Neurosexology. Guidelines for Neurologists. European Federation of Neurological Societies Task Force...

Neurosexology

Guidelines for Neurologists

European Federation of Neurological Societies Task Force on Neurosexology*

P.O. Lundberg, C. Ertekin, A. Ghezzi, M. Swash and D. Vodusek

© 2001 EFNS 2

Search strategy

Four of the members of the task force have reviewedthis topic in recent years: Sexual dysfunction inpatients with neurological disorders (Lundberg, 1992);Sexology (Lundberg, 1994); Sexual dysfunction inselected neurological and endocrinological disorders(Lundberg, 1997a); Diabetes mellitus and sexual dys-function (Ertekin, 1998), Neuro-physiological tests inerectile dysfunction (Vodusek, 1998); Clinical neuro-physiology – in bladder, bowel and sexual dysfunction(Vodusek and Fowler, 1999); Physiology of female sex-ual function and effect of neurological disease(Lundberg 1999); Sexual rehabilitation (Fugl-Meyer etal. 1999); Sexuality and multiple sclerosis (Ghezzi,1999); Neurological disorders: Erectile and ejaculatorydysfunction (Lundberg et al. 2000). Further details onclinical neurosexology can be found in these reviews.The members of the task force are all familiar withneurosexological issues in their clinical work. Besidescommonly used search strategies in standard databas-es (such as Medline and PubMed, which, however, arenot very complete with regard to sexological refer-ences) many other databases, public and private, havebeen used. Members of the group have been searchingreferences pertinent to the field of neurosexology since

the mid-1950s. Exerpta Medica, Neurology/Psychiatry/Gynecology/Urology were used for many years. Sincethe appearance of Current Contents this journal havebeen used and in recent years Index Medicus, Medlineand for the last couple of years NCBI PubMedthrough http://www.ncbi.nlm.nih.gov. Language hasnot been restricted. The sexological terms most fre-quently used have been: sex, sexology, sexuality, sexu-al behaviour, sexual function, sexual dysfunction,desire, libido, erection, erectile dysfunction, ejaculation(absent, retarded, retrograde), orgasmia, anorgasmia,lubrication, priapism. In this review only selected ref-erences are given, mainly in English, and mainly fromthe last 25 years. For review of earlier literature, thereader is referred to reviews by Lundberg (1980, 1992,1997a).

It should be observed that in the literature the sex-ual symptoms and problems of patients with neuro-

Correspondence: P O Lundberg, Department of Neuroscience/Neurology, University Hospital, Uppsala SE-751 85, Sweden.Tel./fax: + 46 18 611 5026; e-mail: [email protected]

*Participants of the European Federation of Neurological SocietiesTask Force on Neurosexology:

Text written by: Professor P. O. Lundberg (chairman; neurologyand sexology, Uppsala, Sweden), Professor C. Ertekin (neurology,Izmir, Turkey), Dr A. Ghezzi (neurology, Gallarate, Italy).Professor M. Swash (neurology, London, UK) and Professor D.Vodusek (clinical neurophysiology, Ljubljana, Slovenia).

Other members of the Task Force: Professor J. de Keyser(Neurology, Groningen, The Netherlands), Professor G. Schmidt(Psychology and Sexology, Hamburg, Germany, and Professor R.Stien (Neurology, Oslo, Norway).

Background

Sexual function can be altered in patients by many neurological disorders affecting the cerebrum, the brain stem,the spinal cord, the spinal roots or the peripheral nerves. It can also be prominently affected in patients with anunderlying undiagnosed neurological disease. Traditionally, practising neurologists have not paid much attentionto sexual dysfunction in their patients, partly because therapeutic possibilities were scant. Sexual dysfunction, how-ever, is observed in many neurological disorders, and may arise as a primary neurogenic disorder. Although notas obviously limiting as, for instance, paresis and pain, it is most disruptive for patients’ lives. With emergingawareness of the primary importance of quality of life as the most important indicator of good patient manage-ment, and with the advent of more effective treatment of sexual dysfunction, it is no longer acceptable to ignorethis very important dimension of life.

In this review we will present the anatomical and physiological basis of the sexual response, the clinicalapproach to patients with sexual dysfunction, the characteristics of symptoms in neurological disorders, how totake a case history, diagnostic procedures and the treatment possibilities.

European Journal of Neurology 2001, 8 (Supp. 3): 2–24

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EFNS Guidelines on Neurosexology 3

© 2001 EFNS European Journal of Neurology 8 (Suppl. 3), 2–24

logical disorders are often poorly described anddefined and can hence be misinterpreted.

Result of literature search

The sexual response phases

Based on their observations of human sexual behav-iour in a laboratory setting, Masters and Johnson con-structed a model of the sexual response cyclecomprising four different phases: Excitement, Plateau,Orgasm and Resolution (EPOR; Masters and Johnson,1966). According to their model, sexual desire is not aphase in itself. This model has reached a wide publicand is used in most textbooks on sexual physiology.However, their observations, although pioneering,were not well controlled and quantified, and their four-phase model has been criticised. Kaplan (1974) hassimplified the sexual response model into three phases:Desire, Excitement and Orgasm (DEO). This modelfits better with physiological observations in humanmales and also with animal research. On the otherhand, it has become more and more evident that fromthe psychological point of view female sexuality ismuch more complex (Basson 2000). From sexual neu-trality, the woman may be seeking for attention andstimuli for non-sexual reasons. This leads to an arousalthat may include a physiological genital responsewhich in turn starts a sexual response cycle with awak-ening of sexual desire and so on. Thus, one could talkalso about a phase of foreplay (‘FDEO’). It is alsoimportant to understand that sexual arousal directly orindirectly results in further stimulation which gives riseto more arousal and so on, thus working as a circuitmechanism.

Viewed from a neurological point of view, the sexu-al response involves a series of neurally controlled phe-nomena occurring in a hormonally defined milieu. Thebehaviour comprises different components: motiva-tion, arousal, genital reactions (erection, lubrication,emission, ejaculation) and orgasm. Obviously, all havetheir neuroanatomical, neurophysiological, neuro-chemical, and neuropsychological dimensions, but onlythe visual reactions in males have been extensivelyexplored to date. The genital components are thereforethe focus of discussions on sexual dysfunction, and fewcontrolled data are available on other physiologicalaspects of sexuality. Essentially reflex in nature, thesexual response has a definite major psychologicaldimension, and can be significantly influenced by neu-ropsychological factors.

Viewed from a functional point of view, and limit-ing ourselves to the male genital components of thenormal sexual functioning, the erection has to be firm

enough for vaginal penetration, and the level of rigid-ity has to be maintained through intercourse to bringabout ejaculation, which in turn should deliver spermto the uterine cervix. The male sexual response cyclehas been extensively studied. Our knowledge about thefemale response cycle is much less complete. A recentreview gives more details (Levin, 1998).

Basic sexual anatomy and physiology

It is beyond the purpose of this review to discuss thedetails of genital anatomy. Genital innervation is bothsomatic and autonomic. Somatic sensory afferentsdeliver information on tactile sexual stimuli, whichinduce local sexual responses (vascular–erectile andglandular) after synapsing in the sacral spinal cord.Sensory information is projected to suprasacral regionsand is important in other reflex activity, leading toawareness and sexual excitation. Parasympatheticefferents travelling through the pelvic plexus and thegreater and lesser cavernosal nerves initiate the erectileresponse. The blood flow in the penile artery increas-es. Smooth muscles lining the cavernosal sinuses in thepenile corpora become relaxed. Helicine arteriolesbranching from cavernosal arteries selectively shuntblood flow to the lacunar spaces of the cavernosal bod-ies, which fill with blood; subtunical venules becomecompressed. Intracorporeal pressure increases and thenstabilizes at a level approximating systolic blood pres-sure and causes penile tumescence and rigidity.Continued parasympathetic activity maintains thiserection (Smith and Bodner, 1993). This parasympa-thetic pathway is, however, not the only proerectilepathway: erections are observed in humans and exper-imental animals after lesions of sacral cord segmentsand pelvic nerves. The best candidates for this alterna-tive proerectile pathway (leading to the so-called psy-chogenic erections in paraplegics with conus or caudaequina lesions but preserved thoracolumbar segments)are the hypogastric nerves. Their role is to some extentstill controversial and may be different in differentspecies.

Continued stimulation eventually triggers orgasmwith seminal emission, rhythmic phasic contractions ofperineal and pelvic floor muscles, and ejaculation ofurethral contents. Actually emission begins duringarousal (Mitsuya et al., 1960). Ejaculation is effected byintegrated sympathetic outflow from T11–L2 segmentstravelling through the sympathetic chain, the hypogas-tric plexus and along pelvic and pudendal nerves(Giuliano et al., 1995), and somatic efferents travellingthrough pudendal nerves. Animal experiments haveshown cross-innervation of the peripheral sympatheticnervous system (Kihara and Degroat, 1997). Thus,

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4 P. O. Lundberg et al.


nerve fibres running in the left mesenteric plexus go tothe pelvic plexus on both sides and innervate vas def-erens on both sides, and vice versa. Sympathetic out-flow causes smooth muscle contraction: in seminalvesicles, vas deferens, the prostate (to deliver seminalfluid to the posterior urethra); in the bladder neck (toprevent retrograde ejaculation); and in the corpora cav-ernosa, to cause detumescence. The latter ‘antierectile’activity is probably inhibited during erection throughspinal coordination of reflex action. In the periphery,the main proerectile transmitter has been shown to beNO (nitric oxide), which is colocalized with VIP(vasoactive intestinal peptide) and acetylcholine. Themain antierectile neurotransmitter is probably nora-drenaline (Giuliano et al., 1995). Although the pre-dominant neural control of the male accessory sexualorgans is sympathetic (adrenergic and purinergic), thereis probably also parasympathetic control over thesecretion of fluids that contribute, overall, to seminalfluid (Hoyle et al., 1994). Appropriate sensory stimula-tion leading to erection and orgasm is not necessarilypurely genital, and erections caused by stimuli deliveredthrough cranial nerves might also be reflexive, althoughthis is usually subsumed under ‘psychogenic’ (Sachs,1995). Mental imagery is the ‘real’ psychogenicdescending activating stimulus of these spinal cord-inte-grated reflex responses.

The pattern of genital neuromuscular activation isthought to be similar in women, in whom parasympa-thetic activity causes clitoral erection, engorgement ofthe labia, and vaginal lubrication. Orgasmic sympa-thetic activity results in contractions of uterus, fallopi-an tubes, and paraurethral glands, and somatic motoractivation causing rhythmic contractions of pelvic floormuscles (Bérard, 1989).

The spinal cord organization of the reflex coordina-tion involved in human sexual responses remains to beelucidated. Spinal cord sites concerned with cavernos-al smooth muscle control have been localized to pre-ganglionic autonomic nuclei in both thoracolumbarand lumbosacral segments in the rat (Marson et al.,1993). Genital afferents probably synapse, viainterneurones, with both somatic and autonomicmotoneurones; those projected to supraspinal struc-tures travel in the anterolateral funiculus. Both thala-mic and cortical areas receive sensory input from thegenitalia, and sexual feelings may be elicited when suchareas are stimulated. In the primary sensory cortex thegenitalia are represented in the parasagittal area(Penfield and Jasper, 1954). In studies using retrogradelabelling in the rat, as revealed by the transneuronaltransport of pseudorabies virus, most of the labellingfrom corpus cavernosum at the level of the brainstemwas in the pons and medulla (Marson et al., 1993).

Descending projections from the brainstem raphenuclei travel in the lateral funiculus. The nucleusparagigantocellularis was shown to have a majority ofserotoninergic neurones that project to the spinal cordand provide tonic inhibition of sexual reflexes in therat (McKenna et al., 1991).

In the diencephalon, the labelled neurones, afterinjection of pseudorabies virus in the corpus caver-nosum, were found only in the hypothalamus, espe-cially the paraventricular nucleus, the tuberal region,the medial preoptic area, and the dorsal hypothalamicarea (Marson et al., 1993). Neurones from the par-aventricular nucleus project to the thoracic and lum-bosacral nuclei concerned with erection.Hypothalamospinal projections are situated in the dor-solateral funiculus (Giuliano et al., 1995). The hypo-thalamus is also directly involved in the control of thegonadotropic functions of the pituitary and thus theprenatal development of the genital organs, pubertaldevelopment, and the menstrual cycle. In the basalhypothalamus, there is a region important for sexualdesire, which is affected by tissue levels of the sexsteroid hormones (testosterone, dihydrotestosteroneand oestradiol).

Animal experiments have delineated a dopaminergicstimulating and a serotoninergic inhibiting mechanismcontrolling sexual desire. Furthermore, the human sex-ual desire is influenced by psychic factors. Androgensare necessary but apparently not essential for normaldesire (Kwan et al., 1983). Sexually dimorphic nucleiare localized in the anterior hypothalamus in the pre-optic region. The medial preoptic area seems to be ofparticular importance in regulating sexual motivationand performance, and dopamine may regulate penileerection at this level. Cortical and subcortical struc-tures related to the limbic system, particularly the hip-pocampus, have been shown to elicit erection whenstimulated (in monkeys; Dua and MacLean, 1964).Overall, the role of the cerebral hemispheres, the restof the brain, and even of the spinal cord in controllingsexual behaviour is far from being fully elucidated. Itis suggested that the brain has an overall inhibitoryinfluence on sexual behaviour, which, at least as far asgenital reflexes are concerned, is organized at thespinal level, where proerectile and antierectile activityis balanced to achieve an appropriate erection, andthen emission is integrated with ejaculatory mecha-nisms to provide semen to the cervix of a female.Particularly important unsolved questions related todiagnostic concepts of erectile dysfunction are whetherpsychogenic and reflex erections are as differentiated ascommonly presumed, and whether sleep-related erec-tions have identical neural control mechanisms to sex-ually elicited erections.

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Case history in patients with sexual dysfunction andneurological disorders

General aspectsFrom the general medical point of view the case histo-ry should include a survey of the patient’s medical his-tory, particularly concerning cardiovascular,endocrine, psychological and psychiatric disturbances,neurological disorders, disorders of the sex organs,prior trauma and surgical procedures, the use of pre-scription drugs, smoking and alcohol habits, and drugabuse.

Detailed history related to sexual dysfunctionFrom the sexological point of view, the case historyshould define the patient’s sexual expectations, needsand behaviour and should identify sexual problems aswell as misconceptions. Psychological factors are fre-quently involved, either as an emotional reaction tosexual dysfunction or as a consequence of a socially orphysically disabling disease. Dependence and lack ofacceptance of the sexual disorder by the patient or thepartner, self-perceived unattractiveness and reducedself-esteem also play a relevant role. It is usuallyimportant also to interview the partner (if the patienthas one) and to evaluate the quality of themarital/partner relationship.

To summarize, it is important to clarify the natureand the characteristics of sexual dysfunction, to dis-cover any underlying (and possibly treatable) organiccause and to document the existence of primary or sec-ondary psychological factors.

Details of the case history of particular neurologicalinterestAs always in neurology, chronology is very important.Has the problem been there all the time, or did it havean onset at a particular time? Was the onset rapid orgradual, the course progressive or episodic?1. The patient should be asked about sexual desire. Isthere a complete lack of desire? Does the patient notexperience spontaneous sexual desire but wishes tohave it or is the desire easily evoked by ordinary situ-ations? Do any particular stimuli, whether visual, tac-tile or emotional play an important role? Is the desirepartner-dependent or not? The term HypoactiveSexual Desire Disorder (HSDD) is sometimes used.HSDD can be defined as the persistent or recurrentdeficiency or absence of sexual fantasies, thoughts,desire for sexual activity, alone or with a partner, andinability to respond to sexual cues that would beexpected to trigger responsive sexual desire. To be sig-nificant, these symptoms need to be causing personaldistress (Basson 2000).

2. Sensory aspects of sexual function should be eluci-dated. The patient should describe sensory experiencesof sexual arousal in different parts of the body. Presentor past disturbances of sensitivity in the region corre-sponding to the sacral segments are of particular inter-est as well as pain during sexual arousal or intercourse,and pelvic or superficial dyspareunia.3. Descriptions of erections are important. Does thepatient have nocturnal erections, morning erections,erections evoked by visual, auditory or psychogenicstimuli and erections evoked or enhanced by genitalstimulation? What is the quality of penile tumescence?Is erection sufficient for penetration? Is there a prema-ture loss of erection during sexual intercourse? Doesthe patient have episodes of priapism or painful noc-turnal erections? Women should be asked about erec-tions of the clitoris and vaginal lubrication. Are thesefemale reactions evoked by visual, emotional or directgenital stimulation?4. Ejaculation should be described. Does the patienthave premature or retarded ejaculation, or evenabsence of ejaculation (anejaculation)? Is the ejacula-tion dribbling, i.e. are there emissions of sementhrough the urethra without contractions of pelvicfloor muscles? Retrograde ejaculation means ejacula-tion into the bladder with presence of spermatozoa inurine. Aspermia means lack of emission of semen.Both can be described as dry ejaculation. The fertilityhistory should be investigated. Is there urinary incon-tinence during sexual intercourse or does the femalehave forceful ejaculation of fluids from the urethraduring orgasm (so-called female ejaculation)?5. Orgasms should be described. Orgasm can bedefined as the sum of all physiological events that hap-pen in the body during the sexual climax and how thisis experienced by the individual. Others define theorgasm solely as the feeling of sexual pleasure. What isthe capacity to achieve an orgasm? Does the person(male or female) actually feel the pelvic floor musclecontractions? How is the quality of orgasmic sensa-tions and experiences? An orgasm may be also anhe-donic, i.e. without pleasurable sensations. Spontaneousorgasms do also occur and orgasms may be painful.6. Menstrual function should be delineated. How oldwas the woman at first menstruation? Has menstrua-tion been regular? When was the last menstruation?Are menstruations painful (dysmenorrhoea)?

Formal questionnaires can be used to obtain stan-dardized information. Frequently used forms are theBrief Male Sexual Function Inventory for Urology(O’Leary et al., 1995) and The International Index ofErectile Function (IIEF, 1997). It has been suggestedthat these protocols are helpful in studies of treatmentefficacy in patients with erectile disorders, but not

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practical for everyday assessment of patient with sexu-al dysfunction (Lue, 1996).

The clinical examination of the patient with sexualdysfunction

Guidelines for the neurological evaluation of male sex-ual dysfunction have also been given by theTherapeutics and Technology AssessmentSubcommittee of the American Academy ofNeurology, 1995.

General clinical examinationSexual development, body length and weight, changesin pigmentation and body hair and the presence ofgalactorrhea are looked for. Inspection of externalgenitalia for any pathology, and evaluation of the sizeof the testes (normal 15–25 mL), the clitoris and theprostatic gland are performed. Distribution of bodyhair and how frequently the man in question has toshave should also be evaluated.

Palpation of peripheral pulses (arms, legs, penis),auscultation of the heart, and blood pressure measure-ment are mandatory.

Neurological examinationA standard neurological examination including assess-ment of the mental state should reveal any signs of anunderlying neurological disease. The examinationshould be related to the particular problem by thepatient. In addition, lower back (naevus, hypertri-chosis, sinus) and feet (deformity, muscle atrophy)should be carefully examined. The sacral segments areespecially important. The bulbocavernosus muscles canbe palpated in the male, and tested for voluntary(‘move the penis’), and reflex contraction. Anal sphinc-ter and levator ani tone (pubococcygeus muscle) andvoluntary and reflex contraction can be palpated inboth sexes. In addition to standard reflexes the cre-masteric reflex (testing the L1 segment), and the bul-bocavernosus and external anal reflex (testing theS2–S4/5 segments) should be evaluated. The bulbocav-ernosus reflex can be elicited by squeezing the glansand assessing contraction of the anal sphincter (in bothsexes) or the bulbocavernosus muscle (in males) bypalpation. The external anal reflex is tested by repeti-tive pricking (or a scratch) delivered to perianal skin(on both sides!) and observing anal sphincter contrac-tion. Skin sensitivity is tested for touch and pain per-ception in the perineum, perianal and genital skin, inaddition to testing over other dermatomes.

Particular laboratory tests (usually classified as neu-rophysiological) are direct extensions of clinical exam-ination of nervous function. Thus, reflex responses can

be recorded with greater sensitivity electromyographi-cally; and perineal sensation can be quantified usingspecial devices and algorithms (see below).

InvestigationsGenerally speaking, investigations will be used toassess sexual function objectively, and then to addressquestions of aetiology. In selected male patients, spon-taneous and physiologically induced erections areexamined; other segments of sexual function (in eithersex) are as a rule not directly evaluated. In males witherectile dysfunction, the capacity to obtain pharmaco-logical erection (with alprostadil) is evaluated andbasic blood and urine tests are performed. In some ofthese further investigation is performed to evaluate theneurogenic, vascular, endocrinological and other possi-ble aetiological factors, but always in relation to ther-apeutic and prognostic considerations.

Investigation of erectile function. Although essentialdata will be obtained by history, objective evaluationof erection is considered the ‘gold standard’ todetermine its quality. Spontaneous and physiologicallyinduced erection can be studied with a variety oftechniques. Spontaneous nocturnal penile tumescenceand rigidity can be measured in the sleep laboratoryusing strain gauges (measuring penile expansion),visual inspection and measuring the buckling force (forassessment of rigidity), with polygraphic confirmationof sleep phases; such a procedure is considered themost accurate for determining erectile function(Karacan and Ilaria, 1978; Wasserman et al., 1980).Various low-cost screening tests for nocturnal penileexpansion have been proposed, but their validity isquestionable (Condra et al., 1987). Continuousmonitoring of nocturnal penile tumescence and rigiditycan be obtained by a rigidometer during normalsleeping conditions at home (Kaneko and Bradley,1986), and also during daytime napping (Morales,1994) or in the conscious sexually stimulated examinee(Thase et al., 1988).

Screening tests for nocturnal penile tumescence(NPT) have been classified as promising in distinguish-ing psychogenic from other causes of erectile dysfunction, but insufficient on their own to arrive at such a conclusion. Home measurements with therigidometer have been classified as promising in establishing the presence and quality of erections. Acomprehensive discussion on the utility and limitationsof the NPT test has been given by Morales et al. (1990).

Investigation of erectile capacity. In addition to theneed to study spontaneous or physiologically inducederections (to verify history data and distinguish

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psychogenic from organic dysfunction), the greatestadvance in diagnosis of erectile failure came with theintroduction of pharmacologically induced erections.Given that no major vascular problem is present(particularly no significant venous incompetence) anintracorporeal injection of a vasoactive substance(papaverine; combination of papaverine +phentolamine; prostaglandin E1) will lead to anerection, thus strengthening the suspicion of aneurogenic or psychogenic aetiology of erectiledysfunction (Mueller and Lue, 1988). The addition ofself-stimulation is considered to increase test sensitivity(Lue, 1990). Intracorporeal injection of vasoactiveagents has been proposed as an established diagnostictool in patients undergoing assessment for possibleneurogenic erectile dysfunction, and safe if performedby experienced physicians, with an acceptablecomplication rate (Haldeman et al., 1995).

Investigation of nervous system function: clinical neuro-physiological and other methods. In patients witherectile (and occasionally ejaculatory) dysfunction and(suspected) neurological disorder a diagnosis ofinvolvement of neural and muscular structures relatedto sexual function may be strengthened, refined anddocumented by neurophysiological tests. There areseveral different methods, classified according to theneuroanatomic subsystem whose function they test.Motor (somatic and autonomic), and sensory(somatosensory and viscerosensory) tests may bedistinguished (Table 1). Most of the tests areelectrophysiological, but quantitative sensory testingand testing lower urinary tract and anorectal function(as indices of sacral autonomic function) can beconveniently discussed under the same heading.

In addition to clinical testing for sensation, specialdevices and algorithms can be used for quantifyingsensory perception on the genital organs. Measuringvibratory perception (biothesiometry/vibrametry) onthe penis has been found to correlate with results ofelectrodiagnostic testing (Padma-Nathan, 1988). Thevibration perception threshold (VPT) in the penis(glans and shaft) in a neurologically healthy man issimilar to that of the feet. In females VPT is best mea-sured in the clitoris, labia majora and perineum(Helström and Lundberg, 1992). The threshold for cli-toris in neurologically healthy women is the same as inthe hands. VPT is of particular importance in womenwith suspected lesions of peripheral sensory nerves inthe pelvic floor area. The test is considered as promis-ing in evaluating penile sensation (Haldeman et al.,1995). Even more informative on nervous control oferection should be tests evaluating small fibre function,i.e. testing for penile thermal sensation (Yarnitsky

et al., 1996). In women vaginal and clitoral warm andcold sensory thresholds may be used to assess neuraldysfunction (Vardi et al. 2000).

Electromyography (EMG) may be used to demon-strate activation patterns of striated muscles (withinthe sexual response [kinesiological EMG]; as forinstance demonstrating the pattern of perineal muscleactivity during ejaculation) (Gerstenberg et al., 1990).However, EMG is mainly used to differentiate normalfrom denervated (reinnervated) muscle. Concentricneedle EMG can identify both changes due to recentdenervation and reinnervation, and is considered themethod of choice to diagnose lower motor neuroneinvolvement in the lower sacral segments (Vodusek &Fowler 1998). Different tests involving stimulation andrecording of somatosensory- and motor-evokedresponses, and sacral reflexes, reflect the function ofdefined parts of the motor and sensory nervous system.These tests measure conduction through nervous path-ways and are sensitive to demyelination, but not toaxonal lesions (which predominate in clinical practice).Tests have been proposed to assess the lumbosacralsympathetic system (the sympathetic skin responses)and penile smooth muscle (the corpus cavernosumEMG). Details of methodology and findings are dis-cussed elsewhere (Vodusek, 1998).

The demonstration of nerve or muscle pathology bythese neurophysiological tests may refine the diagnosisof nervous system involvement, and the procedures aresafe. These tests, however, cannot themselves defineerectile dysfunction as neurogenic (Haldeman et al.,1995); the relationship of any neurophysiological testabnormality to sexual dysfunction per se has proven to

Table 1 Tests of nervous system function

Somatic sensory testsQuantitative sensory testingDorsal penile nerve neurographyPudendal somatosensory evoked potentials (SEP)Measuring bulbocavernosus and anal reflex latenciesVisceral sensory testingSEP to proximal urethra/bladder neck stimulation Testing bladder sensitivity Sacral reflex to proximal urethra/bladder neck stimulationSomatic motor testsElectromyographyPudendal motor latencyMotor evoked potentials (MEP)(above-mentioned reflexes also test the motor part of the reflex arc).Autonomic testsSympathetic skin responsePenile (corpus cavernosum) EMGNeurocardiac testsCystometryAnorectal manometry

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be elusive. However, measurements of dorsal penilenerve conduction, the bulbocavernosus reflex, andpudendal SEP have been classified as promising toolsin evaluating patients with suspected neurogenic erec-tile dysfunction (Haldeman et al., 1995). In particular,the tests measuring penile autonomic innervation andsmooth muscle function would be of value in diagno-sis, but their validity is at present not fully evaluated.Cystometry, other urodynamic tests, and anorectalfunction tests may strengthen a suspicion of sacralautonomic dysfunction.

After delineating (as precisely as required) the presence of a neurological deficit by clinical and labo-ratory examination, further investigations (neuroradio-logical, cerebrospinal fluid, etc.) necessary to diagnosethe neurological disorder are performed.

Laboratory investigation of blood and urine. Basiclaboratory data (including sedimentation rate, bloodcell count, fasting blood sugar, serum lipids,urinanalysis) as well as serum parameters screening forhepatic, kidney and thyroid function should be obtainedin every patient suspected to suffer from organic sexualdysfunction. What hormone to be studied is dependenton the circumstances (sex, age, onset of symptoms).Prolactin and testosterone levels have been proposed asscreening tests for both sexes. In females with menstrualirregularities or signs of masculinization furtherhormone assays may be necessary.

Investigation of vascular function. If intracorporealinjection testing of penile tumescence has strengtheneda suspicion of vascular aetiology in the male patientwith erectile dysfunction, further investigations may becontemplated, as a rule performed by urologists. Penileblood pressure can be measured using a simpleDoppler method and then related to the arm bloodpressure. Vascular competence can be measured byangiography, colour ultrasonography and dynamiccavernosography. It has been stressed that the purposeof testing should always be defined: pharmacotestingmay be sufficient for the majority of patients, and theinvasive tests reserved for those in whom surgery iscontemplated (Meuleman and Diemont, 1995). Infemales, there are also a number of vascular testsavailable. However, their importance in clinicaldiagnosis of sexual dysfunction has so far not beenevaluated.

Psychological testing. If the psychogenic component isconsidered of aetiological relevance, further to dataobtained by history, and the observations made duringthe neurological examination, a clinical psychologicalassessment is useful.

Sexual dysfunction and neurological disorders

Sexual dysfunction with hypothalamo–pituitarydisorders

Decreased or absent sexual desire is the cardinal symp-tom in males with hypothalamo–pituitary disorders. Inmost male cases, this is the first symptom to appear.However, males rarely seek medical advice because ofloss of sexual desire. Hence, the diagnosis is usuallypostponed in men until some other symptom appears.The second symptom to appear is usually hypothy-roidism or visual field defects. However, it may last aslong as a decade after the onset of the sexual problembefore any further symptom develops in a pituitarytumour case.

Studies have shown that 75% of men with hypothal-amo–pituitary disorders report decreased or absentsexual desire at the time of diagnosis. The figures arehigher for those with larger tumours extending into thesuprasellar region than for those with intrasellartumours. A highly significant correlation has beenfound between low serum testosterone levels and adecrease in desire (Lundberg and Wide, 1978). Usuallythe patients also have erectile failure. However,because of lack of desire this does not often present agreat problem to the patient.

Decreased sexual desire is also the first symptom inmost men with smaller pituitary tumours and hyper-prolactinaemia (Muhr et al., 1985). Even in this groupof patients, low serum testosterone is common amongthose with decreased desire. Probably decreased testos-terone levels are more important in causing this symp-tom to develop than hyperprolactinaemia. However,there are some males with hyperprolactinaemia whoreport decreased sexual desire despite normal serumtestosterone.

In women, the situation is somewhat different. Here,amenorrhoea and infertility are usually the problemsthat take the patient to the doctor. In females aged20–60 years with morphologically verified hypothala-mo–pituitary disorders (Hulter and Lundberg, 1994)two-thirds notice absence, or a considerable and trou-blesome decrease in sexual desire. This problem ismuch more common among women with hyperpro-lactinaemia than among those with normal serum pro-lactin. Most of these women have amenorrhoea.Problems with lubrication or orgasms are also verycommon in this group of women.

In most instances hypothalamo–pituitary disorder iscaused by a pituitary adenoma, whether hormone-secreting or not. Less common types of tumours arecraniopharyngiomas, meningiomas, optic gliomas,hypothalamic hamartomas and metastases (Lundberg,1980). Other causes of hypothalamo–pituitary dys-

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function are congenital malformations of the rhinen-cephalon, hypothalamus and/or pituitary such as dys-plasia of the sella turcica or dystopia of the posteriorlobe, olfacto-genital dysplasia or hypogonadotropichypogonadism with anosmia (in its hereditary formcalled Kallmann’s syndrome) and septo-optic dysplasiaor de Morsier’s syndrome. A further group constitutesacquired disorders such as post-traumatic hypothalam-ic bleeding, postpartum pituitary necrosis (Sheehan’ssyndrome), ruptured arterial aneurysms, sequelae of acute asphyxia, spontaneous arrested infantilehydrocephalus, delayed radiation necrosis, meningo-encephalitis, sarcoidosis, histiocytosis-X(Hand–Schüller–Christian syndrome) and a great num-ber of degenerative neurogenetic disorders. The clinicalsymptomatology is dependent upon the age of onsetand the rate of progression. Otherwise, these disordersmore or less regularly result in hypogonadism and lossof libido and potency. In rare cases (spontaneouslyarrested hydrocephalus and hypothalamic tumours,hamartomas in particular) precocious puberty is thecardinal symptom. However, there are many othercauses of secondary hypogonadism. Thus, in aCT/MRI study of 164 impotent males with low serumtestosterone values pathology in thehypothalamo–pituitary region was found in only 11patients (Citron et al., 1996).

In a number of families with cerebellar or spin-ocerebellar ataxia, hypogonadism of the hypogo-nadotropic form, indicating a hypothalamo–pituitaryinsufficiency, has been found (Neuhäuser and Opitz,1975; Berciano et al., 1982; Koskinen et al., 1995).

Sexual dysfunction in patients with brain injuries andencephalopathiesDisability and cognitive impairment occur rather fre-quently after a traumatic brain injury. Sexual impair-ment is not rare, as a consequence of cerebral lesionsor psychological factors. Both decreased and increasedsexual desire have been reported in both sexes. Little isknown about female sexual arousal after traumaticbrain injuries. However, in males both impotence andretarded ejaculation have been reported (Meyer, 1955;Kreutzer and Zasler, 1989). Hypersexuality or alteredsexual preference may also follow brain injury (Milleret al., 1986). Sex offending may also be a psychosocialsequela of traumatic brain injury (Simpson et al.,1999).

Lesions of the frontal and temporal lobe seem tolead more frequently to sexual problems than dolesions of the parieto-occipital part of the brain (DeMorsier and Gronek, 1972). Bilateral lesions of theanterior temporal regions may result in the so-calledKlüver–Bucy syndrome where hypersexuality is a pre-

vailing symptom (Gerstenbrand and Lücking, 1971;Oliveira et al., 1989; Hayman et al., 1998).Pansexuality, that is, sexual drive directed not towardshuman beings but also towards animals and inanimateobjects, is often a major feature. The lesions may betraumatic, sequelae of viral meningoencephalitis, com-plications of SLE or cancer treatment.

Sexual symptoms may also occur in non-traumaticencephalopathies, prion diseases for example (fatalfamilial insomnia, Montagna, 1999; sporadicCreutzfeldt-Jakob disease, Lundberg, unpublishedobservation). Other types of dementia will not be dealtwith in this review.

Sexual dysfunction and strokeAbout three-quarters of stroke patients who have beensexually active before their stroke report abrupt andpermanent decrease in coital frequency. A feeling of anoverall change in sexual life is reported more frequent-ly by male patients. The majority have erectile dys-function after stroke (Kalliomäki et al., 1961; Sjögrenet al., 1983, Monga et al., 1986; Boldrini et al., 1991;Aloni et al. 1998, Korpelainen et al., 1998). Orgasmicdysfunction is seen in three-quarters of the females andtwo-thirds of the males after stroke (Sjögren et al.,1983). Sexual problems in post-stroke patients are usu-ally explained in terms of lack of coping. Diminishedsexual contact for post-stroke patients is due primari-ly to the patient’s overwhelming fears of inadequacy.Other psychological factors have also been suggested.Cognitive impairment may disturb the sexual part of arelationship. Sexual problems are more frequently seenin cases with aphasia (Wiig, 1973). General hemi-hypoaesthesia is associated with decreased sexual driveprobably due to loss of erogenous zones.Hypersexuality may also be the result of a stroke(Monga et al., 1986; Donnet et al., 1997; Absher et al.2000).

Sexual dysfunction in patients with epilepsyNumerous symptoms of sexual dysfunction can be seenin epileptic patients, during the interictal period or inrelation to seizures.

Interictal phenomena. Many men with epilepsy sufferfrom loss of sexual desire, reduced sexual activity,and/or inhibited sexual arousal (Saunders andRawson, 1970; Dansky et al., 1980; Goldner andMorrell, 1996). The figures vary in different studies butare generally higher than those observed in the gener-al population. Inability to maintain erection and, morerarely, ejaculatory dysfunction, decreased satisfactionwith sexual life, reduced sexual fantasies, reduced sex-ual dreams and initiatives, as well as reduced orgasmic

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capacity, are also reported in patients with complex par-tial epilepsy and mesio-basal temporal lobe spike foci,but in these studies data were not compared with pri-mary generalized epilepsy (Taylor, 1969; Shukla et al.,1979). In another study (Morrell et al., 1994) sexual dys-function was more frequent in partial than in general-ized epilepsies. Decreased sexual arousability, vaginismand dyspareunia are reported by many female epilepsypatients (Demerdash et al., 1991). Hypersexual episodesare reported in a few cases (Blumer, 1979). Mostpatients with low sexual desire have complex partialepilepsy and temporal lobe lesions. Sexual interest seemsto be more reduced in patients with right temporal lobeepilepsy compared to patients with the left hemispheredisorders. Whether the patient has undergone surgeryfor their epilepsy or not does not seem to be of impor-tance (Christianson et al., 1995). Life satisfaction andsexuality are higher in patients who are seizure-freecompared to those non-seizure-free. Epileptic patients,especially males, have a lower marriage rate comparedto the general population. Married female patients havefewer children than expected (Dansky et al., 1980).Social and psychological factors play an important role.In fact epileptic patients describe poorer psychologicalhealth compared to healthy subjects. It should also benoticed that antiepileptic drugs, especially the oldertypes (phenytoin, phenobarbital, primidone, carba-mazepine and valproate), may lead to hormonalchanges (particularly increased oestradiol and decreasedfree testosterone levels in men), as well as decreased sex-ual desire and performance in both sexes (Isojärvi et al.,1995; Duncan et al., 1999). Subclinical hypogo-nadotropic hypogonadism caused by the brain damagehas also been suggested as the explanation in somecases. It should be noticed that menstrual irregularitiesare common among epileptic patients (for review, seeLundberg, 1997b).

Seizures and sexual phenomena. Epilepsy and sexualbehaviour may be connected in many ways. Thus, sex-ual activity can provoke an epileptic attack, sexual phe-nomena may be a part of an epileptic seizure, and theepileptic patient may display changes in sexual behav-iour. Such cases have given us important insights in thesexual physiology of the human brain. (For detailsabout sexual phenomena in epileptic patients, seeLundberg, 1992).

Hyperventilation is well known as an inducing eventthat can provoke a generalized epileptic seizure.Hyperventilating during sexual intercourse may some-times provoke an epileptic fit. However, reflex mecha-nisms during sexual behaviour could also trigger apartial epileptic attack from the corresponding corticalarea. Sexual fantasies as well as genital stimuli (mastur-

bation) or orgasm (Berthier et al., 1987; Calleja et al.,1988) may trigger reflex epilepsy. Only few such caseshave been published but this syndrome may be under-reported.

Partial seizures generated from a genital sensory cor-tical area may result in sensations in the genital organs.Such sensations may be described as clitoral warmth, ahot feeling in the vagina, a pleasant sensation of anal orvaginal constriction or of penetration but also asattacks of actual genital pain. Almost all of the very fewdescribed cases have been associated with a parasagittaltumour involving the primary sensory cortex.

Motor symptoms such as erection, lubrication, ejacu-lation or orgasm may also be a part of an epileptic fit.Such genital events may be experienced by patients assexual or as non-sexual. Pelvic sexual movements, as apart of epileptic automatisms, or compulsive masturba-tion in front of other people may occur during or aftera seizure.

Sexual phenomena other than sensory events, occur-ring as part of an epileptic seizure usually feature inpatients with complex partial epilepsy, most often withtemporal lobe lesions. Sexual automatisms may alsooccur with frontal lobe lesions. They are very uncom-mon in primary generalized epilepsy of the grand mal orpetit mal type.

Deviant sexual behaviour, such as exhibitionism,fetishism, frotteurism, sadomasochism, transvestism,and violent sexual behaviour or pansexual behaviour, issometimes displayed by the epileptic patient. Only asmall number of cases have been reported but the fact that the behaviour in question may occurepisodically and sometimes disappears after treat-ment favours a causal connection between the behaviour and the epilepsy or the cerebral lesion behindit. In most cases, there were partial complex epilepticseizures and lesions in one or both temporal lobes.Sometimes it can be shown that the deviant behaviourcorrelated with continuous epileptic discharges in theEEG (psychomotor status). Paranoid delusions of beingviolated, abused or seduced are not uncommon inepileptic patients (for review, see Lundberg, 1992).

Sexual dysfunction in Parkinson’s disease and othermovement disordersDecrease in sexual desire is common in Parkinson’s dis-ease, especially in women. Symptoms of sexual dys-function are also frequent in their partners (Brownet al., 1990). Erectile dysfunction during sexual inter-course occurs in half of the men (Koller et al., 1990;Takahashi, 1991; Wermuth and Stenager, 1995) andnocturnal and morning erections are usually absent.Many affected men are also unable to ejaculate andmany women are unable to achieve an orgasm. During

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sexual arousal, tremor is frequently enhanced whichmakes sexual activity more difficult. Muscle rigidity andakinesia may also contribute to difficulties in perfor-mance of sexual activities. Patients with Parkinson’s dis-ease are frequently depressed and have a tendency toisolate themselves from other people. A correlationbetween sexual dysfunction and depression has beenobserved by some authors. The frequency of sexual dys-function was similar in patients affected by Parkinson’sdisease compared to patients affected by arthritis in onestudy (Lipe et al., 1990)

The mechanisms behind sexual dysfunction in parkin-sonian patients is otherwise not very well understood.Studies of bladder and bowel function have demon-strated a high frequency of bladder detrusor hyper-reflexia and paradoxical contractions of the striatedsphincter muscles during defecation, implying specificautonomic nerve damage in these patients (Berger et al.,1987; Singer et al., 1992).

It is interesting to note that decrease of sexual desireis not directly coupled to the severity of the disease.Treatment with dopaminergic compounds may result inan apparent increase, or rather a normalization, of sex-ual desire without corresponding improvement of themovement disorder. Thus, increase in desire has beenreported as an adverse reaction to dopaminergic drugs(Uitti et al., 1989).

The situation is quite different in Huntington’s dis-ease (HD). Fecundity is increased among these patients:those of the family who are going to develop the diseaseoften have more children than those who do not.Increased sexual activity is seen in around 10% of peo-ple with HD, sometimes in combination with mania orhypomania. Habitual promiscuity and marital infidelitymay be noted at diagnosis of HD. However, HDpatients may have difficulty in achieving sexual arousal.Paraphilias such as sexual aggression, exhibitionism andpaedophilia have been reported in HD patients (Morris,1995).

Disorders of sexual inhibition with pansexuality, e.g.copulation with non-living objects, are not infrequent inTourette’s syndrome (Comings, 1994; Lombroso et al.,1995). Increased sexual activity is also reported inpatients with Wilson’s disease (Akil and Brewer, 1995).Impotence is almost universal among patients with mul-tiple system atrophy, both of the striato–nigral type andthe olivo–pontovcerebellar type. It may be the present-ing symptom (Beck et al., 1994; Hodder, 1997).

Sexual dysfunction in multiple sclerosisChanges in sexual functions are rare at the onset of thedisease but become very common during its evolution inboth sexes.

In a recent study of 47 women with advanced multi-

ple sclerosis (MS), 60% reported decreased sexual desire,36% decreased lubrication and 40% diminished orgas-mic capacity during the course of the disease. Sensorydysfunction in the genital area was experienced by 62%of the women and 77% had weakness of the pelvic mus-cles (Hulter and Lundberg, 1995). In different studies areduced interest was reported by 29–86% of female MSpatients, a reduced sensation by 43–62%, a reducedorgasmic capacity by 24–58%, vaginal dryness by12–40%, and dyspareunia by 6–40% (see review byGhezzi, 1999).

Electrodiagnostic data such as cortical evoked poten-tials of the dorsal nerve of the clitoris (Yang et al. 2000),as well as measurement of vibratory thresholds in theclitoris (Lundberg & Hulter, unpublished data), implythat pudendal somatosensory innervation is necessaryfor at least one type of female orgasmic function. Tocompensate for such a loss, more direct stimulation ofthe anterior vaginal wall is recommended.

Sexual dysfunction also occurs in early and mild casesof multiple sclerosis. Half of the women in one study of25 females aged 20–42 years with a low handicap scorereported sexual problems. Sensory dysfunction seems tobe the most important reason for the sexual problemsin these women. Because of severe external dysaesthesi-ae, some patients reported that during a certain periodthey could not bear direct genital or non-genital contactfrom their partner. The dysaesthesiae were of a maxi-mum intensity from the beginning of an episode of neu-rological symptoms, but resolved fairly rapidly, as isusual in multiple sclerosis (Lundberg, 1978).

Most patients report a diminished sexual desire dur-ing the course of the disease. Some patients may expe-rience a temporary decrease during an episode, in othersthe problem continues. In certain cases, increased sexu-al desire has also been described. When this phenome-non is transitory and concurrent with an episode of newsymptoms, the hypersexuality might well be the result ofa cerebral MS lesion. Other important symptoms of sex-ual dysfunction in women with multiple sclerosis aredeterioration of orgasmic capacity, intensity and quali-ty. In most cases, the orgasmic sensations are reduced.They become shorter, less intense and/or less agreeable.There may be a decrease in orgasmic capacity. Thesechanges may be temporary. However, an orgasmicimprovement has also been noticed. The orgasms maybe more easily triggered, longer lasting, stronger andmore pleasant.

Erectile dysfunction is the most notable sexual dys-function in men with multiple sclerosis (Vas, 1969;Minderhoud et al., 1984; Valleroy and Kraft, 1984;Kirkeby et al., 1988; Betts et al., 1994; Ghezzi et al.,1995; Mattson et al., 1995). Figures given in the litera-ture vary between 34 and 80% (see review by Ghezzi,

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1999). There are no indications of insufficient arterialinflow or venous outflow. Problems with ejaculation arealso frequent, 34–61% have been reported. The figuresare similar for decreased orgasmic capacity (29–64%).Decreased desire also occurs in men (37–86%), but thisproblem has not been studied to a large extent.Occasionally, increased sexual desire can constitute aproblem for both men and women.

Sexual dysfunction correlates with bladder and bowelsphincter dysfunction, but more mildly with motor andsensory dysfunction in the legs (Hulter and Lundberg,1995; Ghezzi et al., 1996; Ghezzi, 1999; Zivadinov et al.,1999). The correlation is poor with disability, clinicalcourse, disease duration. Depression and cognitiveimpairment play an important role. Anorgasmia hasbeen correlated with MRI brain stem and pyramidalabnormalities as well as with total area of lesions onMRI (Barak et al., 1966).

Changes in sexual functions in MS patients usuallystart rather abruptly and correlate both with neurolog-ical symptoms from the sacral segments and with blad-der and bowel dysfunction. Neurophysiological studiesmay give further indications of involvement of thoseparts of the nervous system controlling pelvic structures.

Symptoms related to MS, such as fatigue, muscle con-tractures in the lower limbs, urinary disturbances andthe use of aids to manage incontinence, and paroxysmalmotor and sensory disturbances triggered by sexualintercourse, can indirectly exert a negative effect on sexlife as well as social and physical changes.

Sexual dysfunction in amyotrophic lateral sclerosisIn amyotrophic lateral sclerosis, the neurones of Onuf’snucleus in the sacral spinal cord innervating the pelvicfloor muscles are relatively spared. Sensory and auto-nomic functions are also unaffected. Thus, patients usu-ally have no difficulty with urination and defecation andnormal sexual functions are the rule in males. Despitethe fact that severe paralysis of all voluntary movementseventually make intercourse impossible, erection andejaculation through partner masturbation is possibleand the experience of orgasm is normal (Jokelainen andPalo, 1976).

In Kennedy’s syndrome (X-linked bulbospinal mus-cular atrophy) gynaecomastia is common, and testicularatrophy, decreased libido and impotence may occur(Ertekin and Sirin, 1993; Hokezu et al., 1996).

Sexual dysfunction in spinal cord disordersSpinal cord injuries. The situation for the tetraplegic andparaplegic patient is well understood because of exten-sive studies especially of war victims. The effect of thelesion is very much dependent upon its spinal level. Ifthere is a complete destruction of the genital reflex cen-

tre in the sacral part of the the conus, reflex erectionand reflex lubrication occur but there is complete pare-sis of the striated ejaculatory muscle. Thus, ejaculationis impossible and semen appears as a dribbling from thetip of the penis. In lesions of the upper part of themedulla, reflex erection and reflex lubrication as well asseminal emission and ejaculation may still be possible.However, these patients have impaired sensory percep-tion in the genital organs.

In patients with spinal cord lesion between the levelof the lower thoracic segments and the conus, both cere-bral and reflex erection and lubrication may be possible,despite the fact that the patient cannot feel the sexualorgans. This does not mean that there is no orgasm. Aman with a complete lesion of the spinal cord above theconus can never feel the ejaculatory contractions.However, a number of autonomic phenomena beingparts of the orgasm can be experienced. In spinal cordlesion there is often a hyperaesthetic area of the bodyjust above or at the segment of the lesion. This is, ormay be trained to be, a very strong erogenous zone.

A meta-analysis of 24 studies (Lundberg et al. 2000)of more than 2500 men with spinal cord injuries showedthat a median of 80% (range 54–95%) reported erections(any type) without therapeutic assistance. The percent-age of SCI men reporting ejaculation without therapeu-tic assistance was much lower (median 15%, range0–52%). Fewer (26%) of the patients with completelower sacral lesions had erectile capacity than those withcomplete upper cord lesions or incomplete lesions atany level (90–99%) (Bors and Comarr, 1960).

The semen of men with spinal cord injuries is char-acterized by small volume, low sperm count and lowspermatic mobility. This is at least partly dependent oninsufficient drainage. Ejaculation can be provoked inmany paraplegic males through vibratory stimulation orelectrostimulation. It has been shown that repeatedvibration-induced ejaculations result in increased semenvolume, a larger number of motile sperms and improvedsperm penetration capacity. Insemination with autolo-gous semen obtained in such a way has resulted in preg-nancies. Collection of semen very early after the spinalcord injury makes it possible to store semen of goodquality for future insemination.

A comprehensive review of erectile and ejaculatorydysfunction in spinal cord disorders, and traumaticspinal cord injuries in particular, is given in theInternational Consultation review by Lundberg et al.(2000).

Women with para- or tetra-plegia are in a better sex-ual and reproductive situation than men. Deprived ofsensation in the sacral segments they may still reachorgasm through stimulation of other erogenous zones,suprasegmental to the lesion, such as breasts, lips, ears

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and so on. Preservation of sensory function in the T11-L2 dermatomes is associated with psychogenically medi-ated genital vasocongestion (Sipski et al. 2001) Deeppenetration may provide stimuli enough for orgasmthrough the sympathetic nervous system or possiblythrough the vagus nerve. Such women may also havedysmenorrhoea. Vaginal stimulation has a strong effecton raising the pain threshold in healthy women. Thismechanism may be intact even after complete spinalcord injuries. The majority of para- or tetra-plegicwomen will continue to menstruate, to ovulate and mayhave babies. Sexuality, pregnancy, motherhood andquality of life in women with traumatic spinal cordinjuries are reviewed by Berard (1989) and Westgren(1999).

Spinal cord malformations. The most important malfor-mation giving rise to sexual dysfunction is meningomye-locele. Dependent upon the degree of the malformationthere is a more or less pronounced loss of sexual func-tions. Some boys have no genital sensations at all, somehave erections only and some both both erections andemissions. Loss of genital sensations is the major com-plaint in girls (Dorner, 1977; Wabrek et al., 1978;Sawyer and Roberts, 1999).

In Arnold–Chiari malformation, loss of sexual desireis very common. Some of the men are completely impo-tent and others have reduced potency. The onset of thesexual complaints nearly always follows the beginningof the neurological disturbances (Caetano De Barroset al., 1975).

Spinal canal stenosis. In neurogenic intermittentclaudication, some patients note that after walking ashort distance an unwanted erection appears, unaccom-panied by any libidinous thoughts (Laha et al., 1979;Hopkins et al., 1987). Simultaneously there is pain in thehips on walking radiating to the thighs and legs. The legs tingle and become numb on further walking. If the patients sit down the legsymptoms are relieved and the erection subsides over a few minutes. Erections may also appear after kneelingfor 30 min or so. Decompression by bilateral laminec-tomy results in complete relief of all these symptoms.

Sexual dysfunction in patients with other forms ofmyelopathiesOut of 224 consecutive male patients referred to a sex-ological outpatient department because of impotence,17 patients (31–72 years old) were found to have amyelopathy (Brattberg and Lundberg, 1992; Lundbergand Brattberg, 1992). In most of these patients, theneurological disorder was not diagnosed at the time of

referral. In those patients in whom the neurologicaldisease was known at the time of investigation, the sex-ual problem had started with their falling ill, often avery long time previously. The cause was unknown inseven patients; in the remainder the final diagnosiswas: postmyelitic (five cases), spinal compression (eightcases), posthaematomyelia (one case), and vitamin B12deficiency (one case). Morning erections, psychogenicerections and reflex erections were disturbed in most ofthese men. Disturbances of ejaculation, e.g. retardedejaculation, total loss of ejaculation and dribbling ejac-ulation were reported by 10 patients. Seven patientsreported disturbances of the experience of orgasm; inone patient, the orgasms were painful.

In one study of about 2000 patients of both sexeswith injuries of the cervical spinal cord without paral-ysis, 85 reported sexual dysfunction (Perese et al.,1976). Thirty per cent of patients with vitamin B12deficiency reported sexual dysfunction (Kunze andLeitenmeier, 1976).

Sexual dysfunction in disorders of the spinal roots andperipheral mononeuropathies in the sacral regionOur knowledge of sexual function in patients withspinal root disorders or sacral mononeuropathies ismostly based on single case histories or very limitedmaterial. A review of such cases will be found inLundberg (1992).

Many patients with sacral root lesions are distressedby pain at coitus. Ejaculation may also be painful.Impotence occasionally occurs. As bilateral loss of func-tion is rare some kind of sexual gratification is usuallypossible. Ejaculation may be delayed. Bilateral damageto all S2–5 roots or nerves results in dribbling ejacula-tion, because seminal emission is preserved, but paresisof bulbo- and ischio-cavernosus muscles is present. Insuch a case, reflex erection is not possible but psy-chogenic erection mechanisms are still active. Unilateralloss of all sacral nerves on one side results in ipsilater-al genital anaesthesia. However, sexual function is usu-ally not impaired, because the innervation of the otherside is sufficient for normal genital reflex responses.

Peripheral mononeuropathies of the pudendal nerveor branches of that nerve in particular are not uncom-mon, especially in women. These nerve lesions fre-quently result in so much pain and dyspareunia thatcoitus becomes impossible or at least unpleasant.

Sexual dysfunction in polyneuropathiesDiabetes mellitus. The prevalence of impotence in malediabetics has always been reported to be high. Inreviewing six previous studies comprising a total of1619 cases Neubauer (1971) noted figures of between39 and 75% (mean 55%). Later investigations have

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found similar results (Ellenberg, 1971; Kolodny et al.,1974; Jensen, 1981; Fairburn et al., 1982). In one studywith a healthy control group the difference wasremarkable, 34% vs. 0% (Jensen, 1981). Retrogradeejaculation is reported in diabetic patients as a resultof paresis of the internal bladder sphincter. In moststudies only a few cases have been identified (Greeneet al., 1963; Ellenberg and Weber, 1966; Schirren et al.,1973). However, systematic searches for this symptomusing the proper questions has given figures as high as14% (unpublished observation).A dissociation between different types of erectile fail-

ure is often found. More patients seem to have absentor rare morning erections and absent or weak erectionson visual stimulation than absent or weak erection ongenital stimulation. In sleep laboratory studies(Karacan et al., 1977, 1978; Hirshkowitz et al., 1990)on men with diabetic polyneuropathy, fewer sleep-related erections, shorter tumescence time, diminishedpenile circumference increase and lower penile rigiditywere recorded in those with diabetic polyneuropathythan in non-diabetic men. Much less is known aboutsexual desire in diabetic men (Jensen, 1981).

A correlation between impotence and the occurrenceof peripheral neuropathy is sometimes reported(Ellenberg, 1971; Kolodny et al., 1974; Jensen, 1981;Lehman and Jacobs, 1983). A correlation betweenimpotence and autonomic bladder dysfunction in dia-betic patients has also ben described (Ellenberg, 1971;Buvat et al., 1985; Ertekin et al., 1989). However, toanalyse a diabetic autonomic polyneuropathy, it is notenough to study just the somatic nerves in the legs.

Histological and histochemical studies of the auto-nomic nervous system have been performed in a num-ber of men with diabetic impotence. The content ofnorepinephrine in the corpora cavernosa was found tobe significantly lower in insulin-dependent and diet-controlled diabetic men than in non-diabetic men.Neither changes in choline acetyltransferase activitynor morphological alterations of the nerve fibres oferectile tissue were observed (Melman et al., 1980a;1980b). Using an isotope technique, both choline accu-mulation and acetylcholine synthesis and release weresignificantly reduced in penile tissue from impotentdiabetic patients compared to that from impotent non-diabetic patients. The impairment in acetylcholine syn-thesis worsened with the duration of the diabetes(Blanco et al., 1990). Morphological alterations havebeen demonstrated in unmyelinated nerve fibres in thepenis (Faerman et al., 1974).

Nitric oxide is considered to be the most importantfactor in relaxing corpus cavernosum smooth muscle,thus giving rise to erection (for review, see Anderssonet al. 2000). Hyperglycaemia (and some glycosylation

products) promotes the chemical inactivation of NO(Brodsky et al. 2001). There seems to be a selectivenitrergic neurodegeneration in diabetes (Cellek et al.,1999). This may be an important mechanism behinderectile dysfunction in diabetic men.

In addition, a number of physiological and neuro-physiological tests have been applied. Cardiovascularreflex tests exploring the sympathetic nervous systemcannot be used to differentiate between diabetic menwith erectile failure and those without this problem(Quadri et al., 1989). On the other hand, measurementof bulbocavernosus and urethro-anal reflex latencies aswell as penile evoked potentials have shown a signifi-cantly higher number of abnormalities in impotent dia-betic males than in impotent non-diabetic males andpotent diabetic males (Bemelmans et al., 1994).

Studies of sexual function in women with diabetesmellitus have given conflicting results. In one study35% of the diabetic women were reported to have hadorgasmic dysfunction in the preceding year as com-pared to 6% of the controls (Kolodny, 1971). In anoth-er study the frequency of sexual dysfunction wasaround 25% in both insulin-treated diabetic womenand in age-matched controls (Jensen, 1981). An impor-tant negative impact on sexual life measured as prob-lems with desire, activity, lubrication, orgasmiccapacity, satisfaction and on the relationship with thesexual partner compared to matched control womenwas found only in type II diabetic women (Schreiner-Engel et al., 1987); not in type I. Loss of libido hasbeen reported in some other studies (Newman andBertelson, 1986; Campbell et al., 1989).

In a recent structured interview study, Hulter et al.(1998) found that 26% of 42 women with insulin-dependent diabetes had a decreased sexual desire, 22%had decreased vaginal lubrication and 10% haddecreased capacity to acquire orgasm. Several of thewomen reported more than one dysfunction. Takentogether the figure for sexual dysfunction was 40%.Among age-matched controls without diabetes or anyneurological disease, only 7% of the women reportedsome kind of sexual dysfunction.

Lubrication, the most important female counterpartto penile erection in males, is otherwise rarely studied.Tyrer et al. (1983) found that in insulin-dependent diabetic women vaginal lubrication was frequentlyinadequate or required prolonged stimulation com-pared to controls. There are two psychophysiologicalstudies in women with diabetes mellitus. One of themdid not show any difference compared to controls(Slob et al., 1990). In the other, diabetic women werefound to experience significantly less physiologicalarousal to erotic stimuli than controls (Wincze et al.,1993).

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A number of autonomic and sensory symptoms,such as reduced foot perspiration, increased gustatoryperspiration and impaired subjective vulvar sensibility,were noted more frequently by women with insulin-dependent diabetes than by controls (Hulter et al.,1998). The diabetic patients had significantly highervibration perception thresholds in the hands and in theclitoris than the controls. Reduced foot perspiration,increased gustatory perspiration, constipation andincontinence correlated with sexual dysfunction.

A postmortem study of tissue samples from 17 dia-betic women (Zrustová et al., 1978) showed evidenceof both clitoral nerve degeneration and changes inblood vessels in the clitoris. A non-diabetic controlgroup did not show any signs of neuropathy or vascu-lar damage. However, so far, this study does not seemto have been replicated.

It should be noticed that sexual problems in patientswith diabetes mellitus may be caused by many differ-ent mechanisms besides polyneuropathy. The metabol-ic process in itself, with variations in blood sugar andacidosis certainly plays a role. It is not believed thatother endocrine insufficiency such as hypogonadismplays an important role in either the low sexual desireor the erectile dysfunction. Vascular damage to smallvessels as well as in larger arteries may result indecreased blood flow to the cavernous tissues in bothsexes. Two comprehensive reviews have recently beenpublished about men (Ertekin, 1998) and women(Enzlin et al., 1998) with diabetes.

Other polyneuropathiesAutonomic dysfunction, including sexual dysfunction,is a common complication in peripheral neuropathies(McDougall and McLeod, 1996). Polyneuropathiesdue to vitamin deficiency (Vitamin B1 Tjandra andJanknet, 1997; Vitamin B12 Kunze and Leitenmajer,1978) or plasma cell dyscrasia (Takatsuki and Sanada,1983) may cause erectile dysfunction.

Very little has been written about sexual dysfunctionin hereditary polyneuropathies. However, based onphysiological data, impotence, decreased lubrication,retarded or retrograde ejaculation and orgasmic diffi-culties are to be expected. Thus, impotence and ejacu-lation problems have been observed in patients withGuillain–Barré syndrome, Charcot–Marie–Tooth syn-drome (Bird et al., 1994; Crabtree, 1997), adreno-myeloneuropathy/adrenoleukodystrophy (Sakakibaraet al., 1998; Garside et al., 1999), Refsum’s disease(Lundberg, unpublished observation), Friedreich’sataxia, Riley–Day syndrome, HSAN I-IV (four types ofhereditary sensory and autonomic polyneuropathy) andprimary amyloidotic polyneuropathy (Andersson andHofer, 1974; Obayashi et al. 2000). In a study of 341

consecutive patients with erectile dysfunction, neuro-physiological evaluation for polyneuropathy revealedthe presence of polyneuropathy in 38% of diabetic casesand 10% of impotent cases of other aetiology (Vardiet al., 1996). The adrenoleucodystrophies represent agroup of patients of particular interest because of thesevere prognosis of this disease and the predominanceof CNS lesions (Powers and Schaumberg, 1980). It doesnot seem to be the involvement of the somatic nerves(pudendal nerves) that is of importance in the heredi-tary motor and sensory neuropathies in causing impo-tence (Vodusek and Zidar, 1987).

MyopathiesIn certain types of progressive muscular dystrophies,myotonic dystrophy (Marinkovic et al., 1990;Mastrogiacomo et al., 1994; Olsson et al., 1996), theBecker type (Hallen, 1971) and ocular myopathy of theautosomal progressive external ophthalmoplegia type(Lundberg, 1966; Melberg et al., 1996) in particular,hypogonadism in combination with disturbances oferectile function and desire has been described. Infemales, secondary amenorrhoea is common.Impotence and amenorrhoea may also occur in mito-chondrial encephalomyopathies such as MERFF andMELAS syndromes (Chen and Huang, 1995).

Sexual dysfunction and prescription drugsAntidepressant drugs. For the non-selective monoaminereuptake inhibitors, impotence, decreased desire andproblems with ejaculation are the most frequentunwanted sexual effects. Problems within the orgasmicphase of the sexual response cycle are more prevalentfor the group of selective serotonin reuptake inhibitors(SSRIs) than for the group of non-selectivemonoamine reuptake inhibitors (Kimura et al., 1984;Patterson, 1993; Montejo-González et al., 1997;Lundberg and Biriell, 1998). The most frequentadverse reactions with SSRIs are decreased desire andthe most typical are anorgasmia in females andproblems with ejaculation and anorgasmia in males.Impotence is less frequently reported with the group ofSSRIs than for the group of non-selective monoaminereuptake inhibitors. Trazodone is the antidepressantdrug with the highest number of reports of priapism(Saenz de Tejada et al., 1991; Lundberg and Biriell,1998; Lundberg 2000). Priapism caused by this drug isalso recorded in women.

Antihypertensive drugs. The greatest number of sexualadverse drug reaction reports concern antihypertensivedrugs (Lundberg and Biriell, 1993). All classes amongthese drugs are represented. The different drugs seemto have few pharmacological effects in common

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besides lowering the blood pressure. Hence, theimpotence is probably vascular. However, becausedecrease in libido is also reported with these drugs, thiseffect may be partly central. Alpha-adrenoreceptorblocking agents as well as alpha- and beta-adrenoreceptor agents and guanidine derivatives havebeen reported in connection with ejaculation failure.Priapism has been reported with alpha-adrenoreceptorblocking agents (such as prazosin).

Other types of drugs. Other types of drugs ofimportance in this context are hormonal contraceptivesfor systemic use, anti-epileptic drugs, dopaminergicdrugs, antipsychotic drugs and anxiolytic drugs.Hormonal contraceptives for systemic use are reportedto cause decrease in sexual desire. Anti-epileptic drugsfrequently decrease sexual desire and cause impotence.However, it is difficult to evaluate what is cause by theunderlying brain disorders in itself and what is causedby the drug in question (see paragraph on epilepsy).Dopaminergic drugs (L-dopa, bromocriptine,selegiline) represent the only group more frequentlyassociated with increased desire than with decreaseddesire (see above) (Hyyppä et al., 1975). Antipsychoticdrugs with alpha-adrenoreceptor blocking properties,particularly chlorpromazine, thioridazine, haloperidoland clozapine, have been reported to cause priapismand also ejaculation failure. These drugs may alsocause painful, retrograde or spontaneous ejaculations(Keitner and Selub, 1983; Sitsen, 1988).

Treatment and counselling strategies for sexualdysfunction in neurological disorders

General treatment principlesSexual counselling, discussion and communicationwith the patient and their partner about their sexuallife is an important part of the rehabilitation strategyin neurological disorders. It should be recognized thatsexual dysfunction may be the first symptom of a neurological disorder. The first step is to let the patientknow that it is permitted to discuss sexuality in theclinical setting. The next step is to give limited infor-mation about sexual physiology and practical issuesthat are pertinent to a person with the particular handicap and symptomatology in question. Specificsuggestions about therapeutic methods and strategiesmay then follow. In a limited number of cases, there is also a need for intensive therapy under the supervi-sion of a psychotherapist trained in neurosexology. Itcan be combined with sensate focus exercises. Herebody awareness exercises and psychotherapy aremerged.

Intracavernous injection as a treatment for neurogenicimpotenceIntracavernous injection (ICI) of vasoactive substanceshas become a standard treatment procedure in erectiledysfunction of organic causes for the last decade. Inneurology, it has mostly been used in men with spinalcord injuries or multiple sclerosis (Beretta et al., 1986;Bodner et al., 1987; Sidi et al., 1987; Kirkeby et al.,1988; Costa et al., 1993; Hirsh et al., 1994).

Originally papaverine was used but this drug hasbeen largely replaced by prostaglandin E1. In neuro-genic impotence, rather low doses of PGE 1 are need-ed, 2.5 µg up to a maximum of 10 µg. In arteriogenicimpotence, higher doses are needed. The effect is veryrapid and may last for 2–4 h. Longer-lasting erectionshould be treated as priapism (see below).Haematological malignancies and anticoagulant thera-py are contraindications. Local bleeding and pain arecommon side-effects, and fibrosis may develop in thecorpora cavernosa leading to loss of effectiveness.Moxisylate, an alpha-blocking agent has been studiedagainst placebo in spinal cord-injured patients (Costaet al., 1993) with rather good results. However, long-term studies with this particular drug are lacking.The drugs should be tested during medical supervision,then a suitable dose can be self-administrated by thepatient or his partner.

Prostaglandin E1 can also be administered as a gelintraurethrally (MUSE). This is more practical but lesseffective than ICI (Padma-Nathan et al., 1997; Bodneret al., 1999).

Oral treatment in patients with neurogenic impotenceAs mentioned above, a number of oral prescriptiondrugs may have the potential to be used to treat impo-tence. However, as most of these drugs act centrally,they are not very effective in this regard and have anumber of other side-effects. Recently a specific,peripherally acting vasodilatation agent, sildenafil(Viagra), has become available. This is a specific phos-phodiasterase-5 inhibitor. One hour before desired sex-ual activity, 25, 50 or 100 mg can be given orally. It hasa very discrete spontaneous effect but it enhances erec-tion mechanisms only if the individual is sexually stim-ulated. Sildenafil taken at bedtime significantlyincreases nocturnal erections (Montorsi et al., 2000).

Sildenafil can be used repeatedly with only minorproblems. However, it should be used with great care inthe cardiac patient and is contraindicated in combina-tion with vasodilatation drugs of the nitro type (Boolellet al., 1996; Goldstein et al., 1998). Neurological disor-ders with a disturbed vasoregulation may also be a con-traindication. The most common adverse events areotherwise headache and dyspepsia. Ischaemic optic neu-

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ropathy (Egan 2000) and third nerve palsy (Donahueand Taylor, 1998) have also been associated with silde-nai intake. The drug should not be used in patients withretinitis pigmentosa. It is an effective treatment for erec-tile dysfunction in men with diabetes (Price et al., 1998;Rendel et al., 1999), multiple sclerosis (Fowler et al.,1999; Miller et al., 1999), Parkinson’s disease (Zesiewiczet al. 2000) and spinal cord injury (Derry et al., 1998;Guiliano et al., 1999; Maytom et al., 1999; Hultlinget al. 2000; Schmid et al. 2000). Sildenafil has been usedin women with neurological disorders and lack of sexu-al arousal and anorgasmia. However, so far no con-trolled studies have been published.

A number of other phosphodiasterase-5 inhibitorsare presently under clinical trial for treatment of impo-tence. So far, there are no controlled studies onpatients with neurological disorders.

Treatment with apomorphine sublingually is anoth-er possibility. The action is through a dopaminergiceffect in the hypothalamus (cf. Adverse drug reactionsof l-dopa described above). Doses of 2–4 mg are given.Erection occurs within 10–25 min. Nausea is the mostcommon adverse reaction (Heaton, 2000).

Surgical procedures and technical devicesSurgical revascularization in traumatic cases and severelocal arteriosclerosis is of limited importance in theneurological patients. So are also procedures to treatcavernous insufficiency (venous leakage). Penile pros-

theses, implantation of semifirm or inflatable rods havefrequently been used during the past decades. However,these techniques have so many disadvantages and com-plications that they are rarely to be recommendednowadays when oral medications and ICI are available.However, penile vacuum devices and penile rings stillhave a place in the treatment of impotent neurologicalpatients (Heller et al., 1992; Rivas and Chancellor,1994; Denil et al., 1996; Seckin et al., 1996).

Treatment of anejaculationIn cases of anejaculation/anorgasmia, vibratory stimu-lation may be helpful (Brindley, 1981). The presence ofintact dorsal penile nerves is necessary for the ejacula-tory response to penile vibratory stimulation (Wiederet al. 2000). If the aim is to retrieve sperm for in vitrofertilization or intracytoplasmic sperm injection, elec-troejaculation is preferred (Sato, 1991). Such treatmentshould preferably be supervised by experienced spe-cialists. For that reason, details will not be given inthese guidelines. A comprehensive description can befound in two recent reviews (Kamischke andNieschlag, 1999; Lundberg et al., 2000).

Treatment of priapismPriapism caused by a spinal cord disorder or appear-ing as an adverse drug reaction usually has a goodprognosis with conservative treatment. Painless pri-apism of less than 6 h duration should be treated atfirst by cooling (Bondil et al., 1997; Lundberg, 2000).In the case of failure or if the priapism is painless buthas been more than 6 but less than 24 h in duration,intracavernous injection of an alpha-adrenergic agonistsuch as metaraminol is recommended. In painful pri-apism or when the other conservative methods havefailed, penile puncture should be used. Few cases ofthis type of priapism need open surgery. When ICI orpuncture is performed, it is recommended to draw acavernosal blood sample for blood gas analysis to eval-uate cavernosal anoxia.

Treatment of lack of sexual arousal and anorgasmia inwomenPostmenopausal women as well as women showingsigns of oestrogen insufficiency should be given hor-mone replacement therapy. Androgens may also besupplemented in women with no risk of being preg-nant. Otherwise, there is at present no drug treatmentwith proven effect available. Counselling according tothe first three steps of the PLISSIT model should befollowed. Vibratory stimulation may be very helpfulboth in patients with genital sensory disturbances andthose with paresis and movement disorders. The vibra-tors should preferably be applied against the clitoris.

Table 2 Therapeutic methods in impotence of organic causes

I. Oral and topical pharmacotherapy(A) Central Agents

i-Adrenergic receptor antagonistsPhentolamineDelquamineYohimbine

ii-Dopamine receptor agonistsApomorphineBromocriptine

iii-Serotonergic receptors agonistTrazodone

(B) Peripherally acting agentsNitroglycerinePhosphodiesterase-5 inhibitors (Sildenafil and others)l-ArginineMinoxidil

II. Intracavernosal therapyPapaverinePapaverine-phentolamine mixProstagladine E1Calcitonin Gene-related Peptide (CGRP)LinsidomineTrimix (Papaverine-Phentolamine-Prostaglandin E1)Vasoactive intestinal peptide (VIP)

III. Penile vascular surgeryIV. Penile prosthesisV. Penile vacuum devices

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Dildos of various types, also in combination withvibration, are sometimes very useful.

Treatment of reduced or increased sexual desireHormone insufficiency, testosterone in men andoestrogens in women, should always be corrected.Testosterone may also be helpful in women, but cannot very often be used in fertile women because ofthe risk of masculinization. Hyperprolactinaemiashould be treated with bromocriptine or similar.Dopaminergic drugs may be tried in certain cases withrediced desire. However, the drug of choice in womenwith low sexual desire is still to be found.

Increased sexual desire that has to be treated is rare.Androgen antagonists (cyproterone acetate, medrox-yprogesterone acetate) may be effective. In severecases, neuroleptics may be tried.

Conclusions and recommendations

Sexual disabilities such as loss of sexual desire, erectiledysfunction or decreased lubrication and sexualarousal, as well as disturbances of ejaculation andorgasm, are very common among patients with neuro-logical disorders or those suffering from sequelae ofinjuries. In brain disorders and after brain injuries,sexual desire may be reduced and behavioural distur-bances may occur. Sexual adverse reactions have beenreported with the use of many prescription drugs act-ing on the nervous system. Sexual disabilities may bethe presenting symptom or one of the early symptomsof a neurological disorder.

All neurological patients should have the opportuni-ty of sexual counselling. The majority of them, eventhose with complete spinal cord injuries, can be treat-ed for erectile and ejaculatory dysfunction.

Diagnosis

A careful case history should be taken and the neuro-logical examination should include the sacral segments.In selected patients (particularly those with suspectedperipheral nervous system involvement), neurophysio-logical tests, should, in addition to other indicatedinvestigations, be considered in patients seeking med-ical advice because of a sexual disability.

Treatment

1. Permission: The first step of the counselling is toopen the mind of the patient to the existence of sexu-al disabilities. 2. Limited information: The second step is to give thepatient proper information about sexual issues in thisparticular type of disorder/injury.

3. Specific suggestions: Practical information aboutpositions and stimulation techniques (vibrators) andtechnical aids and devices (vacuum extraction devices)should be given.4. Intensive therapy: The next step is to use specificmedical as well as surgical, treatment available, for thedifferent types of problems; such as decrease in desire,erectile dysfunction, problems with sexual arousal inwomen as well as ejaculatory dysfunction and orgas-mic problems. These treatments include oestrogen sub-stitution in females, testosterone substitution in bothsexes, intracavernous injection of prostaglandin E1,papaverine or moxisylate, transdermal or intraurethralapplication of different drugs, oral administration ofphosphodiesterase-5 inhibitors such as sildenafil and,in a limited number of cases, penile prosthesis.Treatment of bladder dysfunction and spasticity aswell as autonomic dysreflexia is also important.5. In spinal cord injuries, semen can be retrieved by theuse of assisted ejaculation methods such as penilevibratory stimulation (PVS) or rectal probe electroejac-ulation (EEJ). PVS should be used first due to its safe-ty, reliability and low investment in time and money. Italso results in better semen quality. If PVS fails, thepatient should be referred to a specialist centre for EEJ.So far, there are no treatments to normalize semenquality in men with spinal cord injury, although semenquality does not improve when EEJ is used.

ReferencesAbsher JR, Vogt BA, Clark DG et al. (2000). Hypersexuality

and hemiballism due to subthalamic infarction. NeuropsychNeuropsychol Behav Neurol 13:220–229.

Akil M, Brewer GJ (1995). Psychiatric and behavioral abnor-malities in Wilson’s disease. Adv Neurol 65:171–186.

Aloni R, Schwartz J, Ring H (1993). Sexual function in malepatients after stroke – a follow up study. Sexual Disabil11:121–128.

Andersson K-E, Burnett AL, Chen KK, Christ GJ, RampinO, Stief C (2000). Current research and future therapiesIn: Jardin A, Wagner G, Khoury S, Giuliano F, Padma-Nathan H, Rosen R, eds. Erectile Dysfunction Plymouth:Plymbridge Distributors Ltd, pp. 141–203.

Andersson R, Hofer P-Å (1974). Genitourinary disturbancesin familial amd sporadic cases of primary amyloidosiswith polyneuropathy. Acta Med Scand 195:49–58.

Barak Y, Achiron A, Elizur A, Gabbay U, Noy S, Sarova-Pinhas I (1996). Sexual dysfunction in relapsíng-remittingmultiple sclerosis: magnetic resonance imaging, clinical,and psychological correlates. J Psych Neurosci21:255–258.

Basson R (2000). The female sexual response. A differentmodel. J Sex Marital Ther 26:51–65.

Beck RO, Betts CD, Fowler CJ (1994). Genitourinary dys-function in multiple system atrophy. J Urol151:1336–1341.

Bemelmans BLH, Meuleman EJH, Doesburg WH,Notermans SLH, Debruyne FMJ (1994). Erectile dys-

10653_Supp3_pp02_24 19/6/01 17:04 Page 18



function in diabetic men: The neurological factor revisted.J Urol 151:884–889.

Bérard EJ (1989). The sexuality of spinal cord injuredwomen: physiology and pathophysiology. Paraplegia27:99–112.

Berciano J, Amado JA, Freijanes J, Rebollo M, Vaquero A(1982). Familial cerebellar ataxia and hypogonadotropichypogonadism: evidence for hypothalamic LHRH defi-ciency. J Neurol Neurosurg Psych 45:747–751.

Beretta G, Zanolio A, Fancuillacci F, Cantazaro F (1986).Intracavernous injection of papaverine in paraplegicmales. Acta Europaea Fertilitatis 17:283–284.

Berger Y, Blaivas JG, DeLaRocha ER, Salinas JM (1987).Urodynamic findings in Parkinson’s disease. J Urol138:836–838.

Berthier M, Starkstein S, Leiguarda R (1987). Seizuresinduced by orgasm. Ann Neurol 22:394–395.

Betts CD, Jones SJ, Fowler CG, Fowler CJ (1994). Erectiledysfunction in multiple sclerosis. Brain 117:1303–1310.

Bird TD, Lipe HP, Crabtree LD (1994). Impotence associat-ed with Charcot-Marie–Tooth syndrome. Eur Neurol34:155–157.

Blanco R, de Tejada IS, Goldstein I, Krane RJ, Wotiz HH,Cohen RA (1990). Dysfunctional penile cholinergic nervesin diabetic impotent men. J Urol 144:278–280.

Blumer D (1979). Hypersexual episodes in the temporal lobeepilepsy. Am J Psychiatry 126:83–90.

Bodner DR, Haas CA, Krueger B, Seftel AD (1999).Intraurethral alprostadil for treatment of erectile dysfunc-tion in patients with spinal cord injury. Urology53:199–202.

Bodner D, Lindan R, Leffler E, Kursh E, Resnick M (1987).The application of intracavernous injection of vasoactivemedications for erection in men with spinal cord injury. JUrol 138:310–311.

Boldrini P, Basaglia N, Calancq MC (1991). Sexual changesin hemiparetic patients. Arch Phys Med Rehab 72:202–207.

Boolell M, Gepi-Attee S, Gingell JC, Alien MJ (1996).Sildenafil, a novel effective oral therapy for male erectiledysfunction. Br J Urol 78:257–261.

Bondil P, Descottes JL, Salti A, Sabbagh R, Hamza T(1997). Medical treatment of venous priapism apropos of46 cases: puncture, pharmacologic detumescence or penilecooling? Progres en Urologie 7:433–441.

Bors E, Comarr AE (1960). Neurological disturbances of sex-ual function with special reference to 529 patients withspinal cord injury. Urol Surv 10:191–222.

Brattberg A, Lundberg PO (1991). Disorders of the spinalcord in males with sexual dysfunction. Proceedings of the10th World Congress of Sexology, Amsterdam. ElsevierICS 983, 229–232.

Brindley GS (1981). Reflex ejaculation under vibratory stim-ulation in paraplegic men. Paraplegia 30:550–553.

Brodsky SV, Mirrishow AM, Dharia N, Gross SS,Goligorsky MS (2001). Glucose scavenging of nitric oxide.Am J Physiol Renal Physiol 280: F480–F486.

Brown RG, Jahanshahi M, Quinn N, Marsden CD (1990).Sexual function in patients with Parkinson’s disease andtheir partners. J Neurol Neurosurg Psych 53:480–486.

Buvat J, Lemaire A, Buvat-Herbaut M, Guieu JD, BailleulJP, Fossati P (1985). Comparative investigations in 26impotent and 26 nonimpotent diabetic patients. J Urol133:34–38.

Caetano De Barros M, Farias Da Silva W, DeAzevedo Filho

HC, Spinell C (1975). Disturbancies of sexual potency inpatients with basilar impression and Arnold-Chiari mal-formation. J Neurol Neurosurg Psych 38:598–600.

Calleja J, Carpizo R, Berciano J (1988). Orgasmic epilepsy.Epilepsia 29:635–639.

Campbell LV, Redelman MJ, Borkman M, McLay JG,Chisholm DJ (1989). Factors in sexual dysfunction in dia-betic female volunteer subjects. Med J Aust 151: 550–552.

Cellek S, Rodrigo J, Lobos E, Fernandez P, Serrano J,Moncada S. Selective nitrergic neurodegeneration in dia-betes mellitus – a nitric oxide-dependent phenomenon. BrJ Pharmacol 1999:1804–1812.

Chen CM, Huang CC (1995). Gonadal dysfunction in mito-chondrial encephalomyopathies. Eur Neurol 35:281–286.

Christianson S-Å, Silfvenius H, Säisä J, Nilsson M (1995).Life satisfaction and sexuality in patients operated forepilepsy. Acta Neurol Scand 92:1–6.

Citron JT, Ettinger B, Rubinoff H et al. (1996). Prevalenceof hypothalamic-pituitary imaging abnormalities in impo-tent men with secondary hypogonadism. J Urol155:529–533.

Comings DE (1994). Role of genetic factors in human sexu-al behavior based on studies of Tourette syndrome andADHD probands and their relatives. Am J Med Genet54:227–241.

Condra M, Fenemore J, Reid K et al. (1987). Screeningassessment of penile tumescence and rigidity. Clinical testof snap gauge. Urology 29:254–257.

Costa P, Sarrazin B, Bressole F, Colson M, Bondil F,Saudubray F (1993). Efficiency and side effects of intra-cavernous injections of moxisylate in impotent patients: adose findning study versus placebo. J Urol 149:301–305.

Crabtree L (1997). Charcot-Marie-Tooth disease: sex, sexual-ity and self-esteem. Sexual Disab 15:293–311.

Dansky LV, Andermann E, Andermann F (1980). Marriageand fertility in epileptic patients. Epilepsia 21:261–271.

Demerdash A, Shalaan M, Midani A, Kamel F, Bahri M(1991). Sexual behavior of a sample of females withepilepsy. Epilepsia 32: 82–85.

De Morsier G, Gronek B (1972). Sur 92 cas de troubles sex-ual post-traumatiques. Annales Med-Psychologiques130:653–670.

Denil J, Ohl D, Smythe C (1996). Vacuum erection device inspinal cord injured men: Patient and partner satisfaction.Arch Phys Med Rehab77:750–753.

Derry FA, Dinsmore WW, Fraser M et al. (1998). Efficacyand safety of oral sildenafil (Viagra) in men with erectiledysfunction caused by spinal cord injury. Neurology51:1629–1633.

Donahue SP, Taylor RJ (1998). Pupil-sparing third nervepalsy associated with seldenafil citrate (Viagra). Am JOphthalmol 126:376–477.

Donnet A, Schmitt A, Poncet M, Graziani N, Grisoli F(1997). [Hallucinations of supernumerary limbs. LeftHemineglect and Hypersexuality in a Case of RightCapsulo-Lenticular Hematoma] (In French). RevueNeurologiques 153: 587–590 [PubMed abstract].

Dorner S (1977). Sexual interest and activity in adolescentswith spina bifida. J Child Psychol Psych 18:229–237.

Dua S, MacLean PD (1964). Localization of penile erectionin medial frontal lobe. Am J Physiol 207:1425.

Duncan S, Blacklaw J, Beastall GH, Brodie MJ (1999).Antiepileptic drug therapy and sexual function in menwith epilepsy. Epilepsia 40:197–204.

10653_Supp3_pp02_24 19/6/01 17:04 Page 19



Egan R (2000). Sildenafil (Viagra) associated with anteriorischemic optic neuropathy. Arch Ophthamol 118:291.

Ellenberg M (1971). Impotence in diabetes: the neurologicalfactor. Ann Intern Medicine 75:213–219.

Ellenberg M, Weber H (1966). Retrograde ejaculation in dia-betic neuropathy. Ann Intern Medicine 65:1237–1246.

Enzlin P, Mathieu C, Vanderschueren D, Demyttenaere K(1998). Diabetes mellitus and female sexuality: a review of25 year’s research. Diabetes Med 15:809–815.

Ertekin C (1998). Diabetes mellitus and sexual dysfunction.Scand J Sexol 1:3–21.

Ertekin C, Ertekin N, Almis S (1989). Autonomic sympa-thetic nerve involvement in diabetic impotence. NeurourolUrodynamics 8:589–598.

Ertekin C, Sirin H (1993). X-linked bulbospinal muscularatrophy (Kennedy’s syndrome): a report of three cases.Acta Neurol Scand 87:56–61.

Faerman I, Glocer L, Fox D, Jadzinsky MN, Rapaport M(1974). Impotence and diabetes. Histological studies of theautonomic nervous fibers of the corpora cavernosa inimpotent diabetic males. Diabetes 23:971–976.

Fairburn CG, Wu FCW, McCulloch DK et al. (1982). Theclinical features of diabetic impotence: a preliminarystudy. Br J Psychiatry 140:447–452.

Fowler C, Miller J, Sharief M, for the Sildenafil StudyGroup. (1999). Viagra (sildenafil citrate) for the treatmentof erectile dysfunction in men with multiple sclerosis.124th Annual Meeting American Neurological Association,Seattle. [Abstract:].

Fugl-Meyer AG, Fugl-Meyer K, Lundberg PO (1999). Sexualrehabilitation. In: Frommelt P, Grötzbach H eds. NeuroRehabilitation Berlin-Wien: Blackwell, pp. 370–388.

Garside S, Rosebush PI, Levinson AJ, Mazurek MF (1999).Late-onset adrenoleukodystrophy associated with long-standing psychiatric symptoms. J Clin Psychiatry60:460–468.

Gerstenberg TC, Levin RJ, Wagner G (1990). Erection andejaculation in man. Assessment of the electromyographicactivity of the bulbocavernosus and ischiocavernosusmuscles. Br J Urol 65:395–402.

Gerstenbrand F, Lücking CH (1971). Hypersexualität imRahmen der Klüver-Bucy-Symptomatik nach traumatis-chem apallischem Syndrom. J Neuro-Visceral RelationsSupplement X:524–537.

Ghezzi A (1999). Sexuality and multiple sclerosis. Scand JSexol 2:125–140.

Ghezzi A, Malvestiti GM, Baldini S, Zaffaroni M, Zibetti A(1995). Erectile impotence in multiple sclerosis patients. JNeurol 242:123–126.

Ghezzi A, Zaffaroni M, Baldini S, Zibetti A (1996). Sexualdysfunction in multiple sclerosis male patients in relationto clinical findings. Eur J Neurol 53:462–466.

Giuliano FA, Rampin O, Benoit G, Jardin A (1995). Neuralcontrol of penile erection. Urol Clinics North Am22:747–766.

Goldner GT, Morrell MJ (1996). Nocturnal penile tumes-cence and rigidity evaluation in men with epilepsy.Epilepsia 37:1211–1214.

Goldstein I, Lue TIF, Padma-Nathan H, Rosen RC, SteersWD, Wicker PA (1998). Oral Sildenafil in the treatmentof erectile dysfunction. New Eng J Med 338:1397–1404.

Greene LF, Kelalis PP. Weeks PE (1963). Retrograde ejacu-lation of semen due to diabetic neuropathy. Fertil Steril14:617–625.

Guiliano F, Hultling C, El Masry WS et al. (1999).Randomizaed trial of sildenafil for the treatment of erec-tile dysfunction in spional cord injury. Ann Neurol46:15–21.

Haldeman S, Lue T, Krane RJ, Shabsigh R, Bradley W(1995). Assessment. Neurological evaluation of male sex-ual dysfunction. Neurology 45:2287–2292.

Hallen O (1971). Über Potenz- und Libidostörungen bei derprogressiven Muskeldystrophie. J Neuro-VisceralRelations Supplement X:573–579.

Hayman LA, Rexer JL, Pavol MA, Strite D, Meyers CA(1998). Klüver–Bucy syndrome after bilateral selectivedamage of amygdala and its cortical connections. JNeuropsych Clin Neurosci 10. 354–358.

Heaton JPW (2000). Apomorphine: an update of clinical trialresults. Int J Impotence Res 12:S67–S73.

Heller L, Keren O, Aloni R, Davidoff G (1992). An opentrial of vacuum penile tumescence. constriction therapyfor neurological impotence. Paraplegia 30:550–553.

Helström L, Lundberg PO (1992). Vibratory perceptionthresholds in the female genital region. Acta Neurol Scand86:635–637.

Hirsh I, Smith R, Chancellor M, Bagley D, Carsello J, StassW (1994). Use of intracavernous injection ofprostaglandin E1 for neuropathic erectile dysfunction.Paraplegia 32:661–664.

Hirshkowitz M, Karacan I, Rando KC, Williams RL, HowellJW (1990). Diabetes, erectile dysfunction and sleep-relat-ed erections. Sleep 13:53–68.

Hodder J (1997). Shy Drager syndrome. Axone 18:75–79.Hokezu Y, Yanai S, Nagai M, Nagamatsu K, Yamamoto Y

(1996). [A case of Kennedy-Alter-Sung (KAS) syndromepresenting as hypersexuality and elevetad serum CK: use-fulness of genetic analysis]. (In Japanese). RinshoShinkeigaku 36: 471–474 [PubMed abstract].

Hopkins A, Clarke C, Brindley G (1974). Erections on walk-ing as a symptom of spinal canal stenosis. J NeurolNeurosurg Psych 50:1371–1374.

Hoyle CHV, Lincoln J, Burnstock G (1994). Neural controlof pelvic organs. In: Rushton DN, ed. Handbook ofNeuro-Urology New York: Marcel Dekker, pp. 1–54.

Hulter BM, Berne C, Lundberg PO (1998). Sexual functionin women with insulin dependant diabetes mellitus. ScandJ Sexol 1:43–50.

Hulter BM, Lundberg PO (1994). Sexual function in womenwith hypothalamo-pituitary disorders. Arch SexualBehaviour 23:171–183.

Hulter BM, Lundberg PO (1995). Sexual function in womenwith advanced multiple sclerosis. J Neurol NeurosurgPsych 59:83–86.

Hultling C, Giuliano F, Quirk F, Pena B, Mishra A, SmithMD (2000). Quality of life in patients with spinal cordinjury receiving Viagra (sildenafil citrate) for the treatmentof erectile dysfunction. Spinal Cord 38:363–370.

Hyyppä MT, Falck SC, Rinne UK (1975). Is L-DOPA anaphrodisiac in patients with Parkinson’s disease?. In:Sandler M, Gessa GL, eds. Sexual Behavior:Pharmacology and Biochemistry New York: Raven Press,pp. 315–327.

IIEF (1997). The International Index of Erectile Function. Amultidimensional scale for assessment of erectile dysfunc-tion. Urology 49:822–830.

Isojärvi JIT, Repo M, Pakarinen AJ, Lukkarinen O, MyllyläVV (1995). Carbamazepine, phenytoin, sex hormones, and

10653_Supp3_pp02_24 19/6/01 17:04 Page 20



sexual function in men with epilepsy. Epilepsia 36:366–370.Jensen SB (1981). Diabetic sexual dysfunction: a comparative

study of 160 insulin-treated diabetic men and women andan age-matched control group. Arch Sexual Behavior10:493–504.

Jokelainen M, Palo J (1976). Amyotrofic lateral sclerosis andautonomic nervous system. Lancet June 5:1246.

Kalliomäki JL, Markkanen TK, Mustonen VA (1961).Sexual behavior after cerebral vascular accident. FertilSteril 12:156–158.

Kamischke A, Nieschlag E (1999). Treatment of retrogradeejaculation and anejaculation. Human Reprod Update5:448–474.

Kaneko S, Bradley WE (1986). Evaluation of erectile dys-function with continuous monitoring of penile rigidity. JUrol 136:1026–1029.

Kaplan HS (1974). The New Sex Therapy. New York:Brunner, Mazel.

Karacan I, Ilaria RL (1978). Nocturnal penile tumescence(NPT): the phenomenon and its role in the diagnosis ofimpotence. Sexual Disab 1:260–271.

Karacan I, Scott FB, Salis PJ et al. (1977). Nocturnal erec-tions, differential diagnosis of impotence and diabetes.Biol Psychiatry 12:373–380.

Karacan I, Salis PJ, Ware JC et al. (1978). Nocturnal peniletumescence and diagnosis on diabetic impotence. Am JPsych 135:191–197.

Keitner GI, Selub S (1983). Spontaneous ejaculations andneuroleptics. J Clin Psychopharmacol 3:34–36.

Kihara K, Degroat WC (1997). Sympathetic efferent path-ways projecting bilaterally to the vas deferens in the rat.Anatom Records 248:291–299.

Kirkeby H, Petersen T, Poulsen E (1988). Pharmacologicallyinduced erection in patients with multiple sclerosis. ScandJ Urol Nephrol 22:241–244.

Kirkeby HJ, Poulsen EU, Petersen T, Dørup J (1988).Erectile dysfunction in multiple sclerosis. Neurology38:1366–1371.

Koller WC, Vetere-Overfield B, Williamson A, Busenbark K,Nash J, Parrish D (1990). Sexual dysfunction inParkinson’s disease. Clin Neuropharmacol 13:461–463.

Kolodny RC (1971). Sexual dysfunction in diabetic females.Diabetes 20:557–559.

Kolodny RC, Kahn CB, Goldstein HH, Barnett DM (1974).Sexual dysfunction in diabetic men. Diabetes 23:306–309.

Korpelainen JT, Kaulhanen M-L, Kemola H, Malinen U,Myllylä VV (1998). Sexual dysfunction in stroke patients.Acta Neurol Scand 98:400–405.

Koskinen T, Pihko H, Voutilainen R (1995). Primary hypog-onadism in females with infantile onset spinocerebellarataxia. Neuropediatrics 26:263–266.

Kreutzer JS, Zasler ND (1989). Psychosexual consequencesof traumatic brain injury. Brain Injury 3:177–186.

Kunze K, Leitenmaier K (1976). Vitamin B12 deficiency andsubacute combined degeneration of the spinal cord. In:Vinken PJ, Bruyn GW, eds. Handbook of ClinicalNeurology 28: 141–198.

Kwan M, Greenleaf WJ, Mann J, Crapo L, Davidson JM(1983). The nature of androgen action on male sexuality:a combined laboratory-self-report study on hypogonadalmen. J Clin Endocrinol Metab 57:557–562.

Lehman TP, Jacobs JA (1983). Etiology of diabetic impo-tence. J Urol 129:291–294.

Levin RJ (1998). Sex and the human reproductive tract –

what really happens during and after coitus. Int JImpotence Res 10:S14–S21.

Lipe H, Longstreeeth WT, Bird TD, Linde M (1990). Sexualfunction in married men with Parkinson’s disease com-pared to married men with artritis. Neurology40:1347–1349.

Lombroso PJ, Scahill LD, Chappell PB, Pauls DL, CohenDJ, Leckman JF (1995). Tourette’s syndrome: a multi-generational, neuropsychiatric disorder. Adv Neurol65:305–318.

Lue TF (1990). Impotence. A. patient’s goal directedapproach to treatment. World J Urol 8:67–74.

Lue TF (1996). Editorial comment. J Urol 156:552.Lundberg PO (1966). Observations on endocrine function in

ocular myopathy. Acta Neurol Scand 42:39–61.Lundberg PO (1980). Neurological disorders in andrology.

In: Bain J, Hafez ESE, eds. Diagnosis in Andrology TheHague: Nijhoff Publishers, pp. 195–213.

Lundberg PO (1992). Sexual dysfunction in patients withneurological disorders. Ann Rev Sex Res 3:121–150.

Lundberg PO, ed. (1994) Sexologi Stockholm: Liber.Lundberg PO (1997a). Sexual dysfunction in selected neuro-

logical and endocrinological disorders. Bailliere’s ClinPsych 3:113–130.

Lundberg PO (1997b). Catamenial epilepsy. a review.Cephalagia 17:42–45.

Lundberg PO (1999). Physiology of female sexual functionand effect of neurological disease. In: Fowler CJ, ed.Neurology of Bladder Bowel, and Sexual DysfunctionBoston: Butterworth Heinemann, pp. 33–46.

Lundberg PO (2000). Priapism. Review. Scand J Sexol3:13–24.

Lundberg PO, Biriell C (1993). Sexual dysfunction as a sus-pected drug reaction reported to WHO CollaboratingCentre for International Drug Monitoring. Thérapie48:457–459.

Lundberg PO, Biriell C (1998). Sexual dysfunction as a sus-pected adverse reaction to antidepressant drugs. Scand JSexol 1:97–105.

Lundberg PO, Brackett LB, Denys P et al. (2000).Neurological disorders. Erectile and ejaculatory dysfunc-tion. In: Jardin A, Wagner G, Khoury S, Giuliano F,Padma-Nathan H, Rosen R, eds. Erectile DysfunctionPlymouth: Plymbridge Distributors Ltd, pp. 591–645.

Lundberg PO, Brattberg A (1992). Sexual dysfunction inselected neurological disorders. Seminars Neurol12:115–119.

Lundberg PO, Wide L (1978). Sexual function in males withpituitary tumours. Fertil Steril 29:175–179.

Marinkovic Z, Prelevic G, Würzburger M, Nocic S (1990).Gonadal dysfunction in patients with myotonic dystro-phy. Exp Clin Endocrinol 96:37–44.

Marson L, Platt KB, McKenna KE (1993). Central nervoussystem innervation of the penis as revealed by thetransneuronal transport of pseudorabies virus.Neuroscience 55:263–280.

Masters WH, Johnson VE (1966). Human Sexual ResponseBoston: Little, Brown.

Mastrogiacomo I, Pagani E, Novelli G et al. (1994). Malehypogonadism in myotonic dystrophy is related to (CTG)n triplet mutation. J Endocrinol Invest 17:381–383.

Maytom MC, Derry FA, Dinsmore WW et al. (1999). A. two-part stury of sildenafil (Viagra) in men with erectile dys-function by spinal cord injury. Spinal Cord 37:110–116.

10653_Supp3_pp02_24 19/6/01 17:04 Page 21



Mattson D, Petrie M, Srivastava K, McDermott M (1995).Multiple sclerosis. Sexual dysfunction and its response tomedications. Arch Neurol 52:862–868.

McDougall AJ, McLeod JG (1996). Autonomic neuropathy,I. Clinical features, investigation, pathophysiology, andtreatment. J Neurol Sci 137:79–88.

McKenna KE, Chung SK, McVary KT (1991). A model forthe study of sexual function in anesthetized male andfemale rats. Am J Physiol 261:R1276.

Melberg A, Arnell H, Dahl N et al. (1996). Anticipation ofautosomal progressive external ophthalmoplegia withhypogonadism. Muscle Nerve 19:751–757.

Melman A, Henry DP, Felten DL, O’Connnor B (1980a).Effect of diabetes upon penile sympathetic nerves inimpotent patients. Southern Med J 73:307–310.

Melman A, Henry DP, Felten DL, O’Connor B (1980b).Alteration of the penile corpora in patients with erectileimpotence. Invest Urol 17:474–477.

Meuleman EJ, Diemont WL (1995). Investigation of erectiledysfunction. Diagnostic testing for vascular factors inerectile dysfunction. Urol Clinics North Am 22:803–819.

Meyer JE (1955). Die sexuellen Störungen der Hirnverletzten.Arch Psychiatatrie Z Nervenheilkunde 193:449–469.

Miller BL, Cummings JL, McIntyre H, Ebers G, Grode M(1986). Hypersexuality or altered sexual preference follow-ing brain injury. J Neurol Neurosurg Psych 49:867–873.

Miller J, Fowler C, Sharief M, for the Sildenafil Study Group(1999). Effect of Viagra (sildenafil citrate) on quality oflife in men with erectile dysfunction and multiple sclero-sis. 124th Annual Meeting of the American NeurologicalAssociation, Seattle [Abstract].

Minderhoud JM, Leemhuis JG, Kremer J, Labon E, SmitsPML (1984). Sexual disturbances arising from multiplesclerosis. Acta Neurol Scand 70:299–306.

Mitsuya H, Asai J, Suyama K, Ushida T, Hosoe K (1960).Application of X-ray cinematography in urology. 1.Mechanisms of ejaculation J Urol 83:86–95.

Monga TN, Lawson JS, Inglis J (1986). Sexual dysfunctionin stroke patients. Arch Phys Med Rehab 67:19–22.

Monga TN, Monga M, Raina MS, Hardjsudarma M (1986).Hypersexuality in stroke. Arch Phys Med Rehab67:415–417.

Montagna F (1999). [Fatal familial insomnia. clinical, labo-ratory and pathological features.] (In Spanish). RevueNeurologique 29: 1006–1009 [PubMed Abstract.].

Montejo-González AL, Liorca G, Izquierdo JA et al. (1997).SSRI-induced sexual dysfunction. Fluoxetine, paroxetine,sertraline, and fluvoxamine in a prospective, multicenter,and descriptive clinical study of 344 patients. J SexMarital Ther 23: 176–194.

Montorsi F, Maga T, Strambi LF, Salonia A, Berbieri L,Scattonmi V, Guazzoni G, Losa A, Rigatti P, Pizzini G(2000). Sildenafil taken at bedtime significantly increasesnocturnal erection: results of a placebo-controlled study.Urology 56:906–911.

Morales A (1994). Impotence. J Urol 151:1235–1238.Morales A, Condra M, Reid K (1990). The role of nocturnal

penile tumescence monitoring in the diagnosis of impo-tence: a review. J Urol 143:441–446.

Morrell MJ, Sperling MR, Stecker M, Dichter MA (1994).Sexual dysfunction in partial epilepsy. A deficit in physi-ological arousal. Neurology 44:243–247.

Morris M (1995). Dementia and cognitive changes inHuntington’s disease. Adv Neurol 65:187–200.

Mueller SC, Lue TF (1988). Evaluation of vasculogenicimpotence. Urol Clinics North Am 15:650–676.

Muhr C, Hulting A-L, Lundberg PO, Werner S (1985).Pituitary adenomas with hyperprolactinaemia in males.In: Auer LM, Leb G, Tscherne G, Urdl W, Walter GGeds. Prolactinomas An Interdisciplinary Approach Berlin:Walter de Gruyter and Co, pp. 169–178.

Neubauer M (1971). Die Behandlung von Potenzstörungenbeim Diabetiker. Therapiewoche 21:614–620.

Neuhäuser G, Opitz JM (1975). Autosomal recessive syn-drome of cerebellar ataxia and hypogonadotropic hypog-onadism. Clin Genet 7:426.434.

Newman AS, Bertelson AD (1986). Sexual dysfunction indiabetic women. J Behav Med 9:261–270.

Obayashi K, Ando Y, Terazaki H et al. (2000). Effect ofsildenafil citrate (Viagra) on erectile dysfunction in apatient with falilial amyloidotic polyneuropathy ATTRVal30Met. J Auton Nerv Syst 80:89–92.

Oliveira V, Ferro JM, Foreid JP, Costa T, Levy A (1989).Klüver–Bucy syndrome in systemic lupus eruthematosus.J Neurol 236:55–56.

O’Leary MP, Fowler FJ, Lenderking WR et al. (1995). Abrief male sexual function inventory for urology. Urology46:697–706.

Olsson T, Olofsson B-O, Hägg E, Grankvist K, HenrikssonA (1996). Adrenocortical and gonadal abnormalities indystrophia myotonica – a common enzyme defect? Eur JIntern Med 7:29–33.

Padma-Nathan H (1988). Neurological evaluation of erectiledysfunction. Urol Clinics North Am 15:77–80.

Padma-Nathan H, Hellstrom WJ, Kaiser FE et al. (1997).Treatment of men with erectile dysfunction withtransurethral alprostadil. New Eng J Med 336:1–7.

Patterson WM (1993). Fluoxetine induced sexual dysfunc-tion. J Clin Psychiatry 54:71.

Penfield W, Jasper H (1954). Epilepsy and the FunctionalAnatomy of the Human Brain Boston: Little, Brown.

Perese DM, Prezzo JA, Perese EF (1976). Sexual dysfunctioncaused by injuries of the cervical spinal cord withoutparalysis. Spine 1:149–154.

Powers JM, Schaumberg HH (1980). A. fatal cause of sexu-al inadequacy in men: adrenoleukodystrophy. J Urol124:583–585.

Price DE, Boolell M, Gepi-Attee S, Wareham K, Yates P,Gingell JC (1998). Sildenafil. A. study of a novel oraltreatment for erectile dysfunction in diabetic men.Diabetes Med 15:821–825.

Quadri R, Veglio M, Flecchia D et al. (1989). Autonomicneuropathy and sexual impotence in diabetic patients.Analysis of cardiovascular reflexes. Andrologia 21:346–352.

Rendell MS, Rajfer J, Wicker PA, Smith MD (1999). for theSildenafil Diabetes Study Group Sildenafil for treatmentof erectile dysfunction in men with duabetes. J Am MedAssoc 281:421–426.

Rivas D, Chancellor M (1994). Complications associatedwith the use of vacuum contsriciton devices for erectiledysfunction in the spinal cord injured population. J AmParaplegia Soc 17:136–139.

Sachs BD (1995). Placing erection in context: The reflexo-genic psychogenic dichotomy reconsidered. NeurosciBiobehav Rev 19:211–224.

Saenz de Tejada I, Ware JC, Blanco R et al. (1991).Pathophysiology of prolonged penile erection associatedwith trazodone use. J Urol 145:60–64.

10653_Supp3_pp02_24 19/6/01 17:04 Page 22



Sakakibara R, Hatteri T, Fukutake T, Mori M, YamanishiT, Yasuda K (1998). Micturitional disturbances in apatient with adrenomyelo-neuropathy (AMN). NeurourolUrodynamics 17:207–212.

Sato K (1991). Seminal emission by electrical stimulation ofthe spermatic nerve and epidydimis. Intern J Androl14:461–463.

Saunders M, Rawson M (1970). Sexuality in male epileptics.J Neurol Sci 10:577–583.

Sawyer SM, Roberts KV (1999). Sexual and reproductivehealth in young people with spina bifida. Dev Med ChildNeurol 41:671–675.

Schirren C, Rehacek M, De Cooman S, Widman H-U (1973).Die retrograde Ejakulation. Andrologie 5:7–14.

Schmid DM, Schur B, Hauri D (2000). Sildenafil in the treat-ment of sexual dysfunction in spinal cord-injured patients.Eur Urol 38:184–193.

Schreiner-Engel P, Schiavi RC, Vietorisz D, Smith H (1987).The differential impact of diabetes type on female sexual-ity. J Psychosom Res 31:23–33.

Seckin B, Atmaca I, Ozgok Y, Gokalp A, Harmankaya C(1996). External vacuum device therapy for spinal cordinjured males with erectile disorders. Int Urol Nephrol28:235–240.

Shukla GD, Srivastava ON, Katiyar BC (1979). Sexual dis-turbances in temporal lobe epilepsy: a controlled study. BrJ Psychiatry 134:288–292.

Sidi A, Cameron J, Dystra D, Reinberg Y, Lange P (1987).Vasoactive intracavernous pharmacotherapy for the treat-ment of erectile impotence in men with spinal cord injury.J Urol 138:539–542.

Simpson G, Blaszczynski A, Hodgkinson A (1999). Sexoffending as a psychosocial sequela of traumatic braininjury. J Head Trauma Rehab14:567–580.

Singer C, Weiner WJ, Sanchez-Ramos JR (1992). Autonomicdysfunction in men with Parkinson’s disease. Eur Neurol32: 134–140.

Sipski ML, Alexander CJ, Rosen R (2001). Sexual arousaland orgasm in women: Effects of spinal cord injury. AnnNeurol 49:35–44.

Sitsen JMA (1998). Prescription drugs and sexual function.In: Money J, Musaph H eds. Handbook of Sexology Vol.VI. Amsterdam/New York/London: Elsevier, pp.425–461.

Sjögren K, Damber J-E, Lilieqvist B (1983). Sexuality afterstroke. I. Aspects of sexual function. Scand J Rehab Med15:55–61.

Slob AK, Koster J, Radder JK, van der Werff ten Bosch JJ(1990) Sexuality and psychophysiological functioning inwomen with diabetes mellitus. J Sex Marital Ther16:59–69.

Smith EM, Bodner DR (1993). Sexual dysfunction afterspinal cord injury. Urol Clinics North Am 20:535–542.

Takahashi A (1991). Autonomic nervous system disorders inParkinson’s disease. Eur Neurol 31:41–47.

Takatsuki K, Sanada I (1983). Plasma cell dyscrasia withpolyneuropathy and endocrine disorder: clinical and lab-oratory features of 109 reported cases. Jap J Clin Oncol13:543–555.

Taylor DC (1969). Sexual behavior and temporal lobe epilep-sy. Arch Neurol 21:510–516.

Thase ME, Reynolds CF III, Jennings JR et al. (1988).Nocturnal penile tumescence is diminished in depressedmen. Biol Psychiatry 24:33–46.

The Therapeutics and Technology Assessment Subcommitteeof the American Academy of Neurology (1995).Assessment. neurological evaluation of male sexual dys-function. Neurology 45:2287–2292.

Tjandra BS, Janknet RA (1997). Neurogenic impotence andlower urinary tract symptoms due to vitamin B1 deficien-cy in chronic alcoholism. J Urol 157:954–955.

Tyrer G, Steel JM, Ewing DJ, Bancroft J, Warner P, ClarkeBF (1983). Sexual responsiveness in diabetic women.Diabetologia 24:166–171.

Uitti RJ, Tanner CM, Rajput AH, Goetz CG, Klawans HL,Thiessen B (1989). Hypersexuality with antiparkinsoniantherapy. Clin Neuropharmacol 12:375–383.

Valleroy M, Kraft G (1984). Sexual dysfunction in multiplesclerosis. Arch Phys Med Rehab 65:125–128.

Vardi Y, Sprecher E, Kanter Y, Livne PM, Hemli JA,Yarnitsky D (1996). Polyneuropathy in impotence. Int JImpotence Res 8:65–68.

Vardi Y, Gruenwald I, Sprecher E, Gertman I, Yartnisky D(2000). Normative values for female genital sensation.Urology 56:1035–1040.

Vas CJ (1969). Sexual impotence and some autonomic dis-turbances in men with multiple sclerosis. Acta NeurolScand 45:166–182.

Vodusek DB (1998). Neurophysiological tests in erectile dys-function. Scand J Sexol 1:81–95.

Vodusek DB, Fowler CJ (1999). Clinical neurophysiology. In:Fowler CJ eds. Neurology of Bladder, Bowel, and SexualDysfunction Boston: Butterworth Heinemann pp. 109–143.

Vodusek DB, Zidar J (1987). Pudendal nerve involvement inpatients with hereditary motor and sensory neuropathy.Acta Neurol Scand 76:457–460.

Wabrek AJ, Wabrek CJ, Burchell RC (1978). The humantragedy of spina bifida. Spinal myelomeningocele. SexualDisab 1:210–217.

Wasserman MD, Pollak CP, Spielman AJ, Weitzman ED(1980). Theoretical and technical problems in the mea-surement of nocturnal penile tumescence for differentialdiagnosis of impotence. Psychosomatic Med 42:575–585.

Wermuth L, Stenager E (1995). Sexual problems in youngpatients with Parkinson’s disease. Acta Neurol Scand91:453–455.

Westgren N (1999). Women with traumatic spinal cordinjury. Doctoral Thesis. Stockholm: Karolinska Institute.

Wieder JA, Brackett NL, Lynne CM, Green JT, Aballa TC(2000). Anesthetic block of the dorsal penile nerve inhibitsvibratory-induced ejaculation in men with spinal cordinjuries. Urology 55:915–917.

Wiig EH (1973). Counseling the adult aphasic for sexualreadjustment. Rehabilitation Counseling BulletinDecember: 110–119.

Wincze JP, Albert A, Bansal S (1993). Sexual arousal in dia-betic females: physiological and self-report measures. ArchSex Behav 22: 587–601.

Yang CC, Bowen JR, Kraft GH, Uchio EM, Kromm BG(2000). Cortical evoked potentions of the dorsal nerve ofthe clitoris and female sexual dysfunction in multiple scle-rosis. J Urol 164:2010–2013.

Yarnitsky D, Sprecher E, Vardi Y (1996). Penile thermal sen-sation. J Urol 156:391–393.

Zesiewicz TA, Helal M, Hauser RA (2000). Sildenafil citrate(Viagra) for the treatment of erectile dysfunction in menwith Parkinson’s disease. Movement Disorders 15:305–308.

10653_Supp3_pp02_24 19/6/01 17:04 Page 23



Zivadinov R, Zorzon M, Bosco A et al. (1999). Sexual dys-function in multiple sclerosis: II. Correlation analysis.Multiple Sclerosis 5:428–431.

Zrustová M, Rostlapil J, Kabrhelová A (1978). Sexuálniporuchy u zen úplavicí cukrovou. Ceske Gynekologie43:277–280.

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