ORIGINAL ARTICLE

Language abilities and gestural communication in a girlwith bilateral perisylvian syndrome: a clinical and rehabilitativefollow-up

Bruna Molteni • Daniela Sarti • Gloria Airaghi • Chiara Falcone •

Giulia Mantegazza • Giovanni Baranello • Federica Riva •

Veronica Saletti • Nicoletta Paruta • Daria Riva

Received: 16 January 2009 / Accepted: 9 April 2010 / Published online: 2 June 2010

� Springer-Verlag 2010

Abstract We present the neuropsychological and lin-

guistic follow-up of a girl with bilateral perisylvian poly-

microgyria during 4 years of gestural and verbal speech

therapy. Some researchers have suggested that children

with bilateral perisylvian polymicrogyria mentally fail to

reach the syntactic phase and do not acquire a productive

morphology. This patient achieved a mean length of

utterance in signs/gestures of 3.4, a syntactic phase of

completion of the nuclear sentence and the use of mor-

phological modifications. We discuss the link between

gesture and language and formulate hypotheses on the role

of gestural input on the reorganization of compensatory

synaptic circuits.

Keywords Bilateral perisylvian polymicrogyria �Gestural communication � Neuropsychological follow-up

Introduction

There is plenty of information in literature to support the

close relationships between gesture and language [1–8].

The comprehension and production of gestures and lan-

guage evolve in typically developing children in a close

temporal relationship and are mediated by adjacent and

partially shared cortical regions.

Many neuroimaging studies have demonstrated that the

motor representations of oral and manual movements are

adjacent and partially overlap in many frontal and parietal

regions, including the primary motor and ventral pre-motor

areas, and the intraparietal and inferior parietal sulci

[9–12]. A complex neuronal ‘‘mirror’’ system has been

demonstrated in some of these areas.

According to Iacoboni and Wilson [13], the discovery of

the mirror system strongly supports the close link between

gesture and language because it provides the anatomical

substrate that enables human beings to associate the

observation and execution of actions of the mouth and

hands, which is the first step toward imitation. In addition

to Broca’s area, the mirror system also involves the supe-

rior temporal cortex, which overlaps with Wernicke’s area

and has neurons that are activated with movements of the

face and body, and an area in the parietal cortex that

combines visuospatial information. This circuit enables

imitation and amply overlaps with the well-known areas of

language, suggesting a developmental continuity between

action, observation, imitation and language. In addition, a

few studies have recently shown that it might provide the

neuronal substrate for an alternative functional reorgani-

zation, which could be stimulated in the rehabilitation

setting in pathological conditions.

In recent years, numerous studies have concentrated on

the spontaneous gestural production in children with neu-

rological disorders and language impairment [14, 15].

These papers had the purpose of establishing whether ges-

tural development followed typical or atypical patterns in

comparison with typically developing children, and whether

gestures compensated for the deficiency in spoken language

or facilitated language development in such infants.

B. Molteni (&) � D. Sarti � G. Airaghi � G. Mantegazza �G. Baranello � F. Riva � V. Saletti � N. Paruta � D. Riva

Developmental Neurology Division,

Fondazione I.R.C.C.S. Istituto Neurologico ‘‘Carlo Besta’’,

via Celoria, 11, 20133 Milan, Italy

e-mail: bruna.molteni@istituto-besta.it

C. Falcone

Neuroepidemiology Unit,

Fondazione I.R.C.C.S. Istituto Neurologico ‘‘Carlo Besta’’,

via Celoria, 11, 20133 Milan, Italy

123

Neurol Sci (2010) 31:471–481

DOI 10.1007/s10072-010-0309-2

In the last two decades, augmentative communication

systems relying on visual signs and/or symbols have been

used in hearing children with a severely impaired capacity

for oral expression. Analyzing language development in

cognitively impaired children without hearing problems

exposed to a systematic signed input, some authors [16, 17]

found that the mean length of utterance (MLU) remained

below 3, the utterances did not become more complex as

they increased in length and the semantic relationships

expressed were generally very simple, with a large pro-

portion of utterances produced by juxtaposing words or

‘‘chaining items’’ [18]. They also reported that lexical

categories mainly consisted of open-class elements (nouns,

verbs, adjectives and adverbs).

In contrast to the study by Grove and Dockrell [17],

reporting that such children fail to reach morphological

competences of language production, Rudd et al. [19] have

recently conducted an experimental study consisting of

teaching morphological changes in signs to a group of

hearing children with cognitive impairment. Interestingly,

they found that the children were capable of learning

productive morphological rules of the signs.

In relation to the development of pragmatic competences,

a few longitudinal studies have examined the conversational

acts produced by children who use augmentative commu-

nication systems. Some authors have identified a persistent

impairment in the functional use of these systems and a

tendency to adopt a passive role in the interaction [20].

Bilateral perisylvian polymicrogyria (BPP) is of particu-

lar interest among the neurological developmental disorders

associated with severe language impairment. BPP was the

first bilateral polymicrogyria syndrome to be described [21]

and it is the most common [22]. It is clinically characterized

by a pseudobulbar or supranuclear cortical palsy with facial,

pharyngeal, glossal and masticatory diplegia, mental retar-

dation and epilepsy, and by language difficulties that are

often more severe than might be expected from the severity

of the pseudobulbar palsy. Sans and Fernandez–Alvarez [23]

suggested that the reason for these difficulties might lie both

in the oro-motor dyspraxia and in a more extensive dys-

function of the cortical areas responsible for language.

In a recent study [24] evaluating the neuropsychological

characteristics, and language in particular, of six children

with BPP, we found a globally impaired verbal and gestural

communication and the inability to reach a primitive syn-

tactic phase (i.e., incomplete and some nuclear sentences,

according to the model of morphological language devel-

opment proposed by Cipriani et al. [25]). In that study,

however, all the children underwent different types of

rehabilitation programs at different institutions and we

were not able to formulate any hypotheses on the role of

the gestural input in the language reorganization of these

children with bilateral lesions.

In the present study, we used signed Italian (SI), which

combines lexicon from Italian sign language (ISL) with the

grammar of spoken Italian (e.g., ball over table), and exact

signed Italian (ESI), which in addition to SI uses ‘‘finger

spelling’’ (a form of visual and manual alphabet), to

‘‘write’’, in space, those aspects that were missing or dif-

ferently produced in the ISL. The aim of the study was to

analyze the progress in lexical, syntactic, morphological

and pragmatic terms the capabilities of a girl affected by

BPP and exposed to signed Italian and later to exact signed

Italian [26, 27] during a 4-year speech therapy at our

institution.

Materials and methods

Case description

Clinical history

The girl had no family history of neurological diseases and

was the offspring of a reportedly normal pregnancy and

delivery. At birth, she had tremors treated with phenobar-

bital. At 2 months, hematological tests revealed a recent

cytomegalovirus infection. She had sucking/swallowing

difficulties and delayed motor development, learning to

walk independently at 2 years. She also had severely

delayed language development with reduced vocalization

and no babbling, as well as oro-motor problems with

dribbling and eating difficulties. No epileptic seizures were

reported. From the age of 12 months, she underwent psy-

chomotor rehabilitation treatment. At the age of 3 years,

she started speech therapy at another institution, using a

system of visual symbols of augmentative alternative

communication (AAC).

Clinical examination and neuropsychological assessment

The girl came to our observation at the age of 7 years and

4 months for an opinion on the rehabilitative program. She

was attending the second year of primary school. MRI was

performed using a 1.5-T scanner taking T1-weighted sag-

ittal and T2-weighted axial views and scoring the extent of

polymicrogyria and the degree of associated hemispheric

distortion on the images according to the method used by

Jansen and Andermann [22]. The images revealed diffuse

bilateral asymmetrical (right [ left) polymicrogyria grade

3 [22] particularly evident at the perisylvian site (Fig. 1).

The frontal regions were also affected. Lateral ventricles

were asymmetrically enlarged (left [ right), mainly at

the level of the trigons. The EEG showed interictal

abnormalities with epileptiform activity, mainly over the

temporal regions. Neurological assessment revealed

472 Neurol Sci (2010) 31:471–481

123

pseudobulbar palsy with anarthria and both general and

fine motor difficulties. The girl was unable to close her

mouth or move her tongue. Her phonation was limited to

brief, raucous and unmodulated vocalizations. Velum

mobility was reduced and swallowing was accompanied by

a hyperextension of the head. Automatic chewing, cough-

ing or vomiting reflexes were absent. She had bilateral

tactile hypesthesia on her face, more marked on the left.

The imitation of manual gestures was better on the right

and the child could open her hand, extend her fingers, make

a fist, point and bring thumb and index finger in a clamping

action. School teachers and family members used oral

communication modalities and AAC based on visual

symbols, the latter however being poorly spontaneously

used by the child. Her communication consisted of pointing

and about 20 spontaneous isolated representational gestures

made with difficulty and with one hand accompanied by

rare vocalizations and facial mimicry. The girl underwent a

cognitive and language assessment. Scholastic abilities

were also assessed by means of a word spelling test. The

results are shown in Table 1.

We opted to use gestural communication in the reha-

bilitation setting to increase the girl’s language compe-

tences because of her severe dysarthria, cognitive

impairment, spared manual praxic abilities and the spon-

taneous preference for mimicry and gestures. We formu-

lated a speech therapy project aimed to develop all the

language components, following the steps of typical lan-

guage development. The creation of a communication

network that also involved the girl’s family and her school

was essential for this purpose.

The features and goals of the treatment are illustrated in

Table 2.

Assessment of language development

We analyzed the data collected five different times (T0, T1,

T2, T3, T4) at 1-year intervals, starting from the age of

7 years and 4 months. The samples analyzed lasted 20 min

and came from video-recorded sessions conducted in two

different settings: a conversation with the therapist who

communicated using SI and spoken language, and a figure-

describing task. In the conversational setting, the girl was

encouraged to spontaneously communicate with the thera-

pist about a present or past event or activity that she was

interested in. For the figure-describing task, she was asked to

describe 12 figures drawn from the childhood language

assessment tests ‘‘PFLI, Prove per la valutazione del lin-

guaggio infantile’’ [33].

The following gestural productions were considered:

• pointing: when the child used the hand to point to a

particular object or event;

• representational gestures: gestures having a basic

semantic content, relatively stable across different

situations; some were conventional and culturally

defined (e.g., HELLO, GREAT), while others appeared

to be more directly action related (e.g., TO SLEEP) or

object related (TELEPHONE);

• signs: from LIS lexicon used by the deaf community in

Milan;

• finger spelling.

Fig. 1 MRI T1-weighted axial scans showing diffuse bilateral

asymmetrical (right [ left) polymicrogyria grade 3 particularly

evident at the perisylvian site

Table 1 Cognitive, language and scholastic assessment at T0

Test Results

WISC-R [28] PIQ 57

Italian version of MacArthur-Bates Communicative Developmental

Inventory, gestures and words form [29]

Words produced: 0 (younger than 8 months old)

Gestures produced: 45 (50th percentile of 17 months)

Words comprehended: all the 408 words

Language comprehension assessment tests: protocol 3 [30] Average score for children aged 42–48 months

PPVT [31] word comprehension test QV \ 65 (QV 80 for age range 45–50 months)

Writing tests I elementary narration [32]

Self-dictated words in response to figurative stimulus (graduated internal test)

Poor (isolated, flat two-syllable words)

poor (three correct two-syllable words)

Neurol Sci (2010) 31:471–481 473

123

Gestural productions were recorded considering those

between two pauses as a clause and noting not only the

quantity per minute and their morphological and pho-

nological changes, but also the non-manual components

(e.g., facial mimicry). Non-manual elements such as gaze

and facial mimicry were also recorded because they may

contribute to the communicative efficacy of the gestural

communication, amplifying or modifying the meaning of

a given gesture/sign. Vocalizations with a prosodic value

were all noted, but not counted. Double-meaning

signs, e.g., signs that could have a meaning both as a

verb and as a noun, were interpreted on the basis of the

context.

We calculated the MLU of the productions in SI as for

spoken language and the mean number of gestural pro-

ductions per utterance. As a reference for the MLU

development stages, we used the model developed by

Cipriani et al. [25] for Italian-speaking children.

Inter-observer agreement in recording the sign samples

was calculated on a 10-min sample drawn from the fig-

ure-describing scenario and considered as follows: the

presence of the sign or of the gesture, the utterance

boundaries and the length of the utterance produced as

signs by the girl. One of the authors (Gloria Airaghi) was

the first observer, and the girl’s signing school teacher

was the second. The mean inter-observer agreement ran-

ged from 85 to 90%.

We examined the girl’s gestural linguistic abilities using

our dynamic communication evaluation (DCE) grid [34],

adapted to the clinical case considered. We analyzed the

following language levels: syntactic (Table 3), lexical

(Table 4), pragmatic (Table 5) and morphological. The

gestures were classified according to the Italian version of

MacArthur-Bates Communicative Development Invento-

ries [29] into four semantic categories: nouns, actions/

Table 2 Characteristics and objectives of the treatment

Type Twice-weekly individual sessions in the presence of a parent and/or school support teacher; regular sessions for teaching the signs to the

adult and classmates

Tools Vocabulary adapted from the Italian sign language; morpho-syntactic structure of the spoken Italian; all visual channels including video-

recordings

Short-term goals (7 years 4 months to 8 years

4 months)

Medium-term goals (8 years 4 months to 12 years 4 months)

Pragmatics Visual contact; selection and introduction of the

topic; taking turns; extension to different

conversational partners

Maintaining and changing the topic; adjacency and contingency;

cohesion; variation in communicative style; intelligibility and

accuracy; facial expressions

Lexicon Receptive: home and school lexical categories.

Productive: mainly signs for nouns; acceptance of

spontaneous gestures and expansion of categories

already used on the strength of interests and

motives

Receptive: district, work, free time lexical categories. Productive:

signs for verbs, adjectives and functors. Language

decontextualization; gradual correction of all distinctive parameters;

maintenance of a proper execution of the sign in progressively wider

contexts and for increasingly long times

Syntax Comprehension of coordinated phrases Comprehension of subordinate phrases. Phrase production: from

combining two signs to a complete simple nuclear phrase, first with

one and then with two topics. Introduction of morphology with

changes of gender and number; teaching of dactylology

Oro-motor

district

Returning to exploring the girl’s own face;

developing facial sensitivity; movement toward

the mouth and eating unassisted

Biting, chewing and drinking in small sips; facial expressions; first

significant vocalizations

Table 3 Adapted DCE grid: syntactic analysis

Isolated gestures

Syntax

Pre-syntactic phase consisting mainly of single gestures in

succession

Syntactic phase consisting of incomplete and complete simple

nuclear phrases

Phrase expansion phase: incomplete and complete expanded and

complex phrases

Arrangement

Conventional, unconventional, ambiguous, absent

Table 4 Adapted DCE grid: lexical analysis

Nouns Animals, vehicles, toys, food and drinks, clothing, body,

furniture and rooms, familiar objects, outdoors

Predicates Verbs, adjectives

Functors Pronouns, wh questions, prepositions, conjunctions,

articles, quantifiers

Other Sounds and voices of nature, people, routines, time

474 Neurol Sci (2010) 31:471–481

123

adjectives, functors and others (familial ‘‘routines’’, e.g.,

hello, great, thanks; people names).

We coded the phrasal structure of the gestural utter-

ances, drawing from the pattern of phrasal structure

development identified by Cipriani et al. [25] in Italian-

speaking children aged between 19 and 36–39 months. In

our analysis, we recorded the frequency of use of each

phrasal structure produced by the girl.

We analyzed whether the sequence of the gestural pro-

ductions in the utterance followed the sequence of the

words in the sentence in the spoken Italian language,

whether it could be well interpreted and whether it was

absent because the sentence was composed of only one

production. We also analyzed the sign modifications to see

whether the girl was acquiring morphological rules

enabling variations of meaning to be expressed.

First, we recorded the different referential gestures pro-

duced by the girl on the five assessments. The gestures

produced were classified according to the gesture produc-

tion development model in speaking children and for

communicative intentions according to recent models on

the development of intentional communication [26, 35–38].

We calculated the percentages and 95% confidence

intervals (CI) on the data recorded at the five assessments.

We also performed the Cochraine Armitage test for trend,

setting the level of significance at 5%.

We assessed cognitive and scholastic abilities and neu-

rological status again at T4. Cognitive development was

assessed by means of the WISC-R scales. To administer the

verbal scales, we used gestural communication as rein-

forcement for oral communication. Scholastic achievement

was assessed by means of a word spelling test.

Results

Syntactic structuring

The MLU was 1.3 gestural productions at T0 and T1 and

raised to 3.4 at T4 (Fig. 2). Data of the utterances are

shown in Fig. 3. The proportions of isolated gestures/signs

and associations of gestures/signs, classified as belonging

to the ‘‘pre-syntactic phase’’, significantly decreased over

time (p \ 0.001). On the whole, this type of production

represented 100% of the output at T0, but less than 40% at

T4. In particular, the isolated gestures decreased from 68%

at T0 and T1 to 4–16% at T3 and T4, while the pre-syn-

tactic productions dropped from around 30% in the earlier

assessments to around 15–20% at T3 and T4. There was a

corresponding statistically significant increasing trend in

the percentages of productions classified as belonging to

the ‘‘syntactic phase’’, i.e., complete and incomplete

nuclear sentences, and expanded and complex sentences,

both complete and incomplete (p \ 0.001). The nuclear

sentences (absent at T0) accounted for more than 50% of

the productions at T3 and T4, while there was a more

modest increase in the more complex structures, which

reached 11% at T4. With the appearance of the phrasal

structure, the sequence of the signs in the sentence

Table 5 Adapted DCE grid: pragmatic analysis

Communicative

intentions

Requests for attention, requests for action,

requests for information,

declarations on the object, declarations on the

subject

Communicative

role

Spontaneous, in response or in imitation

Communicative

means

Gaze on the focus and on the other party,

Please enter your comments here facial

mimicry, deictic gestures, pointing, referential

gestures, finger spelling

0

1

2

3

4

0 1 2 3 4

Times

ML

U

Fig. 2 Progress of the MLU

16%(8-28)

4%(0-13)

34%(25-45)

68%(58-77)

68%(60-75)

32%(25-40)

14%(6-26)

21%(12-34)

34%(25-45)

29%(20-39)

3%(1-9)

27%(18-37)

76%(62-87)

51%(38-64)

4%(1-10)

6%(1-16)

11%(5-22)

0% 10% 20% 30% 40% 50% 60% 70% 80% 90% 100%

0

1

2

3

4

Isolated gestures Presyntactic phase

Syntactic phase Extended and complexphrases

Fig. 3 Percentages and confidence intervals (95% CI) at the five

assessments of syntactic levels: isolated gestures; presyntactic phase;

syntactic phase; extended and complex phrases

Neurol Sci (2010) 31:471–481 475

123

followed that of the Italian spoken language, instead of LIS

(e.g., I’M NOT GOING HOME’’ instead of ‘‘I HOME

GOING NOT’’).

Vocabulary

The girl showed a statistically significant rising trend

(p \ 0.001) in the different referential gestures she pro-

duced (Fig. 4), starting from a percentage of 20% at T0 and

settling at a percentage varying between 50 and 60% in the

subsequent assessments. The analysis of the lexical cate-

gories (Fig. 5) showed a statistically significant declining

trend (p \ 0.002) for the category ‘‘other’’. The remaining

categories were represented in almost the same percentages

over time, despite the increase in the production of dif-

ferent gestures. In the category ‘‘functors’’, the causal

‘‘why/because’’ and the indeterminate articles and posses-

sive pronouns, which were missing at the earlier evalua-

tions, were recorded at T4. At first, she started to produce

WH-questions, pronouns and prepositions, respectively, by

means of signs. Later, she started to use finger spelling to

produce articles and to specify gender and number of

possessive pronouns.

Morphological sign modifications

Morphological sign modifications (at first according to the

grammar ISL rules expressed through specific alterations of

the sign’s location and of some movement features, such as

direction, duration, intensity or width: e.g., CHILD/

CHILDREN; I GIVE YOU/YOU GIVE ME; I GO/I WILL

GO, and later in addition with finger spelling: e.g., LITTLE

STREET/BIG STREET) were present in proportions below

4% on the first few assessments and occurred in sizeable

percentages only at T4 (70%; 95% CI: 58–81) (Fig. 6).

Communicative intentions (Fig. 7)

The proportion of requests for attention significantly

decreased while the proportion of declarations on the

object (i.e., gestures/signs describing objects or facts: the

ball is red; the weather is rainy) significantly increased

from T0 to T4 (p \ 0.001). Declarations on the subject

(i.e., gestures/signs describing her own action or wish—I

ate everything; I want to play) and requests for information

were absent throughout the period of observation.

Communicative role

The spontaneous productions significantly increased from

T0 to T4 (p \ 0.001) (Fig. 8), reaching nearly 100%

already at T2, while the productions in response and in

imitation tended to disappear.

20%(13-29)

52%(41-63)

61%(52-69)

53%(44-62)

62%(53-70)

0%

10%

20%

30%

40%

50%

60%

70%

80%

90%

100%

Times0 1 2 3 4

Fig. 4 Different referential gestures/all gestures: percentages and

confidence intervals (95% CI) at the five assessments

23%(8-45)

40%(26-56)

33%(24-44)

37%(26-50)

44%(33-55)

49%(37-62)

37%(27-48)

44%(33-55)

31%(18-47)

36%(17-59)

14%(3-35)

13%(5-27)

13%(7-22)

4%(1-13)

15%(8-24)

27%(11-50)

16%(7-30)

10%(5-19)

9%(3-19)

5%(1-11)

0% 10% 20% 30% 40% 50% 60% 70% 80% 90% 100%

0

1

2

3

4

Nouns Predicates Functors Other

Fig. 5 Lexical categories: percentages and confidence intervals

(95% CI) at the five assessments

70%(58-81)

3%(0-11)

4%(1-11)

0%(0-9)

0%(0-20)

0%

10%

20%

30%

40%

50%

60%

70%

80%

90%

100%

0 1 2 3 4Times

Fig. 6 Morphological modifications: percentages and confidence

intervals (95% CI) at the five assessments

476 Neurol Sci (2010) 31:471–481

123

Means of communication

Pointing significantly decreased (Fig. 9), while the use of

referential gestures/signs (Fig. 10) significantly increased

(p \ 0.001) from T0 to T4. Finger spelling also signifi-

cantly increased over time (Fig. 11), ranging between 0

and 4% of the means of communication up to T3 and

becoming 23% at T4 (95% CI: 18–30). Visual contact and

gaze on the focus were normal since T0 as the girl did not

avoid eye contact. Deictic gestures were recorded in very

small proportions at T0 and further declined, disappearing

by T2. At T4 we recorded an adequate use of facial

mimicry, accompanied by vocalizations with a prosodic

value.

Cognitive and scholastic abilities and neurological

status at T4

The data are shown in Table 6.

In particular, the results of the WISC-R scales were

within the range of mild retardation according to the DSM

IV. At T4, the girl was able to close her mouth for a few

seconds without using her hands, bring her lips together,

smile and, with considerable effort, close her eyes. She

could slightly raise her eyebrows and managed to blow

weakly; phonation was possible for a few seconds. The

motility of her tongue was still severely impaired, but she

could move the tip of her tongue to the left. Drooling was

better controlled. The tactile sensitivity of her face

remained deficient on the left, but improved in the region

around the mouth.

98%91-100100%

94-100

95%88-98

77%67-84

65%57-72

0%

10%

20%

30%

40%

50%

60%

70%

80%

90%

100%

Times0 1 2 3 4

Fig. 8 Spontaneous productions: percentages and confidence intervals

(95% CI) at the five assessments

8%4-12

12%7-18

31%25-38

29%22-38

38%32-45

0%

10%

20%

30%

40%

50%

60%

70%

80%

90%

100%

Times0 1 2 3 4

Fig. 9 Pointing gestures: percentages and confidence intervals

(95% CI) at the five assessments

51%44-58

67%59-75

68%61-74

85%78-90

67%61-74

0%

10%

20%

30%

40%

50%

60%

70%

80%

90%

100%

0 1 2 3 4Times

Fig. 10 Referential gestures: percentages and confidence intervals

(95% CI) at the five assessments

63%(53-73)

74%(62-83)

49%(38-60)

6%(1-16)

22%(12-34)

75%(62-85)

94%(84-99)

51%(40-62)

21%(12-32)

19%(12-28)

14%8-23

0% 10% 20% 30% 40% 50% 60% 70% 80% 90% 100%

0

1

2

3

4

Req. attn Decl. obj. Req. action Decl. subj. Req. info

Fig. 7 Percentages and confidence intervals (95% CI) at the five

assessments of communicative intentions: Req. attn requests for

attention, Decl. obj. declarations on the object, Req. action requests

for actions, Decl. subj. declarations on the subject, Req. info requests

for information

Neurol Sci (2010) 31:471–481 477

123

Discussion

In the present study, we found that 4 years after the

beginning of treatment, the phrasal structures produced by

the girl could be placed at the end of phase III of nuclear

sentence according to the classification by Cipriani et al.

[25], with an MLU of 3.4. As reported in typically devel-

oping children [39], the increase in MLU in this child

seemed to be related to the acquisition of morphological

language competences. Although the category ‘‘functors’’

did not increase significantly over time, a qualitative

improvement of language morphology was observed (e.g.,

ON THE/IN THE were produced by means of sign and

finger spelling at the earlier assessments and exclusively by

means of finger spelling later). At variance with the studies

by Udwin and Yule [16] and by Grove and Dockrell [17],

we found that the length of the utterance in the child

seemed related not only to a higher use of open-class

lexical categories (e.g., nouns, adjectives and verbs), but

also to the appearance of functors enabling more complex

semantic relations to be constructed, as at T4 fewer item-

chaining pre-syntactic productions and complete, expanded

nuclear sentences and complex sentences were recorded.

Morphological sign modifications, many of which were

correct, increased from T0 to T4, particularly concerning

consistencies of number and gender, bearing witness to the

emergence of a morpho-syntactic competence and a con-

sequent switch to a grammatical phase. As reported in

some linguistic studies of the sign, changes in meaning

may be indicated using the visuospatial parameters of sign

(i.e., hand shape, movement, location and direction) that

are the equivalent to phonemes in the spoken language

[26].

The patient was taught morphology with the aid of

signed Italian and Italian sign language, adapting the signs

and the changes in the signs to suit her motor impairment.

This is in contrast to the studies by Udwin and Yule [16]

and Grove and Dockrell [17], according to whom the sign

input the children had received from the teachers seemed to

be too limited in terms of quantity, lexical variety and

morpho-syntactic content. As reported in the experimental

study by Rudd et al. [19], our data confirm that children

with mental impairment exposed to treatment using signs

may learn productive morphological rules. Like typically

developing infants, mentally retarded children with severe

expressive language impairment seem to be able to learn

morpho-syntax better from the more complex, contrastive

context than from simple linguistic inputs [39], suggesting

that rehabilitation projects should provide sufficiently

varied morpho-syntactic models.

As shown in Table 6, in parallel with the development

of the gestural communication, there was evidence of an

improvement in the girl’s writing abilities, as at follow-up

she was able to write a simple and correct narrative text

with sentences showing a syntactic structure similar to that

of her gestural productions. Her learning of written lan-

guage seemed to follow a parallel course with her learning

of the signed linguistic input each benefiting from and

facilitating the other’s development.

With regard to pragmatic capabilities, we chose to

analyze the girl’s progress in terms of communicative

intentions in particular declarations and spontaneous pro-

ductions, since previous studies [20, 40] reported them as

being produced less often with respect to answers and

requests for attention. Our aim was to be able to draw some

conclusions on the communicative efficacy of the sign

system used and on the input provided by the therapist,

family and school.

The growth in the spontaneous productions and the

disappearance of the imitative productions demonstrated

the adoption of an increasingly active role in the commu-

nicational exchange. The analysis of the communicative

intentions also showed a consistent increase in the more

well-developed and active intentions, such as declarations

on the object, accompanied by a parallel consistent

23%18-30

3%1-7

1%0-3

0%0-4

0%0-1

0%

10%

20%

30%

40%

50%

60%

70%

80%

90%

100%

Times0 1 2 3 4

Test for trend: p<0.001

Fig. 11 Finger spelling: percentages and confidence intervals

(95% CI) at the five assessments

Table 6 Cognitive, linguistic and scholastic assessment at T4

Test Results

WISC-R TIQ 54; VIQ 55; PIQ 58

Language comprehension

assessment tests: protocol

6–7 [30]

Average score for children

aged 6–7 years

PPVT [31] QV \ 65 (QV 101 for age range

45–50 months)

Writing tests III, elementary

narration [32]

Simple narrative test; complete simple

and amplified nuclear and

incomplete binuclear phrases were

produced; rare spelling mistakes

478 Neurol Sci (2010) 31:471–481

123

reduction in the requests for attention. The means of

communication became more evolved and decontextual-

ized over time: at the last assessment, the girl mainly used

referential gestures accompanied by finger spelling, while

the primitive deictic gestures had disappeared. Our findings

are in contrast with those by Udwin and Yule [20, 40]

reporting a persistently limited use of augmentative com-

munication systems in the children they analyzed, who

tended to maintain a passive role in the communication.

This may be related, however, to the type of rehabilitation

the children received and to an insufficient exposure in the

different environments where the children lived, i.e., at

school and in the family. From the beginning, the aim of

our rehabilitative program was to provide expertise in the

use of signs to the principal parties for exchanging com-

munication with the girl. We were able to count on the

active involvement of her mother and her support teacher at

school. Unlike the situation emerging from the study by

Udwin and Yule [40], the adults believed in the emanci-

patory abilities that the girl could gain from using gestures/

signs, even though they tended to continue to interpret and

anticipate her intentions and communicative output, as

observed in parents of children with severe disabilities.

Linguistic retest (Table 6) at T4 showed that the

impairment mainly involved lexical rather than morpho-

syntactic abilities. These findings are consistent with our

data in a previous study on children with BPP [24]. Bishop

et al. [41] reported a similar pattern in children with

cerebral palsy and severe expressive language impair-

ments. According to the Liberman’s theory [42], the

authors suggested that the lack of an articulatory feedback

may interfere more with the children’s capacity of word

discrimination and phonological memory, and thus with

their word acquisition, rather than with the comprehension

of semantic nexae.

Although the neurological picture at follow-up was

substantially unchanged, the functional assessment of the

phono-articulatory muscles showed a motor and sensory

improvement in the oral district. In our previous study on

six children with BPP, we attributed the scarce progress in

gestural linguistic capabilities to the fact that bilateral

involvement of the perisylvian regions, having an ana-

tomical substrate in common with the motor representation

of mouth and hand movements, prevented any compensa-

tion for the general linguistic deficiency. Our present data

seem to contradict our earlier assumption, although the six

children previously studied underwent different and non-

homogeneous rehabilitative programs.

Up until the 1980s, the relationship between neural

structure and function was seen as being one-way: the

development of the nervous system and the progressive

acquisition of abilities were assumed to be determined

strictly by the maturation of neural structures. Starting

from the 1980s, however, many experimental studies

demonstrated the role of environmental afferences in

influencing the functional development and structural

organization of the nervous system. In this light, the

relationship between structure and function becomes two

way, in that functional changes may induce structural

modifications. The plasticity of the nervous system in the

post-natal period is mainly due to modifications of the

synaptic connections in response to afferences and this

provides the basis for the learning process throughout

life [43]. There are reports in literature [44, 45] on adult

patients with focal lesions secondary to stroke, whose

neuronal aggregates adjacent to lesions involving senso-

rimotor areas progressively acquired the function previ-

ously handled by the damaged neurons in response to

training. These studies showed that the hemisphere

affected by the stroke underwent a remodeling of the

hand’s sensorimotor somatotopic representation beyond

the normal areas, extending into adjacent areas of the

hand’s representation. fMRI studies in children with

perinatal brain lesions [46, 47] support the ‘‘interactive

specialization’’ theory, which suggests that cognitive

functions are mediated, in typical development, by a

constellation of interconnected regions that acquire their

specialized functional role in relation to function-related

connections: this theory maintains that the typical

developmental mechanisms interact with early-onset

disease, giving rise to an atypical neurofunctional anat-

omy. If the balance of the regional competition is dis-

rupted, as in the case of perinatal stroke or structural

cortex anomalies, an alternative functional organization

is possible. In a recent study performed on a group of

children between 5.6 and 17.7 years of age surgically

treated for left hemisphere refractory epilepsy [48], intra-

hemispheric reorganization was documented after sur-

gery, with the displacement of the functional area

assigned to producing oral movements toward more

anterior frontal regions. Despite a bilateral impairment

(which was prevalent in the frontal regions, but also

extended posteriorly) and despite intensive rehabilitation

treatment being started only when she was more than

7 years old, our patient developed her linguistic gestural

abilities at morpho-syntactic level more than in other

case reports in literature or in the six cases we had

previously studied, and even had some, albeit marginal,

improvement in her sensorimotor abilities in the oro-

facial region.

In conclusion, our findings confirm the strict link

between gesture and language, as established by the recent

studies on the mirror system. It may also be interpreted as

the outcome of a reorganization of the compensatory syn-

aptic circuits stimulated by a sufficiently ample and

intensive rehabilitative input.

Neurol Sci (2010) 31:471–481 479

123

Acknowledgments We thank Dr. Tiziana Casalino for her help with

data transcription and coding and Marina Denegri for editing.

References

1. Volterra V, Bates E, Benigni L, Bretherton I, Camaioni L (1979)

First words in language and action: a qualitative look. In: Bates E,

Benigni L, Bretherton I, Camaioni L, Volterra V (eds) The

emergence of symbols: cognition and communication in infancy.

Academic Press, New York, pp 141–222

2. Petitto LA, Marentette PF (1991) Babbling in the manual mode:

evidence for the ontogeny of language. Science 251:1493–1496

3. Volterra V, Erting C (1994) From gesture to language in hearing

and deaf children. Gallaudet University Press, Washington, DC

4. Capirci O, Iverson J, Pizzuto E, Volterra V (1996) Gestures and

words during the transition to two-word speech. J Child Lang

23:645–673

5. Iverson JM, Goldin-Meadow S (1998) The nature and function of

gesture in children’s communication. Jossey-Bass, San Francisco

6. Masataka N (2001) Why early linguistic milestones are delayed

in children with Williams syndrome: late onset of hand banging

as a possible rate-limiting constraint on the emergence of

canonical babbling. Dev Sci 4:158–164

7. Stefanini S, Bello A, Caselli MC, Iverson JM, Volterra V (2009)

Co-speech gestures in a naming task: developmental data. Lang

Cogn process 24:168–189

8. Bates E, Dick F (2002) Language, gesture, and the developing

brain. Dev Psychobiol 40:293–310

9. Gallese V, Fadiga L, Fogassi L, Rizzolatti G (1996) Action

recognition in the premotor cortex. Brain 119:593–609

10. Rizzolatti G, Arbib MA (1998) Language within our grasp.

Trends Neurosci 21:188–194

11. Rizzolatti G, Fogassi L, Gallese V (2001) Neurophysiological

mechanisms underlying the understanding and imitation of

action. Nat Rev Neurosci 2:661–670

12. Buccino C, Vogt S, Ritzl A, Zilles K, Freund HJ, Rizzolatti G

(2004) Neural circuits underlying imitation learning of hand

actions: an event-related study. Neuron 42:323–334

13. Iacoboni M, Wilson SM (2006) Beyond a single area: motor

control and language within a neural architecture encompassing

Broca’s area. Cortex 42:503–506

14. Iverson JM, Longobardi E, Caselli MC (2003) Relationship

between gestures and words in children with Down’s syndrome

and typically developing children in the early stages of commu-

nicative development. Int J Lang Commun Disord 38:179–197

15. Bello A, Capirci O, Volterra V (2004) Lexical production in

children with Williams syndrome: spontaneous use of gesture in a

naming task. Neuropsychologia 42:201–213

16. Udwin O, Yule W (1990) Augmentative communication systems

taught to cerebral-palsied children—a longitudinal study I. The

acquisition of signs and symbols, and syntactic aspects of their

use over time. Br J Disord Commun 25:295–309

17. Grove N, Dockrell J (2000) Multisign combinations by children

with intellectual impairments: an analysis of language skills.

J Speech Hear Res 43:309–323

18. Veneziano E, Sinclair H, Berthoud I (1990) From one word to

two words: repetition patterns on the way to structured speech.

J Child Lang 17:633–650

19. Rudd H, Grove N, Pring T (2007) Teaching productive sign

modifications to children with intellectual disabilities. Augment

Altern Commun 23:154–163

20. Udwin O, Yule W (1991) Augmentative communication systems

taught to cerebral-palsied children—a longitudinal study II.

Pragmatic features of sign and symbol use. Br J Disord Commun

26:137–148

21. Kuzniecky R, Andermann F, Guerrini R (1994) The epileptic

spectrum in the congenital bilateral perisylvian syndrome. CBPS

multicenter collaborative study. Neurology 44:379–385

22. Jansen A, Anderman E (2005) Genetics of the polymicrogyria

syndromes. J Med Genet 42:369–378

23. Sans A, Fernandez-Alvarez E (1996) Neuropsychological find-

ings in congenital bilateral perisylvian syndrome versus early

acquired opercular syndrome. In: Guerrini R, Andermann F,

Canapicchi R, Roger J, Zifkin BG, Pfanner P (eds) Dysplasias of

cerebral cortex and epilepsy. Lippincott-Raven, Philadelphia,

pp 279–283

24. Saletti V, Bulgheroni S, D’Incerti L, Franceschetti S, Molteni B,

Airaghi G, Pantaleoni C, D’Arrigo S, Riva D (2007) Verbal and

gestural communication in children with bilateral perisylvian

polymicrogyria. J Child Neurol 22:1090–1098

25. Cipriani P, Chilosi AM, Bottari P, Pfanner L (1993) L’acqui-

sizione della morfosintassi in italiano: fasi e processi. Unipress,

Padova

26. Caselli MC, Maragna S, Volterra V (2006) Linguaggio e sordita.

Gesti, segni e parole nello sviluppo e nell’educazione. Il Mulino,

Bologna

27. Massoni P, Maragna S (1997) Manuale di logopedia per bambini

sordi. Franco Angeli, Milano

28. Wechsler D (1986) WISC-R Scala di Intelligenza Wechsler per

bambini Riveduta. Organizzazioni Speciali, Firenze

29. Caselli MC, Casadio P (1995) Il primo vocabolario del bambino.

Guida all’uso del questionario MacArthur per la valutazione della

comunicazione e del linguaggio nei primi anni di vita. Franco

Angeli, Milano (Italian version of the MacArthur-Bates comu-

nicative development inventories)

30. Rustioni Metz Lancaster D (1994) Prove di valutazione della

comprensione linguistica. Organizzazioni Speciali, Firenze

31. Dunn LM, Dunn LM (1981) Peabody picture vocabulary test-

revised. American Guidance Service, Circle Pines, MN. [Italian

adaptation and standardization: Stella G, Pizzoli C, Tressoldi PE

(2000) Peabody: Test di vocabolario recettivo. Omega edizioni,

Torino]

32. Tressoldi PE, Cornoldi C (1991) Batteria per la valutazione della

scrittura e della competenza ortografica nella scuola dell’obbligo

Organizzazioni Speciali, Firenze

33. Bortolini U (1995) PFLI Prove per la valutazione fonologica del

linguaggio infantile. Edit Master, Venezia

34. Molteni B, Airaghi G, Mantegazza G, Sarti D (2006) Develop-

mental language delay in early childhood: differential diagnosis

between specific language delay and language delay secondary to

other communication disorders. In: Riva D, Rapin I, Zardini G

(eds) Language: normal and pathological development. Editions

John Libbey Eurotext, Montrouge, pp 217–227

35. Caselli MC (1994) Communicative gestures and first words. In:

Volterra V, Erting CJ (eds) From gesture to language in hearing

and deaf children, 2nd edn. Gallaudet University Press, Wash-

ington, DC, pp 56–67

36. Goldin-Meadow S (2003) Hearing gesture: how our hands help us

think. Harvard University Press, Cambridge

37. Goldin-Meadow S, Butcher C (2003) Pointing toward two-word

speech in young children. In: Kita S (ed) Pointing; where lan-

guage, culture and cognition meet. Lawrence Erlbaum Associ-

ates, Mahwah, pp 85–107

38. Tommasello M, Carpenter M, Liszkowski U (2007) A new look

at infant pointing. Child Dev 78:705–722

39. Pizzuto E, Caselli MC (1992) The acquisition of Italian mor-

phology: implication for models of language development.J Child Lang 19:491–557

480 Neurol Sci (2010) 31:471–481

123

40. Udwin O, Yule W (1991) Augmentative communication systems

taught to cerebral-palsied children–a longitudinal study. III.

Teaching practices and exposure to sign and symbol use in

schools and homes. Br J Disord Commun 26:149–162

41. Bishop DVM, Brown BB, Robson J (1990) The relationship

between phoneme discrimination, speech production, and lan-

guage comprehension in cerebral-palsied individuals. J Speech

Hear Res 33:210–219

42. Liberman AM, Mattingly IG (1985) The motor theory of speech

perception revised. Cognition 21:1–36

43. Bates E (1999) Language and infant brain. J Commun Dis

32:195–205

44. Rossini PM, Dal Forno G (2004) Integrated technology for

evaluation of brain function and neural plasticity. Phys Med

Rehabil Clin N Am 15:263–306

45. Rossini PM, Altamura C, Ferreri F, Melgari JM, Tecchio F,

Tombini M, Pasqualetti P, Vernieri F (2007) Neuroimaging

experimental studies on brain plasticity in recovery from stroke.

Eura Medicophys 43:241–254

46. Liegeois F, Connelly J, Cross JH, Boyd SG, Gadian DG, Vargha-

Khadem F, Baldeweg T (2004) Language reorganization in

children with early-onset lesions of the left hemisphere: an fMRI

study. Brain 127:1229–1236

47. Fair DA, Brown TT, Petersen SE, Schlaggar BL (2006) fMRI

reveals novel functional neuroanatomy in a child with perinatal

stroke. Neurology 67:2246–2249

48. Kadis DS, Iida K, Kerr EN, Logan WJ, McAndrews MP, Ochi A,

Otsubo H, Rutka JT, Snead OC 3rd, Weiss SK, Smith ML (2007)

Intrahemispheric reorganization of language in children with

medically intractable epilepsy of the left hemisphere. J Int Neu-

ropsychol Soc 13:505–516

Neurol Sci (2010) 31:471–481 481

123