Epilepsy : suatu kelainan otak yang yang ditandai oleh adanya faktor predisposisi yang dapat mencetuskan kejang epileptik, perubahan neurobiologis, kognitif, psikologis dan adanya konsekuensi sosial yang diakibatkannya.

Epilepsy terbagi menjadi 3 :

Epilepsy idiopatik

Epilepsy simtomatik

Epilepsy kriptogenik : sindrom west ( infantile spasm biasanya terjadi pada tahun pertama kehidupan biasanya terjadi pada usia 3 sampai 8 bulan dengan klinis secara tiba – tiba badan membungkuk kemudian kaku diikuti lengan dan kaki atau kadang kadang episode berbeda lengan dan kaki melempar kearah luar ( ekstensor kejang), EEG menunjukkan pola yang sangat tidak teratur disebut 'hypsarrhythmia'.

Lennox-Gastaut syndrome (LGS), or childhood epileptic encephalopathy, is a pediatric epilepsy syndrome characterized by multiple seizure types; mental retardation or regression; and abnormal findings on electroencephalography (EEG). See the image below.

Slow spike wave pattern in a 24-year-old awake male with Lennox-Gastaut syndrome. The slow posterior background rhythm has frequent periods of 2- to 2.5-Hz discharges, maximal in the bifrontocentral areas, occurring in trains as long as 8 seconds without any clinical accompaniment.

Signs and symptoms

If not present before symptom onset, neurologic and neuropsychologic deficits inevitably appear during the evolution of LGS. Factors associated with more common or more severe mental retardation include the following:

An identifiable etiology (ie, symptomatic as opposed to cryptogenic LGS) A history of West syndrome ( infantile spasm) Onset of symptoms before age 12-24 months More frequent seizures

Average intelligence quotient (IQ) score is significantly lower in patients with symptomatic LGS than in those with cryptogenic LGS. Earlier age of seizure onset is correlated with higher risk of cognitive impairment.

Ictal clinical manifestations include the following:

Tonic seizures (frequency, 17-95%) - Can occur during wakefulness or sleep but are more frequent during non–rapid eye movement (REM) sleep; may be axial, axiorhizomelic, or global; may be asymmetric

Atypical absence seizures (frequency, 17-100%) – Can have gradual onset, with incomplete loss of consciousness; associated eyelid myoclonias may be noted

Atonic, massive myoclonic, and myoclonic-atonic seizures (frequency, 10-56%) – Can all cause a sudden fall, producing injuries, or may be limited to the head falling on the chest; pure atonic seizures are exceptional

Other types of seizures (generalized tonic-clonic [15%], complex partial [5%], absence status epilepticus, tonic status epilepticus, nonconvulsive status epilepticus)

Findings on general physical examination are normal in many cases. No physical findings are pathognomonic for LGS. Nevertheless, the general physical examination can help identify specific etiologies that have both systemic and neurologic manifestations.

No neurologic examination findings are pathognomonic for LGS. However, neurologic examination of an LGS patient may demonstrate the following:

Abnormalities in mental status function (specifically, deficits in higher cognitive function consistent with intellectual disability)

Abnormalities in level of consciousness, cranial nerve function, motor/sensory/reflex examination, cerebellar testing, or gait (nonspecific findings that are more a reflection of the underlying brain injury or the effect of anticonvulsant medications)

See Presentation for more detail.

Diagnosis

No laboratory investigations are known to aid in the diagnosis of LGS.

EEG (waking and sleep) is an essential part of the workup. Interictal EEG may demonstrate the following characteristics:

A slow background that can be constant or transient Awake – Diffuse slow spike wave Non-REM sleep – Discharges that are more generalized and more frequent, consisting of

polyspikes and slow waves REM sleep – Decreased spike waves

Ictal EEG may demonstrate the following characteristics:

Tonic seizure – Diffuse, rapid, low-amplitude activity pattern that progressively decreases in frequency and increases in amplitude; may be preceded by a brief generalized discharge of slow spike waves or flattening of the recording or followed by diffuse slow waves and slow spike waves; no postictal flattening

Atypical absence seizure – Diffuse, slow, and irregular spike waves; occasionally, discharges of rapid rhythms preceded by flattening of the record for 1-2 seconds, followed by progressive development of irregular fast rhythm in anterior and central regions and ending with brief spike waves

Atonic, massive myoclonic, or myoclonic-atonic seizure – Slow spike waves, polyspike waves, or rapid diffuse rhythms

Absence status epilepticus – Continuous spike wave discharges, usually at a lower frequency than at baseline, and rapid rhythms during tonic status epilepticus

Neuroimaging is an important part of the search for an underlying etiology. Modalities include the following:

Magnetic resonance imaging (MRI) – Generally preferred Computed tomography (CT) – Preferred in selected situations Routine positron emission tomography (PET) and single-photon emission CT (SPECT) –

Lacking current indications for LGS but may be useful in potential candidates for epilepsy surgery

See Workup for more detail.

Management

Medical treatment options may be divided into the following 3 major groups:

First-line treatments based on clinical experience or conventional wisdom – Valproic acid, benzodiazepines (specifically, clonazepam, nitrazepam, and clobazam)

Treatments suspected to be effective on the basis of open-label uncontrolled studies – Vigabatrin, zonisamide

Treatments proven effective by double-blind placebo-controlled studies – Lamotrigine, topiramate, felbamate, rufinamide

Surgical options include the following:

Corpus callosotomy – Effective in reducing drop attacks but not typically helpful for other seizure types; considered palliative rather than curative

Vagus nerve stimulation – FDA-approved as adjunctive treatment for refractory partial-onset seizures in adults and adolescents older than 12 years

Focal cortical resection – May improve seizure control in rare cases

The ketogenic diet—involving a high ratio of fats (ketogenic foods) to proteins and carbohydrates (antiketogenic foods)—may be useful in patients with LGS refractory to medical treatment.