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-yearold 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 nonrapid 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 partialonset seizures in adults and adolescents older than 12 years

Focal cortical resection May improve seizure control in rare cases

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