Académique Documents
Professionnel Documents
Culture Documents
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.
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:
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:
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:
Non-REM sleep Discharges that are more generalized and more frequent, consisting of
polyspikes and slow waves
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
Neuroimaging is an important part of the search for an underlying etiology. Modalities include
the following:
Management
Medical treatment options may be divided into the following 3 major groups:
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
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.