LENNOX GASTAUT SYNDROME

Mandatory seizures

Tonic seizures from sleep (in slow wave sleep or on wakening) are the most characteristic seizure. These are brief (<10 seconds), and may be inconspicuous and therefore not identified. They may be predominantly truncal (with brief vocalization due to involvement of abdominal musculature) or extend to involve proximal and distal muscles (with arms elevating and flexing and legs either flexing or extending). There may be autonomic features in association. They can occur in a series. Tonic status epilepticus may occur.

May have

• Generalized tonic-clonic seizures
• Atypical absence seizures, these are seen in two thirds of patients, atypical absence status epilepticus may occur
• Atonic seizures, these are seen in half of patients and are typically brief, but often cause sudden falls with injury (especially to nose and teeth)
• Myoclonic seizures, these are not a prominent seizure type, they are typically bilateral and symmetrical and involve proximal arm muscles
• Myoclonic-atonic seizures
• Non convulsive status epilepticus is common (seen in half of patients), and can be of long duration (days to weeks), during which time all seizure types may increase in frequency
• Focal seizures
• Epileptic spasms

CAUTION If myoclonic seizures predominate consider Dravet syndrome (especially if prolonged hemiclonic seizures).

CAUTION If myoclonic-atonic seizures predominate consider epilepsy with myoclonic-atonic seizures.