Term
NEONATAL SEIZURES
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Definition
NEONATAL SEIZURES
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Seizure type: Variable
Age: FIRST 4 WEEKS ONLY
Cause: Any.
EEG: Difficult to interpret at this age.
Prognosis: Related to underlying pathology, but overall outcome is not good. 25% die within one year; 50 % have continued seizures or evidence of neurological damage; only 25% make a complete recovery.
Treatment: Depends on cause |
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Term
WEST SYNDROME
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Definition
WEST SYNDROME
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Synonyms: Infantile spasms; Slalaam spasms; Propulsive petit mal
Seizure type: 3 types - flexor: flexion of neck, arms and legs and possibly trunk (salaam seizure); extensor: sudden movement of neck, arms and legs; and mixed: sudden flexion of neck,trunk and arms and extension of legs (most common). Seizures may occur upto 100 times/hour.
Age: ~6months (range 3-9 months)
Cause: 2/3 have identifiable brain lesion such as tuberous sclerosis, cortical dysplasias or ischaemic insults. No aetiology can be found in remaining 1/3.
EEG: Hypsarrhythmia - choatic pattern of high amplitude, irregular, slow activityintermixed with multifocal spike and sharp wave discharges.
Prognosis: Poor. 15% make complete recovery; 20% die in childhood; 65% of survivors have ongoing epilepsy and 50% have persistent neurological handicap
Treatment: Response to treatment with AEDs is generally poor. Some children benefit form ADRENOCORTICOTROPHIC HORMONE if given early. VIGABATRIN is the drug of choice. |
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Term
LENNOX-GASTAUT SYNDROME
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Definition
LENNOX-GASTAUT SYNDROME
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Synonyms: Myoclonic astatic petit-mal epilepsy
Seizure type: Characterised by multiple seizure types including tonic and atonic seizures and complex absences. Clinically vary from brief head nod to violent collapse. HIGH RISK OF DEVELOPING STATUS EPILEPTICUS, whcih is linked to later dementia.
Age: 2-4 yrs (range 1-9 years). Boys more common than girls.
Cause: Progression of West Syndrome in around 40% of cases. Most cases are due to severe brain damage of some type but sometimes aetiology cannot be determined.
EEG: Almost invariably abnormal. Slow, 2.0-2.5 Hz spike and wave and polyspike and wave discharges over anterior and posterior head regions.
Prognosis: Very poor. Cognitive and behavioural problems are very common and it is unusaul for patients to lead independent lives. Seizures control is usually poor and polypharmacy or sedation may worsen them.
Treatment: VALPROATE is the drug of choice, though lamotrigine, topiramate and levetiracetam have shown promise. A KETOGENIC DIET is benefical but usually poorly adhered to. |
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Term
BENIGN PARTIAL EPILEPSY OF CHILDHOOD
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Definition
BENIGN PARTIAL EPILEPSY OF CHILDHOOD
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Synonyms: Rolandic epilepsy; centrotemporal epilepsy
Seizure type: Simple partial seizures, with occaisional progression to complex seizure or secondary generalised. Seizure tend to occur at night or on waking.
Age: Onset usally between 2 and 14 yrs
Cause: 30% have family history of epilepsy.
EEG: Inter-ictal EEG shows frequent paroxysms of slow spike and wave discharges over the centrotemporal region with normal background rhythm.
Prognosis: Excellent. Seizures usually disappear during puberty and EEG becomes normal.
Treatment: Not usually required but, if seizures are frequent, CARBAMAZEPINE is the drug of choice. |
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Term
RASMUSSEN'S SYNDROME
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Definition
RASMUSSEN'S SYNDROME
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Seizure type: Partial seizures, usually motor. Progressive neurological deficits usually appear with a few years of seizure onset.
Age: Between 14 months and 14 years
Cause: Localized autoimmune encephalitic process. Antibodies to glutamate receptors can be demonstrated.
EEG: Multiple contralateral epileptigenic foci.
Prognosis: Progessive debilitating neurological deficit is the norm.
Treatment: AEDs usually ineffective. PLASMAPHERSIS may be effective. Steroids not yet fully evaluated. HEMISPHERECTOMY may be useful is severly effected children. |
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Term
CHILDHOOD ABSENCE SEIZURES
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Definition
CHILDHOOD ABSENCE SEIZURES
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Synonyms: Petit mal epilepsy
Seizure type: Sudden 'stop-and-stare' seizures lasting no more than 30 seconds. Falls and major motor phenomena do not occur, but petit mal automatisms - such as plucking at clothes or mouth movements - may occur. If no other seizure types are present, this is termed PYKNOLEPSY. If seen together with grand mal, this is termed MIXED EPILEPSY.
Age: 2-13 yrs
Cause: Unknown. Possible genetically determined metabolic abnormality as 1/3 have family history of epilepsy.
EEG: Typical ictal 3-4 Hz spike and wave pattern. May be induced by hyperventilation in 90% of patients.
Prognosis: Reasonable. 25% cases resolve with puberty. Some continue to have absences exclusively. More than half develop grand mal seizures, which usually occur on waking.
Treatment: ETHOSUXIMIDE or VALPROATE usually produce good control. |
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Term
JUVENILE MYOCLONIC EPILEPSY
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Definition
JUVENILE MYOCLONIC EPILEPSY
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Synonyms: Janz syndrome; Impulsive petit mal epilepsy;
Seizure type: Brief, forceful, irregularly occuring twitches that may be single or multiple, unilateral or symmetrical and usually invole the neck, shoulders and upper limbs. Conciousness is preserved. More common after waking. PHOTOSENSITIVITY is common. More than half will also develop grand mal seizures, typically on waking.
Age: 8 - 26 years
Cause: Mutations in several genes have been found, including those coding for components of calcium and chloride channels and GABA receptors.
EEG: Ictal EEG reveals multiple peaks interrupted by high-amplitude slow waves. Pattern is photically-inducible.
Prognosis: 80% require life-long AEDs.
Treatment: Valproate is the drug of choice. Lamotrigine and levetiracetam are also effective. |
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