Educationals

Benign Epilepsy with Occipital Paroxysms (BEOP)

Age of onset: 15 months -17 years (mean age 7.5 years)

Neurologically - Normal children

Seizures which include visual symptoms amaurosis, hemianopia, visual hallucinations and illusions, followed by motor or psychomotor manifestation and at times terminates with a migrainous headache.

EEG : Normal background, spike and waves occurring in distinct paroxysms over the occipital and posterior temporal region in bursts or in trains with prompt disappearance on eye opening. Reappearance on eye closure after a latent period.

Prognosis is usually good but not as good as BECT. Seizures cease in adult life.

Landau Kleffner : The acquired aphasia with epilepsy syndrome
Age of onset: 3 years -9 years
Abrupt or gradual onset.
Defect in language function and verbal auditory with an inability
to comprehend language and reduction in spontaneous oral expression. Behavioural and intellectual difficulties are present. Seizures are partial or generalised seen in two thirds of cases and not very severe. They remit by 15 years.

EEG-Multifocal spikes and spike and wave discharges.

Long term recovery of language function is not very good especially in those with early age of onset before age of 5 years.

Early diagnosis is essential as treatment with ACTH or steroids can reverse or arrest the disorder. Valproate or carbamazepine are used for seizures.

Adolescence

Juvenile absence epilepsy.
Juvenile myoclonic epilepsy (Janz syndrome)
Grand mal seizures on awakening

Juvenile Absence

Age of occurrence is around puberty with equal male - female rating.

Seizures: As opposed to childhood absence the seizures are infrequent, longer duration and less impairment of consciousness. Patients may have generalised tonic clonic seizures on awakening or myoclonic seizures.

EEG shows generalised spike and wave discharges with frontal predominance which are more than 3 c/sec. Precipitated by hyper ventilation and sleep deprivation.

Response to treatment is seen not as readily as childhood absence.

Juvenile Myoclonic Epilepsy of Janz. (Impulsive petit mal)

Incidence 5-10% of epilepsy

Age of occurrence : 8-26 years (more between 12-18 years), more in females 2:1 ratio

Seizures : three types of seizures are seen, typical absence seen first, followed by myoclonic jerks and later by generalised tonic clonic seizures. Myoclonic jerks are usually bilateral, arrhythmic, irregular, rapid, more pronounced in the upper limbs. No disturbance of consciousness. The severity of myoclonic seizures is variable and may cause the patient to fall. The seizures are precipitated by sleep deprivation and alcohol. 50% of JME are photosensitive. Myoclonic and generalised tonic clonic seizures occur more often on awakening and patients experience difficulty brushing teeth or carrying a cup in the morning.

Patients have normal intellect and development and no neurological deficits.

EEG: 4-6 Hz polyspike and slow wave generalised discharges with normal background activity and are also found inter ictally. No close correlation between EEG spikes and jerks.

Inheritance : Gene responsible for the syndrome has been localised to chromosome 6.

Treatment : With appropriate medication (valproate) patients are seizure free. There is high relapse rate if medication is withdrawn even if seizure free for several years.

Epilepsy with Generalised Tonic Clonic Seizures on awakening

Incidence : 16-52% of all epilepsies
Age : 9-25 years (peak ar puberty)
Seizures : Generalised tonic clonic

• Seizures within two hours of waking from sleep,
  (whatever time of day.)
• Second seizure peak : evening period of relaxation.

Absences in 50%
Myoclonic seizures in 30%

Neurological examination is unremarkable.
EEG shows 2.5 -4 Hz spike wave activity enhanced by hyperventilation in some, photosensitive in about 13%.

Prognosis : Controlled with medication in 65% but high relapse rate if medication is tapered.