Educationals

Miscellanious syndromes

Chronic Progressive Epilepsia Partialis Continua (EPC) Rasmussen's Syndrome

Age of onset : Mean age 6.8 years, 85% before age of 10 years. Neurological and psychological deterioration in previously normal children.
Cytomegalovirus genomic material may be associated.

Clinical course in three stages :

First stage : Simple partial seizures, complex partial, EPC can occur, but no motor deficit seen, seizures are unresponsive to antiepileptic drugs and gradually increase.

Second stage : Neuropsychological deterioration, progressive hemiparesis, hemianopia and hemisensory loss and if dominant hemisphere involved then results in dysphasia. Epilepsia partialis continua are spontaneous regular or irregular clonic twitching of cerebral cortical origin, sometimes aggravated by action or sensory stimuli confined to one part of body, and continues for hours, days or weeks. It is seen in about 56% cases.

Third stage: Arrest of neuropsychic deterioration and seizures decrease in frequency. The arrest can occur 2 months to 10 years after onset.

EEG : shows progressive brain atrophy and areas of increase T2 weighted signal intensity in the affected hemisphere.

Brain biopsy shows perivascular cuffing, glial nodules, microcystic degeneration with neuronal loss.

Treatment :
Anti epileptic drugs not effective
High dose steroids
IV gamma
Interferon
? Gancyclovir
Functional hemispherectomy

Photosensitive Epilepsy

Photosensitive disorder

Age of onset : 12-14 years, more common in females.

40% are pure photosensitive, 60% have spontaneous along with precipitation by photic stimulation.

Seizures are tonic clonic in 84%, absence in 6%, partial in 2.5%

Flickering lights, TV, video games tend to precipitate seizures.

Treatment is to view TV under bright light from a distance of polaroid lens, dark glasses and sodium valproate.

EEG : Intermittent photic stimulation at 15-18 Hz flash frequency elicit photoconvulsive response.

Isolated Seizure

• Recurrence after first seizure: 51-55%
• After second seizure; 90%
• Recurrence more often in:
  
  
  • Neurologically abnormal
  • EEG showing focal activity
  • Complex partial seizure
  • With history of neonatal seizures
  
  
• Withhold treatment pending second seizure in :
  
  
  • Neurologically and mentally normal children
  • EEG normal / showing generalised neuronal hyperexcitability.
  • History and EEG suggestive of Rolandic epilepsy

Continuous spike and wave during slow sleep (CSWS), or electrical status epilepticus during slow wave sleep (ESES).

Essential component is that spike and waves recorded on the EEG occupy 85% of time of slow wave sleep. Long-lasting syndrome and ESES is present for months to years but eventually disappears spontaneously or following therapy but leaves behind cognitive deficits.

Seizure semiology varies from infrequent nocturnal seizures, motor seizures, absences and frequent falls of any type.

Intellectual deterioration is a constant feature after onset of CSWS.

Electrical status occurs only during sleep, day after day.

CSWS may appear in diverse clinical setting either in previously normal or delayed children.

20-30% have identifiable brain pathology.

Half have normal mental development before status.