This, of course, is the grouping for epilepsies which do not fit the classification schema.

(link not working)   Febrile fits

A febrile fit is a fit, associated with fever, but without a defined cause. A small percentage of children who have had a febrile fit may have epilepsy. Significant risk factors are:

• A family history of epilepsy
• Abnormal neurological or developmental status prior to the febrile fit.
• An atypical febrile fit, such as a prolonged or focal fit.

Children who suffer a febrile fit generally enjoy normal health after the episode. They are, however, at some risk in several respects. 30 to 40% of children who have a febrile fit and who do not receive prophylactic therapy will experience a second fit.

Risk factors for subsequent epilepsy

Epilepsy with continuous spike and waves in slow wave sleep (ESES)

(link not working)   Landau Kleffner syndrome

Landau Kleffner Syndrome is a rare form of childhood epilepsy which results in a severe language disorder. All children with LKS have abnormal electrical activity in one, sometimes both temporal lobes, the area of the brain responsible among other functions for processing language.

The language disorder in most children affects comprehension or understanding. Many LKS children become unable to understand their own name. They are also likely to have difficulty recognising environmental sounds such as the telephone ringing. These children may appear to be deaf. Expressive language - the ability to speak is often seriously affected; some children lose their speech completely. Rarely, a child may be able to understand language, but have difficulty with speaking.

Behavioural problems are common, especially hyper-activity, poor attention, depression and irritability. Some children have episodes of very abnormal "autistic type behaviour" with symptoms such as avoidance of contact with family and friends (avoidance of eye contact is common) extreme pickiness over food, very disturbed sleep, attacks of rage and aggression, insensitivity to pain, bizarre and inappropriate and repetitive play.

There may be temporary other neurological problems such as loss of bladder and bowel control, episodes of visual disturbances may occur, the child can see but does not understand what he sees. He may have difficulty recognising family and friends or common objects, such as food and clothes.

Rasmussen's syndrome

Rasmussen's syndrome is a progressive central nervous system disorder, characterised by seizures, hemiparesis (paralysis on one side), inflammation of the brain, and mental deterioration. This disorder occurs almost exclusively in children under the age of 10. Since this disease was first given its name in 1958, the number of cases recorded in medical literature is small. The cause is unknown, although it is thought to result from an unidentified viral infection.

• very rare
• age of onset: (18 months to 14 years); <10 years in 80%
• focal motor fits or generalised tonic clonic fits
• intellectual deterioration, progressive hemiparesis
• variable severity, eventually plateaus, frequently leaves a neurologically devastated individual
• EEG shows bilateral or multifocal abnormalities with a unilateral predominance
• MRI shows progressive unilateral hemispheric atrophy beginning in the perisylvian region
• Ictal SPECT shows hyper-perfusion in the affected hemisphere
• Neuropathology: perivascular lymphocytic cuffing, glial nodules, microglial proliferation
• Antibody to GluR3 is one pathogenetic mechanism

Epilepsy and inborn errors in metabolism

return to   Types of epilepsy