The
  Infantile
Spasms
Resource




Unclassified epilepsies  

 

 

 

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:

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.

  • generalised fits seen association with fever
  • occur between the age of 3 months and five years
  • CNS infection must be excluded
  • occurs in 2 to 5% of the population
  • most frequently occur from the age of 12 to 18 months
  • fits occur early in the course of the fever
  • about a third recur
  • only 10% have three or more fits
  • Risk factors for subsequent epilepsy

  • prolonged fit lasting longer than 15 minutes
  • recurrence of fit within 24 hours
  • focal neurologic features following a fit
  • Epilepsy with continuous spike and waves in slow wave sleep (ESES)

  • onset in childhood
  • predominantly nocturnal fits, focal or generalised
  • EEG shows generalized epileptiform activity for at least 85% of recording made during slow wave sleep
  • EEG during wakefulness may show a combination of focal and generalised spikes
  • intellectual deterioration frequently occurs
  • treatment regimes are variable and similar to those used in Landau Kleffner syndrome
  • fits disappear by age 20
  • (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.

  • onset occurs at the time of language acquisition
  • major symptom is failure of speech development
  • fits occur in 75% but are infrequent and get less with time
  • generalised fits
  • multifocal EEG spikes
  • etiology unknown at present
  • no imaging abnormalities
  • fits are generally controllable and abate with time but the outcome for speech is less good
  • treatment with steroids and anti-epileptic drugs is presently undertaken
  • 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.

    Epilepsy and inborn errors in metabolism

  • aminoaciduria
  •   adreno leucodystrophy  
  •   non ketotic hyperglycinemia  
  •   neuronal ceroid lipofuscinosis  
  •   pyridoxine deficiency  
  •   phenylketonuria  
  •   peroxisomal disorders  
  • return to   Types of epilepsy

     

     

    (checked: 15 November 2002)
    (update 2.1: 25 July 2002)
    (issue 2: 24 March 1998)