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Landau-Kleffner syndrome
How is it treated?
Treatment with routine antiepilepsy drugs is usually quite effective in seizure control but much less so for the encephalopathy. Corticosteroid drugs are more effective and help more than half of those treated, but have significant side effects.
Occasionally there is a dramatic spontaneous or drug induced total recovery but more commonly problems continue and some long term impairments persist. Occasionally surgical treatment by multiple subpial transactions (severing horizontal connections controlling electrical changes in the brain; this surgical intervention was developed after research showed that the normal structural organisation of the brain relied on vertical connections) is used. Such treatment requires the accurate identification of the source of seizure activity using a range of neurophysiological techniques which may include a methohexitol suppression test and magnetoencephalography. The aim of the methohexitol suppression test is to suppress all the electrical activity in the brain and then to see in which area of the brain the abnormal electrical activity returns first. This area may be the one that is primarily responsible for the problem. Magnetoencephalography is a noninvasive technique that detects and records the magnetic field associated with electrical activity in the brain.
In those children with multiple communication and behavioural impairments, major educational, medical and care support may be required. The active phase of the condition usually burns out in the early part of the second decade, but may leave the child with long term disability.
In addition to the above clinical presentation there are 'variants' with a younger age of onset, and some with abnormal MRI scans which may require different management. There is as yet no evidence to regard the more common presentations of autism in the first two years of life as an early type of LKS
What are the symptoms?
| Inheritance patterns and prenatal diagnosis 
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