Comorbidity of Epilepsy and early onset autism or Landau-Kleffner-Syndrome only?

Aims: Epilepsies as well as autism often are accompanied by behavioural problems, loss of speech and cognitive functions. Typically Landau-Kleffner-Syndroms is described by loss of understanding and production of speech. Patients often show as well concentration deficits, hyperactivity and aggressiveness. The differentiation from early onset autism often is difficult.

Methods: 13 year old boy, after primarily normal motor and speech development, stepwise loss of speech at the age of 3 years. At age 5 years diagnosed with early onset autism followed by a specific therapy for autism. First seizure at age 10;9 years. 4 generalised tonic-clonic seizures in 24h. The EEG (awake and sleep), showed bilateral sharp waves parieto-central. Seizure free with SULTHIAME. After 1½ year discontinuation of SULTHIAME, the boy was still seizure free but he became much more aggressive. He was presented to us to clarifiy whether his aggressiveness was caused by epilepsy.

Results: Without anticonvulsant medication sleep EEG showed repetitive Rolandic sharp waves left and right central. The MRI was normal. Physical and neurological findings were normal except deficits concerning proprioceptive sense and polydipsia of unknown reason. As well he showed strong motoric unrest, auto and extrinsic aggressiveness, self stimulation with loud tones, no other verbal communication and severe sleep disturbances.

With CLOBAZAME and again SULTHIAME no improvement of speech, but increase of motoric unrest and aggressiveness. After change of the medication to OXCARBAZEPIN reduction of aggressiveness and mild improvement of speech.

Conclusion: In the literature a comorbidity of early onset autism and epilepsy is described with 11–39%. The reasons still are indistinct. Until now epilepsy or epileptiform discharges in the EEG could not be defined to be the reason for autism doubtlessly. For both disorders a genetic predisposition is possible. Whether that fact caused a high rate of comorbidity has to be discussed. To differentiate the striking features of behaviour, speech and cognitive function always an interdisciplinary approach is necessary.

Epilepsy - early onset autism - Landau-Kleffner-Syndrome