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PubMed1990

[Paroxysmal kinesigenic choreoathetosis: autonomic disease or reflex epilepsy?].

Motor attacks induced by voluntary movements are infrequent. Paroxysmal kinesigenic choreoathetosis (PKC) is rare and has only recently been individualized (Kertesz, 1967). We report the case of an 8 year-old boy who developed unilateral or bilateral attacks of abnormal, choreoathetotic movements during certain voluntary movements, especially when rising after a rest. The attacks were short (13 to 18 seconds) and frequent. Neurological examination was normal, as were the intelligence quotient, the inter-critical and critical EEG: CT and MRI. The patient's mother had suffered from the same disorder. The attacks disappeared during treatment with phenytoin but reappeared when it was stopped. This case is concordant with data from the literature, with male predominance, age from 6 to 15 years at the onset, shortness of the attacks (less than 1 minute in 80% of the cases) and normality of investigations in almost every patient. A familial factor has been found in 50% of the cases. The frequency of epilepsy in the family is above average. PKC can easily be distinguished from Mount and Reback' syndrome where the attacks are choreoathetotic but longer and unprovoked by movements and where there is also a familial factor. The relationship of PKC with epilepsy is asserted by some authors and denied by others, and in the literature the distinction between movement-induced tonic seizures and PKC is not always clear. Some authors have blamed a disturbance in the maturation of basal ganglia. To sum up, PKC is a very rare condition which is easy to diagnose, has a good prognosis and readily responds to antiepileptic drugs.

Athetosis↗
PubMed1984

Evaluation of anticonvulsant drugs in gerbils with reflex epilepsy.

In more than 98% of randomly bred gerbils epileptic seizures could be evoked by blowing at the animals with compressed air (5 bars for 10 s). In contrast, only few animals showed seizures in response to environmental stimuli (e.g., new cage) and handling. Thus, administration of drugs was possible without precipitating seizures. The severity of the seizures was rated on a seven-point scale. Seizure severity increased with age and reached its maximum at about 7 months. Comparison of pharmacokinetics of common antiepileptic drugs showed that these drugs, except phenobarbital, were rapidly eliminated by the gerbil which, in this respect, resembled the mouse. For determination of anticonvulsant ED50S of antiepileptic drugs in gerbils there was differentiated between "minor" seizures (grade 1-2; myoclonic seizures) and "major" seizures (grade 3-5; generalized clonic-tonic seizures). Phenobarbital, phenytoin and carbamazepine were more potent against major seizures than against minor seizures, whereas the reverse situation was obtained with diazepam, valproic acid and ethosuximide. The relative activity of the different antiepileptic drugs against minor and major seizures in gerbils corresponded to the respective activity in traditional mouse models of petit mal and grand mal epilepsy. In further experiments, a series of GABAmimetic drugs was tested, namely, compounds that selectively increase gamma-aminobutyric acid (GABA)-mediated neuronal inhibition in the brain.(ABSTRACT TRUNCATED AT 250 WORDS)

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