[Electrophysiogical and neuropharmacological studies in a patient with progressive myoclonus-epilepsy (author's transl)].
A progressive form of myoclonus-epilepsy was described in an 21 year old girl. Electrophysiological and neuropharmacological studies were carried out. The typical EEG-pattern, the close association of cortical discharges and myoclonic jerks, the form of myoclonus indicated a "pyramidal" myoclonus. Pharmacological data suggested that two possible mechanisms--a partial deafferentation of cortical neurones and a failure of thalamic, extrapyramidal and brain stem reticular formations--might be responsible for the EEG-abnormalities and concomitant myoclonus. Results from neuropharmacological studies demonstrated a suppressive effect (on EEG-discharges and myoclonic jerks) of clonazepam, diazepam, phenobarbital, taurine, levodopa and budipin. Taurine, levodopa and budipin might induce a new aspect in therapy of progressive myoclonus epilepsy and of myoclonus caused by other etiologic factors.
D000328:Adult; D000927:Anticonvulsants / Q000494:pharmacology / Q000627:therapeutic use; D001921:Brain / Q000187:drug effects / Q000503:physiopathology*; D002998:Clonazepam / Q000627:therapeutic use; D003975:Diazepam / Q000627:therapeutic use; D004569:Electroencephalography; D004831:Epilepsies, Myoclonic / Q000188:drug therapy / Q000503:physiopathology*; D005260:Female; D006801:Humans; D007980:Levodopa / Q000627:therapeutic use; D010634:Phenobarbital / Q000627:therapeutic use; D013654:Taurine / Q000627:therapeutic use
D000927:Anticonvulsants; D013654:Taurine; D007980:Levodopa; D002998:Clonazepam; D003975:Diazepam; D010634:Phenobarbital
Elektrophysiologische und neuropharmakologische Studien bei einer Patientin mit progressiver Myoklonusepilepsie.