Subject(s)
Athetosis/complications , Athetosis/genetics , Chorea/complications , Chorea/genetics , Anticonvulsants/therapeutic use , Athetosis/drug therapy , Carbamazepine/therapeutic use , Child , Chorea/drug therapy , Diazepam/therapeutic use , Drug Therapy, Combination , Humans , Male , Pedigree , Severity of Illness Index , Valproic Acid/therapeutic useABSTRACT
The paper presents an analysis of clinical-neuropsychological peculiarities of the syndrome of atypical benign partial childhood epilepsy (pseudolennox syndrome) of 6 patients (3 boys, 3 girls). An age of the onset of the disease was between 1.5-4 years. There was polymorphism of paroxysms, their high frequency with an obligate presence of hemifacial fits and atypical absences. Night generalized tonic-clonic attacks and the falling attacks were found in 67% of the patients. Spectrum of the neurological disorders included disorders of speech and a slight cerebella symptomatology. Regional "rolandic" activity and diffuse epileptiformed disorders, increasing into a phase of a slow sleep, were registered by EEG. Resistance to anticonvulsive therapy was revealed. The authors had demonstrated a nosologic independence of pseudolennox syndrome and had considered worth while to pick it out in a group of cryptogenic partial forms of epilepsy together with the epileptic aphasia of Landu-Kleffner and an epilepsy with the continuous peak-waves during the slow sleep.