[Benign myoclonic epilepsy in infancy].
Benign myoclonic epilepsy in infancy (NMEI) is one of rare epileptic syndromes. 5 patients (all female sex) aged 4-16 years were observed. NMEI debuted at the age from 7 months till 2.5 years (mean age 1.3 years). Pathology of pregnancy and labor, disorders in both psychomotor development and genetic predisposition were not found. In all the cases the disease began with typical transitory repeated myoclonic paroxysms of different intensity and frequency, without loss of consciousness and with primary involvement of the muscles of the neck and the upper extremities. Most patients had muscular hypotension, mild coordinatory disorders, delayed psycho-speech development, mental retardation, EEG signs of generalized epileptic activity. Valproates, suxilep, clonazepam and lamotrigin (lamiktal) were used for treatment. The most pronounced effect was achieved using either monotherapy with valproates (depakin) or a combination depakin + lamiktal. A stable clinical-encephalographic remission was achieved in all the patients, but during puberty in 2 patients (15 and 16 years old) rare generalized convulsive fits debuted. High frequency of intellectual-mnestic disorders were found even after a complete remission. So benign definition concerns only a course of the fits, but not NMEI prognosis.
Mukhin
K Iu
KIu
Petrukhin
A S
AS
Pylaeva
O A
OA
Iukhalina
N S
NS
Glukhova
L Iu
LIu
Abramova
M F
MF
D017668:Age of Onset; D000927:Anticonvulsants / Q000627:therapeutic use; D001921:Brain / Q000000981:diagnostic imaging; D002675:Child, Preschool; D004359:Drug Therapy, Combination; D004569:Electroencephalography; D004831:Epilepsies, Myoclonic / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D011859:Radiography
D000927:Anticonvulsants