Juvenile Myoclonic Epilepsy In India : Some Interesting Observations

Abstract

Material and Method: Study was conducted in 132 cases of JME. They were selected after screening 1210 patients presenting with syndrome of generalized seizures in age group 10-36 years. Diagnosis was established clinically by standard criterias and confirmed by EEG. Duration of study was 9 years. Results : We observed JME is under diagnosed since majority (27%) were referred as uncontrolled seizures. Other patients were referred as Lenox Gestaut syndrome (19%), progressive myoclonic epilepsy (7.5%) or subacute sclerosing pan-encephalitis (3.7%). There were 25 (15.5%) fresh cases of JME who reported directly. We observed few atypical features in our study. They were in the form of (a) wide range in age of onset (b) gross delay in diagnosis (90%)-lack of clinical suspicion and non-use of activation procedures in EEG appear to be important reasons for this delay (c) negative family history (90%) (d) mild cognitive impairment (14%) and (e) good clinical response to other drugs viz; clobazam, phenytoin (PHT) and carbamazepine (CBZ) besides sodium valproate (VPA) or clonazepam (CLO). However phenobarbitone (PB) was ineffective. Sequential EEG became normal in 63% patients controlled on VPA while it was persistently abnormal in all patients who were well controlled on other drugs. Conclusion : Clinical spectrum of JME appears to be different in India. We suggest that it should be strongly suspected in juvenile patients of generalized epilepsy not responding to treatment.