Epilepsy of Janz in India: Is it different?

(JME) of Janz has attracted plenty of attention. It is important to identify JME early, since it has a favorable prognosis. On the contrary, if the diagnosis is delayed, there may be uncontrolled epilepsy, repeated head injuries, polytherapy and psychosocial trauma. We share our experience about JME in India and its variation from reported observations. Methods: We selected 108 cases of JME after screening 708 patients of generalized seizures, in an age group of 8-32 years. Diagnosis was established using standard clinical and EEG criteria. Patients who responded satisfactorily (>50 % reduction in seizure frequency) with any antiepileptic agent besides sodium valproate (VPA) were advised to continue them as such. If seizures persisted, then substitution by VPA in a dose of 20-40 mg/kg bodyweight was done. Clonazepam was added in a dose of 0.01-0.10 mg/kg bodyweight, only if patients were not seizure free even with maximum dose of VPA (50 mg/ kg). Cognitive assessment was done using Mini-Mental Status Examination and it was repeated twice at intervals of 4 months. All subjects were followed up till they were seizures free for a minimum period of 1.5 years. Total duration of study was 6.5 years. Results: Out of 108 cases of JME, only 5 (5%) patients were referred with this diagnosis, and most of them by neurologists. Majority of them, 62 patients (57%) were referred as intractable seizures. Ther