This study was aimed at documenting different aspects of the expression of the disease based on clinical data from a large number of Indian probands with Juvenile myoclonic epilepsy. Information with regards to the type and frequency of seizures, precipitating factors, EEG data, response to anti-epileptic drugs and family history was collected from 329 JME probands seen in a large tertiary care super-speciality hospital using a uniform protocol. Detailed family pedigrees were constructed to include all the first- and second-degree relatives of probands and information on the seizures/epileptic syndromes in relatives was classified in a uniform way. The clinical expression and EEG features in 329 Indian probands were similar to the data reported on JME probands belonging to different population groups. Most patients (81%) were treated with only valproate. Family history of seizures among first or second-degree relatives was noted in 114 of 329 (35%) probands. the first-degree relatives had a greater chance of being affected and more often expressed a type of idiopathic generalized epilepsy (IGE) as compared to second-degree relatives of probands. It is concluded that the Indian probands express JME in the same manner as that reported in probands of different ethnic backgrounds. The risk of relatives being affected as well as their risk of expressing a type of IGE (including JME) varies as a function of the degree of relation with the probands. The lower incidence of febrile convulsions among relatives of JME probands could be related to recall bias.

Twenty-four cases of sporadic olivo-ponto-cerebellar atrophy (OPCA) of adult onset were studied over a period of two years. Results suggest that this disorder has its usual onset in the 5th and 6th decade of life with a male: female ratio of 2:1. It manifests clinically with gait ataxia in all, dysarthria, other cerebellar signs and autonomic involvement in vast majority. There were features of basal ganglia involvement in some. No known identifiable environmental cause was found and genetically they are quite distinct from the known autosomal dominant spinocerebellar ataxias though sporadic occurrence in recessive inheritance or a de novo mutation could not be ruled out completely, but it is unlikely.

Anthrax is a rare cause of hemorrhagic meningitis in man. This report illustrates the characteristic hemorrhagic manifestations in the brain of a patient dying of anthrax meningitis secondary to overwhelming bacteremia. Gross examination of the brain revealed a thick dense subarachnoid hemorrhage with numerous petechial hemorrhages in the cortex. Histologically, meningoencephalitis with vascular necrosis, edema, perivascular cortical hemorrhages and clumps of Gram positive bacilli in the vascular lumen and invading vessel wall were the salient features. The anthrax bacillus was isolated from CSF and brain tissue and further its pathogenecity was confirmed by animal inoculation.

Recurrence of seizures in a patient in whom a solitary cysticercus granuloma has left a calcific residue is usually attributed to the presence of the calcific residue. We describe a patient in whom the recurrence of seizures was due to a new lesion close to the calcific residue from a solitary cysticercus granuloma. A repeat imaging is therefore necessary even in patients with calcific residues and recurrent seizures. Identification of a new solitary cysticercus granuloma as a cause of the recurrent seizures is important as seizures due to a fresh lesion can be managed with early withdrawal of antiepileptic drug therapy soon after the resolution of the new granuloma.