Adult-onset Rasmussen’s encephalitis.

Rasmussen’s encephalitis (RE) is characterised by refractory focal seizures, unilateral cortical deficits, and progressive unihemispheric focal cortical atrophy of undetermined aetiology. A majority of these cases present in childhood, and three disease stages have been recognised: a ‘prodromal stage’, an ‘acute stage’ and a ‘residual stage’. Adult-onset and variant forms have also been described but are rare, and we report one such case with frequent refractory focal seizures, right-sided hemiparesis, aphasia, and marked atrophy of the left perisylvian cortex with a slow disease progression over 17 years. Immunotherapy with corticosteroids, IVIG, plasma exchange, and tacrolimus has been tried in RE with variable results. Hemispherectomy and disconnective techniques like functional hemispherectomy and hemispherotomy are effective in achieving seizure freedom in 62.5% to 85% cases, but carry a risk of motor and language deficits.

Randomized prospective study of outcome of short term antiepileptic treatment in small single enhancing CT lesion in brain.

The study was conducted in 81 patients of epilepsy with small single enhancing CT (SSECT) lesion in brain to determine the clinical profile and duration of antiepileptic drugs (AEDs) treatment. The patients were randomly divided into group A (41 cases) and group B (40 cases). Group A patients were treated for 6 months and group B for 1 year with AEDS without cysticidal drugs. The most common mode of presentation was simple partial motor seizures with secondary generalization in both the groups. Repeat imaging of brain (CT/MRI) at 6 months showed disappearance of lesion in 82.94% in group A and 87% in group B, while persistence of lesion was present only in 4.87% in group A and 5% in group B. 87.81% patients in group A and 87.17% in group B were seizure free. The recurrence of seizure occurred in 12.19% cases in group A, and 12.82% in group B. 80% of these patients had calcified lesion in both the groups. This study reveals that SSECT lesion with epilpesy is a benign self-limiting disease. It also reveals that 6 months AED treatment is as effective as one year treatment. Patients having calcified lesion or persistence of lesion might require long term AED treatment.

Creutzfeldt-Jakob disease : report of 10 cases from North India.

Creutzfeldt-Jakob disease (CJD) is increasingly being reported over the last three decades as a result of heightened awareness of the disease. Various studies have reported annual incidence of 0.5-1.5 cases of CJD per million of general population. In India, the disease is still under reported. Over the period spanning from 1968-1997, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore recorded 69 cases of CJD from different parts of India in the CJD registry. This paper describes the clinical experience with cases of CJD managed at the Department of Neurology, G.B. Pant Hospital, New Delhi from 1990-1998. In this series, the mean age of the patients was 53.80 (+/- 7.32) years and there were 5 females and 5 males. Myoclonus was present in all the cases and abnormal behaviour with or without other features was the presenting complaint in 7 of the 10 patients, while one patient of CJD had cerebellar ataxia as the presenting feature. One patient with occipital variant of CJD presented with acute onset cortical blindness and myoclonic jerks. One of the patients had acute psychosis precipitated by emotional stress at the onset. Extrapyramidal features were noted in 7 of the 10 patients before death. The mean duration of symptoms from the onset of disease to death was 6.6 (+/- 6.11) months. Classical EEG changes were observed in all the patients, except in one possible case of occipital variant of CJD, where we did not have access to EEG record. Brain biopsy could be undertaken in 3 patients, and in 2 patients the features of subacute spongiform encephalopathy (SSE) were noted.

Congenital myasthenic syndrome: report of four cases and brief review of literature.

The term 'congenital myasthenic syndrome' (CMS) encompasses a number of heterogeneous disorders characterised by myasthenic symptoms since birth, usually with positive family history and absence of acetyl choline receptor antibodies. Recent advances in electrophysiology and ultrastructural analysis of neuromuscular junction have made it possible to identify the various defects underlying these disorders. We report four cases of CMS, with a review of literature.

Subacute sclerosing panencephalitis.

A retrospective study of 36 confirmed cases of subacute sclerosing panencephalitis (SSPE) was carried out to detect any variation in the clinical and EEG patterns previously described. There were 31 males and 5 females, aged 4 to 24 years. Onset of SSPE at or after 15 years of age was observed in 22.2% of cases. Rapid progression of the disease was observed in 52%. The first symptom was myoclonus in 61.6%, mental regression in 22.2% and generalised seizures in 11.1% of cases. Low positive measles antibody titres in the CSF were found in 36.1% of cases. EEG analysis revealed slow background in 69.2% and periodic complexes in 94.4% of cases. Atypical periodic complexes, focal abnormalities and paroxysms of bisynchronus sharp activity were also observed.