Epilepsia partialis continua--a clinical and electroencephalography study.

Epilepsia partialis continua (EPC) is a rare type of localization-related motor epilepsy. Clinical spectrum, electroencephalography (EEG) characteristics and various prognostic factors in EPC were studied in 20 patients. Patients who fulfilled the criteria for EPC between the years 1985 and 1999 were included in this retrospective and prospective study. The mean age was 18 years (range 5 months-70 years). Eleven patients (55%) had Type 1 EPC and in the remaining nine (45%) patients there were features of Type 2 EPC. Among children Rasmussen's encephalitis and viral encephalitis were the commonest cause for EPC. Encephalitis and vascular aetiology were frequently observed in adults. Tuberculous meningitis and tuberculomas occurred evenly in both the groups. The cause was unknown in two cases. Focal EEG abnormalities commonly consisted of discrete spikes, sharp waves (or) slow wave activity and periodic lateralized epileptiform discharges. The mean duration of follow up was 9.6 months with a range between 1 month and 4 years. Cognitive decline, motor deficits and pharmacoresistance to drugs were significantly seen among children with Type 2 EPC. Patients with Type 1 EPC had mild impairment of functional status with good response to treatment. The long-term prognosis depends upon the underlying cause.

Pathology of temporal lobe epilepsy: An analysis of 100 consecutive surgical specimens from patients with medically refractory epilepsy.

The neuropathological features of temporal lobe epilepsy were studied utilising 100 consecutive surgical specimens from patients with medically refractory complex partial seizures. A wide spectrum of neuropathological changes was recorded in 98 specimens. Fifty-eight specimens showed features of Ammon's horn sclerosis. Diffuse accumulation of corpora amylacea were demonstrated in the resected temporal lobes from 54 patients. Six patients had neoplastic lesions of temporal lobe. One unique case of dysembryoplastic neuroepithelial tumour showed a melanotic component within the tumour. The neuropathological features were regarded as nonspecific in 31% of cases. Our results indicate that a majority of patients with medically intractable epilepsy of temporal lobe origin reveal significant neuropathological features. Careful documentation of the neuropathological features and its correlation with radiological, electrophysiological and pre- and post-surgical clinical features will help in predicting the seizure outcome after temporal lobectomy for medically refractory epilepsy.

Prevalence of benign epileptiform variants observed in an EEG laboratory from South India.

No study has investigated the prevalence and pattern of benign epileptiform variants (BEVs) in the electroencephalograph (EEG) of subjects from a developing nation. We addressed this issue by ascertaining the BEVs among subjects referred for EEG to a comprehensive epilepsy program in South India. We identified BEVs in 326 out of 1778 subjects (18.3%) who had both awake and sleep EEG recordings. The frequency distribution of individual BEVs were as follows: benign sporadic sleep spikes 8.2%, wicket waves 1%, 14 and 6 Hz positive spikes 5.7%, 6 Hz spike-waves 2.8% and rhythmic temporal theta burst of drowsiness 0.8%. We conclude that the prevalence rates of BEVs among South Indian subjects are not different from those reported from developed countries. We have highlighted the consequences of misinterpretation of BEVs as epileptiform, which is more likely to occur in Third World countries because of the non-availability of qualified technologists and electroencephalographers.

Substantial spontaneous long-term remission in subacute sclerosing panencephalitis (SSPE).

We report a 25-year-old female who developed clinical and electroencephalographic (EEG) features of subacute sclerosing panencephalitis (SSPE) at the age of 17 years. After almost 17 months of progressive neurological deterioration to a level where she was bedridden and incapable of self-care (Risk and Haddad stage 3a), she experienced a substantial spontaneous clinical and EEG remission (stage 4c) which she has maintained for the last 8 years. The measles antibody titer in the cerebrospinal fluid (CSF), however, progressively increased during follow-up. There are only very few patients with well documented diagnosis of SSPE who have maintained such a prolonged remission. The age at onset of SSPE of > or = 12 years, disappearance of periodic complexes and a tendency for normalization of the background activity in the EEG, and a progressive increase in the measles antibody titer in the CSF appears to predict a favourable outcome of SSPE.

Mitochondrial neurogastrointestinal encephalopathy (MNGIE) in a South Indian family with two affected siblings.

We report a South Indian family in which two living members were affected by a progressive neurogasrointestinal disorder characterized by dysmotility. Evidence for mitochondrial dysfunction was provided by increased arterial blood lactate, and ragged red fibres in muscle biopsy. Our report is believed to be the first description of the autosomal recessively inherited multisystem mitochondrial disease, mitochondrial neurogastrointestinal encephalopathy (MNGIE), from India.

Periodic electroencephalographic pattern in subacute sclerosing panencephalitis modified by preexisting damaged cerebral hemisphere.

We report a 15 year old girl with a childhood hemiplegia, who developed a recent progressive intellectual decline associated with elevated globulins and measles antibody titres in the cerebrospinal fluid, indicating a diagnosis of subacute sclerosing panencephalitis (SSPE). The magnetic resonance imaging revealed left hemispheric atrophy concordant with a long-standing right hemiplegia, and electroencephalography exhibited lateralized periodic complexes (PCs) over the right hemisphere concordant with left-sided myoclonic jerks. The modification of PCs in our patient due to preexisting damaged cerebral hemisphere illustrate that a fairly functional cortex and subcortical white matter are needed for the expression of the PCs of SSPE.

Neurologic complications of infective endocarditis observed in a south Indian referral hospital.

We retrospectively reviewed the records of 110 patients with infective endocarditis (IE) who were hospitalized between 1977 and 1994 at a tertiary referral center in Southern India to assess the occurrence of neurologic complications and the factors that contribute to their development, and to compare our experience from a developing country with the reported data from developed countries. There were 62 males and 48 females, aged 0.6-59 (mean 24.0) years. Rheumatic heart disease (RHD) was the most frequent underlying cardiac lesion accounting for 65 (59.1%) patients. Neurologic complications were observed in 58 (52.7%) patients: cerebral embolism was the most frequent (23 patients). Thirty-five (31.8%) patients died. Mortality in the group with neurologic complications (41.4%) was significantly higher than in the group without (21.2%) (p = 0.04). The duration of symptoms prior to the diagnosis was longer in the group with neurologic complications, mean 174.9 versus 95.6 days (p = 0.03). We conclude that (1) IE occurs at younger ages in the Third World and RHD still constitute the major underlying heart disease; (2) in spite of the differences in the general aspects of IE between developed and developing nations, the frequency and gravity of neurologic complications are similar, (3) mortality is significantly increased in patients with neurologic complications; and (4) delay in the diagnosis of IE contributes to the development of neurologic complications.