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Biomedical subjects

B S Singhal

Publications and source records attributed to B S Singhal.

At least 19 recordsLinked to original sources

Megalencephalic leukodystrophy in an Asian Indian ethnic group.

Over a 10-year period, we reviewed 30 patients with leukodystrophy, megalencephaly, and a relatively benign course. Most of these patients (26) belonged to a distinctive ethnic group called the Agrawals. Head circumference exceeded the 95th percentile in 28 patients; 22 patients had seizures; 22 had pyramidal signs (16 more pronounced and 6 mild); and 16 had cerebellar ataxia. The median age of onset of symptoms was 1.8 years. Interictal electroencephalogram (EEG) was abnormal in 21 of 23 patients. The inheritance is possibly autosomal recessive.

Adolescent

Comparison between multiple sclerosis in India and the United States: a case-control study.

The prevalence of MS in India is low, and it is unclear whether the manifestations of the disease in India are similar to the United States. We carried out a case-control study to compare the disease in the two populations and used clinical, evoked potential, and MRI criteria to assess similarities and differences. Our results indicate that the rate of disease progression and frequency of involvement of the cerebral hemispheres, cerebellum, spinal cord, and brainstem were similar in the two populations. The visual system was more frequently involved in Indian patients. No Indian patient had a family history of MS; this suggests an environmental disease-triggering agent.

Adult

Cervical myelopathy caused by an intracranial dural arteriovenous fistula.

We report a case of progressive myelopathy in a 50-year-old lady with an intracranial dural arteriovenous fistula. Transcatheter embolization followed by surgical resection of the intracranial fistula resulted in reversal of the myelopathy. The diagnosis and management of this potentially reversible condition is discussed and the literature reviewed.

Cerebral Angiography

Vasculitic neuropathy: profile of twenty patients.

Twenty patients with vasculitic neuropathy were analyzed. Sixteen of the twenty presented with classic mononeuritis multiplex but four had distal, symmetrical, sensorimotor polyneuropathy. Though vasculitic neuropathy is classically associated with Collagen vascular syndromes like, polyarteritis nodosa, rheumatoid arthritis and systemic lupus erythematosis, only 13/20 of our patients had definitive Collagen vascular disease. A large proportion (7/20) had vasculitic neuropathy as the only clinical feature.

Adult

Limited sequence divergence of HTLV-I of Indian HAM/TSP patients from a prototype Japanese isolate.

Nucleotide sequences of two HTLV-I proviruses isolated from Indian patients with HAM/TSP were analyzed. The sequence data of the env, pX, and LTR regions showed 98-99% homologies with the prototype HTLV-I, ATK-1, isolated from a Japanese ATL patient, indicating that HTLV-I isolates in India and Japan are similar, with minor variations. However, certain small sequences of noncoding regions in the pX and LTR showed differences of 6.1 and 7.2%, respectively, thus the conclusion could vary depending on the regions and length of the sequences used for comparison.

Adult

Elevated neopterin levels in Guillain-Barré syndrome. Further evidence of immune activation.

Neopterin is a by-product of guanosine triphosphate metabolism and is produced by macrophages in response to lymphocytic activation. We have studied serum neopterin levels in patients with Guillain-Barré syndrome to obtain further evidence of immune activation in this disease. Serum neopterin levels were significantly elevated in patients with Guillain-Barré syndrome compared with patients with other peripheral neuropathies and multiple sclerosis and with healthy control subjects. Serial analysis demonstrated that as neopterin levels fell, the clinical status of the patients with Guillain-Barré syndrome improved and soluble interleukin 2 receptor levels dropped. Thus, lymphocytic and macrophage activation may play a role in the pathogenesis of Guillain-Barré syndrome.

Adolescent

Histopathology and fine structure of the brain in six cases of Creutzfeldt-Jakob disease from western India.

Light and electronmicroscopic changes in 5 formalin-fixed brains, and one glutaraldehyde-fixed brain biopsy, from patients with Creutzfeldt-Jakob disease in the age range of 45 to 65 years, are described. These 6 patients (out of 7 reported clinically earlier and 2 unreported) had classical manifestations with progressive dementia, pyramidal signs and myoclonic jerks. Light microscopy showed neuronal and nerve fibre loss, moderate or severe spongiform change, astrocytic proliferation and absence of inflammatory reaction. Electronmicroscopy confirmed the characteristic membranous profiles of the 'cysts' and 'daughter cysts' constituting the spongiform change. The membranes were generally dark and thin, either concentrically arranged or splitting and with stray pale broad segments. The one glutaraldehyde-fixed brain biopsy specimen showed cisterns of RER in close proximity to these 'cysts', suggesting the source of proteinous material of these membranes. Though mainly in the neurones and dendrites of the cortex, at times they were seen in the myelinated fibres also, a few of which showed dystrophic axons bearing dense bodies. One of the 6 patients had cerebellar signs also, and a total duration of the neurological illness of 36 months, as against 2-8 months in the 5 other patients. The histopathological examination of her brain revealed less spongiform change, and many cerebral cortical glial whorls, the centre of which showed PAS-positive and congo-red-positive material representing amyloid. Fine structural examination confirmed the glial whorls, and the filamentous nature of amyloid in the plaques, which resembled Kuru plaques. All brains also showed more or less intraneuronal lipofuscin.

Amyloid

Practice patterns in neurology in India.

Sixteen Indian neurologists saw 1,850 patients over a sample 6-day working week. The average daily workload comprised 19 patients including 11 new cases. Epilepsy (27.0%), headache (19.0%) and cerebrovascular disorders (7.8%) were the commonest problems. Only 2.5% fell in the ICD-9-CM categories 780-799 consisting of symptoms, signs and ill-defined conditions. In 43% it was felt that the neurologic consult was not necessary. In the Indian setting this would suggest that emphasis should be on neurologic education of primary care physicians and internists for the appropriate utilization of limited resources.

Humans

Prevalence of multiple sclerosis in the Parsis of Bombay.

We carried out a door-to-door-survey to screen a community of 14,010 people (Parsis living in colonies in Bombay, India) for possible neurologic diseases, and used defined diagnostic criteria to evaluate people who tested positive on the screening survey. There were three clinically definite cases of multiple sclerosis (21/100,000). This is the first prevalence survey for multiple sclerosis in a large developing country.

Humans

Multiple sclerosis--Indian experience.

Multiple sclerosis (MS) does occur in India but it is rare. No epidemiological studies have been done. The calculations based on the hospital data suggest an approximate prevalence rate of 0.17 to 1.33 per 100,000 of population in different parts of India. One hundred and five patients with clinically definite MS (2 proven at autopsy) and 14 patients with neuromyelitis optica were seen in the Bombay region from 1957 to 1983. The mean age of onset for MS patients was 27.7 years. There were 60 female and 45 male patients. The clinical data in this larger series confirm the conclusions reached earlier, namely that the Indian MS patients are more akin to those reported from Japan and other Asian countries as compared to the West. The relative incidence of neuromyelitis optica was high (6%). The optico-spinal form of MS was the commonest (71.4%). In patients with clinically definite MS there was a high incidence of optic nerve involvement, both at the onset and during the course of the disease; sensory level occurred in a large number of patients and cerebellar involvement was less frequent. Multiple sclerosis in this series affected patients of all communities and different dietary habits. MS was more common in the higher socioeconomic group whereas neuromyelitis optica was mainly seen in patients in the low socioeconomic group.

Cerebrospinal Fluid Proteins