Generalized myasthenia gravis following use of D-pencillamine in Wilson's disease.
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Biomedical subjects
Publications and source records attributed to M Behari.
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We performed interictal brain 99m Tc-HMPAO study in eight cases of chronic epilepsy. SPECT (single photon emission computed tomography) study showed abnormality in seven cases. As compared to computed tomography (CT) scan and electroencephalogram (EEG) which were positive in 25% and 62% cases respectively it showed abnormality in 87%. In two cases where CT scan and EEG both were normal, SPECT showed areas of hypoperfusion. In one case where EEG indicated a bilateral focus, SPECT study showed a clearly defined unilateral focal hypoperfusion defect. Areas of hyperperfusion were not seen in any of our cases. Our results indicate that HMPAO SPECT is more sensitive than CT scan and EEG, in localising an epileptogenic focus in cases of chronic epilepsy.
OBJECTIVES: To compare the rate of prior environmental exposures between Indian multiple sclerosis (MS) patients and controls in order to identify potential disease triggering factors. MATERIAL AND METHODS: A standard self-administered questionnaire regarding prior exposures was presented to 56 Indian MS patients and 147 other neural disease and healthy controls at two large medical centers in India. RESULTS: The rate of prior foreign travel, surgeries, blood transfusions, clinical chicken pox and mumps infections and exposure to cats and farm animals was not significantly different between MS patients and controls. However, clinical measles infection and dog exposure occurred significantly more often in the MS patients. CONCLUSION: These findings are consistent with but do not prove an association between prior measles infection, dog exposure and MS.
Botulinum toxin (BTX) is the treatment of choice for blepharospasm. To investigate if the locally injected BTX influences the central nervous system and in particular the brain stem, amplitude and latency of compound muscle action potential (CMAP) of orbicularis oculi, blink reflex and brain stem auditory evoked potential were studied in 12 blepharospasm patients before BTX therapy, 9 patients after BTX therapy and 9 age and sex matched healthy controls. The mean amplitude of orbicularis oculi in patients was significantly lower before BTX therapy (p < 0.05) and after BTX therapy (p < 0.02). The amplitudes of R1, R2 and contralateral R2 (CR) was higher in patients than controls but did not reach statistical significance. Following BTX therapy there was a reduction in amplitude of R1, R2 and CR (p < 0.001) and prolongation of latency of R2 and CR (p < 0.05). The ratio of blink reflex amplitude/CMAP amplitude of orbicularis oculi showed a significant decline after BTX therapy (p < 0.01). This small study suggests the involvement of brain stem pathways following BTX therapy in patients with blepharospasm.
SETTING: Since conventional bacteriological methods rarely detect Mycobacterium tuberculosis in cerebrospinal fluid (CSF) and are of limited use in the diagnosis of tuberculous meningitis (TBM), clinical features suggestive of TBM supported by indirect evidence such as CSF examination and computerized tomography (CT) of the head have been used for the early diagnosis of TBM. OBJECTIVE: We evaluated the efficacy of polymerase chain reaction (PCR) in the diagnosis of TBM. METHODS: Coded CSF samples from 40 patients with TBM and from 49 patients with other neurological disorders were processed. In the absence of a reliable sensitive and specific test for M. tuberculosis in CSF, we used a set of established clinical criteria as the gold standard. Accordingly, the patients were divided into definite, highly probable, probable and possible TBM. The samples were decoded only after completion of the laboratory tests. RESULTS: PCR was positive in 2/4, 19/20, 13/16 patients with highly probable, probable and possible TBM respectively. None of the samples were positive by conventional bacteriological methods. However, 3/49 CSF samples from non-TBM patients were also found positive by PCR. PCR detected M. tuberculosis genomic DNA in the CSF of 85% of clinically suspected TBM cases and 6.1% of non-tuberculous controls. CONCLUSION: PCR, along with the suggested clinical criteria, offers a rapid and fairly accurate diagnosis of TBM.
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.
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Fifty five patients with Meige's syndrome were examined for clinical and demographic features. The mean age of onset was 52.3 years. The peak age of onset was in the sixth decade with a male to female ratio of 1.11:1. The mean duration of illness was 3.7 years. Commonest initial symptom was increased blinking, seen in 30 cases (54.5%). Twenty five patients (45.4%) had complete syndrome of blepharospasm with oromandibular dystonia, whereas 24 patients (43.6%) had blepharospasm alone and the rest (6 patients, 10.9%) had oromandibular dystonia. The extension of spasm beyond cranial muscles was observed in 10 patients (18.1%). Eleven patients had family history of dystonia or other extrapyramidal disorders. Incidence of depression was high in these cases.
Fifty patients of multiple sclerosis were reviewed. The commonest initial neurologic symptom was motor weakness (30%) followed by visual impairment (26%). Absence of internuclear opthalmoplegia was noted in addition to the infrequent occurrence of optic nerve involvement and Devic's disease. Evoked potential studies and radiological investigations were in agreement with others series reported.
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SETTING: The patients admitted to the Neurology ward of the All India Institute of Medical Sciences Hospital. OBJECTIVE: To assess the role of dexamethasone as an adjunct to antimicrobial therapy in the treatment of tuberculous meningitis. DESIGN: A randomised controlled trial of 47 patients was conducted over a 13-month period. 41 patients completed the trial. Patients were stratified into mild, moderate and severe groups and randomly allocated to steroid and non-steroid groups. All patients received a standardized antituberculosis drug regime. The end point was 3 months, or death if earlier. The evaluation at the end point included survival, resolution of symptoms, sequelae and activities of daily living. Results were analysed using the Wilcoxon rank sum test. RESULTS: The patients in the dexamethasone group fared better. 75% of this group had mild sequelae as opposed to 62% of the control group. Amongst the survivors, those who received dexamethasone sensorium improved earlier, and there was greater improvement in mental function and daily activities. The difference, however, did not reach statistical significance. CONCLUSIONS: Dexamethasone appears useful as an adjunct in the treatment of tuberculous meningitis especially in patients who have severe disease. The results need confirmation by a larger trial.
SETTING: Tuberculous meningitis (TBM) has high morbidity and mortality. Demonstration of tubercle bacilli in cerebrospinal fluid (CSF), the only reliable method of diagnosis, is time consuming and has a low yield. Early diagnosis and treatment are of utmost importance for favourable outcome. OBJECTIVE: The study was conceived to define easy to use criteria. METHODS: A set of criteria using clinical features, CSF examination and computed tomography (CT) findings were defined. 76 patients suspected of having TBM were divided into definite, highly probable, probable and possible TBM based on the criteria. The validity of criteria was tested using information from bacterial isolation, polymerase chain reaction (PCR) test for tuberculosis, response to treatment and autopsy. RESULTS: PCR was positive in over 75% of patients in the highly probable and probable groups. 91% of patients with highly probable and 66% with probable TBM improved on antituberculosis therapy. CONCLUSION: The criteria are reliable in making early diagnosis of TBM.
Single, small, enhancing lesions that often resolve spontaneously are frequent findings on CTs of Indian patients with seizures. Based on ELISA and biopsy data, the majority of these lesions are probably cysticercosis. To determine if these patients should be treated with albendazole, we performed a double-blind, randomized, placebo-controlled study involving 75 patients with seizures and the appropriate CT abnormality without neurologic abnormality on examination. Patients were randomized to albendazole (15 mg/kg/d) and placebo for 1 week, and we obtained serial CTs at the end of 1 week, 1 month, and 3 months. All patients completed a 3-month follow-up and none had systemic evidence of tuberculosis or cysticercosis. The lesions varied in size from 3 mm to 2.1 cm, with an average size of 1.18 cm. Serum ELISA for cysticercosis was positive in 30 and CSF ELISA was positive in 20 of 45 patients. Forty patients received albendazole and 35 received placebo. At the end of 3 months, a total of 68 patients showed resolution. Thirty-five of 40 patients who received albendazole showed resolution, as opposed to 33 of 35 patients on placebo. We conclude that albendazole therapy was not beneficial.
We report the first Indian experience of botulinum toxin A in the treatment of blepharospasm and hemifacial spasm. Sixteen patients, 7 with essential blepharospasm, 5 with Meige syndrome and 4 with hemifacial spasm received botulinum toxin A injection. One patient received 3 courses of injections, 2 received 2 courses and the rest received only one course. The effect was observed after a latent period of less than 48 hours in all patients and lasted for a mean of 16.65 weeks. More than 70% improvement occurred after 17/20 injections (85%). Poor response was more often seen when blepharospasm was associated with oromandibular dystonia (2/5 injections). Though the duration of response and subjective score of improvement was best in patients with hemifacial spasm, the numbers were very small for any statistical evaluation. The side effects were local, transient, mild and well tolerated. The commonest side effect was blepharoptosis.
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