The symptoms and signs of Leigh's disease are so variable that the diagnosis is extremely difficult. The disease was so far being confirmed only at autopsy. Antemortem clinical diagnosis, however, is important, as some of these patients may respond to high dose of vitamin B-1. We report two patients of Leigh's disease suspected clinically and confirmed on MR imaging. Both the patients responded partially to parenteral thiamine and subsequently remained stable on convenient oral dosage during one year of observation. Follow-up MRI done on patient showed resolution of some of the lesions, no change in some and appearance of some new lesions. There was no definite correlation between the thiamine related clinical recovery and the structural changes observed on MRI. The extreme rarity of involvement of medulla and cervical spinal cord as observed in one of the patients and of external capsule as seen in the other patient, is highlighted.

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Three cases of extrdural spinal agiolipomas of dorsal region presenting as compressive myelopathy are reported. All of them recovered fully following surgery. The role of modern imaging techniques in detecting such lesions along with review of literature is presented. Histopathology in case 2 showed areas suggestive of haemingioma and predominant areas of angiolipoma supporting the hypothesis of common origin of al these from the same pluripotential stem cells.

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A case of well differentiated chondrosarcoma arising from the laminae and spinous processes of cervicodorsal spine is reported in a 22-years-old patient. He had presented with a painless progressive mass in the cervicodorsal region, without any neurological deficit. The tumour had a large exophytic growth without any intraspinal component. Radiological appearance of cartilaginous calcification suggested the diagnosis of a cartilaginous tumour. Fine needle aspiration cytology (FNAC) from the mass failed to differentiate between a chondroma and a well differentiated chondrosarcoma. The final histopathological diagnosis of the surgically excised tumour was possible only after a detailed radiological and clinicopathological correlation. Total surgicalexcision of the tumour was followed by radiation therapy. A review of literature is included in view of the rarity of this condition and scarcity of similar reports in the Indian literature.

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A prospective study was undertaken in 459 cases, undergoing open heart surgery which necessitated media sternotomy, to see the frequency of brachial plexus and peripheral nerve related complications in the upper limbs. Seven cases (1.53) had such complications. These included lower trunk injury (3 cases), ulnar neuropathy at the elbow (3 cases), and radical neuropathy at the level of radical groove (one case). The youngest patient was aged 12 years and the remaining 6 patients were more than 30 years of age. There was no relation between the type of heart disease and these complications. The difference between patients with or without preparation of the internal mammary artery for graft was not significant. All the complications occurred on the left side. Ulnar and radical nerve palsy could be due to the positioning of the left limb. The barchial plexus injury was related to retraction of the sternum during operation. AA : Y

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Two hundred patients treated for supratentorial gliomas and for whom a follow up of one to sixteen years was available have been reviewed retrospectively for recurrence of tumor. All patients were initially treated with radical surgical tumour removal followed by radiotherapy. The series consisted of 82 cases of astrocytomas, 46 cases of mixed gliomas (oligoastrocytomas), 8 of oligodendrogliomas, 33 cases of malignant astrocytomas and 31 glioblastomas. However, period of recurrence could not be correlated to histological grade or presence or absence of residual tumour in a postoperative CT scan. Illustrated cases have been described. Problems of diagnosis and therapy have been discussed.

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A child with vein of Galen malformation whose CT scan showed extensive parenchymal calcification on follow-up, is presented. The possible role of differential venous pressures and medullary veins in the pathogenesis of cerebral calcification in our patient is discussed.

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Lambda-cyhalothrin ia a potent insecticide which acts by producing prolonging the duration of opening of sodium channels in neurons. While it is being used in several countries, no extensive neurophysiologic study of toxic effects in humans is available. We performed multimodality electro-physiologic studies in 15 spraymen in the age range of 19-48 years, before and after the exposure to this insecticide, with a view to detect any neurotoxic manifestation. Facial nerve conduction, blink response, repetitive stimulation test and visual, auditory and somatosensory evoked potentials were normal. Six subjects had mild changes in peripheral nerve conduction parameters. Comparison of mean values of the various nerve conduction studies in three subjects showed normal findings. We conclude that Lambda-cyhalothrin may produce transient, subclinical, electrophysiologic changes in the nerves of upper limb, possibly as a local toxic manifestation.

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This study was undertaken to evaluate the incidence and degree of hearing loss by clinical and audiometric analysis after lumbar puncture and to determine the site of involvement in brainstem auditory evoked response (BAER) studies. We also wanted to determine whether the degree of hearing loss is related to the size of the spinal needle used. Twenty patients were randomised for lumbar puncture into 2 groups, one using 20G and the other using 24G needle. Audiometry and brainstem auditory evoked response studies were carried out before and 24 hours after lumbar puncture. There was no statistically significant change in the hearing levels (on audiometry), before and after lumbar puncture, in cases belonging to both the groups (20G and 24G). There was no statistically significant change in the latency of each wave (on BAER) before and after lumbar puncture in cases belonging to both the groups (20G and 24G). No significant change either in hearing level on audiometry or in latency of BAER waves was noted when the two groups (20G, 24G) were compared with each other. In the present study, there was no indicator to suggest that the size of the lumbar puncture needle influenced auditory function.

[ABSTRACT]   Full text not available    [CITATIONS]  [PubMed]

A case of facioscapulohumeral dystrophy (FSH) with several unusual feature is being presented. It's early onset in infancy as against commonly occurring onset in second decade, the relentless progressive course without abortive or apparent arrest phase as is often seen, the calf hypertrophy, marked skeletal changes and associated retinal changes seen in Coat's disease are the noteworthy features of this unusual case.

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A rare case of extradural dumb-bell schwannoma of brachial plexus, arising from C6 root is reported along with review of literature. The clinical and radiological features useful in diagnosing the lesion are presented. The treatment policy to be adopted and the importance of adequate surgical exposure is discussed.

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Research by traditional methods of collection of data from case files, proformas, registers and master charts is laborious and cumbersome process. Case files in the hospital record section are often not easily accessible and can get lost. Moreover manually recorded and analysed data occupies lot of space. This entire tedious business is highly simplified by using computer software for record keeping and its analysis. We describe the development of a comprehensive, user friendly software programme for neurosurgeons using FoxPro to analyse data of cerebral aneurysm patients. Data entry is done with the help of prompted picklist instead of codes, the selection of which is tapping a key without the need to type it. This entered data is automatically converted into a computer database which generates more than 75 tables and graphs of predetermined nature and covers most of the parameters a neurosurgeon would be interested in. This coded database also generates a discharge summary in a flowing text. The prototype of programme has been used in our first hundred patients of cerebral aneurysm and debugged while analysing the next fifty.

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One hundred and six patients with intracranial meningiomas were evaluated retrospectively, and the degree of peritumoural oedema as noticed on CT, was correlated with the histological features and site and size of the tumour. 89 of the tumours which were 4.5 cms or more and all angioblastic and haemangiopericytic subtypes were associated with significant peritumoural oedema.

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Myelin basic protein (MBP) from one month old rat brain was purified by CM-52 cellulose chromatography and heparin sepharose chromatography. It's lectin properties were studied and conditions for optimum agglutination were observed. MBP behaved likea heparin binding lectin. Carbohydrade binding specificity studies showed that on molar basis MBP was feebly inhibited by lactose and strongly inhibited by heparin. Inhibition due to heparin was not due to electrostatic forces as evidenced by no inhibition due to other glycosaminoglycans. Argine and lysine residues were involved in the agglutination activity of MBP.

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