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Many neurological diseases like myopathy, periodic paralysis, ophthalmoplegia, and myasthenia gravis are known associations of thyrotoxicosis. However the association of neuropathy with thyrotoxicosis is not frequently recognized. First described by Charcot in 1889, thyrotoxic neuropathy or 'Basedow's Paraplegia' is a rarely reported entity. We describe here a case of a young woman with subacute distal neuropathy as the presenting manifestation of thyrotoxicosis. The neuropathy improved on antithyroid treatment. A careful literature search leads us to believe that peripheral neuropathy in thyrotoxicosis is under recognised. Thyroid function tests can be helpful in the diagnosis of this treatable neuropathy and should be included in the routine work up.

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BACKGROUND: To study the clinical and electrophysiological profile of n-hexane neuropathy in a tertiary care center of India. METHODS: Twenty five screen printers from five different factories, with peripheral neuropathy were included in the study. A detailed general physical, systemic and neurological examination was conducted Visual acuity, color vision and field charting was done in all the subjects. All patients were subjected to Folstein mini mental scale examination. Electrophysiological evaluation included motor and sensory conduction studies of the conventionally studied nerves of upper and lower limbs, Needle EMG of various distal and proximal muscles and patterned visual evoked responses. The electrophysiological profile was repeated every three months till one year. Sural nerve biopsy was studied in six patients. The patients were followed for a maximum of 4 years. RESULTS: Twenty three [92%] patients had sensory- motor neuropathy, while pure sensory neuropathy was seen in two. The sensory conductions were affected almost equally in upper as well as the lower limbs, while the motor conductions were affected more in the lower limbs than the upper limbs. The sensory conductions were not recordable in both the upper and the lower limbs in 18 [72%] patients. Motor conduction studies revealed an asymmetric and patchy involvement. Proximal conduction block was seen in 3 patients [12%]. On needle EMG features of denervation were seen in all patients. P100 latency was normal in all. Sural nerve biopsy showed a selective decrease in large myelinated axons with demyelination. Axonal swelling with focal areas of demyelination was observed in two patients. CONCLUSIONS: The electrophysiological patterns as well as the histopathology reflect the pathophysiology of n-hexane neuropathy.

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A 40-year-old woman presented with high fever with chills and rigors. Imaging studies revealed multiple liver abscesses with hepatosplenomegaly and gall-stones. Ultrasound-guided aspirate revealed pus that was negative on Gram and acid-fast staining and for amebic trophozoites. ELISA for echinococcus was strongly positive, but she did not respond to albendazole therapy. At surgery, Fasciola hepatica was detected and she responded well to bithinol postoperative.

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Monoclonal gammopathy of undetermined significance (MGUS) and peripheral neuropathy may be causally linked. In most cases, the M-protein is of IgG or IgM type. Peripheral neuropathy associated with IgA MGUS is uncommon, and there are limited reports. Here, we report a case of a 55-year-old male who was diagnosed to have symmetrical sensorimotor peripheral neuropathy associated with IgA MGUS with deposits of IgA-monoclonal protein in the myelin sheath.

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Gangliogliomas are rare tumors of the Central Nervous System. Five gangliogliomas were diagnosed out of 1560 brain tumours surgically resected out in a period of 5 years accounting for 0. 32%. We have tried to discuss in detail the pathological features of these tumours and have mentioned the clinical and radiological features associated with them. All the slides, tissue blocks and pathology reports of the surgical specimens of gangliglioma were reviewed and the clinical and radiological data reviewed. The ages of the patients ranged from 7-65 years with 4 males and 1 female. The tumors were located in the lateral ventricle (a rare site), temporal, parietal and the frontal lobes with duration of seizures varying from 1-9 years. The tumors were diagnosed by the presence of a dual population of neoplastic ganglionic and glial components. The glial components consisted of pilocytic astrocytes (l case), fibrillary astrocytes (2 cases), oligodendrocytes (1 case) and anaplastic astrocytes and oligodendrocytes (1 case). There was one-grade I GG, three-Grade II GGs and one-grade III GG. Astrocytes were the commonest glial component of GGs, either pilocytic or fibrillary. Oligodendrocytes as the glial component of GGs was seen in 2 cases one of which was anaplastic and this is a rare finding.

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Glioblastoma (GBM) is one of the commonest intra cranial tumors. Infratentorial GBM is, however, a rare entity. We report 3 cases of histologically proven posterior fossa GBM. The clinical presentation was of a rapidly growing posterior fossa mass. The diagnosis was made only on histological examination. The radiological features are non-specific and the clinical course same as GBM elsewhere. We describe their clinical presentation, radiological and pathological features and a review of describe the literature.

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The mitochondrial cytopathies are genetically and phenotypically heterogeneous group of disorders caused by structural and functional abnormalities in mitochondria. To the best of our knowledge, there are very few studies published from India till date. Selected and confirmed fourteen cases of neurological mitochondrial cytopathies with different clinical syndromes admitted between 1997 and 2000 are being reported. There were 8 male and 6 female patients. The mean age was 24.42+/-11.18 years (range 4-40 years). Twelve patients could be categorized into well-defined syndromes, while two belonged to undefined group. In the defined syndrome categories, three patients had MELAS (mitochondrial encephalopathy, lactic acidosis and stroke like episodes), three had MERRF (myoclonic epilepsy and ragged red fibre myopathy), three cases had KSS (Kearns-Sayre Syndrome) and three were diagnosed to be suffering from mitochondrial myopathy. In the uncategorized group, one case presented with paroxysmal kinesogenic dystonia and the other manifested with generalized chorea alone. Serum lactic acid level was significantly increased in all the patients (fasting 28.96+/-4.59 mg%, post exercise 41.02+/-4.93 mg%). Muscle biopsy was done in all cases. Succinic dehydrogenase staining of muscle tissue showed subsarcolemmal accumulation of mitochondria in 12 cases. Mitochondrial DNA study could be performed in one case only and it did not reveal any mutation at nucleotides 3243 and 8344. MRI brain showed multiple infarcts in MELAS, hyperintensities in putaminal areas in chorea and bilateral cerebellar atrophy in MERRF.

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We report a rare case of glossopharyngeal schwannoma whose clinical presentation and the radiological work up suggested an acoustic schwannoma. The diagnosis was made at surgery, once attachment to ninth cranial nerve was seen. The clinical presentation, radiological features and surgical findings of the glossopharyngeal schwannoma are presented along with the review of literature.

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Neurenteric or enterogenous cysts are rare, congenital, benign lesions of the central nervous system. Not more than 35 case reports of neurenteric cysts occurring in the cranial cavity have been documented. We describe two cases of intracranial neurenteric cysts.

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STUDY DESIGN: A case report of purely epidural cavernous haemangioma with MRI appearance and pathological features. OBJECTIVE: To present a rare case of extradural mass with differential diagnosis. SETTING: Delhi, India. METHOD: A 55-year-old man presented with progressive weakness and diminished sensation in both lower limbs. MRI demonstrated a pure extradural mass with no bony invasion. Histopathology of the lesion revealed a typical cavernous haemangioma. RESULT: The patient showed significant improvement after surgery. CONCLUSION: Radiological presentation could be confusing in a purely epidural cavernous haemangioma. Awareness of the characteristics of the lesion will facilitate diagnosis and treatment of the lesion.

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A case of medulloblastoma, which disappeared following steroid administration and a single sitting of radiotherapy (8Gy) is presented. At surgical exploration tumour was not found, multiple biopsies were negative. Post operative CT scan too did not show the tumour. Five months later, tumour reappeared in left cerebellar lobe in cystic and solid form. Patient was reoperated and tumour was decompressed. The biopsy showed medulloblastoma. To our knowledge, medulloblastomas are not known to be completely responsive to either of these modalities.

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A rare case of a young man with a diffusely calcified oligo astrocytoma in right parieto occipital region, which spread along the axonal fibres into right temporal lobe and to the left parietal lobe is presented. Computed tomography (CT) and magnetic resonance imaging (MRI) demonstrated the extent of the lesion. The mass was partially resected.

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The authors report the case of a patient with amyloidoma of the thoracic spine. A 34-year-old man presented with a 2-month history of upper-back pain, bilateral lower-extremity weakness, and numbness below the nipple. A computerized tomography study revealed an extradural mass with destruction of the T-2 lamina and pedicle. Intraoperatively, there was a pinkish, partially suctionable mass infiltrating the muscle plane and causing destruction of the T-2 lamina. Histological examination showed typical amyloid masses that demonstrated apple-green double refraction on examination of the Congo red-stained section under polarized light. Amyloidomas are rare benign lesions that, unlike other forms of amyloidosis, have an excellent prognosis. A cure is possible with complete resection of the mass.

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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.

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Calcifying pseudoneoplasm or fibro-osseous lesion of the CNS is uncommon. We report a case in a 6-year-old boy who presented with generalized tonic-clonic seizures for 6 months. There was a calcified mass in the depth of the left temporal lobe, which on histology showed collagenous nodules surrounded by spindle cells and seperated by chondromyxoid matrix. The only other report of such a lesion in a child is of a 12-year-old boy with an epidural lesion at C-6 level. It is important to recognize this entity since it is slow growing with a good prognosis.

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Dysembryoplastic neuroepithelial tumor (DNT) is a recently recognized tumor entity with distinctive clinicopathological features and an excellent long-term prognosis. We report 10 cases of DNT out of neurosurgical specimens sent for histopathological examination since 1994. Epilepsy surgery is not done at our center, and all the cases were sent with a clinical diagnosis of glioma. On histopathological examination, all the 10 cases contained the specific glioneuronal element. In 6 cases, glial nodules were noted in addition. This study therefore reports 4 cases of simple DNT and 6 cases of complex DNT. There was no nuclear atypia or mitosis in any of the cases. Proliferative index by PCNA varied from negative to <1%. It is very important to recognize this entity, as surgery cures the patient, and radiotherapy or chemotherapy is not required.

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A 53 years old male, a known case of ankylosing spondylitis having recurrent attacks of hypoglycaemia, developed symmetrical distal sensorimotor neuropathy. The neuropathy was axonal with secondary demyelination. Evidence of vasculopathy was also noted on histopathology of the nerve. Serum C-peptide level was low, a feature reported with autoimmune hypoglycaemia with antireceptor antibodies. The patient showed spontaneous recovery.

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A case of thalamic epidermoid cyst is reported. Its clinical presentation, radiological, operative findings and histological findings are discussed. Intraparenchymal epidermoids are uncommon and to our knowledge only one case of thalamic epidermoid has been reported earlier. The literature is reviewed in regard to incidence, location and etiology of Intracerebral epidermoid tumor cysts.

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OBJECTIVE AND IMPORTANCE: Hypophyseal tuberculomas are extremely rare lesions. The recognition of hypophyseal tuberculomas in the differential diagnosis of pituitary tumors is important, even with no evidence of systemic tuberculosis. CLINICAL PRESENTATION: A 27-year-old female patient presented with continuous, dull, generalized headaches and amenorrhea, with no history of visual diminution, galactorrhea, or endocrinological abnormalities and no evidence of systemic tuberculosis. The patient exhibited a normal water balance, without polyuria or polydipsia. A gynecological examination, including an endometrial biopsy for amenorrhea, did not reveal any abnormalities. Perimetric and endocrinological examination results were normal. Contrast magnetic resonance imaging revealed a dense enhancing intrasellar mass, with thickening of the pituitary stalk. INTERVENTION: Sublabial rhinoseptal transsphenoidal decompression of the lesion was performed. The histopathological features were consistent with a diagnosis of tuberculoma, and acid-fast bacilli were demonstrated in the surgically removed tissue with Ziehl-Neelsen staining. As soon as the histopathological features were known, the patient underwent a lumbar puncture for cerebrospinal fluid analysis, which indicated normal findings. An intradermal tuberculin test yielded negative results. The patient was treated with medical therapy for 18 months, and complete resolution of the lesion was observed in follow-up examinations. CONCLUSION: Hypophyseal tuberculomas are often mistaken for pituitary adenomas. The finding of a thickened pituitary stalk in contrast magnetic resonance imaging scans may be useful for the differentiation of these lesions from pituitary adenomas. Direct radiosurgery is not an appropriate primary treatment method for pituitary adenomas and is principally restricted to elderly, medically unfit patients with microadenomas and patients with residual or recurrent tumors after microsurgery. It is contraindicated for patients who exhibit a thickened pituitary stalk in contrast magnetic resonance imaging scans.

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