Background: Peripheral neuropathy is one of the manifestations of primary or familial amyloidosis. Published studies from India are limited.
Materials And Methods: We reviewed the clinical and pathological features of amyloid neuropathy diagnosed at our Institute over the last 39 years.
Introduction: Hypothalamic hamartoma (HH) is a rare developmental disorder presenting with gelastic seizures or precocious puberty attributed to gonadotrophin-releasing hormone expression by the hamartoma. The histogenesis of HH is uncertain, and diagnosis of HH is difficult in small biopsies due to its close resemblance to normal hypothalamic nuclei. TTF-1 and arginine vasopressin (AVP) are associated with gonadotropin-releasing hormone release.
View Article and Find Full Text PDFNeurotropism and infiltration by of peripheral nerves causing neuropathy are well established, but reports of central nervous system (CNS) damage are exceptional. We report CNS magnetic resonance imaging (MRI) abnormalities of the brain and spinal cord as well as lesions in nerve roots and plexus in leprosy patients. Eight patients aged between 17 and 41 years underwent detailed clinical, histopathological, and MRI evaluation.
View Article and Find Full Text PDFNeuronal/glioneuronal tumors are uncommon neoplasms of the CNS with frequent association with refractory epilepsy. Reports documenting the entire spectrum of neuronal/glioneuronal tumors are scarce in the literature. Zulch et al.
View Article and Find Full Text PDFThe clinical manifestations and histopathologic and neuroimaging findings in 4 Indian patients with a variant form of mucolipidosis IV are described. The presenting symptoms were psychomotor delay, spastic paraplegia, and mild mental retardation. One patient also had visual deterioration due to optic atrophy.
View Article and Find Full Text PDFElectrophysiological studies in subjects with HIV/AIDS demonstrate subtle changes in the visual pathway even in the absence of visual symptoms. But the pathological correlate of the electrophysiological abnormalities is largely unknown. This study attempts to correlate pathological changes in the retina and intraorbital portion of optic nerve in four drug naïve patients of AIDS caused by HIV-1 clade C, who had abnormalities in the visual evoked potentials recorded antemortem.
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