Journal of neurology, neurosurgery, and psychiatry
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J. Neurol. Neurosurg. Psychiatr. · Feb 2004
Case ReportsTangier disease--a diagnostic challenge in countries endemic for leprosy.
A case of Tangier disease (TD) is reported from India. The patient had presented with indolent mononeuritis multiplex and trophic ulcers of 16 years duration mimicking Hansen's disease. He received antileprosy treatment for one and a half years. ⋯ This is the first reported case of TD from a tropical country-India. An attempt to establish a correct diagnosis should be made by demonstrating the histopathological and lipoprotein abnormality to avoid long term medications that are chosen empirically and are unnecessary. The importance of recognising this disease in a country where Hansen's disease is highly endemic cannot be overemphasised.
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Patients with familial dysautonomia (FD) manifest episodic hyperhidrosis despite the reduction of sudomotor fibres and sweat glands associated with this autonomic neuropathy. We assessed peripheral sudomotor nerve fibre and sweat gland function to determine if this symptom was due to peripheral denervation hypersensitivity. ⋯ The finding of normal direct and axon reflex mediated sweat output in FD patients supports our hypothesis that, in a disorder with severe sympathetic nerve fibre reduction, sudomotor fibres, but not the sweat gland itself, exhibit chemical hypersensitivity. This might explain excessive episodic hyperhidrosis in situations with increased central sympathetic outflow.
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J. Neurol. Neurosurg. Psychiatr. · Jan 2004
The epidemiology of multiple sclerosis in Devon: a comparison of the new and old classification criteria.
To determine the prevalence of multiple sclerosis in Devon and compare the new McDonald classification guidelines with the Poser criteria currently used. ⋯ This is first survey to use the new recommended guidelines and compare these criteria with the Poser classification. The difficulties encountered with applying the new criteria in research are highlighted, as are the differences between the new and old criteria. This study reports one of the highest prevalences in the south of the UK, adding support for a north-south divide being a step effect rather than a latitudinal gradient.