| Madras motor neuron disease (MMND): clinical description and survival pattern of 116 patients from Southern India seen over 36 years (1971-2007). | |
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MedLine Citation:
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PMID: 18261745 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Madras motor neuron disease (MMND), MMND variant (MMNDV) and Familial MMND (FMMND) have a unique geographic distribution predominantly reported from Southern India. The characteristic features are onset in young, weakness and wasting of limbs, multiple lower cranial nerve palsies and sensorineural hearing loss. We describe the clinical features and survival pattern in 116 patients with Sporadic MMND, MMND variant and FMMND. A retrospective review of patients' medical records for clinical manifestations, electromyography, imaging, audiological and histopathology findings was performed. Over 36 years (1971 to 2007) 116 patients (men: 59; women: 57) particularly hailing from Southern India were seen. Mean age of onset was 15.8+/-7.9 years. Predominant initial manifestations were impaired hearing with wasting and weakness of distal limb muscles and pyramidal dysfunction. All patients had clinical and/or audiological evidence of hearing impairment. Patients with MMNDV in addition had optic atrophy. The overall mean survival duration was 334.9+/-27.9 months. Thus, Madras motor neuron disease is clinically a distinct entity with features of amyotrophic lateral sclerosis but with young age of onset and presence of auditory neuropathy. Studies to look for environmental and genetic basis of this intriguing disease are necessary to find the causation of this rare disorder. |
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Authors:
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A Nalini; K Thennarasu; B K Yamini; D Shivashankar; Nithin Krishna |
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Publication Detail:
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Type: Journal Article Date: 2008-02-07 |
Journal Detail:
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Title: Journal of the neurological sciences Volume: 269 ISSN: 0022-510X ISO Abbreviation: J. Neurol. Sci. Publication Date: 2008 Jun |
Date Detail:
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Created Date: 2008-05-05 Completed Date: 2008-08-13 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0375403 Medline TA: J Neurol Sci Country: Netherlands |
Other Details:
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Languages: eng Pagination: 65-73 Citation Subset: IM |
Affiliation:
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Department of Neurology, National Institute of Mental Health and Neurosciences, Bangalore, India. atchayaramnalini@yahoo.co.in |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Disability Evaluation Female Hearing Loss, Sensorineural / etiology, pathology Humans India / epidemiology Longitudinal Studies Male Motor Neuron Disease / complications, epidemiology*, mortality*, pathology Optic Atrophy / etiology, pathology Retrospective Studies Sex Factors Survival Analysis |
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