Document Detail


Madras motor neuron disease (MMND): clinical description and survival pattern of 116 patients from Southern India seen over 36 years (1971-2007).
MedLine Citation:
PMID:  18261745     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
A Nalini; K Thennarasu; B K Yamini; D Shivashankar; Nithin Krishna
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Publication Detail:
Type:  Journal Article     Date:  2008-02-07
Journal Detail:
Title:  Journal of the neurological sciences     Volume:  269     ISSN:  0022-510X     ISO Abbreviation:  J. Neurol. Sci.     Publication Date:  2008 Jun 
Date Detail:
Created Date:  2008-05-05     Completed Date:  2008-08-13     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0375403     Medline TA:  J Neurol Sci     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  65-73     Citation Subset:  IM    
Affiliation:
Department of Neurology, National Institute of Mental Health and Neurosciences, Bangalore, India. atchayaramnalini@yahoo.co.in
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MeSH Terms
Descriptor/Qualifier:
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

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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