International Journal of Research and Review

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Case Report

Year: 2018 | Month: June | Volume: 5 | Issue: 6 | Pages: 85-87

An Unusual Presentation of Motor Neuron Disease with Overlapping Features of both FTD-ALS and Madras Motor Neuron Disease

Dr Rajalaxmi Satapathy1, Dr Pitamber Behuria2, Dr A. K Mallik3

1Senior Resident Dept. of Neurology,SCB Medical College, Mangalabagh, Cuttack,
2Senior Resident Dept. of Neurology, SCB MCH, Cuttack
3Prof. and HOD Dept. of Neurology, SCB MCH, Cuttack

Corresponding Author: Dr Rajalaxmi Satapathy


Madras motor neuron disease (MMND) presents in the young in the first two decades. The majority of the cases reported are from South India. The hallmark of MMND is sensorineural deafness (hearing loss due to nerve defect), atrophy and weakness of the limb muscles, cranial nerve palsies involving cranial nerves VII, IX and XII, facial and bulbar muscles (kind of muscles that control the chewing, swallowing and speech). MMND variant has the additional features of optic atrophy and cerebellar signs. It is a slowly progressive disease. We report a case of motor neuron disease with sensorineural deafness like Madras motor neuron disease variety but with relatively late presentation and rapid progression and associated with frontotemporaldementia-like FTD –ALS variant. So it’s a very rare case associated with some features of MMND and some features of FTD-ALS.

Key words: MMND,FTD-ALS, South India, sensorineural deafness, optic atrophy

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