Abstract
Motor Neuron Disease (MND) is a heterogenous group of disorders with degeneration of upper and/or lower motor neurons. Limited data is available for clinical characteristics of MND from western India. Methods: We retrospectively observed all cases of MND, evaluated at our centre. Those with conrmed diagnosis, exclusion of secondary causes and with one year of minimal follow up were included, for the anaysis. RESULTS: Out of 51 patients of MND, 36 were diagnosed as Amyotropic Lateral Sclerosis (ALS) and 15 patients were having pure Lower Motor Neuron(LMN) type of MND. Male: female ration was 2.8:1 in ALS group , with mean age of 50 years. Out of 10 bulbar onset MND patients, 5 died in the follow-up period. In the LMN subgroup, younger onset monomelic amyotrophy, of upper limb onset (Hirayama) was commonest subtype. No patients with isolated Upper motor Neuron type of MND was found. SUMMARY: ALS subgroups of patients had younger age of onset in western Indian population, with signicant male rd preponderance. Hirayama disease was commonest LMN type of MND, with onset in 3 decade and more commonly seen in males
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