CLINICAL CHARACTERISTICS OF MOTOR NEURON DISEASE : A SINGLE CENTRE DATA FROM GUJARAT STATE.

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 conrmed 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 signicant male rd preponderance. Hirayama disease was commonest LMN type of MND, with onset in 3 decade and more commonly seen in males