CLINICAL PROFILE OF MOTOR NEURON DISEASE IN KASHMIR VALLEY ? A HOSPITAL BASED STUDY

Background:-This general term is used to designate a progressive degenerative disorder of motor neurons in the spinal cord, brainstem and motor cortex, manifesting clinically by muscular weakness, atrophy and corticospinal tract signs in varying combinations. There is no report on clinical profile of motor neuron disease or amyotropic lateral sclerosis (ALS) from the state of Jammu and Kashmir. Objective: To find the clinical profile of motor neuron disease in Kashmir valley. Methodology:-This hospital based study was conducted on 40 patients of motor neuron disease (MND) visiting OPD / IPD of Department of Medicine, SMHS Hospital. Patients were studied for their presentation, etiology, clinical and laboratory features. Results:-Majority of our patients i.e. 18 (45%) were falling in the age group of 51 to 60 years with males more than females with a male to female ratio of 1.85:1. Weakness of muscles was the most common presenting symptom in our patients with fasciculation as the most common motor system findings. Conclusion:-Motor neuron disorders appear to be a clinically heterogeneous disorder with varied clinical presentation encompassing a range of upper or lower motor neuron dysfunction or both. The patients from Kashmir had disease onset earlier by a decade than most of the studies.