Clinical experience of motor neurone disease

ABSTRACT

Motor Neurone Disease [MND] is an adult onset idiopathic, progressive and ultimately fatal degenerative neurological disorder, involving both upper and lower motor neurons in the cerebral cortex, brain stem and spinal cord. Recent studies revealed additional degeneration of some other areas of brain. No definite cause or treatment is known. Clinically it has been shown to have different onset forms, which frequently evolve into a common clinical entity known as A.l.S. This study, describes clinical pattern of MND in a portion of Pakistani population who presented at Neurology Department of Civil Hospital Karachi during the period 1992 - 1998. Total of 29 cases fulfilled the diagnostic criteria; all the cases were sporadic, except an elderly male patient with pure lower motor neuron illness. Twentyeight cases presented with combination of upper and lower motor neuron signs. Of these, 8 had bulbar onset and later spinal features. Twenty had spinal onset, out of them 7 developed additional bulbar symptoms and 13 retained the features of onset at the time of presentation. Overall mean age of onset was 43 +/- 9 years, about a decade younger than European population and nearer to South Asian studies. Spinal onset form affected mostly young males [male female ratio 191] and bulbar onset form affected elderly patients with equal sex ratio. However male to female ratio, considering all cases, was 4.81. Females were observed to be relatively spared during the reproductive age period. Rate of progression was slow to moderately rapid. Only two patients had fulminant course