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Q J Med 1994; 87: 693-699
© 1994 Association of Physicians

research-article

Motor neurone disease-a study of prevalence and disability

C.M. JAMES1,2,, P.S. HARPER1 and C.M. WILES2

1From the Departments of Medicine (Neurology) Cardiff, UK 2From the Departments of Medical Genetics, University of Wales College of Medicine Cardiff, UK

Address correspondence to Dr C.M. James, Withybush General Hospital, Fishguard Road, Haverfordwest, Pembrokeshire SA61 2PZ

Received 14 June 1994 Accepted for publication 13 July 1994.

We conducted a point prevalence study of motor neurone disease in the counties of South Glamorgan, Mid Glamorgan and Gwent, whose combined population is estimated at 1 394 400. A total of 56 patients were identified, giving a point prevalence for motor neurone disease in this area of 4.02/100 000 on 22/06/92. These cases were reclassified according to the World Federation of Neurology criteria for amyotrophic lateral sclerosis following a further clinical assessment, when disability was also evaluated (in 49 cases). Seven patients were reclassified on review of their medical records alone. Overall, 38 were classified as either definite or probable cases, which gave a point prevalence of 2.73/100 000. Of these patients, 94% (46/49) were living at home, 43% (21/49) scored 15 or less on the Barthel index, 65% (32/49) had some degree of mobility impairment, and 20% (10/49) were wheelchair-dependent. Swallowing speed was reduced (< 10 ml/s) in 67% (33/49), with three requiring feeding through a percutaneous gastrostomy tube. Vital capacity was <70% of predicted value for age, sex and height in 67% (29/43). One patient was receiving domiciliary ventilation. These results give a quantitative indication of the dependency in a population of patients with motor neurone disease.


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