Abstract
Thirty floppy children diagnosed as having primary neuro-muscular disease on muscle biopsy 2 to 6 years earlier have been reviewed to correlate pathological findings with subsequent clinical progress. Spinal muscular atrophies of varying severity formed the largest group with 13 patients, 4 of whom had died during this interval. There were 6 patients each with benign hypotonia (who had completely recovered) and a myopathic disease process. It was felt that clinical examination alone was inadequate for judging the progress in any individual patient, especially, in the differentiation of mild spinal muscular atrophy from benign hypotonia. However a good correlation could be demonstrated between the initial pathological findings and clinical outcome. A combination of clinical, electromyographic and pathological features would seem to be necessary for accurate diagnosis and prognosis.
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