Monomelic amyotrophy of the lower limb: an Australian experience
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摘要
Monomelic amyotrophy has been recognised as a benign form of anterior horn cell disease with a limited segmental involvement. We report 5 patients of Asian or Pacific origin with a history of progressive wasting of the lower limb muscles. The characteristic features noted are sporadic occurrence in young age, male predominance, insidious onset, localized muscle wasting disproportionate to the weakness unilaterally. Neurological examination revealed no evidence of long-tract signs or sensory abnormalities. Nerve conduction velocities were normal. EMG revealed chronic neurogenic changes not only in the affected muscles but also in the clinically uninvolved limb in two cases. Somato-sensory evoked potentials were abnormal in one out of 3 patients. MRI showed no evidence of significant disc disease, canal or foraminal stenosis or wasting of the spinal cord. Follow-up for 2-3 years showed no further progression of the disease. We believe these cases are examples of monomelic amyotrophy which is a well known entity in the eastern countries but is very rare in Australia. This diagnosis should be made retrospectively after a period of observation especially in patients of Asian or Indian background.

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