摘要
The differential diagnosis of parkinsonism includes idiopathic Parkinson's disease multiple system atrophy (MSA), cortical Lewy body disease and progressive supranuclear palsy. Although there is as yet no effective cure for any of these conditions, the correct diagnosis is important to all concerned and if the condition is MSA, treatment of the frequently accompanying urinary symptoms should be by medical not surgical means. Because of the selective atrophy of anterior horn cells in Onuf's nucleus that occurs in MSA, EMG of the striated sphincter to recognise changes of chronic reinnervation has become part of the routine investigation of the condition. Onuf's nucleus is not thought to be affected in other neurodegenerative diseases. This study reports the results of sphincter EMG in 127 patients initially suspected of having MSA and the eventual clinical diagnosis made when the patients' notes were reviewed 1-3 years later. Eighty-nine (70%) had had an abnormal sphincter EMG with a mean duration of 10 motor units >10.0 ms. There were 48 patients with an eventual clinical diagnosis of probably or definite MSA and sphincter EMG was abnormal in 98%of them, as it was in 87%of a further group of 32 patients with possible MSA. Sphincter EMG was abnormal in 20%of patients with neurodegenerative diseases other than MSA. The concordance between an abnormality of sphincter EMG and subsequent clinical diagnosis argues in favour of this being an important investigation in cases of suspected MSA.