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Journal of Neurology, Neurosurgery, and Psychiatry logoLink to Journal of Neurology, Neurosurgery, and Psychiatry
. 1995 May;58(5):618–621. doi: 10.1136/jnnp.58.5.618

Nerve conduction studies, skeletal muscle EMG, and sphincter EMG in multiple system atrophy.

P P Pramstaller 1, G K Wenning 1, S J Smith 1, R O Beck 1, N P Quinn 1, C J Fowler 1
PMCID: PMC1073496  PMID: 7745413

Abstract

Although autonomic failure, parkinsonism, and cerebellar and pyramidal signs are well documented in multiple system atrophy, much less is known about the frequency and severity of involvement of the peripheral nervous system. The frequency and nature of peripheral nerve involvement has therefore been determined in 74 patients with multiple system atrophy using nerve conduction studies and skeletal muscle EMG. These findings were compared with those on sphincter EMG. Ninety per cent of the patients had an abnormal sphincter EMG, indicating denervation and reinnervation consistent with anterior horn cell loss in Onuf's nucleus, but only 40% had either abnormal nerve conduction studies (mixed sensorimotor axonal neuropathy in 17.5%) or abnormal skeletal muscle EMG (suggesting partial denervation in 22.5%). These data indicate a remarkable selective vulnerability of the anterior horn cells of Onuf's nucleus innervating external sphincter muscles relative to those supplying skeletal muscle in patients with multiple system atrophy. If this selective pattern of involvement can be explained it may be a clue to pathogenetic mechanisms in multiple system atrophy.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Beck R. O., Betts C. D., Fowler C. J. Genitourinary dysfunction in multiple system atrophy: clinical features and treatment in 62 cases. J Urol. 1994 May;151(5):1336–1341. doi: 10.1016/s0022-5347(17)35246-1. [DOI] [PubMed] [Google Scholar]
  2. Cohen J., Low P., Fealey R., Sheps S., Jiang N. S. Somatic and autonomic function in progressive autonomic failure and multiple system atrophy. Ann Neurol. 1987 Dec;22(6):692–699. doi: 10.1002/ana.410220604. [DOI] [PubMed] [Google Scholar]
  3. Eardley I., Quinn N. P., Fowler C. J., Kirby R. S., Parkhouse H. F., Marsden C. D., Bannister R. The value of urethral sphincter electromyography in the differential diagnosis of parkinsonism. Br J Urol. 1989 Oct;64(4):360–362. doi: 10.1111/j.1464-410x.1989.tb06043.x. [DOI] [PubMed] [Google Scholar]
  4. Fowler C. J., Kirby R. S., Harrison M. J., Milroy E. J., Turner-Warwick R. Individual motor unit analysis in the diagnosis of disorders of urethral sphincter innervation. J Neurol Neurosurg Psychiatry. 1984 Jun;47(6):637–641. doi: 10.1136/jnnp.47.6.637. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. Galassi G., Nemni R., Baraldi A., Gibertoni M., Colombo A. Peripheral neuropathy in multiple system atrophy with autonomic failure. Neurology. 1982 Oct;32(10):1116–1121. doi: 10.1212/wnl.32.10.1116. [DOI] [PubMed] [Google Scholar]
  6. Guindi G. M., Bannister R., Gibson W. P., Payne J. K. Laryngeal electromyography in multiple system atrophy with autonomic failure. J Neurol Neurosurg Psychiatry. 1981 Jan;44(1):49–53. doi: 10.1136/jnnp.44.1.49. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. Kirby R., Fowler C., Gosling J., Bannister R. Urethro-vesical dysfunction in progressive autonomic failure with multiple system atrophy. J Neurol Neurosurg Psychiatry. 1986 May;49(5):554–562. doi: 10.1136/jnnp.49.5.554. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. Konno H., Yamamoto T., Iwasaki Y., Iizuka H. Shy-Drager syndrome and amyotrophic lateral sclerosis. Cytoarchitectonic and morphometric studies of sacral autonomic neurons. J Neurol Sci. 1986 Apr;73(2):193–204. doi: 10.1016/0022-510x(86)90130-9. [DOI] [PubMed] [Google Scholar]
  9. Lockhart J. L., Webster G. D., Sheremata W., Camuzzi F. A., Carrion H. C. Neurogenic bladder dysfunction in the Shy-Drager syndrome. J Urol. 1981 Jul;126(1):119–121. doi: 10.1016/s0022-5347(17)54406-7. [DOI] [PubMed] [Google Scholar]
  10. Montagna P., Martinelli P., Rizzuto N., Salviati A., Rasi F., Lugaresi E. Amyotrophy in Shy-Drager syndrome. Acta Neurol Belg. 1983 May-Jul;83(3):142–157. [PubMed] [Google Scholar]
  11. Sobue G., Terao S., Kachi T., Ken E., Hashizume Y., Mitsuma T., Takahashi A. Somatic motor efferents in multiple system atrophy with autonomic failure: a clinico-pathological study. J Neurol Sci. 1992 Oct;112(1-2):113–125. doi: 10.1016/0022-510x(92)90140-g. [DOI] [PubMed] [Google Scholar]
  12. Swash M. Vulnerability of lower brachial myotomes in motor neurone disease: a clinical and single fibre EMG study. J Neurol Sci. 1980 Jul;47(1):59–68. doi: 10.1016/0022-510x(80)90025-8. [DOI] [PubMed] [Google Scholar]
  13. Terao S., Sobue G., Hashizume Y., Mitsuma T., Takahashi A. Disease-specific patterns of neuronal loss in the spinal ventral horn in amyotrophic lateral sclerosis, multiple system atrophy and X-linked recessive bulbospinal neuronopathy, with special reference to the loss of small neurons in the intermediate zone. J Neurol. 1994 Feb;241(4):196–203. doi: 10.1007/BF00863768. [DOI] [PubMed] [Google Scholar]
  14. Tohgi H., Tabuchi M., Tomonaga M., Izumiyama N. Selective loss of small myelinated and unmyelinated fibers in Shy-Drager syndrome. Acta Neuropathol. 1982;57(4):282–286. doi: 10.1007/BF00692184. [DOI] [PubMed] [Google Scholar]

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