Skip to main content
PMC home page
Journal of Neurology, Neurosurgery, and Psychiatry logoLink to Journal of Neurology, Neurosurgery, and Psychiatry
. 1995 Feb;58(2):160–166. doi: 10.1136/jnnp.58.2.160

Clinicopathological study of 35 cases of multiple system atrophy.

G K Wenning 1, Y Ben-Shlomo 1, M Magalhães 1, S E Daniel 1, N P Quinn 1
PMCID: PMC1073311  PMID: 7876845

Abstract

The clinical and pathological features of 35 cases with multiple system atrophy collected in the United Kingdom Parkinson's Disease Society Brain Bank (UKPDSBB) between 1985 and 1992 have been analysed. The median age of onset was 55 (range 33.3-75.8) years and median survival was 7.3 (range 2.1-11.5) years. Parkinsonism, usually asymmetric, occurred in all, and autonomic failure in all but one case. Cerebellar signs were noted in 34% and pyramidal features in 54% of the cases. Glial cytoplasmic inclusions were found in all cases with adequate fixation. Lewy bodies were detected in three cases. The substantia nigra was (usually severely) depleted of cells in all cases. With two exceptions the putamen was atrophic; the caudate and pallidum were less commonly and less severely affected. Overall nigrostriatal cell loss correlated with severity of disease at the time of death. The latest, but not the best, recorded levodopa response tended to be inversely related to the degree of putaminal degeneration. The olivopontocerebellar system was involved in 88% of the cases, the cerebellar vermis usually being more severely affected than the hemispheres. The presence of associated cerebellar pathology was, however, unrelated to the presence of cerebellar signs in life.

Full text

PDF
160

Selected References

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

  1. ADAMS R., VAN BOGAERT L., VAN DER EECKEN H. [Nigro-striate and cerebello-nigro-striate degeneration. (Clinical uniqueness and pathological variability of presenile degeneration of the extrapyramidal rigidity type]. Psychiatr Neurol (Basel) 1961;142:219–259. [PubMed] [Google Scholar]
  2. Bannister R., Oppenheimer D. R. Degenerative diseases of the nervous system associated with autonomic failure. Brain. 1972;95(3):457–474. doi: 10.1093/brain/95.3.457. [DOI] [PubMed] [Google Scholar]
  3. Costa C., Duyckaerts C., Cervera P., Hauw J. J. Les inclusions oligodendrogliales, un marqueur des atrophies multisystématisées. Rev Neurol (Paris) 1992;148(4):274–280. [PubMed] [Google Scholar]
  4. De Volder A. G., Francart J., Laterre C., Dooms G., Bol A., Michel C., Goffinet A. M. Decreased glucose utilization in the striatum and frontal lobe in probable striatonigral degeneration. Ann Neurol. 1989 Aug;26(2):239–247. doi: 10.1002/ana.410260210. [DOI] [PubMed] [Google Scholar]
  5. Eidelberg D., Takikawa S., Moeller J. R., Dhawan V., Redington K., Chaly T., Robeson W., Dahl J. R., Margouleff D., Fazzini E. Striatal hypometabolism distinguishes striatonigral degeneration from Parkinson's disease. Ann Neurol. 1993 May;33(5):518–527. doi: 10.1002/ana.410330517. [DOI] [PubMed] [Google Scholar]
  6. Fearnley J. M., Lees A. J. Striatonigral degeneration. A clinicopathological study. Brain. 1990 Dec;113(Pt 6):1823–1842. doi: 10.1093/brain/113.6.1823. [DOI] [PubMed] [Google Scholar]
  7. Graham J. G., Oppenheimer D. R. Orthostatic hypotension and nicotine sensitivity in a case of multiple system atrophy. J Neurol Neurosurg Psychiatry. 1969 Feb;32(1):28–34. doi: 10.1136/jnnp.32.1.28. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. Hughes A. J., Colosimo C., Kleedorfer B., Daniel S. E., Lees A. J. The dopaminergic response in multiple system atrophy. J Neurol Neurosurg Psychiatry. 1992 Nov;55(11):1009–1013. doi: 10.1136/jnnp.55.11.1009. [DOI] [PMC free article] [PubMed] [Google Scholar]
  9. Hughes A. J., Daniel S. E., Kilford L., Lees A. J. Accuracy of clinical diagnosis of idiopathic Parkinson's disease: a clinico-pathological study of 100 cases. J Neurol Neurosurg Psychiatry. 1992 Mar;55(3):181–184. doi: 10.1136/jnnp.55.3.181. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. Lantos P. L., Papp M. I. Cellular pathology of multiple system atrophy: a review. J Neurol Neurosurg Psychiatry. 1994 Feb;57(2):129–133. doi: 10.1136/jnnp.57.2.129. [DOI] [PMC free article] [PubMed] [Google Scholar]
  11. Mochizuki A., Mizusawa H., Ohkoshi N., Yoshizawa K., Komatsuzaki Y., Inoue K., Kanazawa I. Argentophilic intracytoplasmic inclusions in multiple system atrophy. J Neurol. 1992 Jul;239(6):311–316. doi: 10.1007/BF00867586. [DOI] [PubMed] [Google Scholar]
  12. Nakazato Y., Yamazaki H., Hirato J., Ishida Y., Yamaguchi H. Oligodendroglial microtubular tangles in olivopontocerebellar atrophy. J Neuropathol Exp Neurol. 1990 Sep;49(5):521–530. doi: 10.1097/00005072-199009000-00007. [DOI] [PubMed] [Google Scholar]
  13. Papp M. I., Kahn J. E., Lantos P. L. Glial cytoplasmic inclusions in the CNS of patients with multiple system atrophy (striatonigral degeneration, olivopontocerebellar atrophy and Shy-Drager syndrome). J Neurol Sci. 1989 Dec;94(1-3):79–100. doi: 10.1016/0022-510x(89)90219-0. [DOI] [PubMed] [Google Scholar]
  14. Papp M. I., Lantos P. L. Accumulation of tubular structures in oligodendroglial and neuronal cells as the basic alteration in multiple system atrophy. J Neurol Sci. 1992 Feb;107(2):172–182. doi: 10.1016/0022-510x(92)90286-t. [DOI] [PubMed] [Google Scholar]
  15. Quinn N. Multiple system atrophy--the nature of the beast. J Neurol Neurosurg Psychiatry. 1989 Jun;Suppl:78–89. doi: 10.1136/jnnp.52.suppl.78. [DOI] [PMC free article] [PubMed] [Google Scholar]
  16. Robbins T. W., James M., Lange K. W., Owen A. M., Quinn N. P., Marsden C. D. Cognitive performance in multiple system atrophy. Brain. 1992 Feb;115(Pt 1):271–291. doi: 10.1093/brain/115.1.271. [DOI] [PubMed] [Google Scholar]
  17. SHY G. M., DRAGER G. A. A neurological syndrome associated with orthostatic hypotension: a clinical-pathologic study. Arch Neurol. 1960 May;2:511–527. doi: 10.1001/archneur.1960.03840110025004. [DOI] [PubMed] [Google Scholar]
  18. Wenning G. K., Ben Shlomo Y., Magalhães M., Daniel S. E., Quinn N. P. Clinical features and natural history of multiple system atrophy. An analysis of 100 cases. Brain. 1994 Aug;117(Pt 4):835–845. doi: 10.1093/brain/117.4.835. [DOI] [PubMed] [Google Scholar]
  19. Wenning G. K., Quinn N., Magalhăes M., Mathias C., Daniel S. E. "Minimal change" multiple system atrophy. Mov Disord. 1994 Mar;9(2):161–166. doi: 10.1002/mds.870090206. [DOI] [PubMed] [Google Scholar]

Articles from Journal of Neurology, Neurosurgery, and Psychiatry are provided here courtesy of BMJ Publishing Group

RESOURCES