Skip to main content
NCBI home page
Journal of Neurology, Neurosurgery, and Psychiatry logoLink to Journal of Neurology, Neurosurgery, and Psychiatry
. 1995 Aug;59(2):144–151. doi: 10.1136/jnnp.59.2.144

Multiple system atrophy presenting as parkinsonism: clinical features and diagnostic criteria.

A Albanese 1, C Colosimo 1, A R Bentivoglio 1, R Fenici 1, G Melillo 1, C Colosimo 1, P Tonali 1
PMCID: PMC485989  PMID: 7629528

Abstract

To evaluate the possibility that parkinsonian signs may be the only presenting feature of multiple system atrophy (MSA), parkinsonian patients were studied who had no atypical clinical signs and had no symptoms of autonomic dysfunction, but who reported that they had not experienced the anticipated good response to dopaminergic treatment. These stringent criteria identified 20 patients from a series of 298 consecutive parkinsonian outpatients. The following clinical pointers were analysed: (a) rate of disease progression; (b) symmetry of parkinsonian symptoms and signs; (c) occurrence of resting tremor during the first three years from onset. In addition, all patients underwent (d) acute and chronic challenge with dopaminergic drugs; (e) cardiovascular reflex autonomic function tests; (f) high field MRI. Rapid progression of disease was seen in 45% of patients, onset was symmetric in 25%, tremor was absent at onset in 70%, response to dopaminergic drug challenges was inadequate in 40%, abnormal cardiovascular reflexes occurred in 50%, and some abnormal MRI finding occurred in 35% of cases. Each of these features was equally weighted by giving to each patient a 0 to 6 point score corresponding to the number of abnormal findings. Fifteen patients scoring higher than 1 were considered at risk for having MSA: five of them were classified as clinically possible (score 2), six as clinically probable (score 3-4), and four patients were classified as clinically definite multiple system atrophy (score 5). The six pointers considered were variably combined in each patient, none of them being universally abnormal in patients with high scores. The patients were followed up for a mean 2.1 (SEM 0.65) years. All but one of the 10 patients prospectively classified as probable or definite MSA developed unequivocal clinical signs of fully symptomatic MSA. A receiver operator characteristic cure was plotted for the prospective score based on follow up diagnosis. The best compromise for trade off between sensitivity and specificity was a cut off value at a score of 3. The sensitivity and specificity of the individual pointers considered to predict fully symptomatic MSA varied considerably, and no single item could predict whether patients presenting with just parkinsonian signs went on during the two year follow up period to develop fully symptomatic MSA. Instead, the number of abnormalities offered a predictive value for the clinical prognosis of these parkinsonian patients.

Full text

PDF
144

Selected References

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

  1. ADAMS R. D., VANBOGAERT L., VANDEREECKEN H. STRIATO-NIGRAL DEGENERATION. J Neuropathol Exp Neurol. 1964 Oct;23:584–608. [PubMed] [Google Scholar]
  2. Albanese A., Colosimo C., Bentivoglio A. R., Fenici R., Tonali P. Correlation of cardiovascular autonomic function tests, magnetic resonance brain imaging and clinical features in suspect cases of multiple system atrophy. Funct Neurol. 1991 Jul-Sep;6(3):269–273. [PubMed] [Google Scholar]
  3. Brooks D. J., Ibanez V., Sawle G. V., Playford E. D., Quinn N., Mathias C. J., Lees A. J., Marsden C. D., Bannister R., Frackowiak R. S. Striatal D2 receptor status in patients with Parkinson's disease, striatonigral degeneration, and progressive supranuclear palsy, measured with 11C-raclopride and positron emission tomography. Ann Neurol. 1992 Feb;31(2):184–192. doi: 10.1002/ana.410310209. [DOI] [PubMed] [Google Scholar]
  4. Brooks D. J., Ibanez V., Sawle G. V., Quinn N., Lees A. J., Mathias C. J., Bannister R., Marsden C. D., Frackowiak R. S. Differing patterns of striatal 18F-dopa uptake in Parkinson's disease, multiple system atrophy, and progressive supranuclear palsy. Ann Neurol. 1990 Oct;28(4):547–555. doi: 10.1002/ana.410280412. [DOI] [PubMed] [Google Scholar]
  5. Colosimo C., Albanese A., Hughes A. J., de Bruin V. M., Lees A. J. Some specific clinical features differentiate multiple system atrophy (striatonigral variety) from Parkinson's disease. Arch Neurol. 1995 Mar;52(3):294–298. doi: 10.1001/archneur.1995.00540270090024. [DOI] [PubMed] [Google Scholar]
  6. Drayer B. P., Olanow W., Burger P., Johnson G. A., Herfkens R., Riederer S. Parkinson plus syndrome: diagnosis using high field MR imaging of brain iron. Radiology. 1986 May;159(2):493–498. doi: 10.1148/radiology.159.2.3961182. [DOI] [PubMed] [Google Scholar]
  7. Duguid J. R., De La Paz R., DeGroot J. Magnetic resonance imaging of the midbrain in Parkinson's disease. Ann Neurol. 1986 Dec;20(6):744–747. doi: 10.1002/ana.410200618. [DOI] [PubMed] [Google Scholar]
  8. 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]
  9. 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]
  10. Fulham M. J., Dubinsky R. M., Polinsky R. J., Brooks R. A., Brown R. T., Curras M. T., Baser S., Hallett M., Di Chiro G. Computed tomography, magnetic resonance imaging and positron emission tomography with [18F]fluorodeoxyglucose in multiple system atrophy and pure autonomic failure. Clin Auton Res. 1991 Mar;1(1):27–36. doi: 10.1007/BF01826055. [DOI] [PubMed] [Google Scholar]
  11. Gosset A., Pellissier J. F., Delpuech F., Khalil R. Dégénérescence striato-nigrique associée à une atrophie olivo-ponto-cérébelleuse. Etude anatomo-clinique de trois cas. Discussion nosologique. Rev Neurol (Paris) 1983;139(2):125–139. [PubMed] [Google Scholar]
  12. Huber S. J., Chakeres D. W., Paulson G. W., Khanna R. Magnetic resonance imaging in Parkinson's disease. Arch Neurol. 1990 Jul;47(7):735–737. doi: 10.1001/archneur.1990.00530070023006. [DOI] [PubMed] [Google Scholar]
  13. Hughes A. J., Lees A. J., Stern G. M. Apomorphine test to predict dopaminergic responsiveness in parkinsonian syndromes. Lancet. 1990 Jul 7;336(8706):32–34. doi: 10.1016/0140-6736(90)91531-e. [DOI] [PubMed] [Google Scholar]
  14. Konagaya M., Konagaya Y., Iida M. Clinical and magnetic resonance imaging study of extrapyramidal symptoms in multiple system atrophy. J Neurol Neurosurg Psychiatry. 1994 Dec;57(12):1528–1531. doi: 10.1136/jnnp.57.12.1528. [DOI] [PMC free article] [PubMed] [Google Scholar]
  15. Langston J. W., Widner H., Goetz C. G., Brooks D., Fahn S., Freeman T., Watts R. Core assessment program for intracerebral transplantations (CAPIT). Mov Disord. 1992;7(1):2–13. doi: 10.1002/mds.870070103. [DOI] [PubMed] [Google Scholar]
  16. 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]
  17. 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]
  18. Olanow C. W. Magnetic resonance imaging in parkinsonism. Neurol Clin. 1992 May;10(2):405–420. [PubMed] [Google Scholar]
  19. Pastakia B., Polinsky R., Di Chiro G., Simmons J. T., Brown R., Wener L. Multiple system atrophy (Shy-Drager syndrome): MR imaging. Radiology. 1986 May;159(2):499–502. doi: 10.1148/radiology.159.2.3961183. [DOI] [PubMed] [Google Scholar]
  20. 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]
  21. Rajput A. H., Rozdilsky B., Rajput A., Ang L. Levodopa efficacy and pathological basis of Parkinson syndrome. Clin Neuropharmacol. 1990 Dec;13(6):553–558. doi: 10.1097/00002826-199012000-00007. [DOI] [PubMed] [Google Scholar]
  22. Rodriguez M. E., Artieda J., Zubieta J. L., Obeso J. A. Reflex myoclonus in olivopontocerebellar atrophy. J Neurol Neurosurg Psychiatry. 1994 Mar;57(3):316–319. doi: 10.1136/jnnp.57.3.316. [DOI] [PMC free article] [PubMed] [Google Scholar]
  23. Savoiardo M., Bracchi M., Passerini A., Visciani A., Di Donato S., Cocchini F. Computed tomography of olivopontocerebellar degeneration. AJNR Am J Neuroradiol. 1983 May-Jun;4(3):509–512. [PMC free article] [PubMed] [Google Scholar]
  24. Savoiardo M., Strada L., Girotti F., D'Incerti L., Sberna M., Soliveri P., Balzarini L. MR imaging in progressive supranuclear palsy and Shy-Drager syndrome. J Comput Assist Tomogr. 1989 Jul-Aug;13(4):555–560. doi: 10.1097/00004728-198907000-00001. [DOI] [PubMed] [Google Scholar]
  25. Schulz J. B., Klockgether T., Petersen D., Jauch M., Müller-Schauenburg W., Spieker S., Voigt K., Dichgans J. Multiple system atrophy: natural history, MRI morphology, and dopamine receptor imaging with 123IBZM-SPECT. J Neurol Neurosurg Psychiatry. 1994 Sep;57(9):1047–1056. doi: 10.1136/jnnp.57.9.1047. [DOI] [PMC free article] [PubMed] [Google Scholar]
  26. Schwarz J., Tatsch K., Arnold G., Gasser T., Trenkwalder C., Kirsch C. M., Oertel W. H. 123I-iodobenzamide-SPECT predicts dopaminergic responsiveness in patients with de novo parkinsonism. Neurology. 1992 Mar;42(3 Pt 1):556–561. doi: 10.1212/wnl.42.3.556. [DOI] [PubMed] [Google Scholar]
  27. Stern M. B., Braffman B. H., Skolnick B. E., Hurtig H. I., Grossman R. I. Magnetic resonance imaging in Parkinson's disease and parkinsonian syndromes. Neurology. 1989 Nov;39(11):1524–1526. doi: 10.1212/wnl.39.11.1524. [DOI] [PubMed] [Google Scholar]
  28. Testa D., Savoiardo M., Fetoni V., Strada L., Palazzini E., Bertulezzi G., Girotti F. Multiple system atrophy. Clinical and MR observations on 42 cases. Ital J Neurol Sci. 1993 Apr;14(3):211–216. doi: 10.1007/BF02335661. [DOI] [PubMed] [Google Scholar]
  29. Vita G., Princi P., Calabro R., Toscano A., Manna L., Messina C. Cardiovascular reflex tests. Assessment of age-adjusted normal range. J Neurol Sci. 1986 Oct;75(3):263–274. doi: 10.1016/0022-510x(86)90074-2. [DOI] [PubMed] [Google Scholar]
  30. Wakai M., Kume A., Takahashi A., Ando T., Hashizume Y. A study of parkinsonism in multiple system atrophy: clinical and MRI correlation. Acta Neurol Scand. 1994 Oct;90(4):225–231. doi: 10.1111/j.1600-0404.1994.tb02712.x. [DOI] [PubMed] [Google Scholar]
  31. 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]

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

RESOURCES