Advertisement

Exkurs: Multisystematrophie

  • G. K. Wenning
  • W. Poewe

Zusammenfassung

Die Multisystematrophie (MSA) ist eine sporadische, progredient verlaufende neurodegenerative Erkrankung mit Beginn zwischen dem 40. und 70. Lebensjahr, welche klinisch in 90% der Fälle mit einem Parkinson-Syndrom einhergeht und neuropathologisch durch oligodendrogliale Einschlußkörperchen („glial cytoplasmic inclusions“ GCIs, erstmals 1989 beschrieben durch Papp et al.) vor allem in Basalganglien und motorischem Kortex charakterisiert ist (PAPP und Lantos 1994). Vor allem Putamen und Substantia nigra pars compacta sind von neuronalem Zellverlust, Astrogliose und Pigmentdeposition betroffen (Wenning et al. 1995). Jedoch finden sich degenerative Veränderungen variablen Ausmaßes auch in anderen neuronalen Kerngebieten des Zentralnervensystems wie untere Oliven, Pons, Cerebellum, präganglionäre Sympathikusneurone im Rückenmark sowie Onufscher Kern im Sakralmark (Daniel 1992).

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

Literatur

  1. Adams RD, VAN Bogaert L, VAN DER Eecken H (1961) Dégénérescences nigrostriées et céré-bello-nigro-striées. Psychiat Neurol 142: 219–259CrossRefGoogle Scholar
  2. Ben-Shlomo Y, Wenning GR, Tison F, Quinn NP (1997) Survival of patients with pathologically proven multiple system atrophy: a meta-analysis. Neurology 48: 384–393PubMedCrossRefGoogle Scholar
  3. Colosimo C, Merello M, Pontieri F (1996) Amantadine in parkinsonian patients unreponsive to levodopa: a pilot study. J Neurol 243: 422–425PubMedCrossRefGoogle Scholar
  4. Daniel SE (1992) The neuropathology and neurochemistry of multiple system atrophy. In: Bannister R, Mathias C (eds) Autonomic failure. A textbook of clinical disorders of the autonomic nervous system, 3rd ed. Oxford University Press, Oxford, pp 564–585Google Scholar
  5. Dejerine J, Thomas AA (1900) L’atrophie olivoponto-cérébelleuse. Nouv Iconog de le Salpetriere 13: 330–370Google Scholar
  6. Eardley I, Quinn NP, Fowler CJ, Kirby RS, Parkhouse HM, Marsden CD, Bannister R (1989) The value of urethral sphincter electromyography in the differential diagnosis of parkinsonism. Br J Urol 64: 360–362PubMedCrossRefGoogle Scholar
  7. Hughes AJ, Daniel SE, Kilford L, Lees AJ (1992a) Accuracy of clinical diagnosis of idiopathic Parkinson’s disease: a clinico-pathological study of 100 cases. J Neurol Neurosurg Psychiatry 55: 181–184PubMedCrossRefGoogle Scholar
  8. Hughes AJ, Colosimo C, Kleedorfer B, Daniel SE, Lees AJ (1992b) The dopaminergic response in multiple system atrophy. J Neurol Neurosurg Psychiatry 55: 1009–1013PubMedCrossRefGoogle Scholar
  9. Konagaya M, Konagaya Y, Iida M (1994) Clinical and magnetic resonance imaging study of extrapyramidal symptoms in multiple system atrophy. J Neurol Neurosurg Psychiatry 57: 1528–1531PubMedCrossRefGoogle Scholar
  10. Lees AJ (1987) The Steele-Richardson-Olszewski syndrom (progressive supranuclear palsy). In: Marsden CD, Fahn S (eds) Movement disorders 2. Butterworths-Heinemann, London, pp 272–287Google Scholar
  11. Lees AJ (1992) The treatment of multiple system atrophy. In: Bannister R, Mathias C (eds) Autonomic failure. A textbook of clinical disorders of the autonomic nervous system. Oxford University Press, Oxford, pp 646–655Google Scholar
  12. Lees AJ, Bannister R (1981) The use of lisuride in the treatment of multiple system atrophy with autonomic failure (Shy-Drager syndrome). J Neurol Neurosurg Psychiatry 44: 347–351PubMedCrossRefGoogle Scholar
  13. Litvan I et al. (1997) What is the accuracy of the clinical diagnosis of multiple system atrophy? A clinicopathological study. Arch Neurol 54: 937–944PubMedCrossRefGoogle Scholar
  14. Magalhaes M, Wenning GK, Daniel SE, Quinn NP (1995) Autonomic dysfunction in pathologically confirmed multiple system atrophy and idiopathic Parkinson’s disease: a retrospective comparison. Acta Neurol Scand 91: 98–102PubMedCrossRefGoogle Scholar
  15. Papp MI, Lantos PL (1992) Accumulation of tubular structures in oligodendroglial and neuronal cells as the basic alteration in multiple system atrophy. J Neurol Sci 107: 172–182PubMedCrossRefGoogle Scholar
  16. Papp MI, Lantos PL (1994) The distribution of oligodendroglial inclusions in multiple system atrophy and its relevance to clinical symptomatology. Brain 117: 235–243PubMedCrossRefGoogle Scholar
  17. Papp MI, Kahn JE, Lantos PL (1989) Glial cytoplasmic inclusions in the CNS of patients with multilpe system atrophy (striatonigral degeneration, olivopontocerebellar atrophy and Shy Drager syndrome). J Neurol Sci 94: 79–100PubMedCrossRefGoogle Scholar
  18. Pramstaller PP, Wenning GK, Smith SJ, Beck RO, Quinn NP, Fowler CJ (1995) Nerve conduction studies, skeletal muscle EMG, and sphincter EMG in multiple system atrophy. J Neurol Neurosurg Psychiatry 58: 618–621PubMedCrossRefGoogle Scholar
  19. Quinn N (1989) Multiple system atrophy — the nature of the beast. J Neurol Neurosurg Psychiatry [Suppl] 52: 78–89CrossRefGoogle Scholar
  20. Quinn N (1994) Multiple system atrophy. In: Marsden CD, Fahn S (eds) Movement disorders 3. Butterworths-Heinemann, London, pp 262–281Google Scholar
  21. Quinn N (1995) Parkinsonism — recognition and differential diagnosis. BMJ 310: 447–452PubMedCrossRefGoogle Scholar
  22. Quinn NP, Marsden CD (1993) The motor disorder of multiple system atrophy. J Neurol Neurosurg Psychiatry 56: 1239–1242PubMedCrossRefGoogle Scholar
  23. Robbins TW, James M, Owen AM et al. (1994) Cognitive deficits in progressive supranuclear palsy, Parkinson’s disease, and multiple system atrophy in tests sensitive to frontal lobe dysfunction. J Neurol Neurosurg Psychiatry 57: 79–88PubMedCrossRefGoogle Scholar
  24. Schelosky L, Hierholzer J, Wissel J, Cordes M, Poewe W (1993) Correlation of clinical response in apomorphine test with D-2 receptor status as demonstrated by 123-1 IBZM-SPECT. Mov Disord 8: 453–458PubMedCrossRefGoogle Scholar
  25. Schulz JB, Klockgether T, Petersen D, Jauch M, Müller-Schauenburg W, Spieker S, Voigt K, Dichgans J (1994) Multiple system atrophy: natural history. MRI morphology, and dopamine receptor imaging with 123 IBZM-SPECT. J Neurol Neurosurg Psychiatry 57: 1047–1056PubMedCrossRefGoogle Scholar
  26. Schwarz J, Tatsch K, Arnold G, Gasser T, Trenkwalder C, Kirsch CM, Oertel WH (1992) 1231-iodobenzamide-SPECT predicts dopaminergic responsiveness in patients with de novo parkinsonism. Neurology 42: 556–561PubMedCrossRefGoogle Scholar
  27. Shy GM, Drager GA (I960) A neurological syndrome associated with orthostatic hypotension. Arch Neurol 2: 511–527CrossRefGoogle Scholar
  28. Vallderiola F, Valls-Sole F, Marte MJ, Tolosa ES (1995) Striated anal sphincter denervation in patients with progressive supranuclear palsy. Mov Disord 10: 550–555CrossRefGoogle Scholar
  29. Wenning GK, Quinn NP (1996) Facial dystonia in pathologically proven multiple system atrophy: a video report. Mov Disord 11: 107–109PubMedCrossRefGoogle Scholar
  30. Wenning GK, Ben-Shlomo Y, Magalhaes M, Daniel SE, Quinn NP (1994a) Clinical features and natural history of multiple system atrophy. An analysis of 100 patients. Brain 117: 835–845PubMedCrossRefGoogle Scholar
  31. Wenning GK, Jäger R, Kendall B, Kingsley D, Daniel SE, Quinn NP (1994b) Is cranial computerized tomography (CT) useful in the diagnosis of multiple system atrophy? Mov Disord 9: 333–336PubMedCrossRefGoogle Scholar
  32. Wenning GK, Ben-Shlomo Y, Magalhaes M, Daniel SE, Quinn NO (1995) A clinicopathological study of multiple system atrophy. J Neurol Neurosurg Psychiatry 58: 160–166PubMedCrossRefGoogle Scholar
  33. Wenning GK, Granata R, Laboyrie PML et al. (1996) Reversal of behavioural abnormalities by fetal allografts in a novel rat model of stri-atonigral degeneration. Mov Disord 11: 522–532PubMedCrossRefGoogle Scholar
  34. Wenning GK, Kraft E, Beck R, Fowler CJ, Mathias CJ, Quinn NP, Harding AE (1997a) Cerebellar presentation of multiple system atrophy. Mov Disord 12: 115–117PubMedCrossRefGoogle Scholar
  35. Wenning GK, Ben Shlomo Y, Tison F, Daniel SE, Quinn NP (1997b) Multiple system atrophy: a review of 203 cases. Mov Disord 12: 133–147PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Wien 1999

Authors and Affiliations

  • G. K. Wenning
  • W. Poewe

There are no affiliations available

Personalised recommendations