Skip to main content
SpringerLink
Log in

Atypische Parkinson-Syndrome

Atypical Parkinson syndromes

  • CME Weiterbildung • Zertifizierte Fortbildung
  • Published:
Der Nervenarzt Aims and scope Submit manuscript

Zusammenfassung

Atypische Parkinson-Syndrome haben zurzeit eine weitaus ungünstigere Prognose und sprechen kaum auf eine dopaminerge Medikation an. Eine möglichst frühzeitige differenzialdiagnostische Abgrenzung der atypischen Parkinson-Syndrome gegenüber dem idiopathischen Parkinson-Syndrom ist von erheblicher klinischer Relevanz. Im Falle der künftigen Entwicklung kausaler, neuroprotektiver Therapiestrategien wird eine frühe Diagnose einen rechtzeitigen Therapiebeginn ermöglichen. Im Frühstadium können die Multisystematrophie, die progressive supranukleäre Blickparese sowie die kortikobasale Degeneration vom idiopathischen Parkinson-Syndrom schwer zu unterscheiden sein. Zusätzliche elektrophysiologische, bildgebende und nuklearmedizinische Untersuchungen können zu diesem Zeitpunkt die klinische Diagnose stützen. Andererseits sollten im weiteren Verlauf auftretende klinische Hinweise für ein atypisches Parkinson-Syndrom immer zur kritischen Diagnoseüberprüfung führen.

Summary

At the moment atypical Parkinson syndromes have unfavorable prognoses and show little response to dopaminergic medication. Early differential diagnosis of these disorders from idiopathic Parkinson syndrome is of pivotal clinical relevance. In case of future causal, neuroprotective therapeutic strategies, early diagnosis will allow a timely start of therapy. In the early stage of disease, it might be difficult clinically to distinguish multiple system atrophy, progressive supranuclear palsy, and corticobasal ganglionic degeneration from idiopathic Parkinson syndrome. Additional electrophysiological, imaging, and nuclear medical investigations may support the clinical diagnosis. During disease progression clinical signs indicative of an atypical Parkinson syndrome should always warrant reevaluation of the diagnosis.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Abb. 1
Abb. 2
Abb. 3
Abb. 4
Abb. 5

Literatur

  1. Abele M, Schulz JB, Burk K et al. (2000) Evoked potentials in multiple system atrophy (MSA). Acta Neurol Scand 101: 111–115

    Article  PubMed  CAS  Google Scholar 

  2. Agid Y (2000) Une facon d’examiner un patient parkinsonien. Rev Neurol (Paris) 156: 17–22

    Google Scholar 

  3. Althaus A, Becker OA, Spottke A et al. (2008) Frequency and treatment of depressive symptoms in a Parkinson’s disease registry. Parkinsonism Relat Disord (in press)

  4. Baker M, Litvan I, Houlden H et al. (1999) Association of an extended haplotype in the tau gene with progressive supranuclear palsy. Hum Mol Genet 8: 711–715

    Article  PubMed  CAS  Google Scholar 

  5. Boesch SM, Wenning GK, Ransmayr G, Poewe W (2002) Dystonia in multiple system atrophy. J Neurol Neurosurg Psychiatry 72: 300–303

    Article  PubMed  CAS  Google Scholar 

  6. Boeve BF, Lang AE, Litvan I (2003) Corticobasal degeneration and its relationship to progressive supranuclear palsy and frontotemporal dementia. Ann Neurol [Suppl 5] 54: 15–19

    Google Scholar 

  7. Braak H, Del Tredici K (2008) Invited Article: Nervous system pathology in sporadic Parkinson disease. Neurology 70: 1916–1925

    Article  PubMed  Google Scholar 

  8. Braune S (2001) The role of cardiac metaiodobenzylguanidine uptake in the differential diagnosis of parkinsonian syndromes. Clin Auton Res 11: 351–355

    Article  PubMed  CAS  Google Scholar 

  9. Colosimo C, Pezzella FR (2002) The symptomatic treatment of multiple system atrophy. Eur J Neurol 9: 195–199

    Article  PubMed  CAS  Google Scholar 

  10. Colosimo C, Tiple D, Wenning GK (2005) Management of multiple system atrophy: state of the art. J Neural Transm 112: 1695–1704

    Article  PubMed  CAS  Google Scholar 

  11. Comella CL (2007) Sleep disorders in Parkinson’s disease: an overview. Mov Disord [Suppl 17] 22: 367–373

    Google Scholar 

  12. Csoti I, Warmuth-Metz M, Solymosi L et al. (2004) Differentiation of atypical Parkinson syndrome and delineation from idiopathic Parkinson syndrome with routine magnetic resonance tomography. Nervenarzt 75: 128–134

    Article  PubMed  CAS  Google Scholar 

  13. Daniele A, Moro E, Bentivoglio AR (1999) Zolpidem in progressive supranuclear palsy. N Engl J Med 341: 543–544

    Article  PubMed  CAS  Google Scholar 

  14. Dickson DW (1999) Neuropathologic differentiation of progressive supranuclear palsy and corticobasal degeneration. J Neurol [Suppl 2] 246: II6–II15

  15. Dickson DW, Bergeron C, Chin SS et al. (2002) Office of Rare Diseases neuropathologic criteria for corticobasal degeneration. J Neuropathol Exp Neurol 61: 935–946

    PubMed  CAS  Google Scholar 

  16. Diederich NJ (2007) The neurology of REM sleep. A synoptic tour de force. Nervenarzt 78: 406–417

    Article  PubMed  CAS  Google Scholar 

  17. Dubois B, Slachevsky A, Pillon B et al. (2005) „Applause sign“ helps to discriminate PSP from FTD and PD. Neurology 64: 2132–2133

    Article  PubMed  CAS  Google Scholar 

  18. Eggert KM, Oertel WH, Reichmann H et al. (2008) Parkinson-Syndrome: Diagnostik und Therapie. In: Leitlinien für Therapie und Diagnostik in der Neurologie. Thieme, Stuttgart, S. 82–112 (im Druck)

  19. Forstl H, Gratz S, Hahn U et al. (2008) Dementia with Lewy bodies and reduced dopamine transporter binding indicates significant acetylcholine deficiency. Dtsch Med Wochenschr [Suppl 1] 133: 11–14

  20. Fowler CJ (2007) Update on the neurology of Parkinson’s disease. Neurourol Urodyn 26: 103–109

    Article  PubMed  Google Scholar 

  21. Gelb DJ, Oliver E, Gilman S (1999) Diagnostic criteria for Parkinson disease. Arch Neurol 56: 33–39

    Article  PubMed  CAS  Google Scholar 

  22. Gilman S, Low PA, Quinn N et al. (1999) Consensus statement on the diagnosis of multiple system atrophy. J Neurol Sci 163: 94–98

    Article  PubMed  CAS  Google Scholar 

  23. Gilman S, Wenning GK, Low PA et al. (2008) Second consensus statement on the diagnosis of multiple system atrophy. Neurology (in revision)

  24. Graham JG, Oppenheimer DR (1969) Orthostatic hypotension and nicotine sensitivity in a case of multiple system atrophy. J Neurol Neurosurg Psychiatry 32: 28–34

    PubMed  CAS  Google Scholar 

  25. Groschel K, Kastrup A, Litvan I, Schulz JB (2006) Penguins and hummingbirds: midbrain atrophy in progressive supranuclear palsy. Neurology 66: 949–950

    Article  PubMed  Google Scholar 

  26. Hoglinger GU, Michel PP, Champy P et al. (2005) Experimental evidence for a toxic etiology of tropical parkinsonism. Mov Disord 20: 118–119

    Article  PubMed  Google Scholar 

  27. Hughes AJ, Daniel SE, Ben-Shlomo Y, Lees AJ (2002) The accuracy of diagnosis of parkinsonian syndromes in a specialist movement disorder service. Brain 125: 861–870

    Article  PubMed  Google Scholar 

  28. Hughes AJ, Daniel SE, Kilford L, Lees AJ (1992) Accuracy of clinical diagnosis of idiopathic Parkinson’s disease: a clinico-pathological study of 100 cases. J Neurol Neurosurg Psychiatry 55: 181–184

    PubMed  CAS  Google Scholar 

  29. Hussain IF, Brady CM, Swinn MJ et al. (2001) Treatment of erectile dysfunction with sildenafil citrate (Viagra) in parkinsonism due to Parkinson’s disease or multiple system atrophy with observations on orthostatic hypotension. J Neurol Neurosurg Psychiatry 71: 371–374

    Article  PubMed  CAS  Google Scholar 

  30. Jankovic J (2008) Parkinson’s disease: clinical features and diagnosis. J Neurol Neurosurg Psychiatry 79: 368–376

    Article  PubMed  CAS  Google Scholar 

  31. Kim JM, Lee KH, Choi YL et al. (2002) Levodopa-induced dyskinesia in an autopsy-proven case of progressive supranuclear palsy. Mov Disord 17: 1089–1090

    Article  PubMed  Google Scholar 

  32. Kollensperger M, Seppi K, Liener C et al. (2007) Diffusion weighted imaging best discriminates PD from MSA-P: a comparison with tilt table testing and heart MIBG scintigraphy. Mov Disord 22: 1771–1776

    Article  PubMed  Google Scholar 

  33. Kompoliti K, Goetz CG, Boeve BF et al. (1998) Clinical presentation and pharmacological therapy in corticobasal degeneration. Arch Neurol 55: 957–961

    Article  PubMed  CAS  Google Scholar 

  34. Kompoliti K, Goetz CG, Litvan I et al. (1998) Pharmacological therapy in progressive supranuclear palsy. Arch Neurol 55: 1099–1102

    Article  PubMed  CAS  Google Scholar 

  35. Kraft E, Schwarz J, Trenkwalder C et al. (1999) The combination of hypointense and hyperintense signal changes on T2-weighted magnetic resonance imaging sequences: a specific marker of multiple system atrophy? Arch Neurol 56: 225–228

    Article  PubMed  CAS  Google Scholar 

  36. Lannuzel A, Hoglinger GU, Verhaeghe S et al. (2007) Atypical parkinsonism in Guadeloupe: a common risk factor for two closely related phenotypes? Brain 130: 816–827

    Article  PubMed  Google Scholar 

  37. Liepelt I, Maetzler W, Blaicher HP et al. (2008) Treatment for dementia in parkinsonian syndromes. Efficacy of cholinesterase inhibitors. Nervenarzt 79: 36–39, 42–46

    Article  PubMed  CAS  Google Scholar 

  38. Litvan I, Agid Y, Calne D et al. (1996) Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): report of the NINDS-SPSP international workshop. Neurology 47: 1–9

    Article  PubMed  CAS  Google Scholar 

  39. Litvan I, Mangone CA, McKee A et al. (1996) Natural history of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) and clinical predictors of survival: a clinicopathological study. J Neurol Neurosurg Psychiatry 60: 615–620

    PubMed  CAS  Google Scholar 

  40. Mahapatra RK, Edwards MJ, Schott JM, Bhatia KP (2004) Corticobasal degeneration. Lancet Neurol 3: 736–743

    Article  PubMed  Google Scholar 

  41. Marshall V, Grosset D (2003) Role of dopamine transporter imaging in routine clinical practice. Mov Disord 18: 1415–1423

    Article  PubMed  Google Scholar 

  42. McKeith IG, Dickson DW, Lowe J et al. (2005) Diagnosis and management of dementia with Lewy bodies: third report of the DLB Consortium. Neurology 65: 1863–1872

    Article  PubMed  CAS  Google Scholar 

  43. Merlo IM, Occhini A, Pacchetti C, Alfonsi E (2002) Not paralysis, but dystonia causes stridor in multiple system atrophy. Neurology 58: 649–652

    PubMed  Google Scholar 

  44. Nath U, Ben-Shlomo Y, Thomson RG et al. (2001) The prevalence of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) in the UK. Brain 124: 1438–1449

    Article  PubMed  CAS  Google Scholar 

  45. Newman GC (1985) Treatment of progressive supranuclear palsy with tricyclic antidepressants. Neurology 35: 1189–1193

    PubMed  CAS  Google Scholar 

  46. Oba H, Yagishita A, Terada H et al. (2005) New and reliable MRI diagnosis for progressive supranuclear palsy. Neurology 64: 2050–2055

    Article  PubMed  CAS  Google Scholar 

  47. Oertel WH, Bandmann O (1999) Multiple system atrophy. J Neural Transm 56: 155–164

    CAS  Google Scholar 

  48. Oertel WH, Möller JC (2005) Kortikobasale Degeneration (CBD). Thieme, Stuttgart New York

  49. Oertel WH, Wachter T, Quinn NP et al. (2003) Reduced genital sensitivity in female patients with multiple system atrophy of parkinsonian type. Mov Disord 18: 430–432

    Article  PubMed  Google Scholar 

  50. Plazzi G, Corsini R, Provini F et al. (1997) REM sleep behavior disorders in multiple system atrophy. Neurology 48: 1094–1097

    PubMed  CAS  Google Scholar 

  51. Quinn N (1989) Multiple system atrophy–the nature of the beast. J Neurol Neurosurg Psychiatry Suppl 52: 78–89

    Google Scholar 

  52. Quinn NP, Marsden CD (1993) The motor disorder of multiple system atrophy. J Neurol Neurosurg Psychiatry 56: 1239–1242

    Article  PubMed  CAS  Google Scholar 

  53. Rampello L, Butta V, Raffaele R et al. (2005) Progressive supranuclear palsy: a systematic review. Neurobiol Dis 20: 179–186

    Article  PubMed  CAS  Google Scholar 

  54. Ransmayr G, Katzenschlager R, Dal-Bianco P et al. (2007) Dementia with Lewy Bodies and its differentiation from Alzheimer’s disease. Neuropsychiatrie 21: 63–74

    PubMed  Google Scholar 

  55. Rebeiz JJ, Kolodny EH, Richardson EP Jr (1968) Corticodentatonigral degeneration with neuronal achromasia. Arch Neurol 18: 20–33

    PubMed  CAS  Google Scholar 

  56. Rebeiz JJ, Kolodny EH, Richardson EP Jr (1967) Corticodentatonigral degeneration with neuronal achromasia: a progressive disorder of late adult life. Trans Am Neurol Assoc 92: 23–26

    PubMed  CAS  Google Scholar 

  57. Riley DE, Lang AE (1988) Corticobasal ganglionic degeneration (CBGD): further observations on six additional cases. Neurology [Suppl] 38: 360

    Google Scholar 

  58. Rinne JO, Lee MS, Thompson PD, Marsden CD (1994) Corticobasal degeneration. A clinical study of 36 cases. Brain 117: 1183–1196

    Article  PubMed  Google Scholar 

  59. Salazar G, Valls-Sole J, Marti MJ et al. (2000) Postural and action myoclonus in patients with parkinsonian type multiple system atrophy. Mov Disord 15: 77–83

    Article  PubMed  CAS  Google Scholar 

  60. Schrag A, Ben-Shlomo Y, Quinn NP (1999) Prevalence of progressive supranuclear palsy and multiple system atrophy: a cross-sectional study. Lancet 354: 1771–1775

    Article  PubMed  CAS  Google Scholar 

  61. Schrag A, Good CD, Miszkiel K et al. (2000) Differentiation of atypical parkinsonian syndromes with routine MRI. Neurology 54: 697–702

    PubMed  CAS  Google Scholar 

  62. Schwarz J, Tatsch K, Arnold G et al. (1992) 123I-iodobenzamide-SPECT predicts dopaminergic responsiveness in patients with de novo parkinsonism. Neurology 42: 556–561

    PubMed  CAS  Google Scholar 

  63. Schwarz J, Tatsch K, Gasser T et al. (1998) 123I-IBZM binding compared with long-term clinical follow up in patients with de novo parkinsonism. Mov Disord 13: 16–19

    Article  PubMed  CAS  Google Scholar 

  64. Shy GM, Drager GA (1960) A neurological syndrome associated with orthostatic hypotension: a clinical-pathologic study. Arch Neurol 2: 511–527

    PubMed  CAS  Google Scholar 

  65. Sibon I, Fenelon G, Quinn NP, Tison F (2004) Vascular parkinsonism. J Neurol 251: 513–524

    Article  PubMed  Google Scholar 

  66. Silber MH, Levine S (2000) Stridor and death in multiple system atrophy. Mov Disord 15: 699–704

    Article  PubMed  CAS  Google Scholar 

  67. Soliveri P, Monza D, Paridi D et al. (1999) Cognitive and magnetic resonance imaging aspects of corticobasal degeneration and progressive supranuclear palsy. Neurology 53: 502–507

    PubMed  CAS  Google Scholar 

  68. Spillantini MG, Goedert M (1998) Tau protein pathology in neurodegenerative diseases. Trends Neurosci 21: 428–433

    Article  PubMed  CAS  Google Scholar 

  69. Stamelou M, Reuss A, Pilatus U et al. (2008) Short-term effects of coenzyme Q(10) in progressive supranuclear palsy: a randomized, placebo-controlled trial. Mov Disord 23(7): 942–949

    Article  PubMed  Google Scholar 

  70. Steele JC, Richardson JC, Olszewski J (1964) Progressive supranuclear palsy. A heterogeneous degeneration involving the brain stem, basal ganglia and cerebellum with vertical gaze and pseudobulbar palsy, nuchal dystonia and dementia. Arch Neurol 10: 333–359

    PubMed  CAS  Google Scholar 

  71. Tison F, Yekhlef F, Chrysostome V et al. (2002) Parkinsonism in multiple system atrophy: natural history, severity (UPDRS-III), and disability assessment compared with Parkinson’s disease. Mov Disord 17: 701–709

    Article  PubMed  Google Scholar 

  72. Tolosa E, Wenning G, Poewe W (2006) The diagnosis of Parkinson’s disease. Lancet Neurol 5: 75–86

    Article  PubMed  Google Scholar 

  73. Vanek Z, Jankovic J (2001) Dystonia in corticobasal degeneration. Mov Disord 16: 252–257

    Article  PubMed  CAS  Google Scholar 

  74. Vetrugno R, Liguori R, Cortelli P et al. (2007) Sleep-related stridor due to dystonic vocal cord motion and neurogenic tachypnea/tachycardia in multiple system atrophy. Mov Disord 22: 673–678

    Article  PubMed  Google Scholar 

  75. Vlaar AM, Nijs T de, Kessels AG et al. (2008) Diagnostic value of 123I-ioflupane and 123I-iodobenzamide SPECT scans in 248 patients with parkinsonian syndromes. Eur Neurol 59: 258–266

    Article  PubMed  Google Scholar 

  76. Vodusek DB (2001) Sphincter EMG and differential diagnosis of multiple system atrophy. Mov Disord 16: 600–607

    Article  PubMed  CAS  Google Scholar 

  77. Campenhausen S von, Bornschein B, Wick R et al. (2005) Prevalence and incidence of Parkinson’s disease in Europe. Eur Neuropsychopharmacol 15: 473–490

    Article  CAS  Google Scholar 

  78. Walker Z, Jaros E, Walker RW et al. (2007) Dementia with Lewy bodies: a comparison of clinical diagnosis, FP-CIT single photon emission computed tomography imaging and autopsy. J Neurol Neurosurg Psychiatry 78: 1176–1181

    Article  PubMed  Google Scholar 

  79. Watanabe H, Saito Y, Terao S et al. (2002) Progression and prognosis in multiple system atrophy: an analysis of 230 Japanese patients. Brain 125: 1070–1083

    Article  PubMed  Google Scholar 

  80. Watts RL, William JH, Growdon JH (1985) Corticobasal ganglionic degeneration. Neurology [Suppl 1] 35: 178

  81. Weiner WJ (2005) A differential diagnosis of Parkinsonism. Rev Neurol Dis 2: 124–131

    PubMed  Google Scholar 

  82. Wenning GK, Ben Shlomo Y, Magalhaes M et al. (1994) Clinical features and natural history of multiple system atrophy. An analysis of 100 cases. Brain 117: 835–845

    Article  PubMed  Google Scholar 

  83. Wenning GK, Colosimo C, Geser F, Poewe W (2004) Multiple system atrophy. Lancet Neurol 3: 93–103

    Article  PubMed  Google Scholar 

  84. Wenning GK, Litvan I, Jankovic J et al. (1998) Natural history and survival of 14 patients with corticobasal degeneration confirmed at postmortem examination. J Neurol Neurosurg Psychiatry 64: 184–189

    PubMed  CAS  Google Scholar 

  85. Wenning GK, Seppi K, Tison F, Jellinger K (2002) A novel grading scale for striatonigral degeneration (multiple system atrophy). J Neural Transm 109: 307–320

    Article  PubMed  CAS  Google Scholar 

  86. Wenning GK, Tison F, Seppi K et al. (2004) Development and validation of the Unified Multiple System Atrophy Rating Scale (UMSARS). Mov Disord 19: 1391–1402

    Article  PubMed  Google Scholar 

  87. Williams DR, Silva R de, Paviour DC et al. (2005) Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson’s syndrome and PSP-parkinsonism. Brain 128: 1247–1258

    Article  PubMed  Google Scholar 

  88. Williams DR, Holton JL, Strand C et al. (2007) Pathological tau burden and distribution distinguishes progressive supranuclear palsy-parkinsonism from Richardson’s syndrome. Brain 130: 1566–1576

    Article  PubMed  Google Scholar 

  89. Yoshita M (1998) Differentiation of idiopathic Parkinson’s disease from striatonigral degeneration and progressive supranuclear palsy using iodine-123 meta-iodobenzylguanidine myocardial scintigraphy. J Neurol Sci 155: 60–67

    Article  PubMed  CAS  Google Scholar 

Download references

Danksagung

Wir danken Herrn Prof. Dr. Sid Gilman für die Überlassung der MSA-Konsensuskriterien in der 2 Fassung, Herrn Dr. K. Shiratori für die Bereitstellung der neuroradiologischen sowie Herrn PD Dr. H. Höffken für die Bereitstellung der nuklearmedizinischen Bildgebung. Für die kritische Durchsicht des Manuskripts danken wir Frau C. Miesner sowie Frau M. Windholz für die Unterstützung bei der Erstellung der Abbildungen.

Interessenkonflikt

Der korrespondierende Autor gibt an, dass kein Interessenkonflikt besteht.

Author information

Authors and Affiliations

  1. Klinik für Neurologie, Philipps-Universität Marburg, Rudolf-Bultmann-Straße 8, 35039, Marburg, Deutschland

    A. Strzelczyk, J.C. Möller, M. Stamelou, A. Matusch & W.H. Oertel

Authors
  1. A. Strzelczyk
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. J.C. Möller
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. M. Stamelou
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. A. Matusch
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. W.H. Oertel
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to A. Strzelczyk.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Strzelczyk, A., Möller, J., Stamelou, M. et al. Atypische Parkinson-Syndrome. Nervenarzt 79, 1203–1222 (2008). https://doi.org/10.1007/s00115-008-2559-2

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00115-008-2559-2

Schlüsselwörter

Keywords

Search

Navigation