Zur Differenzialdiagnose von Parkinson-Syndromen

Zusammenfassung

Die Differenzialdiagnose der Parkinson-Syndrome gewinnt aufgrund prognostischer und therapeutischer Implikationen zunehmend an Bedeutung. Dabei geht es in erster Linie um die Früherkennung atypischer Parkinson-Syndrome (APS) wie der Multisystematrophie (MSA), der progressiven supranukleären Blicklähmung (PSP), der kortikobasalen Degeneration (CBD) und der Demenz mit Lewy-Körpern (DLB). Diese Erkrankungen zeichnen sich neben der progredienten und häufig nicht L-Dopa-responsiven Parkinson-Symptomatik durch eine Vielfalt sogenannter Plus-Symptome aus. Letztere haben zum Teil Eingang in etablierte Diagnosekriterien gefunden. In den letzten Jahren konnte der Stellenwert von zusätzlichen Untersuchungsverfahren präzisiert werden, wenngleich die Datenlage derzeit noch keine laborgestützten Diagnosekriterien für die APS zulässt. Die folgenden Ausführungen zur Differenzialdiagnose wurden erarbeitet, um klinische Entscheidungsprozesse in der Diagnostik von Patienten mit einem Parkinson-Syndrom zu erleichtern.

Summary

Owing to prognostic and therapeutical implications, the differential diagnosis of parkinsonian syndromes is increasingly appreciated. The major task is the early diagnosis of atypical parkinsonian syndromes (APS) such as multiple system atrophy (MSA), progressive supranuclear palsy (PSP), corticobasal degeneration (CBD) and the dementia with Lewy bodies (DLB). Besides a rapid progression and often non or poorly responsive parkinsonism, these disorders are characterised by a variety of so-called plus symptoms. The latter have partly been included in established clinical diagnostic criteria. Within the last few years the significance of additional investigational techniques have been evaluated precisely, although the results available at present are not in favour of including any laboratory supported techniques into consensus diagnostic criteria. The following recommendations should aid in the differential diagnosis of a parkinsonian patient.

Schlüsselwörter

Differenzialdiagnose - Morbus Parkinson - Demenz mit Lewy Körpern - Multisystematrophie - progressive supranukleäre Paralyse - Kortikobasale Degeneration

Keywords

Differential diagnosis - Parkinson’s disease - Dementia with Lewy bodies - Multiple system atrophy - progressive supranuclear palsy - corticobasal degeneration

• Literatur

• 1 Brenneis C, Seppi K, Schocke MF, Müller J, Luginger E, Bösch S, Löscher W, Büchel C, Poewe W, Wenning GK. Voxel-based morphometry detects cortical atrophy in the parkinson variant of multiple system atrophy. Mov Disord 2003; 18: 1132-8.
• 2 Geser F, Stampfer-Kountchev M, Seppi K, Ndayisaba J, Wenning G, Poewe W. Warning signs (“red flags”) in multiple system atrophy (MSA): a preliminary cross-sectional analysis of 79 European MSA-P patients. Mov Disord 2004; 19 (Suppl. 09) 346.
• 3 Gibb W, Lees AJ. The relevance of the Lewy body to the pathogenesis of idiopathic Parkinson’s disease. Journal of Neurology, Neurosurgery and Psychiatry 1988; 51: 745-52.
• 4 Gilman S, Low PA, Quinn N, Albanese A, Ben Shlomo Y, Fowler CJ. et al. Consensus statement on the diagnosis of multiple system atrophy. J Auton Nerv Syst 1998; 74 (2-3): 189-92.
• 5 Litvan I, Agid Y, Calne D, Campbell G, Dubois B, Duvoisin RC. et al. Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): report of the NINDS-SPSP International Workshop. Neurology 1996; 47: 1-9.
• 6 Litvan I, Mangone CA, McKee A, Verny M, Parsa A, Jellinger K. et al. Natural history of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) and clinical predictors of survival: a clinicopathological study. Journal of Neurology, Neurosurgery and Psychiatry 1996; 61: 615-20.
• 7 Litvan I, Agid Y, Goetz C, Jankovic J, Wenning GK, Brandel JP. et al. Accuracy of the clinical diagnosis of corticobasal degeneration: a clinicopathologic study. Neurology 1997; 48: 119-25.
• 8 McKeith I, Galasko D, Kosaka K, Perry EK. et al. Consensus guidelines for the clinical and pathologic diagnosis of dementia with Lewy bodies (DLB): Report of the consortium on DLB international workshop. Neurology 1996; 47: 1113-24.
• 9 McKeith IG, Perry EK, Perry RH. Report of the second dementia with Lewy body international workshop: diagnosis and treatment. Consortium on Dementia with Lewy Bodies. Neurology 1999; 53 (05) 902-5.
• 10 McKeith I, Mintzer J, Aarsland D, Burn D, Chiu H, Cohen-Mansfield J. et al. Dementia with Lewy bodies. Lancet Neurol 2004; 3 (01) 19-28.
• 11 Schocke M, Seppi K, Esterhammer R, Kremser C, Jaschke W, Poewe W. Diffusion-weighted MRI differentiates the Parinson variant of multiple system atrophy from PD. Neurology 2002; 58: 575-80.
• 12 Schulz J, Skalej M, Wedekind D, Luft AR, Abele M, Voigt K. et al. Magnetic resonance imaging-based volumetry differentiates idiopathic Parkinson’s syndrome from multiple system atrophy and progressive supranuclear palsy. Annals of Neurology 1999; 45: 65-74.
• 13 Seppi K, Schocke M, Esterhammer R, Kremser C, Brenneis C, Mueller J. et al. DWI discriminates PSP from PD, but not from the parkinson variant of multiple system atrophy. Neurology 2003; 60: 922-7.
• 14 Watts R, Mirra SS, Richardson EP. Corticobasal ganglionic degeneration. In: Marsden CD, Fahn S. (ed.). Movement Disorders 3. London: Butterworth-Heinemann; 1994: 282-99.
• 15 Wenning G, Ben-Shlomo Y, Magalhäes M, Quinn NP. Clinicopathological study of 35 cases of multiple system atrophy. Journal of Neurology, Neurosurgery and Psychiatry 1995; 58: 160-6.
• 16 Wenning GK, Litvan I, Jankovic J, Granata R, Mangone CA, McKee A, Poewe W, Jellinger K, Chaudhuri KR, D’Olhaberriague L, Pearce RKB. Natural history and survival of 14 patients with corticobasal degeneration confirmed at postmortem examination. Journal of Neurology, Neurosurgery and Psychiatry 1998; 64: 184-9.
• 17 Wenning G, Seppi K, Jellinger K. et al. Survival of patients of dementia with Lewy bodies: a metaanalysis of 236 post-mortem confirmed cases. Neurology 2000; 54 (Suppl. 03) 391-2.
• 18 Wenning G, Ben-Shlomo Y, Hughes A, Daniel SE, Lees AJ, Quinn NP. What clinical features are most useful to distinguish definite multiple system atrophy from Parkinson’s disease?. Journal of Neurology, Neurosurgery and Psychiatry 2000; 68 (04) 434-40.