Klinische Differenzialdiagnose des Parkinson-Syndroms

Zusammenfassung

Die Differenzialdiagnose des Parkinson-Syndroms umfasst neurodegenerative sowie sekundäre Formen des hypokinetisch-rigiden Syndroms. In dieser Übersichtsarbeit soll vorwiegend auf neurodegenerative Erkrankungen eingegangen werden, den Morbus Parkinson, die Demenz mit Lewy-Körper, die Multisystematrophie, die Progressive Supranukleäre Blickparese und die Kortikobasale Degeneration. Die klinische Diagnosefindung anhand gültiger Diagnosekriterien ist eine Aufgabe, die nach eingehender Anamnese und aufmerksamer neurologischer und neuropsychologischer Untersuchung in vielen Fällen gelingt. Pharmakologische Testungen sowie laborchemische, elektrophysiologische und bildgebende Verfahren untermauern die Verdachtsdiagnose. Gelegentlich muss die endgültige Diagnose im zeitlichen Verlauf aufgrund einer Änderung des klinischen Befundes angepasst werden.

Von relevantem therapeutischem und prognostischem Interesse erscheint auf den ersten Blick nur die Unterteilung in ein idiopathisches Parkinson-Syndrom und atypische Parkinson-Syndrome. Diese Sicht trügt, da sich gerade auf dem Gebiet der spezifischen und unter anderem neuroprotektiven Therapien bei MSA oder PSP ein Anwendungsfeld öffnet und viele therapeutische Ansätze derzeit in Studien untersucht werden. Daher ist die Differenzialdiagnose innerhalb der atypischen Parkinson-Syndrome schon längst keine akademische Frage mehr, sondern von großer Bedeutung, um für die Zukunft wirkungsvolle Therapien zu etablieren. Die nuklearmedizinische Diagnostik wird zur korrekten Einteilung der Patienten sicherlich einen großen Beitrag liefern.

Abstract

Differential diagnosis of Parkinson Syndromes includes neurodegenerative and symptomatic disorders of the hypokinetic-rigid syndrome. This review focuses on neurodegenerative diseases as Parkinson’s disease, dementia with Lewy bodies, multiple system atrophy, progressive supranuclear palsy and corticobasal degeneration. The diagnosis is routinely based on clinical findings which are achieved by a careful history and a comprehensive neurological and neuropsychological examination. Pharmacological tests as well as laboratory and electrophysiological investigations and imaging techniques are currently used to affirm the suspected diagnosis based on established diagnostic criteria. Occasionally, the final diagnosis must be revised due to the occurence of new symptoms in the course of the disease.

It is a common supposition that only differentiation between Parkinson’s disease and atypical Parkinson syndromes is of therapeutic and prognostic interest. However, this is not the case, since the correct identification of the right patient population is mandatory for the development of new therapeutical and even neuroprotective strategies for atypical Parkinson syndromes in future trials. Certainly, the nuclear medicine will contribute to this challenge.

Schlüsselwörter

Morbus Parkinson - Demenz mit Lewy-Körper - Multisystematrophie - Progressive Supranukleäre Blickparese - Kortikobasale Degeneration - Atypisches Parkinson-Syndrom

Key words

Parkinson’s disease - dementia with Lewy bodies - multiple system atrophy - progressive supranuclear palsy - corticobasal degeneration - atypical Parkinson syndrome

• Literatur

• 1 Adachi M, Kawanami T, Ohshima H et al. Morning glory sign: a particular MR finding in progressive supranuclear palsy. Magn Reson Med Sci 2004; 15: 125-132
• 2 Boeve BF, Lang AE, Litvan I. Corticobasal degeneration and its relationship to progressive supranuclear palsy and frontotemporal dementia. Ann Neurol 2003; 54 (Suppl. 05) S15-S19
• 3 Braak H, Del Tredici K, Rub U et al. Staging of brain pathology related to sporadic Parkinson’s disease. Neurobiol Aging 2003; 24: 197-211
• 4 Brodaty H, Seeher K, Gibson L. Dementia time to death: a systematic literature review on survival time and years of life lost in people with dementia. Int Psychogeriatr 2012; 13: 1-12
• 5 Brown RG, Lacomblez L, Landwehrmeyer BG et al. Cognitive impairment in patients with multiple system atrophy and progressive supranuclear palsy. Brain 2010; 133: 2382-2393
• 6 Diem-Zangerl A, Seppi K, Wenning GK et al. Mortality in Parkinson’s disease: a 20-year follow-up study. Mov Disord 2009; 30: 819-825
• 7 Driver JA, Kurth T, Buring JE. Parkinson disease and risk of mortality: a prospective comorbidity-matched cohort study. Neurology 2008; 70: 1423-1430
• 8 Driver JA, Logroscino G, Gaziano JM. Incidence and remaining lifetime risk of Parkinson disease in advanced age. Neurology 2009; 72: 432-438
• 9 Emre M, Aarsland D, Brown R et al. Clinical diagnostic criteria for dementia associated with Parkinson’s disease. Mov Disord 2007; 22: 1689-1707
• 10 Gilman S, Wenning GK, Low PA et al. Second consensus statement on the diagnosis of multiple system atrophy. Neurology 2008; 71: 670-676
• 11 Gröschel K, Kastrup A, Litvan I et al. Penguins and hummingbirds: midbrain atrophy in progressive supranuclear palsy. Neurology 2006; 66: 949-950
• 12 Hagenah J, Seidel G. Sonography of the parenchyma in Parkinson’s disease. Nervenarzt 2010; 81: 1189-1195
• 13 Hughes AJ, Colosimo C, Kleedorfer B et al. The dopaminergic response in multiple system atrophy. J Neurol Neurosurg Psychiatry 1992; 55: 1009-1013
• 14 Hughes AJ, Daniel SE, Kilford L et al. Accuracy of clinical diagnosis of idiopathic Parkinson’s disease: a clinico-pathological study of 100 cases. J Neurol Neurosurg Psychiatry 1992; 55: 181-184
• 15 Josephs KA, Boeve BF, Duffy JR et al. Atypical progressive supranuclear palsy underlying progressive apraxia of speech and nonfluent aphasia. Neurocase 2005; 11: 283-296
• 16 Kato N, Arai K, Hattori T. Study of the rostral midbrain atrophy in progressive supranuclear palsy. J Neurol Sci 2003; 210: 57-60
• 17 Kitada T, Asakawa S, Hattori N et al. Mutations in the parkin gene cause autosomal recessive juvenile parkinsonism. Nature 1998; 392 (6676) 605-608
• 18 Köllensperger M, Krismer F, Pallua A et al. Erythropoietin is neuroprotective in a transgenic mouse model of multiple system atrophy. Mov Disord 2011; 26: 507-515
• 19 Köllensperger M, Seppi K, Liener C et al. Diffusion weighted imaging best discriminates PD from MSA-P: A comparison with tilt table testing and heart MIBG scintigraphy. Mov Disord 2007; 22: 1771-1776
• 20 Kouri N, Whitwell JL, Josephs KA et al. Corticobasal degeneration: a pathologically distinct 4R tauopathy. Nat Rev Neurol 2011; 7: 263-272
• 21 Kraft E, Schwarz J, Trenkwalder C et al. The combination of hypointense and hyperintense signal changes on T2-weighted magnetic resonance imaging sequences: a specific marker of multiple system atrophy?. Arch Neurol 1999; 56: 118-225
• 22 Litvan I, Agid Y, Calne D 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
• 23 Litvan I, Bhatia KP, Burn DJ et al. SIC Task Force appraisal of clinical diagnostic criteria for Parkinsonian disorders. Movement Disorders 2003; 18: 467-486
• 24 Louis ED, Faust PL, Vonsattel JP et al. Neuropathological changes in essential tremor: 33 cases compared with 21 controls. Brain 2007; 130: 3297-3307
• 25 Martinez A, Perez DI. GSK-3 inhibitors: a ray of hope for the treatment of Alzheimer’s disease?. J Alzheimers Dis 2008; 15: 181-191
• 26 McKeith IG, Dickson DW, Lowe J et al. Diagnosis and management of dementia with Lewy bodies: third report of the DLB Consortium. Neurology 2005; 65: 1863-1872
• 27 Merello M, Nouzeilles MI, Arce GP et al. Accuracy of acute levodopa challenge for clinical prediction of sustained long-term levodopa response as a major criterion for idiopathic Parkinson’s disease diagnosis. Mov Disord 2002; 17: 795-798
• 28 O’Sullivan SS, Massey LA, Williams DR et al. Clinical outcomes of progressive supranuclear palsy and multiple system atrophy. Brain 2008; 131: 1362-1372
• 29 Paisan-Ruiz C, Jain S, Evans EW et al. Cloning of the gene containing mutations that cause PARK8-linked Parkinson’s disease. Neuron 2004; 44: 595-600
• 30 Paviour DC, Thornton JS, Lees AJ et al. Diffusion-weighted magnetic resonance imaging differentiates Parkinsonian variant of multiple-system atrophy from progressive supranuclear palsy. Mov Disord 2007; 22: 68-74
• 31 Posada IJ, Benito-Leon J, Louis ED et al. Mortality from Parkinson’s disease: a population-based prospective study (NEDICES). Mov Disord 2011; 26: 2522-2529
• 32 Schrag A, Wenning GK, Quinn N et al. Survival in multiple system atrophy. Mov Disord 2008; 23: 294-296
• 33 Stamelou M, Reuss A, Pilatus U et al. Short-term effects of coenzyme Q10 in progressive supranuclear palsy: a randomized, placebo-controlled trial. Mov Disord 2008; 23: 942-949
• 34 Stefanova N, Poewe W, Wenning GK. Rasagiline is neuroprotective in a transgenic model of multiple system atrophy. Exp Neurol 2008; 210: 421-427
• 35 Tsuboi Y, Josephs KA, Boeve BF et al. Increased tau burden in the cortices of progressive supranuclear palsy presenting with corticobasal syndrome. Movement Disorders 2005; 20: 982-988
• 36 Walter U, Dressler D, Probst T et al. Transcranial brain sonography findings in discriminating between parkinsonism and idiopathic Parkinson disease. Arch Neurol 2007; 64: 1635-1640
• 37 Walter U, Dressler D, Wolters A et al. Sonographic discrimination of corticobasal degeneration vs progressive supranuclear palsy. Neurology 2004; 63: 504-509
• 38 Walter U, Niehaus L, Probst T et al. Brain parenchyma sonography discriminates Parkinson’s disease and atypical parkinsonian syndromes. Neurology 2003; 60: 74-747
• 39 Wenning GK, Granata R. Multiple system atrophy: more on the nature of the beast. Neurologia 1998; 13: 105-110
• 40 Williams DR, de Silva R, Paviour DC et al. Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson’s syndrome and PSP-parkinsonism. Brain 2005; 128: 1247-1258
• 41 Williams DR, Holton JL, Strand K et al. Pure akinesia with gait freezing: A third clinical phenotype of progressive supranuclear palsy. Movement Disorders 2007; 22: 2235-2241
• 42 Williams DR, Lees AJ. Progressive supranuclear palsy: clinicopathological concepts and diagnostic challenges. Lancet Neurology 2009; 8: 270-279
• 43 Wüllner U, Schmitz-Hubsch T, Abele M. Features of probable multiple system atrophy patients identified among 4 770 patients with parkinsonism enrolled in the multicentre registry of the German Competence Network on Parkinson’s disease. J Neural Transm 2007; 114: 1161-1165