Atypische Parkinson-Syndrome – Aktuelles aus Diagnostik und Therapie

Zusammenfassung

Der Begriff „atypische Parkinson-Syndrome“ umfasst die Multisystematrophie (MSA), die progressive supranukleäre Blickparese (PSP) und das kortikobasale Syndrom (CBS). Eine Differenzierung dieser verschiedenen Krankheitsentitäten von der „klassischen“ Parkinson-Krankheit stellt weiterhin selbst für den Spezialisten eine Herausforderung dar, da abhängig von der genauen Diagnose große Unterschiede sowohl im Ansprechen auf die Therapie als auch deren Prognose bestehen. Ziel dieser Arbeit soll sein, zum einen eine Übersicht zu den aktuellen Diagnosekriterien und pragmatisch-therapeutischen Ansätzen zu geben als auch auf rezente Ergebnisse aus klinischen Studien und experimenteller Forschung einzugehen.

Abstract

The term “atypical Parkinson syndromes” usually encompasses the following diseases: multiple system atrophy (MSA), progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS). The differential diagnosis is still a challenge even for a movement disorders specialist, not least because of the distinct therapeutic approaches and disease prognosis. The aim of this review is to provide an overview of current diagnostic criteria and therapeutic approaches and to cite recent findings from clinical and experimental studies.

Schlüsselwörter

atypische Parkinson-Syndrome - Diagnose - Therapie

Key words

atypical Parkinson syndromes - diagnosis - therapy

• Literatur

• 1 Hughes AJ, Daniel SE, Kilford L et al. Accuracy of clinical diagnosis of idiopathic Parkinson’s disease: a clinico-pathological study of 100 cases. Journal of neurology, neurosurgery, and psychiatry 1992; 55: 181-184
• 2 Hughes AJ, Daniel SE, Ben-Shlomo Y et al. The accuracy of diagnosis of parkinsonian syndromes in a specialist movement disorder service. Brain 2002; 125: 861-870
• 3 Schrag A, Ben-Shlomo Y, Quinn NP. Prevalence of progressive supranuclear palsy and multiple system atrophy: a cross-sectional study. Lancet 1999; 354: 1771-1775
• 4 Stefanova N, Reindl M, Neumann M et al. Microglial activation mediates neurodegeneration related to oligodendroglial alpha-synucleinopathy: implications for multiple system atrophy. Mov Disord 2007; 22: 2196-2203
• 5 Scholz SW, Houlden H, Schulte C et al. SNCA variants are associated with increased risk for multiple system atrophy. Annals of neurology 2009; 65: 610-614
• 6 Lee JY, Lee WW, Kim JS et al. Perverted head-shaking and positional downbeat nystagmus in patients with multiple system atrophy. Mov Disord 2009; 24: 1290-1295
• 7 Gilman S, Wenning GK, Low PA et al. Second consensus statement on the diagnosis of multiple system atrophy. Neurology 2008; 71: 670-676
• 8 Lipp A, Sandroni P, Ahlskog JE et al. Prospective differentiation of multiple system atrophy from Parkinson disease, with and without autonomic failure. Arch Neurol 2009; 66: 742-750
• 9 Mathias CJ. Multiple system atrophy and autonomic failure. J Neural Transm Suppl 2006; 70: 343-347
• 10 Benarroch EE, Schmeichel AM, Sandroni P et al. Differential involvement of hypothalamic vasopressin neurons in multiple system atrophy. Brain 2006; 129: 2688-2696
• 11 Kao AW, Racine CA, Quitania LC et al. Cognitive and neuropsychiatric profile of the synucleinopathies: Parkinson disease, dementia with Lewy bodies, and multiple system atrophy. Alzheimer disease and associated disorders 2009; 23: 365-370
• 12 Balas M, Balash Y, Giladi N et al. Cognition in multiple system atrophy: neuropsychological profile and interaction with mood. J Neural Transm 2010; 117: 369-375
• 13 Lipp A, Sandroni P, Low PA. Systemic postganglionic adrenergic studies do not distinguish parkinson’s disease from multiple system atrophy. Journal of the neurological sciences 2009; 281: 15-19
• 14 Wenning GK, Stefanova N, Jellinger KA et al. Multiple system atrophy: a primary oligodendrogliopathy. Annals of neurology 2008; 64: 239-246
• 15 Quinn N. Multiple system atrophy – the nature of the beast. J Neurol Neurosurg Psychiatry 1989; Suppl: 78-89
• 16 Seppi K, Schocke MF, Mair KJ et al. Progression of putaminal degeneration in multiple system atrophy: a serial diffusion MR study. Neuro Image 2006; 31: 240-245
• 17 Brooks DJ, Seppi K. Proposed neuroimaging criteria for the diagnosis of multiple system atrophy. Mov Disord 2009; 24: 949-964
• 18 Kollensperger 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
• 19 Mahlknecht P, Schocke M, Seppi K. Differential diagnosis of parkinsonian syndromes using MRI. Der Nervenarzt 2010; 81: 1168-1179
• 20 Gilman S. Functional imaging with positron emission tomography in multiple system atrophy. J Neural Transm 2005; 112: 1647-1655
• 21 Wenning GK, Ben ShlomoY, Magalhaes M et al. Clinical features and natural history of multiple system atrophy. An analysis of 100 cases. Brain 1994; 117: 835-845
• 22 Wenning GK, Geser F, Poewe W. Therapeutic strategies in multiple system atrophy. Mov Disord 2005; 20 (Suppl. 12) S67-S76
• 23 Wenning GK, Colosimo C, Geser F et al. Multiple system atrophy. Lancet neurology 2004; 3: 93-103
• 24 Winge K, Fowler CJ. Bladder dysfunction in Parkinsonism: mechanisms, prevalence, symptoms, and management. Mov Disord 2006; 21: 737-745
• 25 Beck RO, Betts CD, Fowler CJ. Genitourinary dysfunction in multiple system atrophy: clinical features and treatment in 62 cases. The Journal of urology 1994; 151: 1336-1341
• 26 Stefanova N, Tison F, Reindl M et al. Animal models of multiple system atrophy. Trends in neurosciences 2005; 28: 501-506
• 27 Holmberg B, Johansson JO, Poewe W et al. Safety and tolerability of growth hormone therapy in multiple system atrophy: a double-blind, placebo-controlled study. Mov Disord 2007; 22: 1138-1144
• 28 Dodel R, Spottke A, Gerhard A et al. Minocycline 1-year therapy in multiple-system-atrophy: effect on clinical symptoms and [(11)C] (R)-PK11195 PET (MEMSA-trial). Mov Disord 2010; 25: 97-107
• 29 Bensimon G, Ludolph A, Agid Y et al. Riluzole treatment, survival and diagnostic criteria in Parkinson plus disorders: the NNIPPS study. Brain 2009; 132: 156-171
• 30 Lee PH, Kim JW, Bang OY et al. Autologous mesenchymal stem cell therapy delays the progression of neurological deficits in patients with multiple system atrophy. Clinical pharmacology and therapeutics 2008; 83: 723-730
• 31 Quinn N, Barker RA, Wenning GK. Are trials of intravascular infusions of autologous mesenchymal stem cells in patients with multiple system atrophy currently justified, and are they effective?. Clinical pharmacology and therapeutics 2008; 83: 663-665
• 32 Mathias CJ, Senard JM, Braune S et al. L-threo-dihydroxyphenylserine (L-threo-DOPS; droxidopa) in the management of neurogenic orthostatic hypotension: a multi-national, multi-center, dose-ranging study in multiple system atrophy and pure autonomic failure. Clin Auton Res 2001; 11: 235-242
• 33 Vidailhet M, Rivaud S, Gouider-Khouja N et al. Eye movements in parkinsonian syndromes. Annals of neurology 1994; 35: 420-426
• 34 Litvan I, Agid Y, Jankovic J et al. Accuracy of clinical criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome). Neurology 1996; 46: 922-930
• 35 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
• 36 Williams DR, Holton JL, Strand K et al. Pure akinesia with gait freezing: a third clinical phenotype of progressive supranuclear palsy. Mov Disord 2007; 22: 2235-2241
• 37 Ling H, O’Sullivan SS, Holton JL et al. Does corticobasal degeneration exist? A clinicopathological re-evaluation. Brain 133: 2045-2057
• 38 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
• 39 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
• 40 Stamelou M, de Silva R, Arias-Carrion O et al. Rational therapeutic approaches to progressive supranuclear palsy. Brain 2010; 133: 1578-1590
• 41 Di Maria E, Tabaton M, Vigo T et al. Corticobasal degeneration shares a common genetic background with progressive supranuclear palsy. Annals of neurology 2000; 47: 374-377
• 42 Wenning GK, Litvan I, Jankovic J et al. Natural history and survival of 14 patients with corticobasal degeneration confirmed at postmortem examination. Journal of neurology, neurosurgery, and psychiatry 1998; 64: 184-189
• 43 Litvan I, Grimes DA, Lang AE. Phenotypes and prognosis: clinicopathologic studies of corticobasal degeneration. Advances in neurology 2000; 82: 183-196
• 44 Kompoliti K, Goetz CG, Boeve BF et al. Clinical presentation and pharmacological therapy in corticobasal degeneration. Archives of neurology 1998; 55: 957-961
• 45 Boeve BF, Josephs KA, Drubach DA. Current and future management of the corticobasal syndrome and corticobasal degeneration. Handb Clin Neurol 2008; 89: 533-548
• 46 Cotter C, Armytage T, Crimmins D. The use of zolpidem in the treatment of progressive supranuclear palsy. Clin Neurosci 2010; 17: 385-386
• 47 Takatsu H, Nagashirna K, Murase M et al. Differentiating Parkinson disease from multiple-system atrophy by measuring cardiac iodine-123 metaiodobenzylguanidine accumulation. JAMA 2000; 284: 44-45
• 48 Juh R, Pae CU, Kim TS et al. Cerebral glucose metabolism in corticobasal degeneration comparison with progressive supranuclear palsy using statistical mapping analysis. Neurosci Lett 2005; 383: 22-27
• 49 Köllensperger M, Geser F, Seppi K et al. Red flags for multiple system atrophy. Mov Disord 2008; 23: 1093-1099
• 50 Steele JC, Richardson JC, Olszewski J. Progressive supranuclear palsy. A heterogenous degeneration involving the brain stem, basal ganglia and cerebellum with vertical gaze and pseudobulbar palsy, nuchal dystonia and dementia. Arch Neurol 1964; 10: 333-359
• 51 Wenning GK, Ben-Shlomo Y, Hughes A et al. . What clinical features are most useful to distinguish definite multiple system atrophy from Parkinson's disease?. J Neurol Neurosurg Psychiatry 2000; 68: 434-440
• 52 Wenning GK, Tison F, Ben-Shlomo Y et al. Multiple system atrophy: a review of 203 pathologically proven cases. Mov Disord 1997; 12: 133-147