Parkinson-Syndrome: Klinische Symptomatik, Differenzialdiagnose und Verlauf

Zusammenfassung

Parkinson-Syndrome können im Rahmen des idiopathischen Parkinson-Syndroms (IPS; auch Morbus Parkinson), anderer neurodegenerativer Erkrankungen, den so genannten atypischen Parkinson-Syndromen, und der symptomatischen Parkinson-Syndrome vorkommen. Das IPS ist dabei mit ca. 80 % aller Parkinson-Syndrome die häufigste Ursache. Neurodegenerative atypische Parkinson-Syndrome sind die wichtigsten Differenzialdiagnosen des idiopathischen Parkinson-Syndroms. Die beiden häufigsten Formen sind die Multisystematrophie (MSA) und die progressive supranukleäre Blickparese (PSP). Die meisten Parkinson-Syndrome lassen sich anhand klinischer Kriterien und morphologischer Bildgebung bereits differenzialdiagnostisch einordnen. Klinisch-pathologische Studien haben jedoch gezeigt, dass bei ungefähr 25 % der Patienten mit einem Parkinson-Syndrom eine falsche Diagnose gestellt wird. Eine exakte Diagnosestellung hat jedoch vielfältige therapeutische und prognostische Implikationen. Verschiedene apparative Zusatzuntersuchungen, insbesondere funktionelle bildgebende Verfahren zur Darstellung des nigrostriatalen Systems mittels SPECT oder PET, können die diagnostische Sicherheit erhöhen. Die folgende Übersicht möchte die typischen klinischen Zeichen der verschiedenen Parkinson-Syndrome und deren klinischen Verlauf ins Blickfeld rücken und die Probleme der Früh- und Differenzialdiagnose darstellen.

Abstract

Parkinsonian syndromes include idiopathic Parkinson’s disease (IPD), other neurodegenerative diseases with parkinsonism, the so-called atypical parkinsonian syndromes, and symptomatic parkinsonian syndromes, such as Wilson’s disease. IPD is the most frequent disease with parkinsonism as the main clinical feature and is responsible for approx. 80 % of all parkinsonian syndromes. Atypical parkinsonian syndromes are the most important differential diagnoses of IPD. The two most frequent types are multiple system atrophy (MSA) and progressive supranuclear palsy (PSP). For clinical diagnosis it is essential to take a careful medical history and to examine the patients physically in regular intervals. However, various clinico-pathological studies have shown that approx. 25 % of patients with clinical diagnosis of IPD may have other causes of parkinsonism. Selected technical investigations, in particular functional imaging of the central dopaminergic system using PET or SPECT, may help to make clinical diagnosis more secure. This paper reviews the clinical features and diagnostic findings in diseases with parkinsonism and summarises the difficulties in establishing early and differential diagnoses.

Schlüsselwörter

Morbus Parkinson - atypische Parkinson-Syndrome - Differenzialdiagnose - Neuroprotektion - Neuroimaging

Key words

Parkinson’s disease - atypical parkinsonian syndromes - differential diagnosis - neuroprotection - neuroimaging

Literatur

• 1 Benamer T S, Patterson J, Grosset D G,. et al . Accurate differentiation of parkinsonism and essential tremor using visual assessment of [123I]-FP-CIT SPECT imaging: the [123I]-FP-CIT study group.  Mov Disord. 2000;  15 (3) 503-510
• 2 Brooks D J. et al . Striatal D2 receptor status in patients with Parkinson's disease, striatonigral degeneration, and progressive supranuclear palsy, measured with 11C-raclopride and positron emission tomography.  Ann Neurol. 1992;  31 184-192
• 3 De Bruin V MS, Lees A J. Subcortical neurofibrillary degeneration presenting as Steele-Richardson-Olszewski and other related syndromes: a review of 90 pathologically verified cases.  Mov Disord. 1994;  9 381-389
• 4 Foltynie T, Brayne C, Barker R A. The heterogenity of idiopathic Parkinson’s disease.  J Neurol. 2002;  249 138-145
• 5 Gilman S, Low P A, Quinn N. Consensus statement on the diagnosis of multiple system atrophy.  J Neurol Sci. 1999;  163 94-98
• 6 Heiss W D, Würker M. Möglichkeiten und Grenzen funktioneller bildgebender Verfahren beim Parkinson-Syndrom.  Nervenarzt. 1999;  70 Suppl 1 S2-S10
• 7 Hoehn M M, Yahr M D. Parkinsonism: Onset, progression and martality.  Neurology. 1967;  17 427-442
• 8 Hughes A J, Daniel S E, Kilford L, Lees A J. Accuracy of clinical diagnosis of idiopathic Parkinson’s disease: a clinico-pathological study of 100 cases.  J Neuro Neurosurg Psych. 1992;  55 181-184
• 9 Kish S J, Shannak K, Hornykiewicz O. Uneven pattern of dopamine loss in the striatum of patients with idiopathic Parkinson's disease. Pathophysiologic and clinical implications.  N Engl J Med. 1988;  318 876-880
• 10 Klockgether T. et al . The natural history of degenerative ataxia: a retrospective study in 466 patients.  Brain. 1998;  121 589-600
• 11 Kraft E. 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;  56 225-228
• 12 Lang A E, Lozano A M. Parkinson’s disease. First of two parts.  New Engl J Med. 1998;  339 1044-1053; 1130-1143
• 13 Litvan I. 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
• 14 Magalhaes M. et al . Autonomic dysfunction in pathologically confirmed multiple system atrophy and idiopathic Parkinson's disease - a retrospective comparison.  Acta Neurol Scand. 1995;  91 98-102
• 15 Marek K, Seibyl J, Shoulson I. Dopamine transporter brain imaging to assess the effects of pramipexole vs levodopa on Parkinson disease progression.  JAMA. 2002;  287 1653-1661
• 16 Morrish P K, Sawle G V, Brooks D J. Regional changes in [18F]dopa metabolism in the striatum in Parkinson's disease.  Brain. 1996;  119 2097-2103
• 17 Morrish P K, Rakshi J S, Bailey D L. et al . Measuring the rate of progression and estimating the preclinical period of Parkinson's disease with [18F]dopa PET.  J Neurol Neurosurg Psych. 1998;  64 314-319
• 18 Rajput A H, Rozdilsky B, Rajput A. Accuracy of clinical diagnosis in parkinsonism - a prospective study.  Can J Neurol Sci. 1991;  18 275-278
• 19 Rohl A, Friedrich H J, Ulm G, Vieregge P. The relevance and clinical subtypes for disease course, family history and epidemiological variables in Parkinson’s disease.  Eur J Neurol. 1994;  1 65-72
• 20 Savoiardo M. et al . Magnetic resonance imaging in progressive supranuclear palsy and other parkinsonian disorders.  J Neural Trans. 1994;  42 Suppl 93-110
• 21 Schrag A, Ben-Shlomo Y, Quinn N P. Prevalence of progressive supranuclear palsy and multiple system atrophy: a cross-sectional study.  Lancet. 1999;  354 1771-1775
• 22 Schulz J B. et al . Multiple system atrophy: natural history, MRI morphology, and dopamine receptor imaging with 123IBZM-SPECT.  J Neurol Neurosurg Psychiatry. 1994;  57 1047-1056
• 23 Schwarz J, Linke R, Kerner M. et al . Striatal dopamine transporter binding assessed by [I-123]IPT and single photon emission computed tomography in patients with early Parkinson’s disease: Implications for a preclinical diagnosis.  Arch Neurol. 2000;  57 205-208
• 24 Shoulson I. Experimental therapeutics of neurodegenerative disorders: unmet needs.  Science. 1998;  282 1072-1074
• 25 Storch A, Burkhardt K, Ludolph A C, Schwarz J. Protective effects of riluzole on dopamine neurons: Involvement of oxidative stress and cellular energy metabolism.  J Neurochem. 2000;  75 2259-2269
• 26 Wenning G, Ben-Sholmo Y, Magalhaes M. et al . Clinical features and natural history of multiple system atrophy. An analysis of 100 patients.  Brain. 1994;  117 835-845
• 27 Wenning G K, Pramstaller P P, Ransmyr G, Poewe W. Atypische Parkinson-Syndrome.  Nervenarzt. 1997;  68 102-115
• 28 Whone A L, Remy P, Davis M R. et al . The REAL-PET Study: slower progression in early Parkinson's disease treated with ropinirole compared with L-dopa.  Neurology. 2002;  59 (Suppl 3) A56