Summary
Parkinsonism is a clinical syndrome characterized by bradykinesia, hypo-/akinesia, muscular rigidity, and resting tremor, mainly caused by Parkinson’s disease (PD). Progressive loss of nigral neurons with Lewy bodies is considered an essential neuropathological feature. Recent studies, however, indicate that nigral degeneration is only a part of this synucleinopathy, and clinical symptoms go far beyond motor parkinsonism. Olfactory disturbances, autonomic dysfunction, pain, sleep fragmentation, depression, and dementia with or without psychosis are frequently seen. The variability in the expression of these signs and symptoms suggests multiple causes and/or pathogeneses within the present diagnostic disease entity. In this article, a recently proposed staging of PD-related brain pathology will be correlated with the various clinical expressions. It will be argued that the specific topographical sequence of the pathology, depending on the extent and progression of the degenerative process at defined sites, may explain the individually variable expression of this disease.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
References
Amino T, Orimo S, Itoh Y, Takahashi A, Uchihara T, Mizusawa H (2005) Profound cardiac sympathetic denervation occurs in Parkinson disease. Brain Pathol 15: 29–34
Arnulf I, Konofal E, Merino-Andreu M, Houeto JL, Mesnage V, Welter ML, Lacomblenz L, Golmard JL, Derenne JP, Agid Y (2002) Parkinson’s disease and sleepiness: an integral part of PD. Neurology 58: 1019–1024
Berendse HW, Booij J, Francot Ch, Bergmans PLM, Hijman R, Stoof JC, Wolters ECh (2001) Subclinical dopaminergic dysfunction in asymptomatic Parkinson’s disease patients’ relatives with a decreased sense of smell. Ann Neurol 50: 34–41
Bonuccelli U, Lucetti C, Del Dotto P, Ceravolo R, Gambaccini G, Bernardini S, Rossi G, Piaggesi A (2003) Orthostatic hypotension in de novo Parkinson disease. Arch Neurol 60: 1400–1404
Booij J, Tissingh G, Winogrodzka A, van Royen EA et al. (1999) Imaging of the dopaminergic neurotransmission system using single-photon emission tomography and positron emission tomography in patients with parkinsonism. Eur J Nucl Med 26: 171–182
Bosboom H, Stoffers D, Wolters ECh (2004) Cognitive function in Parkinson’s disease. J Neural Transm 111: 1303–1315
Braak H, Braak E (2000) Pathoanatomy of Parkinson’s disease. J Neurol 247[Suppl 2]: S3–S10
Braak H, Del Tredici K, Rueb U, de Vos RAI, Jansen Steur ENH, Braak E (2003) Staging of brain pathology related to sporadic Parkinson’s disease. Neurobiol Aging 24: 197–211
Braak H, Rub U, Gai WP, Del Tredici K (2003) Idiopathic Parkinson’s disease: possible routes by which vulnerable neuronal types may be subject to neuroinvasion by an unknown pathogen. J Neural Transm 110: 517–536
Braak H, Ghebremedhin E, Rueb U, Bratzke H, Del Tredici K (2004) Stages in the development of Parkinson’s disease-related pathology. Cell Tissue Res 318: 121–134
Braak H, Rub U, Jansen Steur EN, Del Tredici K, de Vos RA (2005) Cognitive status correlates with neuropathologic stage in Parkinson’s disease. Neurology 64: 1404–1410
Bronner G, Royter V, Korczyn AD, Giladi N (2004) Sexual dysfunction in Parkinson’s disease. J Sex Marital Ther 30: 95–105
Brown RG, Soliveri P, Jahanshahi M (1998) Executive processes in Parkinson’s disease — random number generation and response suppression. Neuropsychol 36: 1355–1362
Calne D (2005) A definition of Parkinson’s disease. J Parkinsonism Relat Disord 1: S39–S40
Chaudhuri KR, Yates L, Martinez-Martin P (2005) The Non-motor symptom complex of Parkinson’s disease: a comprehensive assessment is essential. Curr Neurol Neurosci Rep 5: 275–283
Cummings JL (2004) Dementia with lewy bodies: molecular pathogenesis and implications for classification. J Geriatr Psychiatry Neurol 17: 112–119
Del Tredici K, Rub U, De Vos RA, Bohl JR, Braak H (2002) Where does parkinson disease pathology begin in the brain? J Neuropathol Exp Neurol 61: 413–426
Dubois B, Pillon B (1997) Cognitive deficits in Parkinson’s disease. J Neurol 244: 2–8
Fantini ML, Ferini-Strambi L, Montplaisir J (2005) Idiopathic REM sleep behavior disorder: toward a better nosologic definition. Neurology 64: 780–786
Fearnley JM, Lees AJ (1991) Ageing and Parkinson’s disease: substantia nigra regional selectivity. Brain 114: 2283–2301
Giuffrida R, Vingerhoets FJ, Bogousslavsky J, Ghika J (2005) Pain in Parkinson’s disease. Rev Neurol (Paris) 161: 407–418
Gonera EG, van’t Hof M, Berger HJ, van Weel C, Horstink MW (1997) Symptoms and duration of the prodromal phase in Parkinson’s disease. Mov Disord 12: 871–876
Hague K, Lento P, Morgello S, Caro S, Kaufmann H (1997) The distribution of Lewy bodies in pure autonomic failure: autopsy findings and review of the literature. Acta Neuropathol (Berl) 94: 192–196
Hocherman S, Giladi N (1998) Visuomotor control abnormalities in patients with unilateral parkinsonism. Neurology 50: 1648–1654
Huisman E, Uylings HB, Hoogland PV (2004) A 100% increase of dopaminergic cells in the olfactory bulb may explain hyposmia in Parkinson’s disease. Mov Disord 19: 687–692
Iwanaga K, Wakabayashi K, Yoshimoto M, Tomita I, Satoh H, Takashima H, Satoh A, Seto M, Tsujihata M, Takahashi H (1999) Lewy body-type degeneration in cardiac plexus in Parkinson’s and incidental Lewy body diseases. Neurology 52: 1269–1271
Jellinger KA (1999) Post mortem studies in Parkinson’s disease-is it possible to detect brain areas for specific symptoms? J Neural Transm 56: 1–29
Kaufmann H, Nahm K, Purohit D, Wolfe D (2004) Autonomic failure as the initial presentation of Parkinson disease and dementia with Lewy bodies. Neurology 63: 953–954
Koller WC (1998) Neuroprotection for Parkinson’s disease. Ann Neurol 44[Suppl 1]: S155–S159
Larner AJ, Mathias CJ, Rossor MN (2000) Autonomic failure preceding dementia with Lewy bodies. J Neurol 247: 229–231
Lewis SJG, Foltynie T, Blackwell AD, Robbins TW, Owen AM, Barker RA (2005) Heterogeneity of Parkinson’s disease in the early clinical stages using a data driven approach. J Neurol Neurosurg Psychiatry 76: 343–348
Magerkurth C, Schnitzer R, Braune S (2005) Symptoms of autonomic failure in Parkinson’s disease: prevalence and impact on daily life. Clin Auton Res 15: 76–82
Mahieux F, Fenelon G, Flahault A, Manifacier MJ, Michelet D, Boller F (1998) Neuropsychological prediction of dementia in Parkinson’s disease. J Neurol Neurosurg Psychiatry 64: 178–183
Marek K, Innis R, van Dyck C, Fussell B, Early M, Eberly S, Oakes D, Seybil J (2001) [(123)I]beta-CIT SPECT imaging assessment of the rate of Parkinson’s disease progression. Neurology 57: 2089–2094
Micieli G, Tosi P, Marcheselli S, Cavallini A (2003) Autonomic dysfunction in Parkinson’s disease. Neurol Sci 24[Suppl 1]: S32–S34
Montgomery EB, Baker KB, Lyons K, Koller W (1999) Abnormal performance on the PD test battery by asymptomatic first degree relatives. Neurology 52: 757–762
Morris RG, Downes JJ, Sahakian BJ, Evenden JL, Heald A, Robbins TW (1988) Planning and spatial working memory in Parkinson’s disease. J Neurol Neurosurg Psychiatry 51: 757–766
Morrish PK, Rakshi JS, Bailey DL, Sawle GV, Brooks DJ (1998) Measuring the rate of progression and estimating the preclinical period of Parkinson’s disease with [18F]dopa PET. J Neurol Neurosurg Psychiatry 64: 314–319
Oppenheimer DR (1980) Lateral horn cells in progressive autonomic failure. J Neurol Sci 46: 393–404
Pacchetti C, Manni R, Zangaglia R, Mancini F, Marchioni E, Tassorelli C, Terzaghi M, Ossola M, Martignoni E, Moglia A, Nappi G (2005) Relationship between hallucinations, delusions, and rapid eye movement sleep behavior disorder in Parkinson’s disease. Mov Disord, October 14 (Epub ahead of print)
Ponsen MM, Stoffers D, Booij J, van Eck-Smit BLF, Wolters ECh, Berendse HW (2004) Idiopatic hyposmia as a preclinical sign of Parkinson’s disease. Ann Neurol 56: 173–181
Przuntek H, Muller T, Riederer P (2004) Diagnostic staging of Parkinson’s disease: conceptual aspects. J Neural Transm 111: 201–216
Remy P, Doder M, Lees A, Turjanski N, Brooks D (2005) Depression in Parkinson’s disease: loss of dopamine and noradrenaline innervation in the limbic system. Brain 128: 1314–1322
Sage JI, Mark MH (1995) Drenching sweats as an off phenomenon in Parkinson’s disease: treatment and relation to plasma levodopa profile. Ann Neurol 37: 120–122
Schenck CH, Bundlie SR, Mahowald MW (1996) Delayed emergence of a parkinsonian disorder in 38% of 29 older men initially diagnosed with idiopathic REM sleep disorder behavior. Neurology 46: 388–393
Scherder E, Wolters E, Polman C, Sergeant J, Swaab D (2005) Pain in Parkinson’s disease and multiple sclerosis: its relation to the medial and lateral pain systems. Neurosci Biobehav Rev 29: 1047–1056
Schuurman AG, van den Akker M, Ensinck KTJL, Metsemakers JF, Knottnerus JA, Leentjens AF, Buntix F (2002) Increased risk of Parkinson’s disease after depression: a retrospective cohort study. Neurology 58: 1501–1504
Simard M, van Reekum R, Cohen T (2000) A review of the cognitive and behavioral symptoms in dementia with Lewy bodies. J Neuropsychiatry Clin Neurosci 12: 425–450
Singleton A, Gwinn-Hardy K, Sharabi Y, Li ST, Holmes C, Dendi R, Hardy J, Singleton A, Crawley A, Goldstein DS (2004) Association between cardiac denervation and parkinsonism caused by alphasynuclein gene triplication. Brain 127: 768–772
Soler JM, Le Portz B (2004) Bladder sphincter disorders in Parkinson’s disease Ann Urol (Paris) 38[Suppl 2]: S57–S61
Stiasny-Kolster K, Doerr Y, Moller JC, Hoffken H, Behr TM, Oertel WH, Mayer G (2005) Combination of ‘idiopathic’ REM sleep behaviour disorder and olfactory dysfunction as possible indicator for alpha-synucleinopathy demonstrated by dopamine transporter FP-CIT-SPECT. Brain 128: 126–137
Stoffers D, Berendse HW, Deijen JB, Wolters ECh (2001) Motor perseveration is an early sign in Parkinson’s disease. Neurology 57: 2111–2113
Tissingh G, Bergmans P, Booij J, Winogrodzka A, van Royen EA, Stoof JC, Wolters EC (1998) Drug-naive patients with Parkinson’s disease in Hoehn and Yahr stages I and II show a bilateral decrease in striatal dopamine transporters as revealed by [123I]beta-CIT SPECT. J Neurol 245: 14–20
Tissingh G, Booij J, Bergmans P et al. (1998) Iodine-123-N-omega-fluoropropyl-2beta-carbomethoxy-3beta-(4-iod ophenyl)tropane SPECT in healthy controls and early-stage, drug-naive Parkinson’s disease. J Nucl Med 39: 1143–1148
Tissingh G, de Waard R, Bergmans P, Drukarch B, Stoof JC, Wolters ECh (2001) Loss of olfactory discrimination in de-novo Parkinson’s disease patients. Mov Disord 16: 41–46
Uitti RJ, Baba Y, Wszolek ZK, Putzke DJ (2005) Defining the Parkinson disease phenotype: initial symptoms and baseline characteristics in a clinical cohort. J Parkinsonism Relat Disord 11: 139–145
Vanderhaeghen JJ, Perier O, Sternon JE (1970) Pathological findings in idiopathic orthostatic hypotension: its relationship with Parkinson’s disease. Arch Neurol 22: 207–214
Waxman MJ, Durfee D, Moore M, Morantz RA, Koller W (1990) Nutritional aspects and swallowing function of patients with Parkinson’s disease. Nutr Clin Pract 5: 196–199
Yu M, Roane DM, Miner CR, Fleming M, Rogers JD (2004) Dimensions of sexual dysfunction in Parkinson’s disease. Am J Geratr Psychiatry 12: 221–226
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2006 Springer-Verlag
About this paper
Cite this paper
Wolters, E.C., Braak, H. (2006). Parkinson’s disease: premotor clinico-pathological correlations. In: Riederer, P., Reichmann, H., Youdim, M.B.H., Gerlach, M. (eds) Parkinson’s Disease and Related Disorders. Journal of Neural Transmission. Supplementa, vol 70. Springer, Vienna . https://doi.org/10.1007/978-3-211-45295-0_47
Download citation
DOI: https://doi.org/10.1007/978-3-211-45295-0_47
Publisher Name: Springer, Vienna
Print ISBN: 978-3-211-28927-3
Online ISBN: 978-3-211-45295-0
eBook Packages: MedicineMedicine (R0)