Abstract
The ‘Parkinsonism-Plus’, or atypical Parkinsonian syndromes, are a group of movement disorders that present with significant Parkinsonism and may initially mimic idiopathic Parkinson’s Disease (IPD). They are however a diverse group of proteinopathies and their diagnosis is important, both in terms of management and prognosis. There are general “red flags” that may herald the diagnosis, including atypical rapid progression, poor response to domaminergic medications, and very early occurrence of features that do eventually arise in IPD. Examples of the latter include frequent falls with marked postural instability, autonomic failure or dementia/hallucinations. This chapter covers pathogenesis, diagnostic features and specific management issues of the four main Parkinsonism-Plus disorders: Multiple Systems Atrophy [MSA], Progressive Supranuclear Palsy [PSP], Corticobasal Degeneration [CBD] and Dementia with Lewy Bodies [DLB]. The diagnosis remains clinical and vigilant neurological monitoring along with a strong multidisciplinary team support are the cornerstone of management.
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Suggested Reading
Armstrong MJ, et al. Criteria for the diagnosis of corticobasal degeneration. Neurology. 2013;80:496–503.
Alexander SK, et al. Validation of the new consensus criteria for the diagnosis of corticobasal degeneration. J Neurol Neurosurg Psychiatry. 2014;85:923–7.
Donaghy PD, McKeith IG. The clinical characteristics of dementia with Lewy bodies and a consideration of prodromal diagnosis. Alzheimers Res Ther. 2014;6(4):46.
Gilman S, et al. Second consensus statement on the diagnosis of multiple system atrophy. Neurology. 2008;71(9):670–6.
Golbe LI. Progressive supranuclear palsy. In: Watts RL, Koller WC, editors. Movement disorders neurological principles and practice. Pa: McGraw-Hill; 1997. p. 279–97.
Lang AE, Riley DE, Bergeron C. Cortical-basal ganglionic degeneration. In: Calne DB, editor. Neurodegenerative diseases. Philadelphia: WB Saunders; 1994. p. 877–94.
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):1–9.
McKeith IG, et al. Diagnosis and management of dementia with Lewy bodies: third report of the DLB consortium. Neurology. 2005;65:1863–72.
Rebeiz JJ, Kolodny EH, Richardson Jr EP. Corticodentatonigral degeneration with neuronal achromasia. Arch Neurol. 1968;18(1):20–33.
Respondek G, et al. The phenotypic spectrum of progressive supranuclear palsy: a retrospective multicenter study of 100 definite cases. Mov Disord. 2014;29(14):1758–66.
Wenning GK, et al. The natural history of multiple system atrophy: a prospective European cohort study. Lancet Neurol. 2013;12(3):264–74.
Wenning GK, Litvan I, Tolosa E. Milestones in atypical and secondary parkinsonisms. Mov Disord. 2011;26(6):1083–95.
Williams DR, Lees AJ. What features improve the accuracy of the clinical diagnosis of progressive supranuclear palsy-parkinsonism (PSP-P). Mov Disord. 2010;25(3):357–62.
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O’Dowd, S., Healy, D., Bradley, D. (2016). Parkinsonism-Plus Syndromes. In: Hardiman, O., Doherty, C., Elamin, M., Bede, P. (eds) Neurodegenerative Disorders. Springer, Cham. https://doi.org/10.1007/978-3-319-23309-3_10
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DOI: https://doi.org/10.1007/978-3-319-23309-3_10
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