Diagnostic Approach to Atypical Parkinsonian Syndromes

Nikolaus R. McFarland, MD, PhD Movement Disorders p. 1117-1142 August 2016, Vol.22, No.4 doi: 10.1212/CON.0000000000000348
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Purpose of Review: Although increasingly recognized, atypical parkinsonian syndromes remain challenging to diagnose and are underrecognized due to overlap with other parkinsonisms. This article provides a diagnostic approach to atypical parkinsonian syndromes, including progressive supranuclear palsy (PSP), multiple system atrophy (MSA), corticobasal degeneration (CBD), and dementia with Lewy bodies. The goal of this review is to aid the clinician in recognizing key clinical and pathologic features and to raise awareness of recent advances in diagnostics and treatment.

Recent Findings: Diagnostic criteria for atypical parkinsonian syndromes are evolving to encompass increasingly recognized heterogeneity in the presentation of these disorders and information gleamed from clinicopathologic correlations. PSP and CBD in particular now share similar pathologic clinical features and include a number of phenotypic variants. Pathologic diagnoses are increasingly used in clinical practice, and there is frequent reference now by clinicians to tauopathies, including PSP and CBD, and the synucleinopathies, which include MSA and dementia with Lewy bodies (as well as Parkinson disease). Research into biomarkers, including both tissue and imaging modalities and genetics, has the potential to increase disease recognition and make earlier diagnosis and treatment possible. Although novel therapeutics are being studied for atypical parkinsonian syndromes such as PSP, no new breakthrough interventions have emerged for the treatment of PSP, CBD, and MSA. Current therapeutic management for these disorders frequently uses a multidisciplinary team approach.

Summary: The approach to atypical parkinsonian syndromes requires recognition of a constellation of overlapping but distinct clinical features that help with identifying and distinguishing them from Parkinson disease and other similar disorders.

Address correspondence to Dr Nikolaus R. McFarland, 1149 South Newell Dr, L3-100, PO Box 100236, Gainesville, FL 32610, nikolaus.mcfarland@neurology.ufl.edu.

Relationship Disclosure: Dr McFarland is supported by a career development grant from the National Institutes of Health/National Institute of Neurological Disorders and Stroke (K09 NS067024) and the Michael J. Fox Foundation.

Unlabeled Use of Products/Investigational Use Disclosure: Dr McFarland reports no disclosure.

Supplemental digital content: Videos accompanying this article are cited in the text as Supplemental Digital Content. Videos may be accessed by clicking on links provided in the HTML, PDF, and app versions of this article; the URLs are provided in the print version. Video legends begin on page 1139.

Copyright © 2016 by the American Academy of Neurology.