{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,2]],"date-time":"2026-09-02T17:07:58Z","timestamp":1788368878951,"version":"build-2803163510"},"reference-count":93,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2017,9,28]],"date-time":"2017-09-28T00:00:00Z","timestamp":1506556800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2018,1]]},"abstract":"<jats:p>The differential diagnosis of atypical parkinsonian syndromes is challenging. These severe and often rapidly progressive neurodegenerative disorders are clinically heterogeneous and show significant phenotypic overlap. Here, clinical, imaging, neuropathological and genetic features of multiple system atrophy, progressive supranuclear palsy, corticobasal degeneration and frontotemporal lobar degeneration (<jats:styled-content style=\"fixed-case\">FTLD<\/jats:styled-content>) are reviewed. The terms corticobasal degeneration and <jats:styled-content style=\"fixed-case\">FTLD<\/jats:styled-content> refer to pathologically confirmed cases of corticobasal syndrome and frontotemporal dementia (<jats:styled-content style=\"fixed-case\">FTD<\/jats:styled-content>). <jats:styled-content style=\"fixed-case\">Frontotemporal lobar degeneration<\/jats:styled-content> clinically presents as the behavioral variant <jats:styled-content style=\"fixed-case\">FTD<\/jats:styled-content>, semantic variant primary progressive aphasia (<jats:styled-content style=\"fixed-case\">PPA<\/jats:styled-content>), non\u2010fluent agrammatic variant <jats:styled-content style=\"fixed-case\">PPA<\/jats:styled-content>, logopenic variant <jats:styled-content style=\"fixed-case\">PPA<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">FTD<\/jats:styled-content> associated with motor neuron disease. While progressive supranuclear palsy and corticobasal syndrome have been called Parkinson\u2010plus syndromes in the past, they are now classified as <jats:styled-content style=\"fixed-case\">FTD<\/jats:styled-content>\u2010related disorders, reflecting that they pathologically differ from \u03b1\u2010synucleinopathies like multiple system atrophy and Parkinson disease. The contribution of genetic factors to atypical parkinsonian syndromes is increasingly recognized. Genes involved in the etiology of <jats:styled-content style=\"fixed-case\">FTLD<\/jats:styled-content> include <jats:italic><jats:styled-content style=\"fixed-case\">MAPT<\/jats:styled-content><\/jats:italic>,<jats:italic> <jats:styled-content style=\"fixed-case\">GRN<\/jats:styled-content><\/jats:italic> and <jats:italic>C9orf72<\/jats:italic>. Novel neuroimaging techniques, including tau positron emission tomography imaging, are being investigated. Multimodal magnetic resonance imaging approaches and automated magnetic resonance imaging volume segmentation techniques are being evaluated for optimized differential diagnosis. Current treatment options are symptomatic, and disease modifying therapies are under active investigation.<\/jats:p>","DOI":"10.1111\/ene.13412","type":"journal-article","created":{"date-parts":[[2017,8,13]],"date-time":"2017-08-13T04:06:35Z","timestamp":1502597195000},"page":"41-58","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":71,"title":["Atypical parkinsonian syndromes: a general neurologist's perspective"],"prefix":"10.1111","volume":"25","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4071-9090","authenticated-orcid":false,"given":"A. 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