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Diagnostic challenges in multiple system atrophy

Authors Obelieniene D, Bauzaite S, Kulakiene I, Keleras E, Eitmonaite I, Rastenyte D

Received 11 July 2017

Accepted for publication 26 September 2017

Published 4 January 2018 Volume 2018:14 Pages 179—184


Checked for plagiarism Yes

Review by Single-blind

Peer reviewer comments 4

Editor who approved publication: Dr Roger Pinder

Diana Obelieniene,1 Sandra Bauzaite,1 Ilona Kulakiene,2 Evaldas Keleras,2 Indre Eitmonaite,3 Daiva Rastenyte1

1Department of Neurology, 2Department of Radiology, 3Medical Academy, Lithuanian University of Health Sciences, Kaunas, Lithuania

Abstract: Multiple system atrophy is a progressive neurodegenerative disorder that is characterized by autonomic failure, cerebellar ataxia and parkinsonism syndrome in various combinations. In spite of the presence of well-established clinical criteria for multiple system atrophy, ante-mortem diagnosis is difficult. In our case report, we present a 78-year-old female patient who presented with early progressive aphasia and severe autonomic dysfunction. Two years after appearance of the first symptoms, she fulfilled all the major criteria for probable multiple system atrophy with rapid progression. In addition, brain magnetic resonance imaging and fluorodeoxyglucose-positron emission tomography findings were more typical for progressive supranuclear palsy. Clinically differentiating multiple system atrophy from progressive supranuclear palsy and other similar neurodegenerative disorders may be challenging in all stages of the disease, especially with atypical disease presentation.

Keywords: multiple system atrophy, progressive aphasia, dementia, neurodegenerative diseases, progressive supranuclear palsy, FDG-PET, MRI, MRA

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