Frontotemporal lobar degeneration‐TDP with ‘multiple system atrophy phenocopy syndrome’

Abstract

Multiple system atrophy (MSA) is a neurodegenerative disorder presenting with parkinsonism, cerebellar involvement, autonomic dysfunction and pyramidal signs (1). Two main clinical subtypes of MSA are recognized: a parkinsonian-type (MSA-P) associated with predominant nigrostriatal degeneration and a cerebellar-type (MSA-C) with predominant olivopontocerebellar atrophy. A ‘definite’ diagnosis requires pathological confirmation with demonstration of glial cytoplasmic inclusions comprising alpha-synuclein protein aggregates (1). Here we present a patient with an MSA-P clinical phenotype and a novel pathological profile of frontotemporal lobar degeneration (FTLD). This article is protected by copyright. All rights reserved.