title: Multiple system atrophy of the cerebellar type (MSA-C) with concomitant beta-amyloid and tau pathology.
abstract: Multiple system atrophy (MSA) is a rapidly progressive sporadic α-synucleinopathy with adult onset characterized by progressive cerebellar ataxia, basal ganglia symptoms, autonomic dysfunction and pyramidal tract signs. While full-blown dementia is considered an exclusion criterion according to Consensus Guidelines, mild cognitive deficits such as fronto-executive dysfunction have been reported in some MSA individuals. However, the underlying anatomic correlate still has to be elucidated. We here report a 74-year-old patient with a clinical diagnosis of "probable MSA of the cerebellar type (MSA-C)" who developed pronounced clinical symptoms of fronto-executive dysfunction. Neuropathologic investigations revealed (1) numerous glial cytoplasmic inclusions (GCI) in the putamen, mesencephalon and cerebellum, (2) pronounced betaamyloid pathology in the frontal lobe and (3) mild hippocampal τ-pathology. In this patient, fronto-executive dysfunction can easily be explained by frontal degeneration typical for AD. These findings challenge the concept of cognitive dysfunction as a core feature of MSA as long as concomitant pathology other than MSA has not been reliably excluded by post mortem analysis.
authors: Department of Neuropathology, Philipps-University of Marburg, Marburg, Germany.
authors: Bartosz
authors: B
authors: Bujan
authors: Markus J
authors: MJ
authors: Hofer
authors: Wolfgang H
authors: WH
authors: Oertel
authors: Axel
authors: A
authors: Pagenstecher
authors: Katrin
authors: K
authors: Bürk
mesh_terms: D000368:Aged; D016229:Amyloid beta-Peptides / Q000032:analysis* / Q000378:metabolism; D006801:Humans; D008297:Male; D019578:Multiple System Atrophy / Q000378:metabolism / Q000473:pathology*; D051844:alpha-Synuclein / Q000032:analysis / Q000096:biosynthesis; D016875:tau Proteins / Q000032:analysis* / Q000096:biosynthesis
chemical_list: D016229:Amyloid beta-Peptides; D051844:alpha-Synuclein; D016875:tau Proteins