Glial cytoplasmic inclusions in neurologically normal elderly: prodromal multiple system atrophy?

Abstract:

:In this study, we used immunohistochemistry to screen for alpha-synuclein pathology in the brains of 241 individuals without clinical evidence of neurologic disease, and discovered 36 cases (15%) with incidental Lewy bodies (LBs) and one case, a 96-year-old woman (0.4%), with inclusions similar to those seen in multiple system atrophy (MSA), a non-familial neurodegenerative disorder characterized by parkinsonism, cerebellar ataxia and autonomic dysfunction and alpha-synuclein immunoreactive glial cytoplasmic inclusions (GCI). In a routine hospital autopsy series of 125 brains, we detected GCI in a neurologically normal 82-year-old man (0.8%). Both cases showed widespread GCI in the central nervous system, as well as a few neuronal cytoplasmic inclusions, but no neuronal loss or gliosis in vulnerable brain regions, including the substantia nigra, putamen, inferior olive and pontine base. Applying a recently proposed grading scale for MSA, the two cases showed pathology far below that detected in patients with clinically overt MSA, suggesting the possibility that these two individuals had preclinical MSA. The prevalence of clinically overt MSA is estimated to be about 4 per 100,000 persons (0.004%), which is far less than the frequency of GCI in this series (0.4-0.8%). Further studies are needed to determine if GCI in neurologically normal elderly represents prodromal MSA or a rare non-progressive age-related alpha-synucleinopathy.

journal_name

Acta Neuropathol

journal_title

Acta neuropathologica

authors

Fujishiro H,Ahn TB,Frigerio R,DelleDonne A,Josephs KA,Parisi JE,Eric Ahlskog J,Dickson DW

doi

10.1007/s00401-008-0398-7

subject

Has Abstract

pub_date

2008-09-01 00:00:00

pages

269-75

issue

3

eissn

0001-6322

issn

1432-0533

journal_volume

116

pub_type

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