Glial cytoplasmic inclusions in neurologically normal elderly: prodromal multiple system atrophy?
- 14 June 2008
- journal article
- case report
- Published by Springer Science and Business Media LLC in Acta Neuropathologica
- Vol. 116 (3), 269-275
- https://doi.org/10.1007/s00401-008-0398-7
Abstract
In this study, we used immunohistochemistry to screen for α-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 α-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 α-synucleinopathy.Keywords
This publication has 26 references indexed in Scilit:
- Multiple system atrophyThe Lancet Neurology, 2004
- Olfactory bulb in multiple system atrophyMovement Disorders, 2003
- Progression and prognosis in multiple system atrophyBrain, 2002
- Argyrophilic Grain Disease: Neuropathology, Frequency in a Dementia Brain Bank and Lack of Relationship with Apolipoprotein EBrain Pathology, 2002
- Frequency of bowel movements and the future risk of Parkinson’s diseaseNeurology, 2001
- Subclinical dopaminergic dysfunction in asymptomatic Parkinson's disease patients' relatives with a decreased sense of smellAnnals of Neurology, 2001
- Distinctive neuropathology revealed by α-synuclein antibodies in hereditary parkinsonism and dementia linked to chromosome 4pActa Neuropathologica, 2000
- Prevalence of progressive supranuclear palsy and multiple system atrophy: a cross-sectional studyThe Lancet, 1999
- Neuropathological stageing of Alzheimer-related changesActa Neuropathologica, 1991
- The relevance of the Lewy body to the pathogenesis of idiopathic Parkinson's disease.Journal of Neurology, Neurosurgery & Psychiatry, 1988