Changes in the cell population in brain white matter in multiple system atrophy

ABSTRACT Background: M ultiple system atrophy (MSA) is a sporadic progressive neurodegenerative disorder with adult onset and unknown etiology. Clinically it is characterized by autonomic failure, cerebellar ataxia, parkinsonism, and corticospinal dysfunction in any combination and with varying severity. Objectives and Methods: T o establish the extent of involvement of the white matter in the disease, we have used stereology to quantify the total number of neurons and glial cells (oligodendrocytes, astrocytes, and microglia) in the brains from 10 MSA patients and 11 controls. Results: T he mean total number of white matter interstitial neurons in the patient brains was 0.5 × 10 9 (coefficient of variation = standard deviation/mean = 0.37), which was significantly lower than the 1.1 × 10 9 (0.41) in the control brains ( P = .001) and equal to a reduction by ∼50%. The patient brains had a significantly higher number of white matter microglia, 1.5 × 10 9 (0.47) versus 0.7 × 10 9 (0.39) microglia in the control subjects ( P = .003) and equal to an increase by ∼ 100%. There was no significant difference in mean total numbers of white matter oligodendrocytes and astrocytes between the groups. Conclusions: We found widespread microgliosis without concomitant astrogliosis in brain white matter in MSA patients and demonstrated an absence of significant oligodendrocyte degeneration. The exact role of oligodendrocytes in MSA pathogenesis, including neurodegeneration, remains to be elucidated. © 2017 International Parkinson and Movement Disorder Society