Introduction: Multiple System Atrophy (MSA) is a progressive neurodegenerative disease that affects multiple regions of the brain. Previously known as three separate diseases, they were united by their considerable clinical and pathological overlap. The subsets being: Olivopontocerebellar Atrophy, Shy-Drager Syndrome and Striatonigral Degeneration. The unifying pathology of these diseases is the accumulation of Glial Cytoplasmic Inclusions (GCI) within oligodendrocytes, which are composed mostly of alpha-synuclein protein.
Methods: We reviewed sections from a 58-year-old female brain with noticeable autonomic failure and a clinical diagnosis of Shy-Drager Syndrome. Sections from multiple brain regions were immunostained with antibodies against alpha-synuclein, ABCA8, and Ubiquitin proteins.
Results: Results demonstrated increased ABCA8 expression within oligodendrocytes in the cerebellar white matter, putamen and pons that matched Alpha-synuclein and ubiquitin protein bearing GCI within oligodendrocytes in the same regions. To compare, a control normal brain was stained that failed to show similar expression profile within oligodendrocytes.
Conclusions: Expression of ABCA8 in MSA Shy-Drager brain is abnormal and suggests a possible role of ABC8 in the etiology of this disease.
Patient Care: Better understanding of MSA will lead to possible neurosurgical interventions to assist in symptom alleviation and treatment of this debilitating disease.
Learning Objectives: Elucidating the molecular mechanisms by which neuronal dysfunction occurs in MSA.