“Minimal change” multiple system atrophy with limbic-predominant α-synuclein pathology

Excerpt

Multiple system atrophy (MSA) is a progressive neurodegenerative disease characterized by autonomic failure, parkinsonism, and cerebellar ataxia [3]. The striatonigral and olivopontocerebellar systems are most vulnerable regions in MSA. A variable degree of neuronal loss and astrogliosis are observed in addition to glial cytoplasmic inclusions (GCIs). “Minimal change” or “preclinical” MSA has been reported to as an early pathologic form of MSA [2, 4‐7]. These cases have minimal neuronal loss confined to the striatonigral and/or olivopontocerebellar systems [2, 5‐7]. Some have no neuronal loss [4], but GCIs are observed throughout the brain, even in regions without neuronal loss. This finding suggests that the formation of GCI may precede neuronal loss, and both striatonigral and olivopontocerebellar systems are the brain regions affected in the earliest stage of the disease. We herein report a limbic counterpart of “minimal change” MSA with abundant α-synuclein pathology in the limbic system. …