Multiple System Atrophy With Predominant Striatonigral Degeneration and TAR DNA‐Binding Protein of 43 kDa Pathology: An Unusual Variant of Multiple System Atrophy

摘要

The pathological hallmark in MSA is oligodendrocytic glial cytoplasmic inclusions (GCIs) containing α‐synuclein, in addition to neuronal loss and astrogliosis especially involving the striatonigral and olivopontocerebellar systems. Rarely, TAR DNA‐binding protein of 43 kDa (TDP‐43), a component of ubiquitinated inclusions observed mainly in amyotrophic lateral sclerosis and frontotemporal lobar degeneration has been demonstrated in cases of MSA and, more recently, was shown to colocalize with α‐synuclein pathology in GCIs in 2 patients.