Minimal change multiple system atrophy: an aggressive variant?

摘要: Glial cytoplasmic inclusions containing α-synuclein are the pathological hallmark of multiple system atrophy (MSA). Minimal change (MC-MSA) is an unusual MSA subtype with neuronal loss largely restricted to substantia nigra and locus coeruleus. Immunohistochemistry on selected brain regions semiquantitative assessment were performed six MC-MSA eight control cases. More seen in caudate than controls (P = 0.002), without any statistical difference glial inclusion load region. Severe was found ventrolateral medulla (P = 1.0) nucleus raphe obscurus (P = 0.4) both groups. When compared controls, three cases who had died sudden unexpected death earlier age onset (mean: 38 vs. 57.6 y, P = 0.02), a numerically shorter disease duration 5.3 8 P = 0.2) more rapid clinical progression most milestones reached within 3 y presentation, suggesting aggressive variant MSA. Another cases, unrelated concurrent diseases, 57.7 y) temporal course similar less severe gliosis medial dorsolateral subregions (P < 0.05) could be considered as unique group interrupted progression. Significant respiratory dysfunction early orthostatic hypotension observed all Our findings suggest that α-synuclein-associated oligodendroglial pathology may lead sufficient cause symptoms before overt © 2015 International Parkinson Movement Disorder Society.