TY - JOUR PY - 2022// TI - Pathological Appearance of a Case of Preclinical Multiple System Atrophy: A Comparison With Advanced Cases JO - Journal of neuropathology and experimental neurology A1 - Okada, Keitaro A1 - Hata, Yukiko A1 - Ichimata, Shojiro A1 - Yoshida, Koji A1 - Nishida, Naoki SP - 965 EP - 974 VL - 81 IS - 12 N2 - We aimed to investigate the frequency of multiple system atrophy (MSA) in a large number of forensic autopsies and characterize the pathological appearance of preclinical MSA. We investigated a series of 1930 brains from forensic autopsies. In addition to performing immunohistochemistry for phosphorylated α-synuclein, the levels of 3 autonomic nervous system markers (catecholaminergic, serotonergic, and cholinergic) were used to assess the peripheral nerve (heart and superior cervical ganglion) and medulla oblongata. The results were compared to those of healthy control and Parkinson disease (PD) cases. Four cases (0.21%) were identified as having MSA. Cases 1-3 were symptomatic, and Case 4 was incipient; that is, although no neuronal loss was evident, the cerebellar dentate nucleus exhibited marked grumose degeneration. Immunohistochemistry revealed a marked reduction in autonomic nervous system marker levels expressed in the medulla; this reduction was more prominent in the 3 symptomatic MSA cases than in the PD case. The opposite occurred for the peripheral nerve. Case 4 exhibited mild cholinergic nerve reduction. Two cases showed possible significant pathological changes in the heart. Grumose degeneration, few oligodendroglial cytoplasmic inclusions without neuronal loss, and less reduction of autonomic nervous tissue were more prominent in the preclinical case than in symptomatic cases.
Language: en
LA - en SN - 0022-3069 UR - http://dx.doi.org/10.1093/jnen/nlac096 ID - ref1 ER -