Multiple System Atrophy (MSA) is a rare, fatal neurodegenerative disorder. Its etiology and exact pathogenesis still remain poorly understood and currently no disease-modifying therapy is available to halt or slow down this detrimental neurodegenerative process. Hallmarks of the disease are α-synuclein rich glial cytoplasmic inclusions (GCIs). Neuropathologically, various degrees of striatonigral degeneration (SND) and olivopontocerebellar atrophy (OPCA) can be observed. Since the original descriptions of this multifaceted disorder, several steps forward have been made to clarify its neuropathological hallmarks and key pathophysiological mechanisms. The Austrian neuropathologist Kurt Jellinger substantially contributed to the understanding of the underlying neuropathology of this disease, to its standardized assessment and to a broad systematical clinic-pathological correlation. On the occasion of his 90th birthday, we reviewed the current state of the art in the field of MSA neuropathology, highlighting Prof. Jellinger’s substantial contribution.

多系统萎缩 (MSA) 是一种罕见的致命神经退行性疾病。它的病因和确切发病机制仍然知之甚少，目前还没有可用于阻止或减缓这种有害的神经退行性过程的疾病修饰疗法。该疾病的标志是富含 α-突触核蛋白的胶质细胞质内含物 (GCI)。神经病理学上，可以观察到不同程度的纹状体黑质变性（SND）和橄榄脑桥小脑萎缩（OPCA）。自从对这种多方面疾病的最初描述以来，已经采取了几个步骤来阐明其神经病理学特征和关键的病理生理机制。奥地利神经病理学家 Kurt Jellinger 对了解这种疾病的潜在神经病理学做出了重大贡献，其标准化评估和广泛的系统临床病理学相关性。在他 90 岁生日之际，我们回顾了 MSA 神经病理学领域的最新技术，突出了 Jellinger 教授的重大贡献。