Kretzschmar H A, Berg B O, Davis R L
Acta Neuropathol. 1987;73(2):138-44. doi: 10.1007/BF00693779.
Giant axonal neuropathy (GAN) is a disease characterized by a slowly progressive neuropathy and signs of central involvement, manifested by visual impairment, corticospinal tract dysfunction, ataxia, and dementia. Pathological hallmarks of the disease include axonal swellings packed with neurofilaments in both peripheral and central nervous systems, and accumulations of intermediate filaments in Schwann cells, fibroblasts, melanocytes, endothelial, and Langerhans cells. Rosenthal fibers, sometimes appearing in masses and mimicking Alexander's disease, emerge as a conspicuous characteristic in longstanding GAN.
巨轴索神经病（GAN）是一种以缓慢进展性神经病和中枢受累体征为特征的疾病，表现为视力障碍、皮质脊髓束功能障碍、共济失调和痴呆。该疾病的病理特征包括外周和中枢神经系统中充满神经丝的轴突肿胀，以及施万细胞、成纤维细胞、黑素细胞、内皮细胞和朗格汉斯细胞中中间丝的积聚。罗森塔尔纤维有时大量出现并酷似亚历山大病，是长期GAN的一个显著特征。
