Shirafuji Toshihiko, Kanda Fumio, Sekiguchi Kenji, Higuchi Masatsugu, Yokozaki Hiroshi, Tanaka Keiko, Takahashi Hitoshi, Toda Tatsushi
Division of Neurology, Kobe University Graduate School of Medicine, Japan.
Intern Med. 2012;51(17):2423-7. doi: 10.2169/internalmedicine.51.6884. Epub 2012 Sep 1.
A 63-year-old woman had anti-Hu-associated paraneoplastic encephalomyelitis (anti-Hu syndrome) caused by esophageal small cell carcinoma (SCC). The patient developed bilateral limbic encephalitis, followed by myelitis, brain stem encephalitis, and autonomic failure. Extensive examination demonstrated SCC of the abdominal lymph nodes that was retrospectively diagnosed as metastasis of esophageal SCC on autopsy. The neuropathological findings were characterized by widespread neuronal loss and gliosis in the central nervous system, as well as patchy loss of myelin and axons in the spinal nerve roots with perivascular lymphocytic infiltration. This is the first detailed clinical and neuropathological report of anti-Hu syndrome caused by esophageal SCC.
一名63岁女性因食管小细胞癌（SCC）引发抗Hu相关副肿瘤性脑脊髓炎（抗Hu综合征）。患者先是出现双侧边缘叶脑炎，随后发生脊髓炎、脑干脑炎及自主神经功能衰竭。全面检查发现腹部淋巴结有SCC，尸检时回顾性诊断为食管SCC转移。神经病理学检查结果的特征为中枢神经系统广泛神经元丢失和胶质细胞增生，以及脊神经根节段性脱髓鞘和轴突丢失伴血管周围淋巴细胞浸润。这是首例关于食管SCC所致抗Hu综合征的详细临床及神经病理学报告。
