title: Anti-Hu-associated paraneoplastic encephalomyelitis with esophageal small cell carcinoma.
abstract: 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.
authors.affiliation: Division of Neurology, Kobe University Graduate School of Medicine, Japan.
authors.forename: Toshihiko
authors.initials: T
authors.lastname: Shirafuji
authors.forename: Fumio
authors.initials: F
authors.lastname: Kanda
authors.forename: Kenji
authors.initials: K
authors.lastname: Sekiguchi
authors.forename: Masatsugu
authors.initials: M
authors.lastname: Higuchi
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Yokozaki
authors.forename: Keiko
authors.initials: K
authors.lastname: Tanaka
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Takahashi
authors.forename: Tatsushi
authors.initials: T
authors.lastname: Toda
mesh_terms: D000888:Antibodies, Anti-Idiotypic / Q000097:blood*; D001344:Autopsy; D001369:Axons / Q000378:metabolism / Q000473:pathology; D018288:Carcinoma, Small Cell / Q000150:complications* / Q000473:pathology; D051959:ELAV Proteins / Q000276:immunology*; D000067779:ELAV-Like Protein 4; D004938:Esophageal Neoplasms / Q000150:complications* / Q000473:pathology; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008207:Lymphatic Metastasis / Q000473:pathology; D008875:Middle Aged; D009186:Myelin Sheath / Q000378:metabolism; D020361:Paraneoplastic Syndromes, Nervous System / Q000209:etiology* / Q000276:immunology* / Q000473:pathology
chemical_list: D000888:Antibodies, Anti-Idiotypic; D051959:ELAV Proteins; D000067779:ELAV-Like Protein 4; C498010:ELAVL4 protein, human