title: Clinicopathological studies of peripheral neuropathy in Churg-Strauss syndrome.
abstract: Clinicopathological studies were performed on the visceral organs and the sural nerve of a male patient with Churg-Strauss syndrome (CSS) in order to understand the mechanisms of peripheral nervous system damage. A 67-year-old man, with a 2-year history of bronchial asthma, developed acutely painful paraplegia and dyspnea. Laboratory data showed a leukocytosis, an elevated serum creatinine kinase (CK) and marked eosionophilia. Autoantibodies including p- and c-ANCA were negative. Electrophysiological studies revealed a severe sensory-motor neuropathy of multiple mononeuritis type. Steroid pulse therapy performed a day after biopsy of skin, muscle and sural nerve was effective in resolving his respiratory and neurological dysfunction but a perforation of an intestinal ulcer occurred which required surgical intervention. In the biopsied sural nerve and the surgically resected intestine and mesentery there was vasculitis with fibrinoid necrosis accompanied by numerous eosinophils and macrophages containing eosinophil cationic protein (ECP). These findings suggest that in addition to ischemic changes due to vasculitis some neurotoxic substances generated by the eosinophils may be involved in the development of neuropathy in CSS.
authors.affiliation: Department of Neurology, Teine Keijinkai Hospital, Hokkaido University, School of Medicine, Sapporo, Japan.
authors.forename: Toshiko
authors.initials: T
authors.lastname: Nagashima
authors.forename: Bingzhen
authors.initials: B
authors.lastname: Cao
authors.forename: Naoto
authors.initials: N
authors.lastname: Takeuchi
authors.forename: Takayo
authors.initials: T
authors.lastname: Chuma
authors.forename: Yukio
authors.initials: Y
authors.lastname: Mano
authors.forename: Matoko
authors.initials: M
authors.lastname: Fujimoto
authors.forename: Mitsuru
authors.initials: M
authors.lastname: Nunomura
authors.forename: Taro
authors.initials: T
authors.lastname: Oshikiri
authors.forename: Kyohsuke
authors.initials: K
authors.lastname: Miyazaki
authors.forename: Mituru
authors.initials: M
authors.lastname: Dohke
authors.forename: Nobukazu
authors.initials: N
authors.lastname: Kashimura
authors.forename: Toshiya
authors.initials: T
authors.lastname: Shinohara
authors.forename: Yasuko
authors.initials: Y
authors.lastname: Orba
authors.forename: Shin
authors.initials: S
authors.lastname: Ishizawa
authors.forename: Kazuo
authors.initials: K
authors.lastname: Nagashima
mesh_terms: D000368:Aged; D001798:Blood Proteins / Q000378:metabolism; D015267:Churg-Strauss Syndrome / Q000150:complications* / Q000378:metabolism / Q000473:pathology* / Q000503:physiopathology; D004594:Electrophysiology; D047091:Eosinophil Granule Proteins; D004804:Eosinophils / Q000378:metabolism / Q000473:pathology*; D005355:Fibrosis; D006801:Humans; D007150:Immunohistochemistry; D008264:Macrophages / Q000378:metabolism / Q000473:pathology; D008297:Male; D009336:Necrosis; D009431:Neural Conduction; D010523:Peripheral Nervous System Diseases / Q000209:etiology* / Q000378:metabolism / Q000473:pathology* / Q000503:physiopathology; D012260:Ribonucleases; D013497:Sural Nerve / Q000473:pathology / Q000503:physiopathology
chemical_list: D001798:Blood Proteins; D047091:Eosinophil Granule Proteins; D012260:Ribonucleases