Lévy S, Degott C, Redondo A, Benhamou J P, Bernuau J
Fédération d'Hépato-Gastroentérologie et INSERM U-481, Hôpital Beaujon, Clichy.
Gastroenterol Clin Biol. 2001 Jan;25(1):100-2.
Whipple's disease is a rare infectious disease with potential central nervous system manifestations and a poor prognosis. We report the case of a young woman who presented with acute intracranial hypertension associated with cholestasis which revealed Whipple's disease without digestive involvement. The diagnosis was supported by the presence of PAS-diastase positive hepatic granulomas. A long course of antibiotics resulted in complete remission of the disease without relapse. An acute neurologic syndrome associated with cholestasis should suggest Whipple's disease.
惠普尔病是一种罕见的传染病，可能出现中枢神经系统表现，预后较差。我们报告了一例年轻女性病例，该患者表现为与胆汁淤积相关的急性颅内高压，经检查发现患有惠普尔病，但无消化系统受累。PAS-淀粉酶阳性的肝脏肉芽肿支持了该诊断。长期使用抗生素导致疾病完全缓解且未复发。与胆汁淤积相关的急性神经综合征应提示惠普尔病。
