神经系统Whipple病1例并文献复习

doi:10.6040/j.issn.1671-7554.0.2015.273

摘要/Abstract

摘要： 目的提高临床医师对Whipple病的认识和诊治水平。方法报道1例中枢神经系统Whipple 病的临床特点和诊治过程,并进行文献复习。结果患者,男,27岁。首发症状为记忆力下降、头痛。慢性腹泻5年,反复发作的双侧膝关节痛2年。病情进展中出现意识模糊、言语不清、四肢抽动、小便失禁、视力下降。腰椎穿刺脑脊液检测示:压力280 mmH₂O,有核细胞数升高,蛋白定量升高,潘氏试验阳性。脑电图示弥漫性重度异常。颅脑MRI示双侧大脑半球多发类圆形长T₂信号,病灶、相邻脑膜、小脑幕明显不均质强化。脑组织活检病理检查示脑白质疏松解离,有液化性坏死和新鲜出血,含大量过碘酸雪夫氏(PAS)染色和六胺银染色阳性的颗粒状物质。结论中枢神经系统Whipple病进展迅速,诊治困难,病死率高。详细了解病史,及时行脑组织活检有助于明确诊断,正确规范的治疗可改善患者预后。

关键词: 中枢神经系统, 治疗, Whipple病, 诊断

Abstract: Objective To improve the understanding of clinicians on the standard of diagnosis and treatment of Whipple's disease. Methods We reported a case of central nervous system Whipple's disease and analyzed its clinical characteristics, process of diagnosis and treatment. Meanwhile, relevant literatures were also reviewed. Results The patient is 27-year-old male. Memory loss and headache were the main symptoms, accompanied with chronic diarrhea for 5 years and recurrent bilateral knee pain for 2 years. During the course of the disease, the patient successively presented confusion, slurred speech, extremity convulsion, urinary incontinence and decreasing vision. Cerebrospinal fluid test showed that the pressure was 280 mmH₂O, and the number of nucleated cells and protein contents increased. Pan's test showed positive result. Electrocardiography (EEG) showed diffused severe abnormalities. Cerebral magnetic resonance imaging (MRI) revealed long T₂ signals in bilateral cerebral hemisphere, and the lesions, adjacent meninges and tentorium showed obvious irregular contrast. Brain biopsy indicated loose dissociation in cerebral white matter, accompanied with liquefaction necrosis and fresh bleeding. Positive Periodic Acid-Schiff (PAS) and methenamine silver staining revealed large amount of granular materials. Conclusion Central nervous system Whipple's disease has rapid progress and high fatality. Its diagnosis and treatment are difficult. Both detailed medical history and early decision to perform brain biopsy are important for the explicit diagnosis, while, correct and standardized treatment could improve the prognosis of patients.

Key words: Whipple's disease, Central nervous system, Treatment, Diagnosis

中图分类号:

R742

刁晓君, 陈春富. 神经系统Whipple病1例并文献复习[J]. 山东大学学报（医学版）, 2015, 53(10): 46-50.

DIAO Xiaojun, CHEN Chunfu. Central nervous system Whipple's disease: a case report and review of the literature[J]. JOURNAL OF SHANDONG UNIVERSITY (HEALTH SCIENCES), 2015, 53(10): 46-50.

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