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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 mm H2O,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M RI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。
Objective To improve clinicians’ understanding and diagnosis and treatment of Whipple’s disease. Methods One case of Whipple’s disease of central nervous system was reported. The clinical features, diagnosis and treatment of Whipple’s disease were reviewed and reviewed. Results patients, male, 27 years old. The first symptom is memory loss, headache. Chronic diarrhea 5 years, recurrent bilateral knee pain for 2 years. Sickness in the progress of confusion, unclear speech, limbs twitching, urinary incontinence, vision loss. Lumbar puncture cerebrospinal fluid test showed: the pressure of 280 mm H2O, the number of nucleated cells increased, the protein increased, Pan test positive. EEG showed diffuse severe abnormalities. Cerebral M RI showed multiple round semicircular long T2 signal, lesion, adjacent meninges, cerebellar obvious inhomogeneous enhancement. Brain biopsy pathology showed leukoaraiosis dissociation, with liquefied necrosis and fresh bleeding, containing a large number of periodic acid Schiff (PAS) staining and hexamine silver staining-positive particulate matter. Conclusion The central nervous system Whipple disease progresses rapidly, it is difficult to diagnose and treat, and the mortality rate is high. A detailed understanding of medical history, timely brain biopsy helps to confirm the diagnosis, the correct and standardized treatment can improve the prognosis of patients.