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