Case report
Rapidly progressive Whipple's disease of the central nervous system

https://doi.org/10.1016/0967-5868(95)90013-6Get rights and content

Abstract

We describe a patient with rapidly progressive Whipple's disease confined to the central nervous system (CNS). The diagnosis was made pre-mortem following stereotactic and open brain biopsis and confirmed at autopsy. Despite appropriate antibiotic treatment, the disease ran a fulminant course to death after nine weeks.

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      Whipple's disease should be considered in the differential diagnosis of patients with fluctuating cognitive impairment, rapidly progressing dementias and brainstem encephalitis. On MRI, the presence of high midline T2 signal intensity involving the hypothalamus, brainstem, or mesial temporal lobes [107,89] and high T2 signal intensity alterations in a peripheral disposition in white matter [89,108], in the clinical context of symptoms suggestive of WD, should prompt the physician to test for the involvement of the CNS. The myriad, non-specific symptoms of WD make for a broad differential diagnosis, which includes autoimmune diseases, vasculitis and even tumors.

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