Whipple's disease presenting with isolated neurological symptoms

Case report

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✓ Whipple's disease is infrequently considered in the differential diagnosis of patients presenting with progressive neurological deterioration. This is in part a result of the relative rarity of this entity and in part due to the more frequent initial presentation of the disease with gastrointestinal, musculoskeletal, or cardiovascular symptoms. A case is described in which the neurological symptoms of progressive dementia and weakness were seen in the relative absence of non-neurological symptomatology. The diagnosis of Whipple's disease was made from a brain biopsy. The neuropathology of Whipple's disease of the central nervous system is described and the importance of considering it as a treatable entity in the differential diagnosis of progressive neurological deterioration, despite the absence of systemic symptomatology, is stressed.

Article Information

Address reprint requests to: Adam P. Brown, M.D., Department of Neurosurgery, Washington University School of Medicine, Campus Box 8057, 660 South Euclid Avenue, St. Louis, Missouri 63110.

© AANS, except where prohibited by US copyright law.

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Figures

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    T2-weighted magnetic resonance image demonstrating diffuse white matter changes and ventriculomegaly. The focal region of high signal intensity in the right temporal lobe (arrow) was excised at brain biopsy.

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    Left: Photomicrograph demonstrating numerous macrophages in the leptomeninges (M), and brain parenchyma. Prominent perivascular cuffing (asterisk) is evident. PAS. Right: High magnification of the area indicated by the asterisk shown left. PAS.

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    Left: Electron micrograph of the biopsy specimen revealing a macrophage around a blood vessel. Lipofuscin (L), unique membrane-bound inclusions with numerous membranous profiles (arrowheads), and transitional forms (arrows), are ubiquitous. Right: Higher magnification of the unique membranous inclusion shown left. Dense bodies (D) are present in the middle of the membranous profiles.

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