A patient with cerebral Whipple's disease and a stroke-like syndrome

Abstract

The central nervous system (CNS) may be affected in up to 50% of patients with Whipple's disease and this can occur even with little or no gastrointestinal involvement. We describe a 63-year-old patient in whom CNS involvement with Whipple's disease had the clinical and imaging features of a brain infarction. Treatment with aspirin and ceftriaxone followed by trimethoprim-sulfamethoxazole resulted in a good neurological recovery and complete remission of the malabsorption syndrome. Cerebral Whipple's disease resembling a stroke syndrome has so far been reported in only two other patients and in both cases it represented the first presentation of the disease. Arterial or arteriolar fibrosis, thrombosis and thickening associated with the inflammation of adjacent brain parenchyma and leptomeninges, and cerebral vasculitis caused by the hematogenous spread of Tropheryma whippelii to the brain may all be important triggers of brain infarction in patients with Whipple's disease. Our case report highlights the important point that cerebral Whipple's disease with the features of a stroke syndrome, if recognized early and treated aggressively with antibiotics, could have a favorable course with no long-term disability sequelae.