Whipple disease and central nervous system.

Pedro Abreu, Elsa Azevedo, Luís Lobo, Conceição Souto Moura, Celso Pontes


Whipple disease (WD) is a rare systemic infection caused by the bacteria Tropheryma whipplei. Slowly progressive dementia, supranuclear ophthalmoplegia, headache, myoclonus, hypothalamic dysfunction, oculomasticatory-skeletal myorythmia are characteristic manifestations when WD involves the central nervous system (CNS). Treatment of cerebral WD should be performed with antibiotics that reach the CNS for at the least one-year. We alert for the early diagnosis of WD in patients with exclusively neurological clinical pictures, sometimes with atypical or non-definitive diagnosis, because it is a potential treatable disease.

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