Objective: To describe a case of primary Whipple disease (WD) of the brain, which may manifest as an amnesic syndrome.
Materials and methods: A 46-year-old woman developed primary WD of the brain. The onset was characterized by a short-term amnesic syndrome for several months before the onset of generalized tonic-clonic seizures. Her amnesia was stable throughout her illness.
Results: Our patient had impairment of verbal and visual memory tasks with reduced learning. Her amnesic syndrome was secondary to asymmetric bilateral hippocampal atrophy with gliosis involving the mesial temporal structures-all secondary to primary WD of the brain.
Conclusions: WD may present as an amnesic syndrome and needs to be thought of as a treatable cause of cognitive dysfunction in young adults.