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.