Objective: To describe a relapse of Whipple disease revealed by isolated limbic encephalitis with no other signs of systemic involvement.
Design: Case report.
Setting: University Hospital of Strasbourg, Strasbourg, France. Patient A 41-year-old patient.
Main outcome measures: Cognitive functions and results of cerebrospinal fluid analysis and brain magnetic resonance imaging.
Results: A 41-year-old patient was hospitalized for headache associated with anterograde amnesia and temporospatial disorientation. Whipple disease with systemic manifestations was diagnosed 4 years previously and insufficiently treated. The neuropsychological evaluation showed impaired episodic memory and executive functions. Analysis of the cerebrospinal fluid showed increased lymphocytes and a positive Tropheryma whipplei polymerase chain reaction result. Cerebral magnetic resonance imaging revealed a typical pattern of limbic encephalitis with an intense signal in the amygdalae and hippocampi. The outcome under antibiotic treatment was marked by partial improvement of the cognitive disorders, disappearance of the positive T whipplei polymerase chain reaction result in cerebrospinal fluid, and a clear decrease of inflammation on brain magnetic resonance imaging.
Conclusions: Whipple disease can present as limbic encephalitis. Few cases have been previously described in the literature. Such diagnosis is of importance because of the specific treatment.