Cognitive and behavioral abnormalities in a case of central nervous system Whipple disease

Background: Whipple disease is a rare condition characterized by migratory polyarthralgias, fever, and chronic diarrhea. A subset of patients with the disease may either initially have or eventually develop symptoms of central nervous system involvement. Design and Methods: The cognitive and behavioral functioning of a patient with central nervous system involvement from Whipple disease was studied during a 7-month period. Serial neuropsychological evaluations were used to quantify the nature of his cognitive and behavioral profile. Setting: Neurology department of a university medical center. Results: A variety of cognitive impairments were noted, most prominently in the domains of sustained attention, memory, executive function, and constructional praxis. There were striking behavioral manifestations as well, including disinhibition and confabulation. Conclusions: The case demonstrates a degree of higher-order central nervous system dysfunction rarely observed and quantified in connection with Whipple disease, and with important implications for differential diagnosis of certain neurologic conditions. We also call attention to some of the neuroanatomical correlates of this encephalopathic condition.