Cognitive and behavioral abnormalities in a case of central nervous systemWhipple 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 centra l nervous system involvement. Design and Methods: The cognitive and behavioral functioning of a patient w ith central nervous system involvement from Whipple disease was studied dur ing a 7-month period. Serial neuropsychological evaluations were used to qu antify 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 constr uctional praxis. There were striking behavioral manifestations as well, inc luding disinhibition and confabulation. Conclusions: The case demonstrates a degree of higher-order central nervous system dysfunction rarely observed and quantified in connection with Whipp le disease, and with important implications for differential diagnosis of c ertain neurologic conditions. We also call attention to some of the neuroan atomical correlates of this encephalopathic condition.