Neurocognitive impairment in Whipple disease with central nervous system involvement

Cognitive and behavioral neurology: official journal of the Society for Behavioral and Cognitive Neurology (Impact Factor: 0.95). 03/2014; 27(1):51-6. DOI: 10.1097/WNN.0000000000000016
Source: PubMed

ABSTRACT Young-onset dementias pose a major challenge to both clinicians and researchers. Cognitive decline may be accompanied by systemic features, leading to a diagnosis of "dementia plus" syndromes. Whipple disease is a rare systemic illness characterized by arthralgias, chronic diarrhea, weight loss, fever, and abdominal pain. Central nervous system involvement, including severe cognitive deterioration, may precede systemic manifestations, appear during the course of the disease, or even be the only symptom. We report a previously highly functional 48-year-old man whom we first suspected of having early-onset neurodegenerative dementia but then diagnosed with Whipple disease based on a detailed clinical and laboratory evaluation. Initial neuropsychological evaluation revealed marked impairment in the patient's fluid intelligence and severe cognitive deficits in his information processing speed, complex attention, memory, visuomotor and construction dexterities, problem solving, and executive functions. At neuropsychological follow-up 21 months later, his information processing speed had improved only slightly and deficits persisted in his other cognitive functions. Repeat brain magnetic resonance imaging at that time showed that he had responded to antibiotic treatment. Because Whipple disease can cause young-onset "dementia plus" syndromes that may leave patients with neurocognitive deficits even after apparently successful treatment, we recommend comprehensive neuropsychological assessment for early detection of residual and reversible cognitive processes and evaluation of treatment response.

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    ABSTRACT: 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. 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. Neurology department of a university medical center. 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. 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.
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