Neurocognitive impairment in Whipple disease with central nervous system involvement.

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.

Progressive dementia associated with ataxia or obesity in patients with Tropheryma whipplei encephalitis.

BACKGROUND: Tropheryma whipplei, the agent of Whipple's disease, causes localised infections in the absence of histological digestive involvement. Our objective is to describe T. whipplei encephalitis. METHODS: We first diagnosed a patient presenting dementia and obesity whose brain biopsy and cerebrospinal fluid specimens contained T. whipplei DNA and who responded dramatically to antibiotic treatment. We subsequently tested cerebrospinal fluid specimens and brain biopsies sent to our laboratory using T. whipplei PCR assays. PAS-staining and T. whipplei immunohistochemistry were also performed on brain biopsies. Analysis was conducted for 824 cerebrospinal fluid specimens and 16 brain biopsies. RESULTS: We diagnosed seven patients with T. whipplei encephalitis who demonstrated no digestive involvement. Detailed clinical histories were available for 5 of them. Regular PCR that targeted a monocopy sequence, PAS-staining and immunohistochemistry were negative; however, several highly sensitive and specific PCR assays targeting a repeated sequence were positive. Cognitive impairments and ataxia were the most common neurologic manifestations. Weight gain was paradoxically observed for 2 patients. The patients' responses to the antibiotic treatment were dramatic and included weight loss in the obese patients. CONCLUSIONS: We describe a new clinical condition in patients with dementia and obesity or ataxia linked to T. whipplei that may be cured with antibiotics.

Whipple disease.

Whipple disease (WD) is a rare disease caused by Tropheryma whipplei. The classic profile of the patient is that of a middle-aged man presenting with fever, chronic diarrhea, and arthralgias. Extragastrointestinal manifestations are not rare. A high degree of clinical suspicion for the disease is needed in atypical cases. Trimethoprim-sulfamethoxazole is the treatment of choice. We present two patients with WD. The first presented with melena and generalized hyperpigmentation. The second had depression for two years before the typical symptoms. Both hyperpigmentation and long-lasting depression without the typical manifestations of the disease are rare. Histologic examination of tissue biopsies was diagnostic for WD. Both patients were treated successfully with trimethoprim-sulfamethoxazole.

Enfermedad de Whipple: una infrecuente causa bacteriana de demencia / Whipple’s Disease: an uncommon cause of dementia

Presentamos un caso de demencia diagnosticada como Alzheimer, cuya evolución condujo al diagnóstico final de una enfermedad de Whipple cerebral (AU)

We present a dementia case, diagnosed as Alzheimer type. Nevertheless, the final diagnosis of Whipple’s disease was delayed (AU)

A systematic literature review of psychological factors and the development of late whiplash syndrome.

This systematic literature review aims to assess the prognostic value of psychological factors in the development of late whiplash syndrome (LWS). We included prospective cohort studies that provided a baseline measure of at least one psychological variable and used outcome measures relating to LWS (i.e. pain or disability persisting 6 months post injury). A search of electronic databases (Pubmed, Medline, Cinahl, Embase and Psychinfo) up to August 2006 was done using a predetermined search strategy. Methodological quality was assessed independently by two assessors. Data extraction were carried out using a standardised data extraction form. Twenty-five articles representing data from 17 cohorts were included. Fourteen articles were rated as low quality with 11 rated as adequate quality. Meta-analysis was not undertaken due to the heterogeneity of prognostic factors, outcome measures and methods used. Results were tabulated and predefined criterion applied to rate the overall strength of evidence for associations between psychological factors and LWS. Data on 21 possible psychological risk factors were included. The majority of findings were inconclusive. Limited evidence was found to support an association between lower self-efficacy and greater post-traumatic stress with the development of LWS. No association was found between the development of LWS and personality traits, general psychological distress, wellbeing, social support, life control and psychosocial work factors. The lack of conclusive findings and poor methodological quality of the studies reviewed highlights the need for better quality research. Self-efficacy and post-traumatic distress may be associated with the development of LWS but this needs further investigation.

[Clinical case of the month. Whipple's disease: inaugural psychiatric symptoms]. / Le cas clinique du mois. Maladie de Whipple: tableau psychiatrique inaugural.

First considered to be a metabolic illness of the gastro-intestinal tract, Whipple's disease has progressively been recognized as a systemic bacterial disorder due to Tropheryma Whippelii and responding to antibiotics. Neurological symptoms occur in 10 to 20% of cases either initially or during the disease course. Involvement of the central nervous system alone is rare. Modern molecular biology methods, i.e. PCR amplification of 16S rRNA can be applied to accessible biological fluids and/or tissue allowing an early diagnosis, which can lead to earlier administration of adequate antibiotics as well as to a more accurate monitoring of disease evolution. We report a patient suffering from Whipple's disease and presenting initially with severe mental and behavioral disturbances which were thought to be due to a psychiatric disorder.

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.