Cases reported "Whipple Disease"

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1/38. Whipple's disease confined to the central nervous system: case report and review of the literature.

    Whipple's disease confined exclusively to the CNS without systemic involvement appears to be very rare, with only 8 cases reported in the literature. We present here a further case of primary cerebral Whipple's disease in which the neurological symptoms were seen in the absence of systemic involvement and emphasize the importance of diagnosing this treatable disease.
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2/38. 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.
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3/38. Whipple's disease with isolated central nervous system symptomatology diagnosed by molecular identification of tropheryma whippelii in peripheral blood.

    We report a new case of Whipple's disease (WD) confined to the central nervous system. The patient presented with ataxia, ophthalmoplegia, hypersomnia, hemiparesis and generalized myorhythmia. The diagnosis was confirmed by identification of specific sequences of the causal agent of WD, the actinobacteria tropheryma whippelii (TW), by PCR of dna extracted from peripheral blood. An epidemiological survey of TW in patients with dementia suggests that WD is an uncommon cause of dementia in our population. Molecular methods may allow rapid identification of TW in peripheral fluids, and non-invasive diagnosis of this disorder.
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4/38. Ocular Whipple's disease: earlier definitive diagnosis.

    PURPOSE: Whipple's disease is a rare, chronic, and multiorgan bacterial disease that predominantly involves the gut and its lymphatic drainage in middle-aged Caucasian men but may involve the eye. It is often difficult to diagnose and treat. We report results of one more polymerase chain reaction (PCR) analysis-diagnosed ocular Whipple's disease (OWD) case and the care of three new patients with ocular inflammation caused by OWD. DESIGN: Retrospective noncomparative case series. PARTICIPANTS: Three patients. methods: Diagnostic PCR was performed on a vitreous sample, medical records of three patients with OWD were reviewed, and a literature review (1907 to present) located 74 OWD cases. Analysis of published OWD cases to seek out factors that contribute to earlier, definitive diagnosis and treatment of OWD is made. MAIN OUTCOME MEASURES: Response of ocular inflammation and vision to treatment. RESULTS: One of 3 new and 4 of 74 published OWD cases reviewed were diagnosed with PCR on vitreous samples and subsequently successfully treated with antibiotics without devastating central nervous system (CNS) sequelae. CONCLUSION: Diagnostic PCR on vitreous samples and recognition of key presenting symptoms in patients with OWD allows earlier definitive diagnosis, when the disease may be more amenable to antibiotic treatment, compared with "late" CNS Whipple's disease in the cases reviewed.
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5/38. Primary diagnosis of whipple disease manifesting as lymphadenopathy: use of polymerase chain reaction for detection of tropheryma whippelii.

    whipple disease is a rare, chronic multisystem disease associated with the recently characterized organism tropheryma whippelii. Extraintestinal manifestation involving the central nervous system, heart, and joints occasionally occurs. Involvement of the abdominal lymph nodes, especially the mesenteric and periaortic nodes, is not uncommon. However, peripheral lymphadenopathy as the sole clinical manifestation of whipple disease is rare. We describe 2 patients with whipple disease whose initial manifestation was lymphadenopathy. lymph nodes from both patients showed infiltration of the sinuses by macrophages containing periodic acid-Schiff-positive, diastase-resistant, sickle-like structures. Electron microscopic evaluation confirmed the presence of rod-like organisms. dna from each sample was amplified by the polymerase chain reaction using a specific set of oligonucleotide primers developed against the 16S ribosomal rna coding sequence of T. whippelii. The histopathologic features and differential diagnosis of lipogranulomatous lymphadenopathy secondary to whipple disease, as well as use of molecular-based assays, are discussed.
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6/38. whipple disease with supranuclear ophthalmoplegia diagnosed by polymerase chain reaction of cerebrospinal fluid.

    An elderly man developed acute progressive supranuclear ophthalmoplegia and other central nervous system manifestations that suggested whipple disease. Results of small intestinal biopsy were negative but polymerase chain reaction testing of the cerebrospinal fluid confirmed the diagnosis.
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7/38. Cerebral Whipple's disease with a stroke-like presentation and cerebrovascular pathology.

    Although neurological symptoms are common in Whipple's disease, patients rarely have a purely neurological presentation and involvement restricted to the central nervous system is uncommon. A 39 year old woman presented with a meningoencephalitic illness, which responded to penicillin. Eleven months later she developed recurrent stroke-like episodes. Patchy enhancing meningeal, cortical, and subcortical lesions thought to be vascular in origin developed within nine days of the onset of symptoms. No evidence was found of a cardiovascular source of emboli, vasculitis, or thrombophilic condition. A brain biopsy showed meningoencephalitic features suspicious of Whipple's disease associated with leptomeningeal arterial fibrosis and thrombosis. dna polymerase chain reaction confirmed tropheryma whippelii in both blood and brain tissue. The neurological manifestations of cerebral Whipple's disease are varied and very rarely include stroke-like symptoms. The pathogenesis of cerebral infarction in Whipple's disease is not well established but arterial fibrosis and endocarditis complicated by embolisation have been reported. This case emphasises the importance of early brain biopsy in unusual cases of stroke and illustrates the clinical utility of polymerase chain reaction to confirm Whipple's disease.
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keywords = nervous system
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8/38. Intracerebral whipple disease: unusual location and bone destruction. Case report.

    whipple disease is a rare systemic bacterial infection characterized by migratory polyarthralgia and chronic diarrhea. In 5 to 20% of patients with whipple disease, the infection may present initially with or eventually develop symptoms related to the central nervous system (CNS). Although CNS involvement is a known feature of systemic whipple disease, intracerebral mass lesions are uncommon. Mass lesions in these cases are typically deep seated and multifocal. Corticosubcortical regions are unusual sites of CNS involvement in cases of whipple disease. In the present paper, the authors describe the first case of whipple disease to feature a single corticosubcortical solid frontoparietal mass lesion that displayed homogeneous contrast enhancement on neuroimaging and was associated with bone destruction of the calvaria. Although CNS involvement has been observed in the form of deep-seated mass lesions in cases of systemic whipple disease, unusual manifestations should be kept in mind during diagnosis and follow-up review in these patients.
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9/38. Neurologic presentation of whipple disease: report of 12 cases and review of the literature.

    We report 12 cases of whipple disease in patients with prominent neurologic symptoms, along with 122 cases of whipple disease with nervous system involvement reported in the literature. We analyzed the clinical signs and results of additional examinations in 2 groups: the first group included patients with predominantly but not exclusively neurologic signs, and the second included patients with clinically isolated neurologic presentation of the disease. whipple disease is a multisystemic infectious disease due to tropheryma whippelii that may present with prominent or isolated symptoms of either the central or the peripheral nervous system. Recent reports stress the importance of polymerase chain reaction (PCR) analysis of cerebrospinal fluid, magnetic resonance imaging (MRI) during follow-up, and prolonged antibiotic therapy with drugs able to cross the blood-brain barrier. cerebrospinal fluid should be analyzed repeatedly during follow-up, and treatment should be discontinued only when the results of PCR assay performed on cerebrospinal fluid are negative. Other examinations to be done include searching for gastrointestinal tract involvement with multiple duodenal biopsies and searching for systemic involvement with lymph node biopsies, which should be analyzed with light microscopy, electron microscopy, and PCR. When all examinations are negative, if whipple disease is suspected and a lesion is found on brain MRI, a stereotactic cerebral biopsy should be performed. Treating whipple disease with long-term trimethoprim-sulfamethoxazole is usually effective, but the use of third-generation cephalosporins in case of incomplete response deserves further attention.
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10/38. Whipple's disease with destructive arthritis, abdominal lymphadenopathy, and central nervous system involvement.

    We describe a patient with Whipple's disease who had an unusual erosive and destructive polyarthritis, massive abdominal lymphadenopathy, asymptomatic central nervous system involvement, and rare manifestations of orbital pseudotumor and orchitis with epididymitis. Taking oral therapy with trimethoprim-sulfamethoxazole he had recurrent flares of orbital pseudotumor, an episode of orchitis with epididymitis, and persistent polymerase chain reaction T. whipplei-positive cerebrospinal fluid. Resolution was achieved with a one month course of intravenous ceftriaxone and a 6 month course of azithromycin, and no relapse occurred during 24 months of followup.
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ranking = 1.25
keywords = nervous system
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