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      Tomografía axial computarizada y resonancia magnética en el diagnóstico de la enfermedad de Whipple con afectación del sistema nervioso central Translated title: Diagnostic features of central nervous system involvement in Whipple’s disease by magnetic resonance imaging and computed tomography

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          Abstract

          Se presenta un paciente con enfermedad de Whipple y síntomas exclusivamente neurológicos, entre los que destacaron alteraciones cognoscitivas, cuadro comicial, movimientos oculares anormales y síndrome piramidal. En las técnicas de imagen (TAC y RM) se observaron en diversas regiones cerebrales masas que, en la RM, se realzaban con gadolinio. Se identificó además importante ependimitis, así como una lesión de volumen en la médula cervical. La PCR fue negativa y el diagnóstico se confirmó con microscopía electrónica. Lo más notable de este caso fue la excelente respuesta a la antibioterapia.

          Translated abstract

          We report a patient diagnosed with Whipple’s disease who presented only with neurological symptoms including memory loss and confusion, seizures, abnormal ocular movements and a pyramidal syndrome. Neuro-imaging (CT and MRI) showed masses located in several areas of the brain which enhanced after gadolinium. There was also severe ependymitis, and a mass in the cervical cord. PCR in CSF was negative and the diagnosis was confirmed with electron microscopy. The patient had a dramatic response to an appropriate course of antibiotics.

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          Most cited references37

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          Identification of the uncultured bacillus of Whipple's disease.

          Whipple's disease is a systemic disorder known for 85 years to be associated with an uncultured, and therefore unidentified, bacillus. We used a molecular genetic approach to identify this organism. The bacterial 16S ribosomal RNA (rRNA) sequence was amplified directly from tissues of five unrelated patients with Whipple's disease by means of the polymerase chain reaction, first with broad-range primers and then with specific primers. We determined and analyzed the nucleotide sequence of the amplification products. A unique 1321-base bacterial 16S rRNA sequence was amplified from duodenal tissue of one patient. This sequence indicated the presence of a previously uncharacterized organism. We then detected this sequence in tissues from all 5 patients with Whipple's disease, but in none of those from 10 patients without the disorder. According to phylogenetic analysis, this bacterium is a gram-positive actinomycete that is not closely related to any known genus. We have identified the uncultured bacillus associated with Whipple's disease. The phylogenetic relations of this bacterium, its distinct morphologic characteristics, and the unusual features of the disease are sufficient grounds for naming this bacillus Tropheryma whippelii gen. nov. sp. nov. Our findings also provide a basis for a specific diagnostic test for this organism.
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            A hitherto undescribed disease characterized anatomically by deposits of fat and fatty acids in the intestinal and mesenteric lymphatic tissues

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              Diagnostic guidelines in central nervous system Whipple's disease.

              Many cases of central nervous system (CNS) Whipple's disease are not diagnosed until postmortem. Few reviews of CNS Whipple's disease have delineated the frequencies of abnormalities on neurological examination, cerebrospinal fluid studies, neuroimaging, and intestinal biopsy studies. Guidelines for diagnosis and treatment have not been proposed. In this review we present 3 new cases of CNS Whipple's disease and summarize the literature to determine the frequencies of neurological signs and abnormalities on diagnostic testing. We propose guidelines for diagnostic screening, selection for biopsy, and treatment. Review of the 84 cases of CNS Whipple's disease (81 in the literature, 3 new) revealed that 80% of the patients had systemic signs. Cognitive changes were frequent (71%), and 47% with cognitive changes also had psychiatric signs. Oculomasticatory myorhythmia and oculo-facial-skeletal myorhythmia, pathognomic for CNS Whipple's disease, were present in 20% of patients, and were always accompanied by a supranuclear vertical gaze palsy. Tissue biopsy was a sensitive technique; 89% of those who had biopsies had positive biopsy results. Diagnosis and treatment of definite CNS Whipple's disease should be based on the presence of pathognomic signs (oculomasticatory myorhythmia or oculo-facial-skeletal myorhythmia) or positive biopsy or polymerase chain reaction results. Possible CNS Whipple's disease should be diagnosed in the setting of unexplained systemic symptoms and neurological signs (supranuclear vertical gaze palsy, rhythmic myoclonus, dementia with psychiatric symptoms, or hypothalamic manifestations). Those with possible CNS Whipple's disease should undergo small-bowel biopsy.
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                Author and article information

                Journal
                rchnp
                Revista chilena de neuro-psiquiatría
                Rev. chil. neuro-psiquiatr.
                Sociedad de Neurología, Psiquiatría y Neurocirugía (Santiago, , Chile )
                0717-9227
                April 2003
                : 41
                : 2
                : 123-130
                Affiliations
                [01] orgnameUniversidad Autónoma de Madrid orgdiv1Hospital Universitario La Paz orgdiv2Servicio de Medicina Interna y Radiodiagnóstico España
                Article
                S0717-92272003000200006 S0717-9227(03)04100206
                10.4067/S0717-92272003000200006
                ca7ec68f-ea6d-4e20-986c-e9eee1353d80

                This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

                History
                : June 2003
                : December 2002
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 29, Pages: 8
                Product

                SciELO Chile

                Categories
                Artículos Originales

                computed tomography,diagnosis magnetic resonance imaging,Whipple´s disease,PCR (polymerase chain reaction),Resonancia magnética,Tomografía axial computarizada,Enfermedad de Whipple

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