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XXIV Congreso Nacional de la Sociedad Española de Neurocirugía
Madrid, 15-17 September 2021
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P. Barbero Aznárez, R. Pérez Tanoira, D.T. Aguirre Mollehuanca, Á. Trascasa Caño, J. Fortes Alén, F. Manzarbeitia Arrambari, J. Castillo Álvarez, J. Montoya Bordón, E. Petkova Saiz and L. Prieto Pérez

Neurocirugía, Hospital Quirónsalud Zaragoza, Zaragoza, Spain. Microbiología, Hospital Universitario Príncipe de Asturias, Alcalá de Henares, Spain. Neurocirugía, Fundación Jiménez Díaz, Madrid, Spain. Anatomía Patológica, Hospital Infanta Elena, Valdemoro, Spain. Anatomía Patológica, Fundación Jiménez Díaz, Madrid, Spain. Medicina Interna, Fundación Jiménez Díaz, Madrid, Spain. Radiología, Fundación Jiménez Díaz, Madrid, Spain.

Objectives: Our objectives are to describe a case of isolated central nervous system Whipple disease presenting as a brain mass and to present a review of the literature about this topic.

Methods: We review our case and perform a literature search in Cochrane, Scielo and Pubmed. We make a revision of the subject and also make a table with all the cases in the literature of isolated CNS Whipple disease.

Results: We present the case of a 51 year-old-man who complained about difficulty in nomination, blurred vision and impaired gait. After image tests, a glioma was suspected, and a brain biopsy was then offered. The result was consistent with Whipple disease, with no systemic disease. In our review of the literature, we have found 35 cases of isolated CNS Whipple disease. 22 of them were men and 13 women. The mean age was 42 years-old with a median of 43 years-old. 13 of the patients presented like a brain tumor in the MRI. The most common diagnostic clue in the brain biopsy was the PAS+ macrophagues. Only 5 of the cases had the patognomonic sign of oculomasticatory myorhythmia. 13 of the cases were dead in the follow-up while another 13 improved. The other 9 patients had little improvement or were stable.

Conclusions: Whipple disease is an infectious disease which has three different forms: classic, localized and isolated in the central nervous system (CNS). Isolated CNS Whipple disease is a rare illness but should be considered among the differential diagnosis of CNS mass lesions. Brain biopsy is necessary to establish the diagnosis of isolated CNS Whipple disease. It is stressed in the literature that a prolonged course of antibiotic therapy is required to prevent relapses and to control the disease.

Neurocirugía (English edition)

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