Nota Clínica

Neuro-Behçet: clinicopathological findings in an autopsy case

J. Landeyro, L. Vidaur-Tello, J.F. García-Fontgivell, A. Elguezábal, M. Gené-Hijós, E. Mayayo-Artal [REV NEUROL 2008;47:575-578] PMID: 19048537 DOI: https://doi.org/10.33588/rn.4711.2008453

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Volumen 47 | Number 11 | Nº of views of the article 5.364 | Nº of PDF downloads 461 | Article publication date 01/12/2008

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ABSTRACT

INTRODUCTION Behçet’s disease (BD) was first described by Hulusi Behçet in 1937 as a triad of oral aphthae, genital ulcers and uveitis. It affects most of tissues and organs without exception and has a prevalence of neurologic involvement between 5 and 30%. Histopathological research of autopsy cases is limited, even though the cause of death is confirmed in this study.

CASE REPORT A 30-years-old man without prior medical history of interest debuting with a BD with progressive neurological manifestations and fatal evolution. The autopsy showed, in gross examination, oral and scrotal ulcers. Moreover, the brain revealed a marked cerebral edema, opaque leptomeninges and hemorrhagic infarction of the left frontal lobule extended to the homolateral ventricular cavity. In the brain stem, multiples hemorrhagic infarctions and hemorrhage in the fourth ventricle. The microscopic examination showed a generalized perivascular lymphocytic and neutrophilic inflammation in small and medium vessels with intense diapedesis bleeding. This phenomenon was predominant in brain stem.

CONCLUSIONS The necropsy studies of patients with neuro-Behçet’s disease are scarce. We report the clinicopathological findings in a patient with BD and neurological involvement, in which stands out a cerebral vascular affectation predominant in brain stem. KeywordsAutoimmune disease Autopsy Behçet disease Neuro-Behçet Ulcer Vasculitis

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Neuro-Behçet: clinicopathological findings in an autopsy case