Neuro-Behçet’s disease: A report of sixteen patients

Radi Shahien, Abdalla BowirratDepartment of Neurology, Ziv Medical Center, Safed, IsraelBackground: Neurologic involvement in Behçet’s disease (BD), also known as neuro-Behcet’s disease (NBD), is one of the most devastating manifestations of the disease. Th...

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Autores principales: Radi Shahien, Abdalla Bowirrat
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Publicado: Dove Medical Press 2010
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spelling oai:doaj.org-article:4727f33b36cb4a51bd05b7acbca36a232021-12-02T04:27:02ZNeuro-Behçet’s disease: A report of sixteen patients1176-63281178-2021https://doaj.org/article/4727f33b36cb4a51bd05b7acbca36a232010-05-01T00:00:00Zhttp://www.dovepress.com/neuro-behccediletrsquos-disease-a-report-of-sixteen-patients-a4366https://doaj.org/toc/1176-6328https://doaj.org/toc/1178-2021Radi Shahien, Abdalla BowirratDepartment of Neurology, Ziv Medical Center, Safed, IsraelBackground: Neurologic involvement in Behçet’s disease (BD), also known as neuro-Behcet’s disease (NBD), is one of the most devastating manifestations of the disease. The etiology of BD remains obscure and speculative. NBD usually occurs 1–10 years after the first symptom of BD has occurred, and its incidence is 18% (range of 4%–49%).Objective: This study was conducted to describe the clinical and prognostic aspects of neurologic involvement in BD among patients attending a multidisciplinary hospital clinic.Methods: Eighty patients with BD were diagnosed according to the International Study Group Criteria for BD at our hospital. Sixteen patients (20%, including 11 men and five women) had evidence of and fulfilled the diagnostic criteria for NBD. These patients underwent laboratory and imaging investigations, including human leukocyte antigen-typing, lumbar puncture, electroencephalographic studies, and computed tomography scanning.Results: Fourteen of the 16 patients received high-dose steroids, and four of these 14 patients were treated with a combination of steroids and cytotoxic agents. Relief was observed in 14 of 16 patients (87.5%). The remaining two patients were untreated males who suffered severe brainstem lesions, and later died.Conclusion: In contrast with previous reports of a poor prognosis with NBD, our study shows that early aggressive intervention with corticosteroids and cytotoxic agents may ameliorate the prognosis in these patients, and can diminish and stabilize the negative effect of neurologic involvement.Keywords: neuro-Behçet’s disease, neurologic involvement, aphthous ulceration, genital ulceration, iridocyclitis Radi ShahienAbdalla BowirratDove Medical PressarticleNeurosciences. Biological psychiatry. NeuropsychiatryRC321-571Neurology. Diseases of the nervous systemRC346-429ENNeuropsychiatric Disease and Treatment, Vol 2010, Iss Issue 1, Pp 219-225 (2010)
institution DOAJ
collection DOAJ
language EN
topic Neurosciences. Biological psychiatry. Neuropsychiatry
RC321-571
Neurology. Diseases of the nervous system
RC346-429
spellingShingle Neurosciences. Biological psychiatry. Neuropsychiatry
RC321-571
Neurology. Diseases of the nervous system
RC346-429
Radi Shahien
Abdalla Bowirrat
Neuro-Behçet’s disease: A report of sixteen patients
description Radi Shahien, Abdalla BowirratDepartment of Neurology, Ziv Medical Center, Safed, IsraelBackground: Neurologic involvement in Behçet’s disease (BD), also known as neuro-Behcet’s disease (NBD), is one of the most devastating manifestations of the disease. The etiology of BD remains obscure and speculative. NBD usually occurs 1–10 years after the first symptom of BD has occurred, and its incidence is 18% (range of 4%–49%).Objective: This study was conducted to describe the clinical and prognostic aspects of neurologic involvement in BD among patients attending a multidisciplinary hospital clinic.Methods: Eighty patients with BD were diagnosed according to the International Study Group Criteria for BD at our hospital. Sixteen patients (20%, including 11 men and five women) had evidence of and fulfilled the diagnostic criteria for NBD. These patients underwent laboratory and imaging investigations, including human leukocyte antigen-typing, lumbar puncture, electroencephalographic studies, and computed tomography scanning.Results: Fourteen of the 16 patients received high-dose steroids, and four of these 14 patients were treated with a combination of steroids and cytotoxic agents. Relief was observed in 14 of 16 patients (87.5%). The remaining two patients were untreated males who suffered severe brainstem lesions, and later died.Conclusion: In contrast with previous reports of a poor prognosis with NBD, our study shows that early aggressive intervention with corticosteroids and cytotoxic agents may ameliorate the prognosis in these patients, and can diminish and stabilize the negative effect of neurologic involvement.Keywords: neuro-Behçet’s disease, neurologic involvement, aphthous ulceration, genital ulceration, iridocyclitis
format article
author Radi Shahien
Abdalla Bowirrat
author_facet Radi Shahien
Abdalla Bowirrat
author_sort Radi Shahien
title Neuro-Behçet’s disease: A report of sixteen patients
title_short Neuro-Behçet’s disease: A report of sixteen patients
title_full Neuro-Behçet’s disease: A report of sixteen patients
title_fullStr Neuro-Behçet’s disease: A report of sixteen patients
title_full_unstemmed Neuro-Behçet’s disease: A report of sixteen patients
title_sort neuro-behçet’s disease: a report of sixteen patients
publisher Dove Medical Press
publishDate 2010
url https://doaj.org/article/4727f33b36cb4a51bd05b7acbca36a23
work_keys_str_mv AT radishahien neurobehampccediletamprsquosdiseaseareportofsixteenpatients
AT abdallabowirrat neurobehampccediletamprsquosdiseaseareportofsixteenpatients
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