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      Neuro-Behçet: report of three clinically distinct cases Translated title: Neuro-Behçet: relato de três casos clinicamente distintos

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          Abstract

          We report three patients who collectively have very representative clinical forms of neuro-Behçet and different neurological findings. The first case, male, 49 years old, presents symptoms similar to multiple sclerosis. The second case, male 15 years old, presents with parenchymatous compromise and an association with antiphospholipid antibody. And the third case, female 25 years old, presents an acute meningitis. Neuro-Behçet must always be included as a differential diagnosis of neurological disorders that have any difficulties in establishing a definite diagnosis.

          Translated abstract

          Relatamos três pacientes com formas bem representativas de neuro-Behçet e com diferentes apresentações clínicas e manifestações neurológicas. O primeiro caso é o de um homem, 49 anos e que apresenta manifestações clínicas similares à esclerose múltipla. O segundo caso é o de um adolescente de 15 anos, com comprometimento parenquimatoso e doença de Behçet associada à presença de anticorpo antifosfolipide. E o terceiro é caso de uma mulher de 25 anos apresentando quadro de meningite. Neuro-Behçet deve ser sempre incluída em casos de desordens neurológicas que apresentem dificuldades para o estabelecimento de um diagnóstico definitivo.

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          Most cited references34

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          Criteria for diagnosis of Behcet's disease

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            Cerebrospinal fluid in diseases of the nervous system

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              Epidemiology of Adamantiades-Behçet's disease.

              Adamantiades-Behçet's disease is a universal disorder with varying prevalence, i.e. 80-370 patients per 100,000 inhabitants in Turkey, 2-30 patients per 100,000 inhabitants in the Asian continent and 0.1-7.5 patients per 100,000 inhabitants in Europe and the USA. Certain ethnic groups are mainly affected, while the prevalence of the disease seems to be strongly dependent on the geographic area of their residence. These data indicate environmental triggering of a genetically determined disorder. The disease usually occurs around the third decade of life, however, early and late onsets (first year of life to 72 years) have been reported. Juvenile onset disease rates from 7 to 44% in different ethnic groups; juvenile disease is less frequent, i.e. 2-21%. Both genders are equally affected. Familial occurrence has been reported in 1-18% of the patients, mostly of Turkish, Israeli and Korean origin, and is increased in patients with juvenile disease. Oral aphthous ulcers represent the onset sign in the majority of patients worldwide (47-86%). Oral aphthous ulcers (92-100%), genital ulcerations (57-93%), skin lesions (38-99%), ocular lesions (29-100%) and arthropathy (16-84%) are the most frequent clinical features; sterile pustules (28-66%) and erythema nodosum (15-78%) are the most common encountered skin lesions. The positivity of pathergy test varies widely in different populations (6-71%). HLA-B51 is associated with high relative risk for the disease in a small geographic area of the Mediterranean Sea countries and Southern Asia. Diagnosis can be established 2 to 15 years after the onset of the disease. Male gender, early development of the disease, and HLA-B51 positivity are markers of severe prognosis (mortality rates of 0-6%).
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                Author and article information

                Contributors
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Journal
                anp
                Arquivos de Neuro-Psiquiatria
                Arq. Neuro-Psiquiatr.
                Academia Brasileira de Neurologia - ABNEURO (São Paulo )
                1678-4227
                June 2001
                : 59
                : 2A
                : 250-254
                Affiliations
                [1 ] Universidade Federal do Paraná Brazil
                Article
                S0004-282X2001000200020
                10.1590/S0004-282X2001000200020
                aec69f43-d37a-4584-8479-d0a94c925dd1

                http://creativecommons.org/licenses/by/4.0/

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                SciELO Brazil

                Self URI (journal page): http://www.scielo.br/scielo.php?script=sci_serial&pid=0004-282X&lng=en
                Categories
                NEUROSCIENCES
                PSYCHIATRY

                Neurosciences,Clinical Psychology & Psychiatry
                neuro-Behçet,Behçet's disease,cerebrospinal fluid,doença de Behçet,liquor

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