Neuro-Behçet: report of three clinically distinct cases

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. NeuroBehçet must always be included as a differential diagnosis of neurological disorders that have any difficulties in establishing a definite diagnosis.KEY WORDS: neuro-Behçet, Behçet's disease, cerebrospinal fluid. Neuro-Behçet: relato de três casos clinicamente distintosRESUMO -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. NeuroBehçet deve ser sempre incluída em casos de desordens neurológicas que apresentem dificuldades para o estabelecimento de um diagnóstico definitivo.PALAVRAS-CHAVE: neuro-Behçet, doença de Behçet, liquor.

Neuro-Behçet syndrome presentıng as acute meningeal syndrome

“…When all four major criteria are found, the patient is said to have complete BD, and when only two or three major criteria are found, the patient has incomplete BD. 8 As four major criteria were found in our patient, the diagnosis was definite.…”

“…6 In the first group, the most common findings are pyramidal signs, pseudo-bulbar paralysis, hemiparesis, cranial nerve palsies, cerebellar signs, behavioral changes, sphincter disturbance, and can usually be found in patients with symptoms of meningitis. 2,8 In the second group, raised intracranial pressure due to dural sinus thrombosis is the main clinical manifestation. 2 Vascular complications as a result of dural sinus thrombosis occur in about 16% of cases, and are found in 80% of cases with isolated intracranial hypertension; the CSF is generally normal and arterial thrombosis is rare.…”

Atypical manifestations in Brazilian patients with neuro-Behçet’s disease

“…NBD also presents as a demyelinating syndrome, as subcortical white-matter lesions without typical involvement of the brainstem [18,24,25]. ON is an uncommon NBD manifestation and might occur uni-or bilaterally, usually in association with other parenchymal manifestations [9].…”

A Complex Case of Neuro-Behçet's Disease in a Patient Previously Diagnosed with multiple Sclerosis: A Case Report

“…8 To date, there have only been two case reports of patients misdiagnosed with MS who were later confirmed to have NBD. 7,9 Ashjazadeh and colleagues 10 also prospectively reviewed neuropsychiatric symptoms in six patients with NBD, two of whom had not been previously diagnosed with NBD.…”