Characteristics of Neuro-Behçet's Disease in a Case-Series from a Single Centre in Northern Portugal

Abstract

Introduction: Behçet's disease (BD) is a multisystem inflammatory disease of unknown etiology that may affect the CNS - Neuro-Behçet (NB). Our aim was to evaluate the frequency of neurological involvement and characterize a cohort of our NB patients. Methods: We retrospectively revised the clinical, laboratory and imaging data of a cohort of BD patients, followed in our hospital outpatient clinic. Results: We identified 138 BD patients. Twenty-five out of 138 had NB (15 female). Four patients presented with neurological symptoms. We identified a total of 37 attacks. Twenty-one attacks were classified as parenchymatous, four non-parenchymatous and 12 as other syndromes. Seventeen patients had CSF analysis performed (20 samples). Five samples were normal, 15 showed CSF pleocytosis. The most frequent finding on MRI performed in the acute phase was extensive lesions involving the brainstem. Two patients died due to the neurological involvement of BD. Conclusion: We found 18.1% prevalence of NB and a higher female-to-male ratio in our group than in other series. Gastrointestinal and vascular involvement was more frequent in the NB group. The fact that neurological involvement may be the first manifestation of BD with therapeutic implications and associated morbidity points out the relevance of an early diagnosis.

Hints for Genetic and Clinical Differentiation of Adult-Onset Monogenic Autoinflammatory Diseases

“…Among polygenic AIDs, BD might start with severe signs of neurological involvement, consisting of vascular affections with variable severity inducing stroke-related manifestations [222][223][224].…”

Psychological and Neurocognitive Impact of Behcet’s Disease

“…There is also variable regional prevalence in the extent of neurological manifestations observed in the disease. Higher rates of central nervous system abnormalities (between 17 and 44%) have been reported in studies with cohorts from Saudi Arabia, Jordan, Morocco, Italy and Portugal [11][12][13][14][15]. In contrast lower rates (2.3 to 4.6%) have been reported in studies from Iran, Turkey and Korea [16][17][18].…”

A Clinical Odyssey: a case report on the diagnostic complexity and neurological management of Behçet's disease

“…Neurological involvement (Neuro-Behçet's disease), which is rarer, is around 9%, ranging from 3% to 30% [3]. It generally affects more men and young adults [4] and may be the first manifestation or appear during the disease [5].…”