Behçet's disease and the nervous system

Abstract

Behçet's disease is a multisystem inflammatory disorder with unknown aetiology. It is a disease of young adults with a more severe course in males subjects. Its prevalence is high in the Mediterranean basin and Japan and has been linked with human leucocyte antigen B5 (HLA-B5) in those countries. According to the diagnostic criteria formed by the International Study Group, recurrent oral ulceration is a prerequisite, with two more typical symptoms or signs. Neurological involvement is one of the most devastating manifestations of Behçet's disease. The involvement is either caused by primary neural parenchymal lesions (neuro-Behçet) or secondary to major vascular involvement (vasculo Behçet). The course is relapsing-remitting, secondary progressive or primary progressive. The most commonly involved area is the brain stem, with additional symptoms or signs, hemispherical involvement with mental changes being the most common. Intracranial hypertension, usually owing to dural sinus thrombosis, has a special place in Behçet's disease. The most common clinical findings are pyramidal signs. Sensory symptoms or signs are much less frequent, and hemianopia and higher cortical function disturbances as well as pure cerebellar syndrome are rare features. Cerebrospinal fluid usually has a high protein content and/or pleocytosis. Notably, in the acute period most patients have lesions shown by magnetic resonance imaging (MRI) extending from the brain-stem to diencephalic structures. Differential diagnosis from multiple sclerosis can be difficult in patients with hemispheric white matter MRI hyperintensities. Immunosuppressives are used in treatment.

Clinical characteristics of neuro-Behcet’s disease in Japan: a multicenter retrospective analysis

“…Only 1 of the 144 patients showed cerebral vein thrombosis (CVT), and this patient was classified as having non-NBD, confirming that the incidence of CVT in BD in Japan is much lower than that in Middle-Eastern or European counties [2–5]. The reason for the paucity of CVT in BD patients in Japan remains unclear.…”

“…The disease is characterized by recurrent episodes of remission and exacerbation of various symptoms, whereas chronic sustained inflammation in certain tissues is rare [1]. Central nervous system (CNS) involvement in BD is caused either by primary neural parenchymal lesions [neuro-Behçet’s disease (NBD)] or is secondary to major vascular involvement [2–4]. The latter type should be called vasculo-BD [2].…”

“…Central nervous system (CNS) involvement in BD is caused either by primary neural parenchymal lesions [neuro-Behçet’s disease (NBD)] or is secondary to major vascular involvement [2–4]. The latter type should be called vasculo-BD [2]. Several recent studies have disclosed that the parenchymal lesions (NBD) can be classified as acute NBD and chronic progressive (CP) NBD depending on their differential clinical courses, especially on their differential responses to steroids [3, 5–8].…”

Cognitive Event-Related Potential and Neuropsychological Findings in Behcet's Disease without Neurological Manifestations

“…However, neurological involvement due to BD occurs prior to its characteristic oral and cutaneous lesions in some patients (Serdaroğlu 1998). Additionally, subclinical neurological involvement has been clearly demonstrated in some previous series and some of these patients have developed acute attacks during the clinical follow-up period (Serdaroğlu 1998). Early detection of neural in- volvement may provide early establishment of appropriate treatment for neuro-Behçet's disease.…”

NF‐κB protects Behçet's disease T cells against CD95‐induced apoptosis up‐regulating antiapoptotic proteins

“…Originally described as a triad of ocular inflammation, oral ulcerations, and genital ulcerations, BD is better understood as a multisystem disease involving skin, joints, the central nervous system (1,2), the large bowel, and peripheral veins (3). Patients with BD may manifest all or only some of these clinical features.…”