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Behçet's disease and the nervous system
1Department of Neurology, University of Istanbul, Faculty of Medicine, Turkey.
Insights
Behçet's disease is a rare inflammatory condition affecting multiple body systems. Neurological involvement, particularly brainstem lesions, is a severe manifestation requiring immunosuppressive treatment.
Area of Science:
- Neurology
- Immunology
- Rheumatology
Background:
- Behçet's disease is a multisystem inflammatory disorder of unknown cause.
- It primarily affects young adults, with higher prevalence in Mediterranean regions and Japan, and is associated with HLA-B5.
- Recurrent oral ulcers are a key diagnostic criterion, alongside two other typical symptoms.
Purpose of the Study:
- To summarize the key aspects of neurological involvement in Behçet's disease.
- To highlight the diagnostic challenges and common presentations of neuro-Behçet.
- To outline current treatment approaches.
Main Methods:
- Review of existing literature on Behçet's disease and its neurological manifestations.
- Analysis of diagnostic criteria and clinical findings.
- Discussion of neuroimaging findings, particularly MRI.
Main Results:
- Neurological involvement in Behçet's disease presents as neuro-Behçet (parenchymal lesions) or vasculo-Behçet (vascular involvement).
- Commonly affected areas include the brainstem and diencephalon, leading to symptoms like pyramidal signs and intracranial hypertension (often from dural sinus thrombosis).
- Cerebrospinal fluid typically shows high protein and/or pleocytosis; MRI reveals characteristic lesions, though differential diagnosis with multiple sclerosis can be challenging.
Conclusions:
- Neurological complications significantly impact Behçet's disease prognosis.
- Early diagnosis and recognition of neuroimaging findings are crucial.
- Immunosuppressive therapy is the mainstay treatment for managing neurological involvement.
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.