Fact-sheet: Behcet syndrome - disease
Updated on 08/31/2018 at 12:11 PM
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Definition
Behçet's disease is a vasculitis of unknown etiology.
It affects vessels of all calibers, more often veins than arteries.
Neuro-Behçet encompasses the neurological manifestations of the disease. They occur within 10 years of the onset of the first symptoms. Neurological involvement affects 5 to 25% of patients, more often men than women, young adults between 20 and 40 years old/occasionally children.
Clinical features
Systemic disease:
- Mucocutaneous involvement (oral and genital aphthosis)
- ocular involvement (posterior uveitis)
- neurological involvement: headaches, seizures, strokes.
- rheumatologic involvement (joint and muscle involvement)
- cardiac, pulmonary, digestive, renal manifestations...
CT
Brain CT often normal, sometimes shows hypodensities.
MRI
Brain MRI:
Best diagnostic criterion:
- association of T2 hyperintense brainstem lesions on MRI with oral and/or genital aphthosis.
On MRI:
- uni- or multifocal involvement.
- lesion location: brainstem (pons and cerebral peduncles), basal ganglia (occasionally white matter).
- rarely: spinal cord involvement.
- look for: dural sinus thrombosis (+++), arterial occlusion, aneurysm of central nervous system.
- protocol: diffusion-weighted imaging, T1-weighted, T2-weighted, T2 FLAIR sequences, T1 sequences with gadolinium injection.
We look for:
- lesions with T2 hyperintensity, T1 iso- or hypointensity, variable diffusion signal, enhancing after injection.
Management
Drug treatment:
- corticosteroid therapy
- immunosuppressants
Differential diagnosis
Neuro-Behçet:
- Cerebral gliomatosis
- ADEM
- Lymphoma of brain
- Multiple sclerosis