Neurological Manifestations in Behçet's Disease

Clinical Presentation and Diagnosis

Cerebral venous sinus thrombosis is a common neurological manifestation in Behçet's disease, particularly in patients with retinal vasculitis 1

The European League Against Rheumatism recommends the use of cyclophosphamide and methotrexate for managing acute phase neurological involvement in Behçet's disease 1
For parenchymal involvement, the use of TNF-alpha antagonists is recommended 1, 2
For dural sinus thrombosis, corticosteroids are recommended 1, 2
Cyclosporine A should not be used in Behçet's patients with central nervous system involvement unless necessary for intraocular inflammation 2, 3

Any patient with Behçet's disease and inflammatory eye disease affecting the posterior segment should be on a treatment regimen that includes azathioprine and systemic corticosteroids 1, 4
For severe eye disease, the use of either cyclosporine A or infliximab in combination with azathioprine and corticosteroids is recommended 5, 6
Corticosteroids should never be used alone in patients with posterior uveitis 6

Regular ophthalmologic examinations are essential for patients with ocular involvement 2
Young men with early disease onset have a higher risk of severe disease and may benefit from early systemic immunosuppression with azathioprine 2
Monitoring should include assessment of clinical symptoms and inflammatory markers regularly, with consideration of repeat imaging for neurological involvement 2