From the Guidelines
Neurobehçet's disease treatment should prioritize a combination of immunosuppressive medications, including high-dose corticosteroids and immunosuppressants like azathioprine or cyclophosphamide, to control inflammation in the central nervous system, as recommended by the 2018 EULAR update 1. The goal of treatment is to suppress the autoimmune inflammatory process that damages the nervous system, as Neurobehçet's involves vasculitis and immune-mediated damage to neural tissues.
- First-line therapy typically includes high-dose corticosteroids such as intravenous methylprednisolone 1000mg daily for 3-5 days, followed by oral prednisone 1mg/kg/day with a slow taper over months.
- This should be combined with immunosuppressants like azathioprine (2-3mg/kg/day) or cyclophosphamide (monthly pulses of 750-1000mg/m²).
- For refractory cases, biologic agents such as infliximab (5mg/kg at weeks 0,2,6, then every 8 weeks) or adalimumab (40mg every other week) have shown effectiveness, as suggested by previous EULAR recommendations 1.
- Anticoagulation may be necessary if cerebral venous thrombosis is present, although the management of major vessel disease in BD is not well-established, with no firm evidence to guide treatment 1. Treatment duration is typically long-term, often lasting years, with careful monitoring for side effects.
- Regular neurological assessments, MRI monitoring, and laboratory tests are essential to evaluate treatment response and adjust therapy accordingly. The 2018 EULAR update provides the most recent and highest quality evidence for the management of Behçet's syndrome, including Neurobehçet's disease, and should be prioritized in clinical decision-making 1.
From the Research
Treatment Options for Neuro-Behcet's Disease
- The treatment of neuro-Behcet's disease (NBD) depends on the severity and type of neurological involvement, with options including glucocorticoids, immunosuppressants, and biologic agents 2, 3, 4.
- For patients with parenchymal NBD without poor prognostic factors, azathioprine or methotrexate and corticosteroids are recommended as first-line treatment 2.
- High-risk patients may require intravenous cyclophosphamide and corticosteroids, with TNF-alpha-blocking drugs added if these regimens fail 2.
- Alternating IFN-alpha and then chlorambucil or experimental treatments may be considered for refractory patients 2, 4.
Treatment of Specific Neuro-Behcet's Disease Manifestations
- Cerebral venous sinus thrombosis (CVST) is typically treated with anticoagulation and short-term corticosteroids, with or without immunosuppressants 2, 3, 4.
- Parenchymal NBD attacks can be treated with glucocorticoids alone or in combination with azathioprine, with more potent immunosuppressive drugs used for relapsing-remitting or progressive courses 3.
Challenges in Treating Neuro-Behcet's Disease
- The heterogeneity of neurologic involvement in NBD makes it difficult to predict the course and prognosis of the disease, as well as its response to treatment 5.
- There is currently a lack of evidence to support or refute the benefit of various treatments for NBD, highlighting the need for well-designed multicenter randomized controlled trials to inform clinical practice 6.