Treatment of neuro-Behçet’s disease: an update

Abstract

Neurological manifestations of Behçet’s disease (neuro-Behçet’s disease) present in 5–30% of cases. They are classified into parenchymal and nonparenchymal categories. Poor prognostic factors include multifocal involvement, spinal presentations, more than two attacks per year, progressive course and increased cerebrospinal fluid cell count and protein content at the time of neurologic manifestations. For patients with parenchymal neuro-Behçet’s disease without any poor prognostic factor, azathioprine or methotrexate and corticosteroids are recommended as the first-line treatment. For high-risk patients, intravenous cyclophosphamide and corticosteroids are recommended. If these regimens failed, TNF-α-blocking drugs, such as infliximab or etanercept, should be added. Alternating IFN-α and then chlorambucil or experimental treatments are the last resorts for most refractory patients. Treatment of venous sinus thrombosis is achieved by using anticoagulation and short-term corticosteroids with or without immunosuppressants.

Keywords::

• Behçet’s syndrome
• nervous system
• neuro-Behçet’s disease
• practice guidelines
• treatment

Acknowledgments

I am indebted to Fereydun Davatchi and Saeedeh Shenavadeh for their critical reading and very useful comments. I am also thankful to Behruz Astaneh for his assistance in editing the manuscript.

Financial & competing interests disclosure

The author has no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

No writing assistance was utilized in the production of this manuscript.