Management for neurological features of Behçet syndrome

Yazici, 2021Hatemi, 2018Ozguler, 2018

Note: If neurological disease associated with Behçet syndrome is suspected, refer patients to a specialist.

Neurological disease associated with Behçet syndrome must be treated in consultation with a specialist. For patients with significant features of central nervous system (CNS) disease (whether parenchymal or nonparenchymal), treatment choice depends on:

  • the severity of symptoms and course of disease
  • whether the patient’s CNS disease responds to systemic corticosteroids
  • whether the patient has had prior CNS disease
  • the presence of other features of Behçet syndrome.

In the acute phase of neurological disease, high-dose pulse intravenous corticosteroids are recommended. Pulse intravenous corticosteroids should be followed by slow tapering of oral corticosteroids, plus an immunomodulatory drug such as azathioprineYazici, 2021. For patients with severe or refractory neurological disease, TNF inhibitors or cyclophosphamide may be usedYazici, 2021. Ciclosporin should be avoided because it is associated with an increased risk of developing parenchymal CNS diseaseHatemi, 2018.