Neuro-Behçet syndrome

摘要: Behcet syndrome (BS) is an idiopathic chronic relapsing multisystem vascular-inflammatory disease of unknown origin. As the affects many organs and systems shows a wide range clinical manifestations presentations, it prefereable to call Behcet's rather than disease. Nervous system involvement, known as "neuro-BS" (NBS), seen in about 5-10% all cases. Clinical imaging evidence suggests that primary neurologic involvement BS may be subclassified into two major forms: first, which majority patients, characterized central nervous with focal or multifocal parenchymal mostly presenting subacute brainstem hemiparesis (intra-axial NBS); other, has few symptoms better prognosis, caused by isolated cerebral venous sinus thrombosis intracranial hypertension (extra-axial NBS), occurring 10-20% These types are rarely same individual, their pathogenesis likely different. Isolated behavioral syndromes peripheral rare, whereas vascular type headache relatively common independent from involvement. Neurologic complications secondary systemic BS, well related treatments considered syndrome. The core histopathologic phenomenon seems vasculitic some cases, low-grade nonspecific inflammation others. this so heterogeneous, difficult predict its course response treatment. Currently, treatment options for NBS limited attack therapies high-dose intravenous methylprednisolone followed prolonged oral taper, symptomatic management, generally use azathioprine, cyclophosphamide, interferon-α anti-TNF agents long-term preventive treatment, although there no efficacy.