Online first

Acta Reumatológica Portuguesa - Online First: 2020-07-01
Revisão de Caso

Neuro-Behcet Disease - Case Report and Review

Tsalta-Mladenov M, Georgieva D, Andonova S


Introduction: Behcet’s disease (BD) is a multisystem autoimmune relapsing vasculitis with almost unknown etiology, which involves both large and small vessels. The involvement of the central nervous system (CNS) is rare, divided into two main sub-types- parenchymal nad and non-parenchymal. The peripheral nervous system is generally preserved or involved in very rare cases. Case report: We present a rare case of neuro-Behcet’s Disease (NBD) in a young 25-year-old female. The patient presented to our clinic with sudden onset complaints of general weakness, quadriparesis more prevalent for the right limbs, slurred speech, and swallowing disorders. Initially, a clinical diagnosis of cerebral infarction was made, but later the condition was defined as parenchymal Neuro-Behcet disease and the patient underwent corticosteroid and immunosuppressive treatment. Discussion: NBD remains a difficult diagnosis to establish as other diseases and conditions may have a similar clinical presentation. The diagnosis is based on the clinical presentation and the typical lesions in brain magnetic resonance imaging (MRI). The development and disappearance of lesions at MRI in relation with disease-specific treatment may correlate with the course of clinical neurologic deficits. Conclusions: Differential diagnosis of NBD should be considered in cerebrovascular disease, brain tumors, and demyelinating processes.