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Nervous system involvement in Behçet's syndrome

Uygunoğlu, Uğur; Siva, Aksel

Current Opinion in Rheumatology: January 2019 - Volume 31 - Issue 1 - p 32–39
doi: 10.1097/BOR.0000000000000562
VASCULITIS SYNDROMES: Edited by Hasan Yazici and Yusuf Yazici

Purpose of review Neurological involvement in Behçet's syndrome is defined as ‘the occurrence of neurological symptoms and signs in a patient who meets the International Diagnostic Criteria for BS not otherwise explained by any other known systemic or neurological disease or treatment, and in whom objective abnormalities consistent with neuro-Behçet's syndrome (NBS) are detected either on neurological examination, neuroimaging studies (magnetic resonance imaging [MRI]), and/or on cerebrospinal fluid (CSF) examination’. Given that the neurological involvement of Behçet's syndrome carries a poor prognosis, we aimed to describe the differential diagnosis of NBS and highlight the different radiological patterns together with the treatment options.

Recent findings Two distinct MRI patterns of spinal cord involvement in Behçet's syndrome according to T2-weighted axial images were described: ‘Bagel Sign’ pattern: a central lesion with hypointense core and hyperintense rim with or without contrast enhancement; and ‘Motor Neuron’ pattern: a symmetric involvement of the anterior horn cells. Infliximab prevents patients from having further attacks and even led to improvement in the neurological examination.

Summary As the treatment options completely differ, a NBS diagnosis should be carefully made in patients with clinical and MRI features mimicking other central nervous system inflammatory disorders.

Department of Neurology, Istanbul University Cerrahpaşa School of Medicine, Istanbul, Turkey

Correspondence to Aksel Siva, MD, Department of Neurology, Istanbul University Cerrahpaşa School of Medicine, 34098 Istanbul, Turkey. Tel: +90 212 4143152; fax: +90 212 5290886; e-mail: akselsiva@gmail.com

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