Nervous system involvement in Beh?et's syndrome

摘要

Purpose of review Neurological involvement in Beh?et'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-Beh?et'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 Beh?et'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 Beh?et'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.