Behçet Syndrome and the Nervous System

  • Ugur Uygunoglu
  • Aksel Siva


The neurologic involvement of BS is termed as neuro-Behçet syndrome (NBS). Directly, neurological involvement of BS may be classified into two forms: (1) parenchymal (p-NBS) and (2) vascular involvement. Cranial neuropathy, dysarthria, ataxia, hemiparesis, and headache are the major symptoms of p-NBS, with headache being the most prominent. The most common areas affected in p-NBS are the mesodiencephalic junction (MDJ), pons, and medulla oblongata. MDJ lesions tend to extend upward to involve the diencephalic structures and downward to involve the pontobulbar region which is the most common radiological finding observed in p-NBS. In addition to cerebral involvement, two distinct patterns of spinal cord involvement in the long myelopathic lesions of BS, based on T2-weighted axial MRI scans, were described (1) the “bagel sign” pattern, characterized by a central lesion with a hypo-intense core and a hyper-intense rim, with or without contrast enhancement, and (2) the motor neuron pattern, characterized by symmetrical involvement of anterior horn cells.

Taken into account all published autopsy cases together with the clinical and MRI findings, the central nervous system involvement in BS is observed in the areas where venous supply is insufficient. Infliximab is the first-line treatment option in patients having poor prognostic factors.


Parenchymal neuro-Behçet syndrome Cerebral venous sinus thrombosis Bagel sign Venous disease Infliximab 


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Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  • Ugur Uygunoglu
    • 1
  • Aksel Siva
    • 1
  1. 1.Istanbul University Cerrahpaşa School of Medicine, Department of NeurologyIstanbulTurkey

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