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Behçet’s Syndrome and the Nervous System Involvement

  • Ugur Uygunoglu
  • Aksel Siva
Chapter
Part of the Contemporary Clinical Neuroscience book series (CCNE)

Abstract

Behçet’s syndrome (BS), a distinct disease with orogenital ulceration and uveitis known as the triple-symptom complex, is an idiopathic chronic relapsing multisystem vascular-inflammatory disease of unknown origin. The neurological involvement of BS is termed as neuro-Behçet’s syndrome (NBS). Direct neurological involvement of BS may be classified into two forms: (1) parenchymal (p-NBS) and (2) vascular involvement. p-NBS with the rate of 75% among neurological involvements of BS usually presents with an acute-subacute brain stem syndrome. Headache, cranial neuropathy, dysarthria, ataxia, and hemiparesis are the most prominent symptoms. 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. The only drug that has been shown to be effective based on the Class IV evidence is infliximab for the treatment of p-NBS.

Keywords

Behçet’s syndrome Parenchymal neuro-Behçet’s syndrome Cerebral venous sinus thrombosis Bagel Sign Infliximab 

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© Springer Nature Switzerland AG 2019

Authors and Affiliations

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

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