An uncommon disease included commonly in the differential diagnosis of neurological diseases: Neuro-Behcet's syndrome

Uygunoglu U. , Siva A.

JOURNAL OF THE NEUROLOGICAL SCIENCES, vol.426, 2021 (Journal Indexed in SCI) identifier identifier identifier

  • Publication Type: Article / Review
  • Volume: 426
  • Publication Date: 2021
  • Doi Number: 10.1016/j.jns.2021.117436


Behcet's Syndrome (BS) may present with different neurological problems, related either directly (primary) or indirectly (secondary) to BS. Primary neurological involvement is named as neuro-Behcet's syndrome (NBS), and its two major subtypes that are classified mainly on the clinical and MRI findings are (1) Parenchymal-NBS (pNBS) and (2) Extra parenchymal neuro-vascular involvement mostly seen as cerebral dural venous sinus thrombosis (CVST). The less commonly seen forms of NBS are cognitive and behavioral syndromes and peripheral nervous system involvement. Parenchymal-NBS is the most common clinical neurological presentation of BS. It is a rare disease with distinct MRI features and is often included in the differential diagnosis of neuro-vascular and neuro-inflammatory disorders. The most commonly affected neuro-anatomical site in p-NBS is the meso-diencephalic junction (MDJ), followed by the ponto-bulbar and thalamic regions, the basal ganglia, and the spinal cord. These varied locations may explain to a certain extent why BS is considered in the differential of so many neurological disorders. The other relatively common form of NBS that results in CVST may also be confused with other conditions resulting in CVST, especially when the systemic clinical features suggestive of BS are missed.