Behçet disease (CNS manifestations)

Last revised by Craig Hacking on 18 Aug 2021

CNS manifestations of Behçet disease, also known as neuro-Behçet disease, corresponds to the neurological involvement of the systemic vasculitis Behçet disease and has a variety of manifestations. 

For a discussion of the disease, in general, please refer to Behçet disease article. 

CNS involvement is seen in 4-49% of patients with systemic Behçet disease and has the same predilection of patients of middle eastern and Japanese descent 1

In the vast majority of cases, ulcerative lesions preceded neurological involvement, aiding in the diagnosis. In 3% of cases, central nervous system manifestations occur first, making diagnosis significantly more challenging 1. Signs and symptoms include 1:

  • headaches
  • sensory disturbances
  • personality changes
  • dysarthria
  • cerebellar signs

Neuro-Behçet disease, depending on the stage or degree of the inflammation, shows perivascular infiltration of leukocytes and microglia, degeneration of oligodendroglia, and perivascular softening or necrosis 3.

Neuro-Behçet disease has a wide variety of manifestations in the central nervous system, including 1

Meningoencephalitis and cerebral vein thrombosis are discussed separately in general articles related to these conditions. 

Lesions in neuro-Behçet disease typically involve 1,3, in order of preference: 

Lesions in neuro-Behçet disease typically demonstrate the following signal characteristics 1:

  • T1: usually hypointense
  • T2:
    • usually hyperintense
    • associated with vasogenic edema
    • in acute phase, lesions cause mass effect 
  • T1 C+ (Gd): typically moderate patchy enhancement
  • DWI: isointense to slightly hyperintense
  • MRS: drop in NAA, with elevated lipid and choline/creatine ratio 4
  • corticosteroids: intravenous methylprednisolone infusion then oral prednisone 
  • immunosuppression: azathioprine, methotrexate, and TNFα inhibitors 2

General imaging differential considerations include

Consider other causes of T2 hyperintensity of the basal ganglia

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Cases and figures

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