Behçet disease (CNS manifestations)

CNS manifestations of Behçet disease (or, also known as neuro-Behçet disease) is one of the more, correspond to a common manifestations ofinvolvement related to systemicBehçet disease and has a variety of manifestations. 

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

Epidemiology

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. 

Clinical presentation

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 symtomssymptoms include ¹:

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

Radiographic features

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

• focal or multifocal lesions
• meningoencephalitis 
• cerebral vein thrombosis

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

MRI

Neuro: Behçet disease typically involves ^1,3, in order of preference: 

• brainstem: most common
• basal ganglia: bilateral in a third of cases
• brainstem
• thalamus
• subcortical white matter: less common
• spinal cord: less common

Lesions of Behçet disease typically demonstrate the following signal characteristics ¹:

• T1: usually hypo-intense
• T2:
  • usually hyperintense
  • associated with vasogenic oedema
  • in acute phase lesions cause mass effect 
• T1 C+ (Gd): typically moderate patchy enhancement
• DWI: isointense to slightly hyperintense [Ed: T2 shine through or true restricted diffusion?]

Treatment and prognosis

• corticosteroids: i.v. methlyprednisolonemethylprednisolone infusion then oral prednisone 
• immunosuppression: azathioprine, methotrexate, and anti TNFα ² 

Differential diagnosis

• multiple sclerosis
• sarcoidosis
• primary lymphoma
• gliomatosis cerebri 
• antiphospholipid syndrome
• Sweet syndrome

Consider other causes of T2 hyperintensity of the basal ganglia.