Behçet disease (CNS manifestations)
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:
- sensory disturbances
- personality changes
- cerebellar signs
Neuro-Behçet disease, depending on the stage or degree of the inflammation, shows a perivascular infiltration of leukocytes and microglia, degeneration of oligodendroglia, and perivascular softening or necrosis 3.
Unfortunately, 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:
- brainstem: most common
- basal ganglia: bilateral in a third of cases
- subcortical white matter: less common
- spinal cord: less common
Lesions in neuro-Behçet disease typically demonstrate the following signal characteristics 1:
- T1: usually hypointense
- 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. methylprednisolone infusion then oral prednisone
- immunosuppression: azathioprine, methotrexate, and anti-TNFα 2
General imaging differential considerations include
- multiple sclerosis
- primary CNS lymphoma
- gliomatosis cerebri
- antiphospholipid syndrome
- Sweet syndrome
Consider other causes of T2 hyperintensity of the basal ganglia.
- 1. Hegde AN, Mohan S, Lath N et-al. Differential diagnosis for bilateral abnormalities of the basal ganglia and thalamus. Radiographics. 31 (1): 5-30. doi:10.1148/rg.311105041 - Pubmed citation
- 2. Noel N, Hutié M, Wechsler B et-al. Pseudotumoural presentation of neuro-Behcet's disease: case series and review of literature. Rheumatology (Oxford). 2012;51 (7): 1216-25. doi:10.1093/rheumatology/ker449 - Pubmed citation
- 3. Matsuo K, Yamada K, Nakajima K et-al. Neuro-Behçet disease mimicking brain tumor. AJNR Am J Neuroradiol. 2005;26 (3): 650-3. AJNR Am J Neuroradiol (full text) - Pubmed citation