Behçet disease (CNS manifestations)
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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.
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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 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:
- focal or multifocal lesions
- cerebral vein thrombosis
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, and typically in the pons
- basal ganglia: bilateral in one-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 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
Treatment and prognosis
- corticosteroids: intravenous methylprednisolone infusion then oral prednisone
- immunosuppression: azathioprine, methotrexate, and TNFα inhibitors 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
- 4. Scully SE, Stebner FC, Yoest SM. Magnetic resonance spectroscopic findings in neuro-Behçet disease. (2004) The neurologist. 10 (6): 323-6. doi:10.1097/01.nrl.0000144559.07378.3a - Pubmed