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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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 precede 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
Pathology
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.
Radiographic features
Neuro-Behçet disease has a wide variety of manifestations in the central nervous system, including 1:
focal or multifocal lesions
Meningoencephalitis and cerebral venous thrombosis are discussed separately in general articles related to these conditions.
MRI
Parenchymal lesions in neuro-Behçet disease typically involve the following sites 1,3,5,6:
mesodiencephalic junction (thalamus, midbrain, and internal capsule): most common, on coronal images appearing as the cascade sign 7
pons: second most common
basal ganglia: bilateral in one-third of cases
subcortical white matter: less common
spinal cord: less common, often as longitudinally extensive transverse myelitis with the bagel sign
Lesions in neuro-Behçet disease typically demonstrate the following signal characteristics 1:
T1: usually hypointense
-
T2:
usually hyperintense
associated with vasogenic edema
in the 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
steroid-sparing immunosuppression: azathioprine, methotrexate, and TNF-α inhibitors 2
Differential diagnosis
General imaging differential considerations include
Consider other causes of T2 hyperintensity of the basal ganglia.
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