Neuro-Behçet disease

Case contributed by Dalia Ibrahim
Diagnosis almost certain

Presentation

Dizziness and blurred vision since 5 months.

Patient Data

Age: 35 years
Gender: Male
mri

Abnormal signal of the right basal ganglia, midbrain, and mesodiencephalic junction and pons.

Small hemorrhagic focus evident on T1 and GRE. No diffusion restriction.

Case Discussion

An abnormal signal involving the mesodiencephalic junction is typical for neuro-Behçet disease.

The patient was not known to have Behçet disease. However, clinical examination revealed orogenital ulcers and a positive skin prick test.

Behçet disease is a multisystem vasculitis of unknown origin. It is more common in the Middle East.

Neurologic involvement includes typical and atypical parenchymal neuro-Behçet disease, dural sinus thrombosis, cerebral arterial aneurysm, occlusion, dissection, and meningitis.

Parenchymal neuro-Behçet primarily affects the brainstem (especially the mesodiencephalic junction, around the cerebral peduncles and the pons) or the basal ganglia, the thalami, or subcortical white matter.

An abnormal signal of the mesodiencephalic junction can also be seen in neuromyelitis optica (NMO).

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