Neuro-Behçet disease

Case contributed by Dalia Ibrahim

Presentation

Aphasia and lower limb weakness.

Patient Data

Age: 20 years
Gender: Male
mri

Bilateral abnormal signal involving the mesodiencephalic junctions (larger on the left side), eliciting high signal on T2WI and FLAIR.

Left nasolabial fold cyst (Incidental finding).

Abnormal signal of the left mesodiencephalic junction (arrow).

Case Discussion

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

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

Behçet disease (BD) 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).

Thanks to Dr. Nae'ma, Resident of general radiology, Kasr Al Ainy Hospital, for her assistance

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