Neuro-Behçet disease

Case contributed by Dalia Ibrahim
Diagnosis almost certain

Presentation

Involuntary movement in both upper limbs.

Patient Data

Age: 40 years
Gender: Male
mri

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

Photo

Typical involvement of mesodiencephalic junction in Neuro-Behcet disease.

Case Discussion

The case demonstrates typical parenchymal involvement of neuro-Behcet involving the basal ganglia, midbrain, and the mesodiencephalic junction.

The patient has a history of Behçet disease with recurrent orogenital ulcers.

Behçet disease (BD) is a multisystem vasculitis of unknown origin.

Neurologic involvement could potentially include typical or 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, thalami, or subcortical white matter. Parenchymal changes are usually unilateral or asymmetric bilateral.

Orogenital ulcers usually proceed the neurological manifestations of Behcet disease.

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