Neuro-Behçet disease

Case contributed by Dalia Ibrahim
Diagnosis almost certain

Presentation

Upper and lower limb weakness and dysphasia

Patient Data

Age: 30-year-old
Gender: Male
mri

Abnormal signal of the left midbrain, left mesodiencephalic junction, pons, right middle cerebellar peduncle,anterior medulla and bilateral cerebellar hemispheres.

Right basal ganglia, left thalamic, pontine and left medullary multiple small foci of CSF like signal, likely old lacunar infarcts.

Annotated image

Typical involvement of mesodiencephalic junction in Neuro-Behcet disease.

Case Discussion

The patient has a history of Behçet disease.

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.

Orogenital ulcers usually proceed the neurological manifestations of Behcet disease.

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