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Brain Perfusion SPECT in Juvenile Neuro-Behçet’s Disease

II Division of Pediatrics, Giannina Gaslini Institute; Clinical Neurophysiology Service; and Nuclear Medicine Service, Department of Internal Medicine, University of Genoa, Genoa, Italy

Regional cerebral blood flow was evaluated by ^99mTc-hexamethylpropyleneamine oxime SPECT in 7 patients (age range, 7–18 y; mean age, 9.1 y) affected with Behçet’s disease and signs or symptoms of central nervous system involvement at different times of their clinical history. Methods: Three patients suffered from seizures, 3 patients were affected with severe persistent headache that was refractory to common analgesic and nonsteroidal antiinflammatory drugs, and 1 patient had recurrent episodes of acute intracranial hypertension. Electroencephalography was performed on all patients, MRI on 5 patients, and CT on 1 patient. Brain SPECT was performed using a high-resolution, brain-dedicated camera. After conventional visual analysis by 2 expert readers, 2 transaxial sections were drawn parallel to the bicommissural line: the first across the thalami and the second across the temporal lobe at the level of the mesiotemporal structures. Cortical regions of interest were drawn automatically on the cortical ribbon on the 2 sections, whereas other regions of interest were drawn by hand around the basal ganglia, the thalami, and the mesiotemporal structures. Asymmetry analysis was then applied, and hypoperfusion was considered when the asymmetry value was >10%. Results: Hypoperfusion was observed in all patients by visual and asymmetry analyses; this finding was localized mainly in the basal ganglia, the thalami, and the temporal cortex, including its mesial portion. Temporal hypoperfusion was found primarily in patients with seizures, and hypoperfusion of deep gray nuclei was found mainly in the other patients. Electroencephalography disclosed brain functional impairment in 5 of 6 patients, where- as MRI showed multiple bilateral white matter lesions in 1 patient suffering from persistent headache. Conclusion: As in adults, perfusion SPECT seems to be very sensitive in disclosing brain abnormalities in children and adolescents with Behçet’s disease and signs or symptoms of central nervous system involvement, even with negative findings on brain MRI.

Key Words: juvenile Behçet’s disease • brain SPECT • neuro-Behçet’s disease • pediatrics • regional cerebral blood flow