Follow-up findings in regional cerebral blood flow (r-CBF)-SPECT in a caseof idiopathic childhood hemidystonia: Functional neuroimaging and pathophysiological implications

A 9 1/2-year-old girl suffered from intermitting tremor and jitteriness of her left hand and oral muscles every 4 to 6 weeks with long lasting episode s. Clinically myoclonias and dystonic positioning of the left arm, hand and facial muscles were seen. No evidence of trauma, infection or inborn error s of metabolism was found. Successful therapy with carbamazepine was initia ted while L-DOPA failed. An ictal 99m-Tc-HMPAO-SPECT showed severe asymmetr y with focal hyperperfusion of the contralateral right thalamus and basal g anglia as well as of the bifrontal cortex, whereas no anatomical lesions we re found by MRI. in contrast, an interictally performed 99m-Tc-HMPAO SPECT showed hypoperfusion of the right thalamus and normalisation of the frontal perfusion under medical treatment. These 99m-Tc-HMPAO-SPECT findings may p rovide new insights into the localisation and pathophysiological pathways o f idiopathic childhood dystonia.