RESUMEN
A patient with severe, generalized dystonia and 6 age range-matched controls were studied with the regional cerebral blood flow tracer technetium-99m hexamethylpropyleneamine oxime by single-photon emission computed tomography to test the hypothesis that cerebellar function is abnormal in dystonia. Analysis was performed by drawing regions of interest around the caudate head nuclei, hemithalami, deep cerebellar nuclei, and cerebellar hemicortices. The counts in each region of interest were normalized to whole brain cerebral blood flow in an identical manner for each subject. The dystonic patient had a difference in regional cerebral blood flow between the right and left deep cerebellar nuclei, increased regional cerebral blood flow in subcortical motor structures, and an abnormal relationship between right cerebellar cortical and right deep cerebellar nuclear regional cerebral blood flow. The findings in this patient provide evidence that the cerebellum may play a role in the pathophysiology of motor signs in some patients with dystonia.
Asunto(s)
Encéfalo/irrigación sanguínea , Distonía/congénito , Tomografía Computarizada de Emisión de Fotón Único , Mapeo Encefálico , Núcleos Cerebelosos/irrigación sanguínea , Niño , Medios de Contraste , Dominancia Cerebral/fisiología , Distonía/diagnóstico por imagen , Distonía/fisiopatología , Femenino , Estudios de Seguimiento , Humanos , Examen Neurológico , Compuestos de Organotecnecio , Oximas , Flujo Sanguíneo Regional/fisiología , Exametazima de Tecnecio Tc 99mRESUMEN
UNLABELLED: We performed initial and follow-up regional cerebral blood flow (rCBF) studies using 99mTc-hexamethylpropyleneamine oxime (HMPAO) SPECT on a patient with manganese-induced central nervous system (CNS) neurotoxicity. METHODS: The patient had a history of long-term exposure to manganese at the time of the first scan, while the follow-up scan was performed 9 mo after removal from the toxic environment. The patient's serum level of manganese was five- to tenfold greater than normal at the time of the initial rCBF brain SPECT scan. RESULTS: The rCBF brain SPECT scan demonstrated significantly decreased rCBF in the right caudate nucleus and both thalami. A MRI scan obtained at the same time was normal. The follow-up rCBF brain SPECT scan was normal. CONCLUSIONS: This report supports the utilization of functional rCBF brain SPECT imaging to provide objective evidence of a function CNS abnormality due to neurotoxicity at an early clinical stage. Our results emphasize that rCBF brain SPECT may provide a confirmational test to support the diagnosis of neurotoxicity in the appropriate clinical setting.