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Iodine-123-IBZM Dopamine D2 Receptor and Technetium-99m-HMPAO Brain Perfusion SPECT in the Evaluation of Patients with and Subjects at Risk for Huntington's Disease

Department of Nuclear Medicine, Mount Sinai Hospital and University of Toronto, Toronto, Ontario, Canada
Department of Radiology, Fujita Health University, Toyoake, Japan
Department of Neurology, Clarke Institute of Psychiatry, Toronto, Ontario, Canada
Department of Nuclear Medicine, Princess Margaret Hospital, Toronto, Ontario, Canada

Correspondence: For correspondence or reprints contact: Masanori Ichise, MD, FRCPC, Room 635, Nuclear Medicine, Radiologica1 Sciences, Mount Sinai Hospital, 600 University Ave., Toronto, Ontario, Canada M5G 1X5.

ABSTRACT

Huntington's disease (HD) is pathologically characterized by neuronal loss and neuroreceptor alterations in the striatum, including a reduction in dopamine receptor density. We evaluated the clinical usefulness of ¹²³I-iodobenzamide (IBZM) D2 receptor SPECT imaging and ^99mTc-hexamethylpropyleneamineoxime (HMPAO) brain perfusion SPECT imaging by studying four early symptomatic HD patients, 20 asymptomatic subjects at risk for HD and 22 controls. Striatal D2 receptor binding and perfusion were measured semiquantitatively by calculating striatum-to-frontal cortex IBZM and HMPAO uptake ratios, respectively. The control IBZM ratio (1.58 ± 0.06) declined with age at 1.5% per decade (r = –0.58, p < 0.005), whereas the HMPAO ratio (1.15 ± 0.05) did not. All four symptomatic patients had decreased IBZM ratios and three patients also had decreased HMPAO ratios. Five of 20 at-risk subjects had decreased IBZM ratios and two subjects also had decreased HMPAO ratios. Three of the five at-risk subjects showed subtle nonchoreic neurological abnormalities. Decreased striatal D2 receptor binding thus may be detected by IBZM-SPECT in the asymptomatic as well as symptomatic groups, and these changes were more marked than perfusion deficits detected by HMPAO-SPECT. IBZM-SPECT thus appears to be a promising method for early diagnosis and preclinical detection of HD.

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