Ichise M, Toyama H, Fornazzari L, Ballinger J R, Kirsh J C
Department of Nuclear Medicine, Mount Sinai Hospital, Toronto, Ontario, Canada.
J Nucl Med. 1993 Aug;34(8):1274-81.
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.
亨廷顿舞蹈病（HD）的病理特征为纹状体神经元丢失和神经受体改变，包括多巴胺受体密度降低。我们通过研究4例早期有症状的HD患者、20例有HD风险的无症状受试者和22例对照，评估了123I - 碘苄胺（IBZM）D2受体单光子发射计算机断层扫描（SPECT）成像和99m锝 - 六甲基丙烯胺肟（HMPAO）脑灌注SPECT成像的临床实用性。分别通过计算纹状体与额叶皮质的IBZM和HMPAO摄取率，对纹状体D2受体结合和灌注进行半定量测量。对照的IBZM比率（1.58±0.06）随年龄每十年下降1.5%（r = -0.58，p < 0.005），而HMPAO比率（1.15±0.05）则不然。所有4例有症状的患者IBZM比率均降低，3例患者HMPAO比率也降低。20例有风险的受试者中有5例IBZM比率降低，2例受试者HMPAO比率也降低。5例有风险的受试者中有3例表现出轻微的非舞蹈样神经异常。因此，在无症状和有症状组中，IBZM - SPECT均可检测到纹状体D2受体结合降低，且这些变化比HMPAO - SPECT检测到的灌注缺损更为明显。因此，IBZM - SPECT似乎是HD早期诊断和临床前检测的一种有前景的方法。
