Yen T C, Yeh S H
Department of Nuclear Medicine, Veterans General Hospital-Taipei, Taiwan, R.O.C.
Zhonghua Yi Xue Za Zhi (Taipei). 1995 Nov;56(5):287-91.
Mitochondrial encephalomyopathies are heterogeneous diseases with common clinical features of muscle and/or the central nervous system. Although molecular and histological diagnoses have been established, imaging modalities for the functioning evaluation of these patients are still obscure. In this study, we tried to use 99mTc-HMPAO brain SPECT images to analyze various mitochondrial encephalomyopathies.
We examined 99mTc-HMPAO Brain SPECT studies of 15 patients with various types of mitochondrial encephalomyopathy (3 Leber's hereditary optic neuropathy (LHON), 4 Kearns-Sayre syndrome (KSS), 4 mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS), 3 myoclonic epilepsy and ragged-red fiber disease (MERRF), and 1 Leigh syndrome), diagnosed by molecular studies.
The results of our studies show obviously decreased radiotracer accumulation in the parieto-temporal regions after stroke-like episodes in patients with MELAS and MERRF: Relatively diminished brain perfusion in patients with KSS is noted, probably secondary to severe encephalomyopathy or subdural effusion. However, there seems to be no significant correlation between the clinical manifestations and the imaging findings in LHON and Leigh.
99mTc-HMPAO Brain SPECT is useful in the diagnosing and assessment of the progress of MERRF, MELAS and KSS. However, its role in LHON and Leigh syndrome seems to be debatable.
线粒体脑肌病是一类具有肌肉和/或中枢神经系统常见临床特征的异质性疾病。尽管已经建立了分子和组织学诊断方法，但用于评估这些患者功能的影像学检查方法仍不明确。在本研究中，我们尝试使用99mTc-HMPAO脑单光子发射计算机断层扫描（SPECT）图像来分析各种线粒体脑肌病。
我们对15例经分子研究确诊的各种类型线粒体脑肌病患者（3例Leber遗传性视神经病变（LHON）、4例凯-塞尔综合征（KSS）、4例线粒体肌病、脑病、乳酸酸中毒和卒中样发作（MELAS）、3例肌阵挛性癫痫伴破碎红纤维病（MERRF）和1例 Leigh综合征）的99mTc-HMPAO脑SPECT检查进行了分析。
我们的研究结果显示，MELAS和MERRF患者在卒中样发作后顶颞区放射性示踪剂摄取明显减少；KSS患者脑灌注相对减少，可能继发于严重的脑肌病或硬膜下积液。然而，LHON和Leigh综合征的临床表现与影像学表现之间似乎没有显著相关性。
99mTc-HMPAO脑SPECT有助于MERRF、MELAS和KSS的诊断及病情评估。然而，其在LHON和Leigh综合征中的作用似乎存在争议。
