Fujii T, Okuno T, Ito M, Hattori H, Mutoh K, Go T, Shirasaka Y, Shiraishi H, Iwasaki Y, Asato R
Department of Pediatrics, Kyoto University Faculty of Medicine, Japan.
Brain Dev. 1995 Mar-Apr;17(2):89-94. doi: 10.1016/0387-7604(94)00115-e.
We performed N-isopropyl-[123I]p-iodoamphetamine (IMP) single photon emission computed tomography (SPECT) in three patients with Leigh syndrome, two patients with mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS), and two siblings with progressive external ophthalmoplegia (PEO). The SPECT images were compared with the findings on magnetic resonance imaging (MRI) and computed tomography (CT). All Leigh syndrome patients showed low accumulation areas (LAA) bilaterally in the frontal lobes and the basal ganglia. The frontal lobe LAA was seen even in an area without abnormalities on CT/MRI. Each MELAS patient showed a focal LAA. SPECT could also detect an old stroke-like lesion that was no longer shown by CT/MRI. However, SPECT did not show LAA in the basal ganglia, which showed calcification on CT or abnormal signal intensity on MRI. MRI in the 2 PEO patients showed lesions bilaterally in the basal ganglia in one, and in the internal capsules in the other. SPECT showed LAA not only in corresponding areas, but also in the occipital lobes, where no lesions were revealed by MRI. Thus, 123I-IMP SPECT was more sensitive than CT/MRI for detecting stroke-like lesions in MELAS patients, although it did not detect small lesions in the basal ganglia. LAA in the frontal lobes and occipital lobes may be SPECT findings characteristic of Leigh syndrome and PEO, respectively.
我们对3例Leigh综合征患者、2例线粒体脑肌病伴乳酸酸中毒和卒中样发作（MELAS）患者以及2例进行性眼外肌麻痹（PEO）的同胞进行了N-异丙基-[123I]对碘安非他明（IMP）单光子发射计算机断层扫描（SPECT）。将SPECT图像与磁共振成像（MRI）和计算机断层扫描（CT）的结果进行比较。所有Leigh综合征患者双侧额叶和基底神经节均显示低积聚区（LAA）。即使在CT/MRI无异常的区域也可见额叶LAA。每例MELAS患者均显示一个局灶性LAA。SPECT还能检测到一个CT/MRI不再显示的陈旧性卒中样病变。然而，SPECT未显示基底神经节的LAA，而基底神经节在CT上显示钙化或在MRI上显示异常信号强度。2例PEO患者中的1例MRI显示双侧基底神经节病变，另1例显示内囊病变。SPECT不仅在相应区域显示LAA，而且在MRI未显示病变的枕叶也显示LAA。因此，123I-IMP SPECT在检测MELAS患者的卒中样病变方面比CT/MRI更敏感，尽管它未检测到基底神经节的小病变。额叶和枕叶的LAA可能分别是Leigh综合征和PEO的SPECT特征表现。
