Araki S, Hayashi M, Yasaka A, Maruki K
Department of Clinical Neuropathology, Tokyo Metropolitan Institute for Neuroscience, Japan.
Pediatr Neurol. 1997 May;16(4):329-33. doi: 10.1016/s0887-8994(97)00020-9.
We evaluated brainstem dysfunction in a girl with Leigh disease. Neuroradiological and neurophysiological tests included brain magnetic resonance imaging, [123I]IMP single-photon emission computed tomography (SPECT), auditory brainstem responses (ABRs), short-latency somatosensory evoked potentials (SSEPs), electrically elicited blink reflexes (BRs) and all night polysomnography (PSG). The basal ganglia and midbrain contained areas of low accumulation on [123I]IMP SPECT images. Serial analysis of ABRs and SSEPs showed progressive disturbances of the brainstem wave components. The R2 components of the BRs were impaired. All night PSG revealed disturbances in both the tonic and phasic components of REM sleep. These findings suggested the multimodality tests in combination with neuroradiologic examinations are useful for assessing brainstem dysfunction in patients with Leigh disease.
我们评估了一名患有 Leigh 病的女孩的脑干功能障碍。神经放射学和神经生理学检查包括脑磁共振成像、[123I]IMP 单光子发射计算机断层扫描（SPECT）、听觉脑干反应（ABR）、短潜伏期体感诱发电位（SSEP）、电诱发眨眼反射（BR）以及整夜多导睡眠图（PSG）。[123I]IMP SPECT 图像显示基底神经节和中脑存在低聚集区域。对 ABR 和 SSEP 的系列分析显示脑干波成分存在进行性紊乱。BR 的 R2 成分受损。整夜 PSG 显示快速眼动睡眠的紧张性和相位性成分均有紊乱。这些发现表明，多模态检查与神经放射学检查相结合，有助于评估 Leigh 病患者的脑干功能障碍。
