The nucleus raphe interpositus in the Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy)
The nucleus raphe interpositus in the Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy)
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DOI：
10.1093/brain/119.4.1137
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发表时间：
1996-08-01
期刊：
影响因子：
14.5
通讯作者：
Daniel, SE
中科院分区：
文献类型：
--
作者：
Revesz, T;Sangha, H;Daniel, SE
由于位于中缝间位核的全间歇神经元的完整性是正常眼球运动的先决条件，因此在13例Steele-Richardson-Olszewski综合征（SROS）病例[8例患有核上性凝视麻痹（SGP），而Jive没有]和6例对照中测定了细胞和神经元缠结密度。与正常对照组相比，SGP患者的神经细胞丢失率接近50%（P < 0.001），而无SGP患者的数据无显著差异（P = 0.18）。SGP组神经元密度低于无SGP组（P = 0.016），神经元缠结密度高于无SGP组（P = 0.011）。这些结果表明，参与omnipause神经元，这是甘氨酸，有助于异常的眼运动在SROS。这些甘氨酸能神经细胞的参与表明，在SROS脑干结构的变性影响神经化学多样的系统，到目前为止，其他脑干核与眼运动有关，并已知在SROS中受到影响，是胆碱能。本研究结果提供的证据表明，临床上不同的亚组的SROS可以区分组织学时，适当的形态测量技术。
As the integrity of the omnipause neurons located in the nucleus raphe interpositus is a prerequisite of normal ocular motility, cell and neurofibrillary tangle densities were determined in 13 Steele-Richardson-Olszewski syndrome (SROS) cases [eight with supranuclear gaze palsy (SGP) and Jive without] and six controls. Compared with normal controls, cases with SGP were associated with similar to 50% nerve cell loss (P < 0.001), whereas data from cases without SGP were not significantly different (P = 0.18). Furthermore, cases with SGP had lower neuronal cell (P = 0.016) and higher neurofibrillary tangle densities than those without (P = 0.011). These results indicate that the involvement of the omnipause neurons, which are glycinergic, contributes to abnormal eye motility in SROS. Involvement of these glycinergic nerve cells suggests that the degeneration of brainstem structures in SROS affects neurochemically diverse systems; so far other brainstem nuclei concerned with eye motility, and known to be affected in SROS, are cholinergic. The results of this study provide evidence that clinically distinct subgroups of SROS may be differentiated histologically when adequate morphometric techniques are applied.