Damage to the reticulotegmental nucleus of the pons in spinocerebellar ataxia type 1, 2, and 3
Damage to the reticulotegmental nucleus of the pons in spinocerebellar ataxia type 1, 2, and 3
复制标题
DOI：
10.1212/01.wnl.0000140498.24112.8c
复制
发表时间：
2004-10-12
期刊：
影响因子：
9.9
通讯作者：
Braak, H
中科院分区：
文献类型：
--
作者：
Rüb, U;Bürk, K;Braak, H
背景：脑桥网状被盖核（RTTG）是脑干小脑前核团之一。虽然它代表了一个重要的组成部分，眼电路的准确性至关重要的水平扫视和水平平滑的追求，RTTG从未被认为是CAG重复或多聚谷氨酰胺疾病。研究方法：对10例脊髓小脑共济失调（SCA）患者（2例SCA-1患者，4例SCA-2患者和4例SCA-3患者）的RTTG厚连续切片进行神经元脂褐素色素和尼氏物质染色。结果如下：非常规的厚组织切片揭示了迄今为止被忽视的RTTG参与SCA-1、SCA-2和SCA-3的退行性过程，其中在SCA-1患者中的一个、SCA-2患者中的两个和所有SCA-3患者中，RTTG经历了其神经细胞的明显损失。结论：神经变性不仅影响脑神经核（即，SCA-1、SCA-2和SCA-3患者的眼神经和外展神经核）整合到回路中，有助于水平扫视的准确性和水平平滑追踪的产生，但同样涉及这些回路的运动前神经网络。这可能解释了为什么SCA-1，SCA-2，SCA-3患者在本研究中与一个严重受损的网状被盖核的脑桥发展不对称的水平扫视和受损的顺利追求在病程中。
Background: The reticulotegmental nucleus of the pons (RTTG) is among the precerebellar nuclei of the human brainstem. Although it represents an important component of the oculomotor circuits crucial for the accuracy of horizontal saccades and the generation of horizontal smooth pursuits, the RTTG has never been considered in CAG repeat or polyglutamine diseases. Methods: Thick serial sections through the RTTG of 10 patients with spinocerebellar ataxias (SCAs) assigned to the CAG repeat or polyglutamine diseases ( 2 SCA-1 patients, 4 SCA-2 patients, and 4 SCA-3 patients) were stained for neuronal lipofuscin pigment and Nissl material. Results: The unconventionally thick tissue sections revealed the hitherto overlooked involvement of the RTTG in the degenerative processes underlying SCA-1, SCA-2, and SCA-3, whereby in one of the SCA-1 patients, in two of the SCA-2 patients, and in all of the SCA-3 patients, the RTTG underwent a conspicuous loss of its nerve cells. Conclusions: Neurodegeneration may not only affect the cranial nerve nuclei (i.e., oculomotor and abducens nuclei) of SCA-1, SCA-2 and SCA-3 patients integrated into the circuits, subserving accuracy of horizontal saccades and the generation of horizontal smooth pursuits, but likewise involves the premotor networks of these circuits. This may explain why the SCA-1, SCA-2, and SCA-3 patients in this study with a heavily damaged reticulotegmental nucleus of the pons developed dysmetric horizontal saccades and impaired smooth pursuits during the course of the disease.