Isozaki Eiji, Naito Rie, Kanda Takemasa, Mizutani Toshio, Hirai Shunsaku
Department of Neurology, Tokyo Metropolitan Neurological Hospital, 2-6-1, Musashidai, Fuchu, Tokyo 183-0042, Japan.
J Neurol Sci. 2002 May 15;197(1-2):37-43. doi: 10.1016/s0022-510x(02)00046-1.
While multiple system atrophy (MSA) is frequently associated with vocal cord paralysis (VCP) causing severe respiratory failure, it is still unknown whether hereditary types of spinocerebellar degeneration develop similar laryngeal paralysis. We analyzed the laryngeal function from the viewpoints of fiberoptic laryngoscopy and laryngeal myopathology and then attempted to clarify the difference of the mechanism of VCP among the patients with spinocerebellar ataxia type 1 (SCA 1), type 3 (SCA 3), and MSA. Seven patients with SCA 1, nineteen with SCA 3, and eleven with MSA were studied. Vocal cord movement was analyzed by fiberoptic laryngoscopy during wakefulness and diazepam-induced sleep (sleep load test). Paraffin-embedded sections or cryosections of the intrinsic laryngeal muscles from five autopsied cases (one with SCA 1 and four with SCA 3) were histologically examined. VCP was found in two of the seven SCA 1 patients (29%), three of the nineteen SCA 3 patients (16%), and in nine of the eleven MSA patients (82%). VCP observed in SCA 1 and SCA 3 was various in the severity and showed no exacerbation on sleep load test in all of the eight patients but one SCA 3 patient. In this patient, the findings of fiberoptic laryngoscopy were quite similar to those found in MSA. All the intrinsic laryngeal muscles including cricothyroid (CT), interarytenoid (IA), and posterior cricoarytenoid (PCA) muscles showed neurogenic atrophy in one autopsied SCA 1 and four SCA 3 patients. Our conclusion is that VCP in SCA 1 and SCA 3 contrasts with that in MSA in its occurrence, response to the sleep load test, and the distribution of the neurogenic abnormalities among the intrinsic laryngeal muscles.
虽然多系统萎缩（MSA）常伴有声带麻痹（VCP），导致严重呼吸衰竭，但遗传性脊髓小脑变性是否会发展为类似的喉麻痹仍不清楚。我们从纤维喉镜检查和喉肌病理学的角度分析了喉功能，试图阐明脊髓小脑共济失调1型（SCA 1）、3型（SCA 3）和MSA患者中VCP机制的差异。研究了7例SCA 1患者、19例SCA 3患者和11例MSA患者。在清醒和地西泮诱导睡眠期间（睡眠负荷试验），通过纤维喉镜检查分析声带运动。对5例尸检病例（1例SCA 1和4例SCA 3）的喉内肌石蜡包埋切片或冰冻切片进行组织学检查。7例SCA 1患者中有2例（29%）出现VCP，19例SCA 3患者中有3例（16%）出现VCP，11例MSA患者中有9例（82%）出现VCP。在SCA 1和SCA 3中观察到的VCP严重程度各不相同，除1例SCA 3患者外，其余8例患者在睡眠负荷试验中均未加重。在该患者中，纤维喉镜检查结果与MSA中发现的结果非常相似。在1例尸检的SCA 1患者和4例SCA 3患者中，所有喉内肌，包括环甲肌（CT）、杓间肌（IA）和环杓后肌（PCA）均显示神经源性萎缩。我们的结论是，SCA 1和SCA 3中的VCP在发生率、对睡眠负荷试验的反应以及喉内肌神经源性异常的分布方面与MSA中的VCP形成对比。
