Matsuishi T, Sakai T, Nagamitsu S, Shoji H, Ueda N, Kaneko S, Kano T, Iwashita H, Kato H
Department of Pediatrics and Child Health, Kurume University School of Medicine, Fukuoka, Japan.
J Neurol Sci. 1996 Oct;142(1-2):107-10. doi: 10.1016/0022-510x(96)00159-1.
To clarify the mechanism of brain impairment in Machado-Joseph disease (MJD), we measured the cerebrospinal fluid (CSF) levels of substance P in 7 patients (mean age 45.7 +/- 12.09 years) with this disease. Four patients had type I and three had type II disease. Findings were compared with those obtained in 14 age-matched controls, 8 patients with Parkinson's disease, 7 patients with multiple system atrophy, and 6 patients with myopathy. The CSF level of substance P was significantly (p = 0.0000) lower in the patients with MJD, being 44.5% of the control value. However, the mean CSF levels of substance P in the patients with Parkinson's disease, multiple system atrophy, or myopathy did not differ significantly from that in the control subjects. The alteration in the CSF level of substance P may be related to the neurological impairment observed in MJD.
为阐明马查多-约瑟夫病（MJD）患者脑损伤的机制，我们检测了7例（平均年龄45.7±12.09岁）MJD患者脑脊液（CSF）中P物质的水平。其中4例为I型，3例为II型。将研究结果与14例年龄匹配的对照者、8例帕金森病患者、7例多系统萎缩患者及6例肌病患者的结果进行比较。MJD患者CSF中P物质水平显著降低（p = 0.0000），仅为对照值的44.5%。然而，帕金森病、多系统萎缩或肌病患者CSF中P物质的平均水平与对照者相比无显著差异。CSF中P物质水平的改变可能与MJD中观察到的神经功能损害有关。
