Matsuishi T, Sakai T, Nagamitsu S, Komori H, Iwashita H, Kato H
Department of Pediatrics and Child Health, Kurume University School of Medicine, Japan.
Ann Neurol. 1994 Sep;36(3):441-3. doi: 10.1002/ana.410360317.
We measured the cerebrospinal fluid (CSF) levels of beta-endorphin in 7 Japanese patients with Joseph disease and compared them with control values. The 7 patients included 4 with type I and 3 with type II disease; their mean age was 45.7 +/- 12.09 years. Diseased controls were matched in age to the patients studied. In these patients, CSF beta-endorphin level was significantly lower than in the controls (40% of normal values). An alteration in CSF beta-endorphin level may explain some of the neurological impairment found in Joseph disease.
我们检测了7例患有约瑟夫病的日本患者脑脊液中β-内啡肽的水平，并将其与对照值进行比较。这7例患者中，4例为I型疾病，3例为II型疾病；他们的平均年龄为45.7±12.09岁。患病对照组的年龄与所研究的患者相匹配。在这些患者中，脑脊液β-内啡肽水平显著低于对照组（为正常值的40%）。脑脊液β-内啡肽水平的改变可能解释了约瑟夫病中发现的一些神经功能损害。
