Stibler Helena, Edström Lars, Ahlbeck Karsten, Remahl Sten, Ansved Tor
Department of Neurology, Karolinska Hospital, Stockholm, Sweden.
Intensive Care Med. 2003 Sep;29(9):1515-27. doi: 10.1007/s00134-003-1894-9. Epub 2003 Aug 12.
To develop a rapid method to quantify myosin in muscle biopsy specimens from patients with critical illness myopathy (CIM).
Percutaneous muscle biopsy specimens at different stages of CIM were examined by light microscopy and transmission electron microscopy (TEM) and by horizontal pore gradient SDS electrophoresis (SDS-PAGE). The myosin/actin ratio was calculated densitometrically. Neurophysiological examinations were performed at various times during the course of CIM.
All patients were treated in intensive care units at Karolinska Hospital.
We studied 11 patients with CIM, 5 patients with axonal neuropathies, and 42 control individuals.
The histopathological changes included in all cases muscle fiber atrophy, degeneration, regeneration, nuclear changes, and reduction in myofibrillar ATPase activity in both type I and II fibers. In severely affected muscles fiber type differentiation was lost. On TEM preferential loss of thick filaments was the dominant finding. In some patients changes were present only in parts of the specimen. The neurophysiological examinations indicated myopathy in five patients and combined myopathy and neuropathy in five and suggested neuropathy in one. The SDS-pore PAGE used showed a technical variation of 4-5%. Quantitative results were obtained within 1 day and night. The mean value of the myosin/actin ratio in controls was 1.37+/-0.21 and in CIM patients 0.37+/-0.17, without overlapping with the control values.
Considering the diagnostic difficulty using morphological and neurophysiological methods, especially in early stages of CIM, we suggest including SDS-pore PAGE to determine the myosin/actin ratio for rapid diagnosis of CIM.
开发一种快速方法来定量测定危重病性肌病(CIM)患者肌肉活检标本中的肌球蛋白。
对处于CIM不同阶段的经皮肌肉活检标本进行光学显微镜、透射电子显微镜(TEM)检查以及水平孔梯度SDS电泳(SDS-PAGE)。通过光密度法计算肌球蛋白/肌动蛋白比率。在CIM病程的不同时间进行神经生理学检查。
所有患者均在卡罗林斯卡医院的重症监护病房接受治疗。
我们研究了11例CIM患者、5例轴索性神经病患者和42名对照个体。
组织病理学变化在所有病例中均包括肌纤维萎缩、变性、再生、核变化以及I型和II型纤维中肌原纤维ATP酶活性降低。在严重受累的肌肉中,纤维类型分化消失。在TEM下,主要发现是粗肌丝优先丢失。在一些患者中,仅标本的部分区域出现变化。神经生理学检查表明5例患者存在肌病,5例患者存在肌病和神经病合并情况,1例患者提示存在神经病。所使用的SDS孔PAGE显示技术变异为4-5%。在1个昼夜内获得定量结果。对照组中肌球蛋白/肌动蛋白比率的平均值为1.37±0.21,CIM患者为0.37±0.17,与对照值无重叠。
考虑到使用形态学和神经生理学方法进行诊断存在困难,尤其是在CIM的早期阶段,我们建议纳入SDS孔PAGE来测定肌球蛋白/肌动蛋白比率以快速诊断CIM。