Edström L, Wroblewski R
Department of Neurology, Karolinska Hospital, Stockholm, Sweden.
Acta Neurol Scand. 1989 Nov;80(5):419-24. doi: 10.1111/j.1600-0404.1989.tb03903.x.
Seven patients with myotonic muscle dystrophy (MD), 6 with muscle dystrophy without myotonia (limb-girdle and facio-scapulo-humeral dystrophy) and 6 healthy volunteers were subjected to biopsy from the anterior tibial muscle (TA). Light microscopic examination of cryostat sections revealed pathological changes of different kind and degree--the occurrence of ring fibres and multiple central nuclei was the most consistent in advanced MD. X-ray microanalysis (XRMA) of single muscle fibres demonstrated the intracellular content of such elements as Na, Cl, K, Mg, S and P. The most conspicuous finding was the increase in Na and Cl and decrease in K demonstrated in MD. The highest levels of Na and Cl were found in ring fibres. Decrease in K was as high in structurally normal fibres of MD biopsies as in ring fibres and was also found in the muscle dystrophies without myotonia, but to a lesser degree than in MD. Thus, the decrease in K was not correlated to increase in Na and Cl. These changes in muscle fibre elemental content are discussed in relation to plasma membrane changes of erythrocytes in MD and especially the indications of disturbed anion channels.
选取7例强直性肌营养不良（MD）患者、6例无肌强直的肌营养不良患者（肢带型和面肩肱型肌营养不良）以及6名健康志愿者，对其胫前肌（TA）进行活检。对低温恒温器切片进行光学显微镜检查，发现了不同类型和程度的病理变化——在晚期MD中，环形纤维和多个中央核的出现最为一致。对单根肌纤维进行X射线微分析（XRMA），测定了钠、氯、钾、镁、硫和磷等元素的细胞内含量。最显著的发现是MD患者中钠和氯增加而钾减少。在环形纤维中发现钠和氯的含量最高。MD活检标本中结构正常的纤维中钾的减少程度与环形纤维相同，在无肌强直的肌营养不良中也有发现，但程度低于MD。因此，钾的减少与钠和氯的增加无关。本文结合MD患者红细胞质膜变化，特别是阴离子通道紊乱的迹象，讨论了肌纤维元素含量的这些变化。
