低钾型周期性麻痹的临床及病理研究  

Clinical and pathological study on hypokalemic periodic paralysis

作　　者：张卫清[1] 吕强[1] 张英谦[1] 张宏[1] 陈雪涛[1] 王健[1] 石进[1] 宋东林[1] 

机构地区：[1]空军总医院神经内科,北京100036

出　　处：《中华神经科杂志》2004年第3期203-205,共3页Chinese Journal of Neurology

摘　　要：目的　总结低血钾型周期性麻痹 (hypoPP)的临床表现及其肌肉组织病理学改变的特点。方法　回顾分析 4 2例hypoPP患者的主要临床表现及辅助检查 ,并对其中的 3例行肌肉组织病理学检查。结果　总体来看 ,血钾越低患者临床症状及其他辅助检查变化也就越重、治疗效果越差 ,但患者的肌无力和腱反射的变化与血钾的变化并不完全平行 ,肌力下降也可不对称 ,2例患者出现呼吸肌麻痹 ,其中 1例还同时伴有脑神经支配肌肉的受损 ,11例患者出现主观感觉障碍 ,5 4 5 % (18/ 33例 )的患者肌酸磷酸激酶 (CPK)增高。患者骨骼肌病理学检查中可见空泡样肌纤维及管状集合物。结论　血清钾水平与肌无力症状不完全平行 ;主观感觉异常、CPK增高、恢复时间较长与肌肉组织的病理改变有关 ;不典型低钾型周期性麻痹应与吉兰 巴雷综合征、多发性肌炎等进行鉴别 ,必要时应进行骨骼肌病理学检查。Objective To investigate the clinical and pathological features of hypokalemic periodic paralysis.Methods The clinical manifestations in 42 patients and pathological features of biopsied muscle specimens from 3 patients were analyzed and summarized.Results The lower was the serum kalemia level,the severer of the clinical symptoms and the worse was the cure effect. However,there is no association between the level of the serum kalemia and myoasthenia. The muscle weakness may not be symmetric. Respiratory paralysis was found in two patients and in one of them cranial nerve innervated muscles were affected. Subjective sensation disturbance was complained by 11 cases. Increased serum CPK was detected in 18 cases. In pathological examination,the rimmed vacuoles and tubular aggregates in muscle fibers were observed.Conclusion There should be no correlation between the level of serum kalemia and muscle weakness. Sensation disturbance,increased serum CPK and prolonged healing time might be related to the muscular pathological changes. Atypical hypokalemic periodic paralysis should be distinguished from Guillain-Barré syndrome and polymyositis. In some cases,pathological examination of biopsied muscles is needed.

关 键 词：低钾型周期性麻痹 病理研究 肌酸激酶 病理学 

分 类 号：R746[医药卫生—神经病学与精神病学]