低钾型周期性麻痹的临床特点及肌肉病理研究

发布时间：2019-03-09 20:10

【摘要】：目的：通过回顾性分析，总结低钾型周期性麻痹患者（HOKPP）的一般临床特点及病理学改变特点。方法：收集2004年12月～2014年1月在吉林大学第一医院神经内科住院和门诊临床明确诊断为低钾型周期性麻痹43例患者的临床表现、实验室检查，并对其中8例患者进行了骨骼肌活检，对其肌肉标本分别进行了组织化学和免疫组化的检查。结果： 1.临床特点：男性37例（86.05%），女性6例（13.95%）。42例无家族史（97.68%）。平均发病年龄35.42±9.03岁。首发症状以四肢迟缓性瘫痪的27例（62.79%），主要以双下肢无力的14例（32.56%），伴四肢酸痛15例（34.88%）。发病前有明显诱因28例（65.12%）。43例腱反射均有不同程度减弱（100%），其中7例腱反射未引出（16.28%）。 2.辅助检查：发作时血钾中重度下降28例（65.12%），16例进行了心肌酶学检验，CK轻中度升高8例（50.00%）。32例进行了甲功五项检验，其中甲亢性低钾型周期性麻痹（TPP）10例（31.25%）。9例进行了肌电检查，其中肌源性损害4例（44.44%）。对8例进行肌肉活检，4例（50.00%）可见管状聚集现象。结论： 1. HOKPP男性发病多于女性，多为散发。平均发病年龄在20～40岁。 2.首发症状多为四肢无力，以双下肢为主。腱反射均有减弱或消失。超过60%的患者发病前有明显诱因。 3. HOKPP发作时血钾均有不同程度降低，，以中重度下降为主。心肌酶主要以CK升高为主，多为轻中度升高为主。TPP较为常见。肌电图异常主要以肌源性改变为主。 4. HOKPP骨骼肌病理学损害特点主要以管状聚集较多见。
[Abstract]:Objective: to summarize the clinical and pathological features of (HOKPP) in patients with hypokalemic periodic paralysis by retrospective analysis. Methods: from December 2004 to January 2014, 43 patients with hypokalemic periodic paralysis were diagnosed as hypokalemic periodic paralysis in the Department of Neurology, first Hospital of Jilin University. Skeletal muscle biopsies were performed in 8 of them, and their muscle specimens were examined by histochemistry and immunohistochemistry, respectively. Results: 1. Clinical features: male 37 cases (86.05%), female 6 cases (13.95%), 42 cases without family history (97.68%). The mean age of onset was 35.42 卤9.03 years old. The first symptom was paralysis of limbs in 27 cases (62.79%), weakness of both limbs in 14 cases (32.56%) and pain of limbs in 15 cases (34.88%). There were 28 cases (65.12%) with obvious inducement before onset and 43 cases (100%) with weak tendon reflex. Among them, 7 cases (16.28%) had no tendon reflex. 2. Auxiliary examination: 28 cases (65.12%) had moderate and severe decrease of serum potassium, 16 cases had myocardial enzyme test, 8 cases (50.00%) had mild to moderate elevation of CK, 32 cases had five tests of thyroid function. Among them, 10 cases (31.25%) of hyperthyroidism hypokalemic periodic paralysis were examined by electromyography (EMG), of which 4 cases (44.44%) were myogenic damage. Tubular aggregation was observed in 4 cases (50.00%) after muscle biopsy in 8 cases. Conclusions: 1. The incidence of HOKPP in men is more than that in women, and most of them are sporadic. The average age of onset was 20 to 40. 2. Most of the initial symptoms were weakness of extremities, and the main symptoms were lower extremities. Tendon reflex was weakened or disappeared. More than 60% of patients had significant predisposing factors before onset. 3. During the onset of HOKPP, serum potassium decreased to different degrees, mainly in moderate and severe degree. The myocardial enzymes were mainly increased in CK, most of them were mild to moderate elevation, which was more common. Electromyography (EMG) abnormalities were mainly caused by myogenic changes. 4. The pathological damage of HOKPP skeletal muscle was mainly characterized by tubular aggregation.
【学位授予单位】：吉林大学
【学位级别】：硕士
【学位授予年份】：2014
【分类号】：R746.3

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