【摘要】：目的：通過回顧性分析，總結(jié)低鉀型周期性麻痹患者（HOKPP）的一般臨床特點(diǎn)及病理學(xué)改變特點(diǎn)。 方法：收集2004年12月～2014年1月在吉林大學(xué)第一醫(yī)院神經(jīng)內(nèi)科住院和門診臨床明確診斷為低鉀型周期性麻痹43例患者的臨床表現(xiàn)、實(shí)驗(yàn)室檢查，并對其中8例患者進(jìn)行了骨骼肌活檢，對其肌肉標(biāo)本分別進(jìn)行了組織化學(xué)和免疫組化的檢查。 結(jié)果： 1.臨床特點(diǎn)：男性37例（86.05%），女性6例（13.95%）。42例無家族史（97.68%）。平均發(fā)病年齡35.42±9.03歲。首發(fā)癥狀以四肢遲緩性癱瘓的27例（62.79%），主要以雙下肢無力的14例（32.56%），伴四肢酸痛15例（34.88%）。發(fā)病前有明顯誘因28例（65.12%）。43例腱反射均有不同程度減弱（100%），其中7例腱反射未引出（16.28%）。 2.輔助檢查：發(fā)作時血鉀中重度下降28例（65.12%），16例進(jìn)行了心肌酶學(xué)檢驗(yàn)，CK輕中度升高8例（50.00%）。32例進(jìn)行了甲功五項(xiàng)檢驗(yàn)，其中甲亢性低鉀型周期性麻痹（TPP）10例（31.25%）。9例進(jìn)行了肌電檢查，其中肌源性損害4例（44.44%）。對8例進(jìn)行肌肉活檢，4例（50.00%）可見管狀聚集現(xiàn)象。 結(jié)論： 1. HOKPP男性發(fā)病多于女性，多為散發(fā)。平均發(fā)病年齡在20～40歲。 2.首發(fā)癥狀多為四肢無力，以雙下肢為主。腱反射均有減弱或消失。超過60%的患者發(fā)病前有明顯誘因。 3. HOKPP發(fā)作時血鉀均有不同程度降低，，以中重度下降為主。心肌酶主要以CK升高為主，多為輕中度升高為主。TPP較為常見。肌電圖異常主要以肌源性改變?yōu)橹鳌?4. HOKPP骨骼肌病理學(xué)損害特點(diǎn)主要以管狀聚集較多見。
[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 鹵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.
【學(xué)位授予單位】：吉林大學(xué)
【學(xué)位級別】：碩士
【學(xué)位授予年份】：2014
【分類號】：R746.3

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