【醫(yī)學(xué)超級(jí)全】肌肉疾病課件

郭二十章肌肉疾病神經(jīng)病學(xué)教研室Valadononry.chAsposeslidesforNET4odientPEvaluationonly.CreatedwithAsposeSlidesforNET4.0dientProfilo71Copyright2019-2019AsposePtyL肌肉疾病-概述肌肉疾病:是指各種原因所致的骨骼肌疾病。常見(jiàn)的有周期性癱瘓肌營(yíng)養(yǎng)不良癥各種原因所致的肌炎■代謝性肌病■線(xiàn)粒體肌病等Valadononry.chAsposeslidesforNET4odientPEvaluationonly.CreatedwithAsposeSlidesforNET4.0dientProfilo71Copyright2019-2019AsposePtyL肌肉疾病口概述口周明咝癱瘓口進(jìn)行咝肌營(yíng)養(yǎng)不良癥口多發(fā)性肌炎口其他肌疾病Valadononry.chAsposeslidesforNET4odientPEvaluationonly.CreatedwithAsposeSlidesforNET4.0dientProfilo71Copyright2019-2019AsposePtyL二曠周期性癖瘓Valadononry.chAsposeslidesforNET4odientPEvaluationonly.CreatedwithAsposeSlidesforNET4.0dientProfilo71Copyright2019-2019AsposePtyL概念周期性癱瘓:以反復(fù)弛緩性肌無(wú)力或麻痹發(fā)作為特征的一組疾病,發(fā)作期血鉀異常,發(fā)作間期肌力正常?？谟蒀avae(1863)首先描述口分型:低鉀型、高鉀型和正常鉀型。口臨床上以低鉀型者最常見(jiàn)。Valadononry.chAsposeslidesforNET4odientPEvaluationonly.CreatedwithAsposeSlidesforNET4.0dientProfilo71Copyright2019-2019AsposePtyL病因及發(fā)病機(jī)制◆低鉀型周期性癱瘓(HoPP)為常染色體顯性遺傳性疾病,我國(guó)為散發(fā)。◆本病作為第一個(gè)離子通道病由Ptacek(1991)提出,認(rèn)為其與通道基因的突變有關(guān)。◆離子通道是貫穿于質(zhì)膜或細(xì)胞器膜的大分子蛋白質(zhì),是信號(hào)轉(zhuǎn)導(dǎo)的基本元件。Valadononry.chAsposeslidesforNET4odientPEvaluationonly.CreatedwithAsposeSlidesforNET4.0dientProfilo71Copyright2019-2019AsposePtyL病因及發(fā)病機(jī)制◆HoPP為骨骼肌鈣通道病◆高鉀型和正常血鉀型周期性癱瘓屬于骨骼肌鈉通道病Valadononry.chAsposeslidesforNET4odientPEvaluationonly.CreatedwithAsposeSlidesforNET4.0dientProfilo71Copyright2019-2019AsposePtyL病因及發(fā)病機(jī)制■Goldman公式:神經(jīng)肌肉細(xì)胞的膜電位Em=-61logK++001Na=-88mVK*e+0.01(Na*le■正常時(shí)興奮的閾值電位(Et)為-65mV低血鉀時(shí),Em-氏t值增大,此時(shí)閾刺激增加去極化困難,細(xì)胞應(yīng)激性下降,AP不易形成,N-M興奮性下降,出現(xiàn)肌無(wú)力高血鉀時(shí),Em-Et值縮小,但Em<E時(shí),肌細(xì)胞不能復(fù)極化而癱瘓Valadononry.chAsposeslidesforNET4odientPEvaluationonly.CreatedwithAsposeSlidesforNET4.0dientProfilo71Copyright2019-2019AsposePtyL病理口肌漿網(wǎng)的空泡化?？诩≡w維被圓形或卵圓形空泡分隔?？陔婄R下可見(jiàn)空泡由肌漿網(wǎng)和橫管系統(tǒng)擴(kuò)張形成。口病變晚期可有肌纖維變性Valadononry.chAsposeslidesforNET4odientPEvaluationonly.CreatedwithAsposeSlidesforNET4.0dientProfilo71Copyright2019-2019AsposePtyL臨床表現(xiàn)般情況:四季均可發(fā)病,男性多于女性,多為散發(fā);>遺傳史:常染色體顯性遺傳;發(fā)病年齡:任何年齡,以20-40歲多見(jiàn);誘發(fā)因素:飽餐大量碳水化合物、酗酒、受寒、過(guò)度疲勞、月經(jīng)前期、感染、創(chuàng)傷、情緒激動(dòng)等應(yīng)激反應(yīng)。Valadononry.chAsposeslidesforNET