運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病課件

1、運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病1運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病 運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病2總論（疾病分類）總論（疾病分類）運(yùn)動(dòng)神經(jīng)元病運(yùn)動(dòng)神經(jīng)元病 上下運(yùn)動(dòng)神經(jīng)元受累上下運(yùn)動(dòng)神經(jīng)元受累 上運(yùn)動(dòng)神經(jīng)元受累上運(yùn)動(dòng)神經(jīng)元受累下運(yùn)動(dòng)神經(jīng)元受累下運(yùn)動(dòng)神經(jīng)元受累單肢肌萎縮單肢肌萎縮(hirayama病病) 副腫瘤運(yùn)動(dòng)神經(jīng)元病副腫瘤運(yùn)動(dòng)神經(jīng)元病 hopkins綜合癥綜合癥 后脊髓灰質(zhì)炎綜合癥后脊髓灰質(zhì)炎綜合癥多灶運(yùn)動(dòng)神經(jīng)病多灶運(yùn)動(dòng)神經(jīng)病 急性軸索運(yùn)動(dòng)神經(jīng)病急性軸索運(yùn)動(dòng)神經(jīng)病 卟啉病卟啉病 中毒中毒 運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病3感染感染 脊髓灰質(zhì)炎脊髓灰質(zhì)炎 cjdcjd嵌壓性神經(jīng)病嵌壓性神經(jīng)病糖尿病性肌萎縮糖尿病性肌萎縮 重癥肌無(wú)力重癥

2、肌無(wú)力肌病肌病n遠(yuǎn)端型肌病遠(yuǎn)端型肌病n強(qiáng)直性肌萎縮強(qiáng)直性肌萎縮n包涵體肌炎包涵體肌炎總論（疾病分類）總論（疾病分類）運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病4運(yùn)動(dòng)神經(jīng)元病運(yùn)動(dòng)神經(jīng)元病上下運(yùn)動(dòng)神經(jīng)元受累上下運(yùn)動(dòng)神經(jīng)元受累肌萎縮性側(cè)索硬化癥肌萎縮性側(cè)索硬化癥散發(fā)性散發(fā)性 遺傳性遺傳性 散發(fā)性運(yùn)動(dòng)神經(jīng)元病伴癡呆散發(fā)性運(yùn)動(dòng)神經(jīng)元病伴癡呆西太平洋西太平洋als 額葉癡呆繼發(fā)運(yùn)動(dòng)系統(tǒng)疾病額葉癡呆繼發(fā)運(yùn)動(dòng)系統(tǒng)疾病 副腫瘤運(yùn)動(dòng)神經(jīng)元病副腫瘤運(yùn)動(dòng)神經(jīng)元病 運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病5病變限于上運(yùn)動(dòng)神經(jīng)元病變限于上運(yùn)動(dòng)神經(jīng)元原發(fā)性側(cè)索硬化癥原發(fā)性側(cè)索硬化癥（primary lateral sclerosis）遺傳性痙攣性截癱遺傳性痙攣性截癱（he

3、reditary spastic paraparesis）運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病6病變限于下運(yùn)動(dòng)神經(jīng)元病變限于下運(yùn)動(dòng)神經(jīng)元進(jìn)行性肌肉萎縮癥進(jìn)行性肌肉萎縮癥成人發(fā)病的脊髓性肌萎縮癥成人發(fā)病的脊髓性肌萎縮癥性連鎖的球脊型肌萎縮癥性連鎖的球脊型肌萎縮癥單肢肌萎縮單肢肌萎縮(hirayama(hirayama病病) ) 后脊髓灰質(zhì)炎綜合癥后脊髓灰質(zhì)炎綜合癥遠(yuǎn)端下運(yùn)動(dòng)神經(jīng)元綜合癥（抗遠(yuǎn)端下運(yùn)動(dòng)神經(jīng)元綜合癥（抗gm1gm1抗體抗體 ）近端下運(yùn)動(dòng)神經(jīng)元綜合癥近端下運(yùn)動(dòng)神經(jīng)元綜合癥 asialo-gm1 igm asialo-gm1 igm 抗抗huhu抗體抗體 運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病7病變限于運(yùn)動(dòng)神經(jīng)病變限于運(yùn)動(dòng)神經(jīng)免

4、疫介導(dǎo)性免疫介導(dǎo)性多灶性運(yùn)動(dòng)神經(jīng)病多灶性運(yùn)動(dòng)神經(jīng)病急性運(yùn)動(dòng)軸索性神經(jīng)病急性運(yùn)動(dòng)軸索性神經(jīng)病副蛋白血癥性副蛋白血癥性硬化性骨髓瘤硬化性骨髓瘤 waldenstroms waldenstroms 巨球蛋白血癥巨球蛋白血癥 未明原因的單克隆丙球蛋白血癥未明原因的單克隆丙球蛋白血癥 腫瘤性：淋巴瘤腫瘤性：淋巴瘤代謝性代謝性卟啉病卟啉病 中毒性中毒性鉛，萊姆氏病鉛，萊姆氏病 運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病8嵌壓性神經(jīng)病嵌壓性神經(jīng)病正中神經(jīng)正中神經(jīng)腕管綜合征腕管綜合征（carpal tunnel syndrome）前前骨間神經(jīng)綜合癥骨間神經(jīng)綜合癥（anterior interosseous nerve syndrome

5、）旋前肌綜合癥旋前肌綜合癥（pronator syndrome）尺神經(jīng)尺神經(jīng)guyonguyon氏管氏管 橈神經(jīng)橈神經(jīng) 后骨間神經(jīng)綜合癥后骨間神經(jīng)綜合癥（posterior interosseous nerve syndrome ）橈管綜合癥橈管綜合癥運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病9遠(yuǎn)端型肌病遠(yuǎn)端型肌病welanderwelander肌?。ǔ扇送戆l(fā)肌病（成人晚發(fā)型）型） markesbery-griggs/uddmarkesbery-griggs/udd肌?。ǔ扇送戆l(fā)肌?。ǔ扇送戆l(fā)型）型） nonakanonaka肌?。ǔ扇嗽绨l(fā)肌?。ǔ扇嗽绨l(fā)型）型） miyoshimiyoshi肌?。ǔ扇嗽绨l(fā)肌病（成人早發(fā)

6、型）型） langlang肌病（成人早發(fā)肌?。ǔ扇嗽绨l(fā)型）型） 運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病10其它有遠(yuǎn)端肌無(wú)力的肌病其它有遠(yuǎn)端肌無(wú)力的肌病 結(jié)蛋白肌病結(jié)蛋白肌病強(qiáng)直性肌營(yíng)養(yǎng)不良強(qiáng)直性肌營(yíng)養(yǎng)不良 面面- -肩肩- -肱型肌營(yíng)養(yǎng)不良肱型肌營(yíng)養(yǎng)不良 emery-dreifussemery-dreifuss肌營(yíng)養(yǎng)不良肌營(yíng)養(yǎng)不良 炎性肌病炎性肌病 散發(fā)包涵體肌炎散發(fā)包涵體肌炎 多發(fā)性肌炎多發(fā)性肌炎 運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病11其它有遠(yuǎn)端肌無(wú)力的肌病其它有遠(yuǎn)端肌無(wú)力的肌病代謝性肌病代謝性肌病 酸性麥芽糖酶缺乏酸性麥芽糖酶缺乏 磷酸化酶磷酸化酶b b激酶缺乏激酶缺乏 脂質(zhì)沉積肌病脂質(zhì)沉積肌病 debrancher缺乏缺乏 先

7、天性肌病先天性肌病 桿狀體肌病桿狀體肌病中央軸空肌病中央軸空肌病中央核肌病中央核肌病運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病12各論（重點(diǎn)討論疾病）各論（重點(diǎn)討論疾?。゛lsals脊髓性肌萎縮脊髓性肌萎縮副腫瘤運(yùn)動(dòng)神經(jīng)元病副腫瘤運(yùn)動(dòng)神經(jīng)元病單肢肌萎縮單肢肌萎縮(hirayama病病)hopkins綜合癥綜合癥 后脊髓灰質(zhì)炎綜合癥后脊髓灰質(zhì)炎綜合癥多灶性運(yùn)動(dòng)神經(jīng)病多灶性運(yùn)動(dòng)神經(jīng)病急性運(yùn)動(dòng)軸索性神經(jīng)病急性運(yùn)動(dòng)軸索性神經(jīng)病運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病13el escorial criteria for diagnosis als運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病14運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病15進(jìn)行性肌萎縮癥進(jìn)行性肌萎縮癥廣泛性下運(yùn)動(dòng)神經(jīng)元綜合癥廣泛性下運(yùn)動(dòng)神經(jīng)

8、元綜合癥 大約大約20als病例最初表現(xiàn)為純下運(yùn)動(dòng)神經(jīng)病例最初表現(xiàn)為純下運(yùn)動(dòng)神經(jīng)元病，其中半數(shù)隨著病程發(fā)展出現(xiàn)元病，其中半數(shù)隨著病程發(fā)展出現(xiàn)umn臨床表現(xiàn)臨床表現(xiàn)肌無(wú)力：遠(yuǎn)近端均較顯著肌無(wú)力：遠(yuǎn)近端均較顯著，不對(duì)稱不對(duì)稱，常累及脊旁肌常累及脊旁肌和呼吸?。缓秃粑。患〖’d攣常見(jiàn)于下肢，夜間多發(fā)痙攣常見(jiàn)于下肢，夜間多發(fā) ；肌束顫肌束顫動(dòng)動(dòng)，無(wú)上運(yùn)動(dòng)神經(jīng)元征象無(wú)上運(yùn)動(dòng)神經(jīng)元征象 肌肉病理：群組萎縮肌肉病理：群組萎縮 電生理檢查電生理檢查：無(wú)傳導(dǎo)阻滯無(wú)傳導(dǎo)阻滯 運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病16成人發(fā)病的脊髓性肌萎縮癥成人發(fā)病的脊髓性肌萎縮癥sma ivsma iv型：型：7070常染色體隱性遺傳常染色體隱性遺傳

9、發(fā)病年齡發(fā)病年齡30306060歲，病程較歲，病程較alsals和和pmapma慢慢以下肢開(kāi)始的緩慢進(jìn)展性肢帶肌無(wú)力以下肢開(kāi)始的緩慢進(jìn)展性肢帶肌無(wú)力早期表現(xiàn)為行走困難、不能上樓、從坐早期表現(xiàn)為行走困難、不能上樓、從坐椅上站起費(fèi)力，很少累及球部肌肉和呼椅上站起費(fèi)力，很少累及球部肌肉和呼吸肌。吸肌。運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病17kennedyskennedys病病symptoms generally begin in older age (after age 30 years). it affects only malesprominent fasciculation around the tongue a

10、nd chin, muscle cramps, and bulbar and spinal lmn weakness, usually prominent in the pectoral girdle（上肢帶）（上肢帶）distributionmodest involvement of the sensory systemgynecomastia男子女性型乳房男子女性型乳房endocrine abnormalities include testicular atrophy, diabetes mellitus.x-linked recessive運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病18副腫瘤性運(yùn)動(dòng)神經(jīng)元病副腫瘤性

11、運(yùn)動(dòng)神經(jīng)元病近年發(fā)現(xiàn)：?jiǎn)慰寺「钡鞍籽Y和淋巴增殖性疾近年發(fā)現(xiàn)：?jiǎn)慰寺「钡鞍籽Y和淋巴增殖性疾病與運(yùn)動(dòng)神經(jīng)元病存在相關(guān)性病與運(yùn)動(dòng)神經(jīng)元病存在相關(guān)性副蛋白血癥：副蛋白血癥：monoclonal gammopathy of unknown monoclonal gammopathy of unknown significance, waldenstroms macroglobulinemia, significance, waldenstroms macroglobulinemia, osteosclerotic myelomaosteosclerotic myeloma淋巴增殖性疾?。毫馨驮鲋承约?/p>

12、病：hodgkins hodgkins 和和 non-hodgkins non-hodgkins lymphomalymphoma目前認(rèn)為其它腫瘤（目前認(rèn)為其它腫瘤（lung, colon or thyroid lung, colon or thyroid and insulinomaand insulinoma）與運(yùn)動(dòng)神經(jīng)元病無(wú)因果關(guān)系）與運(yùn)動(dòng)神經(jīng)元病無(wú)因果關(guān)系運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病19單肢肌萎縮單肢肌萎縮(hirayamas disease)(hirayamas disease) 成年早期，成年早期，1525歲發(fā)病歲發(fā)病 80為男性為男性 ，偶爾家族性發(fā)病偶爾家族性發(fā)病 肌無(wú)力常限于上肢肌無(wú)力常

13、限于上肢，c7, c8 & t1支配支配的肌群的肌群 ，肌束顫動(dòng)在病側(cè)肌束顫動(dòng)在病側(cè)(66%) 病程進(jìn)展病程進(jìn)展: 1至至3年，然后停止年，然后停止 感覺(jué)喪失：輕微感覺(jué)喪失：輕微 肌電圖肌電圖:慢性失神經(jīng)支配慢性失神經(jīng)支配 運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病20hopkins 綜合癥綜合癥發(fā)病年齡發(fā)病年齡: 113 歲歲發(fā)病發(fā)病急性哮喘發(fā)作后發(fā)病：潛伏期急性哮喘發(fā)作后發(fā)?。簼摲?18天天 輕微疼痛：四肢，頸或假性腦膜炎輕微疼痛：四肢，頸或假性腦膜炎 迅速發(fā)生肌無(wú)力迅速發(fā)生肌無(wú)力 肌無(wú)力肌無(wú)力 單肢；不對(duì)稱；近端可能重于遠(yuǎn)端單肢；不對(duì)稱；近端可能重于遠(yuǎn)端 感覺(jué)感覺(jué):正常正常 腦脊液細(xì)胞增多，蛋白升高腦脊

14、液細(xì)胞增多，蛋白升高 運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病21后脊髓灰質(zhì)炎綜合癥后脊髓灰質(zhì)炎綜合癥脊髓灰質(zhì)炎史，部分脊髓灰質(zhì)炎史，部分或完成恢復(fù)，或完成恢復(fù)，1515年后年后出現(xiàn)癥狀；出現(xiàn)癥狀；重新出現(xiàn)疲勞、肌肉重新出現(xiàn)疲勞、肌肉疼痛、肌萎縮和無(wú)力；疼痛、肌萎縮和無(wú)力；電生理檢查：大運(yùn)動(dòng)電生理檢查：大運(yùn)動(dòng)單位電位，纖顫電位單位電位，纖顫電位（可見(jiàn)于穩(wěn)定期）（可見(jiàn)于穩(wěn)定期）運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病22summary of clinical course of postpolio syndrome, beginning with acute paralysis and continuing through partial r

15、ecovery, stability, and new weakness (postpolio syndrome). overlapping boxes during periods of stability and postpolio syndrome indicate continuing subclinical instability of motor units despite clinically stable condition. postpolio syndrome occurs when motor neurons in stable postpolio state can n

16、o longer maintain all distal axonal sprouts 運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病23多灶性運(yùn)動(dòng)神經(jīng)病多灶性運(yùn)動(dòng)神經(jīng)病male female: 2 to 1onset most between 30 and 50 years weakness: 100%distal proximal (87%) asymmetric (94%)upper lower extremity (80%) muscle atrophy (80%) fasciculations: 25% to 50% cramps: 50%sensory: normal or minimal subjective symptoms 運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病24mmn electrophysiology motor conduction block: especially 50% reduction of proximal vs distal cmap amplitude axonal lossemg: no paraspinous