醫(yī)藥-臨床-護(hù)理運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病ppt課件

1、歡迎進(jìn)入學(xué)習(xí) 課堂運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病 總論（疾病分類）運(yùn)動(dòng)神經(jīng)元病 上下運(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)元受累單肢肌萎縮(Hirayama病) 副腫瘤運(yùn)動(dòng)神經(jīng)元病 Hopkins綜合癥 后脊髓灰質(zhì)炎綜合癥多灶運(yùn)動(dòng)神經(jīng)病 急性軸索運(yùn)動(dòng)神經(jīng)病 卟啉病 中毒 感染 脊髓灰質(zhì)炎 CJD嵌壓性神經(jīng)病糖尿病性肌萎縮 重癥肌無(wú)力肌病遠(yuǎn)端型肌病強(qiáng)直性肌萎縮包涵體肌炎總論（疾病分類）運(yùn)動(dòng)神經(jīng)元病上下運(yùn)動(dòng)神經(jīng)元受累肌萎縮性側(cè)索硬化癥散發(fā)性 遺傳性 散發(fā)性運(yùn)動(dòng)神經(jīng)元病伴癡呆西太平洋ALS 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 病變限于上運(yùn)動(dòng)神經(jīng)元原發(fā)性側(cè)索硬化癥（Primary lateral sc

2、lerosis）遺傳性痙攣性截癱（Hereditary spastic paraparesis）病變限于下運(yùn)動(dòng)神經(jīng)元進(jìn)行性肌肉萎縮癥成人發(fā)病的脊髓性肌萎縮癥性連鎖的球脊型肌萎縮癥單肢肌萎縮(Hirayama病) 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GM1抗體 ）近端下運(yùn)動(dòng)神經(jīng)元綜合癥 asialo-GM1 IgM 抗Hu抗體 病變限于運(yùn)動(dòng)神經(jīng)免疫介導(dǎo)性多灶性運(yùn)動(dòng)神經(jīng)病急性運(yùn)動(dòng)軸索性神經(jīng)病副蛋白血癥性硬化性骨髓瘤 Waldenstroms 巨球蛋白血癥 未明原因的單克隆丙球蛋白血癥 腫瘤性：淋巴瘤代謝性卟啉病 中毒性鉛，萊姆氏病 嵌壓性神經(jīng)病正中神經(jīng)腕管綜合征（Carpal Tunnel

3、 syndrome）前骨間神經(jīng)綜合癥（Anterior interosseous nerve syndrome）旋前肌綜合癥（Pronator syndrome）尺神經(jīng)Guyon氏管 橈神經(jīng) 后骨間神經(jīng)綜合癥（posterior interosseous nerve syndrome ）橈管綜合癥遠(yuǎn)端型肌病welander肌病（成人晚發(fā)型） markesbery-griggs/udd肌?。ǔ扇送戆l(fā)型） nonaka肌?。ǔ扇嗽绨l(fā)型） miyoshi肌?。ǔ扇嗽绨l(fā)型） lang肌?。ǔ扇嗽绨l(fā)型） 其它有遠(yuǎn)端肌無(wú)力的肌病 結(jié)蛋白肌病強(qiáng)直性肌營(yíng)養(yǎng)不良 面-肩-肱型肌營(yíng)養(yǎng)不良 Emery-dreifu

4、ss肌營(yíng)養(yǎng)不良 炎性肌病 散發(fā)包涵體肌炎 多發(fā)性肌炎 其它有遠(yuǎn)端肌無(wú)力的肌病代謝性肌病 酸性麥芽糖酶缺乏 磷酸化酶B激酶缺乏 脂質(zhì)沉積肌病 Debrancher缺乏 先天性肌病 桿狀體肌病中央軸空肌病中央核肌病各論（重點(diǎn)討論疾?。〢LS脊髓性肌萎縮副腫瘤運(yùn)動(dòng)神經(jīng)元病單肢肌萎縮(Hirayama病)Hopkins綜合癥 后脊髓灰質(zhì)炎綜合癥多灶性運(yùn)動(dòng)神經(jīng)病急性運(yùn)動(dòng)軸索性神經(jīng)病El Escorial criteria for diagnosis ALS進(jìn)行性肌萎縮癥廣泛性下運(yùn)動(dòng)神經(jīng)元綜合癥 大約20ALS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)UMN臨床表現(xiàn)肌無(wú)力：遠(yuǎn)近端均較顯著，不

5、對(duì)稱，常累及脊旁肌和呼吸??；肌痙攣常見于下肢，夜間多發(fā) ；肌束顫動(dòng)，無(wú)上運(yùn)動(dòng)神經(jīng)元征象 肌肉病理：群組萎縮 電生理檢查：無(wú)傳導(dǎo)阻滯 成人發(fā)病的脊髓性肌萎縮癥SMA IV型：70常染色體隱性遺傳發(fā)病年齡3060歲，病程較ALS和PMA慢以下肢開始的緩慢進(jìn)展性肢帶肌無(wú)力早期表現(xiàn)為行走困難、不能上樓、從坐椅上站起費(fèi)力，很少累及球部肌肉和呼吸肌。Kennedys病symptoms generally begin in older age (after age 30 years). It affects only malesProminent fasciculation around the tongu

6、e and chin, muscle cramps, and bulbar and spinal LMN weakness, usually prominent in the pectoral girdle（上肢帶）distributionModest involvement of the sensory systemGynecomastia男子女性型乳房endocrine abnormalities include testicular atrophy, diabetes mellitus.X-linked recessive副腫瘤性運(yùn)動(dòng)神經(jīng)元病近年發(fā)現(xiàn)：?jiǎn)慰寺「钡鞍籽Y和淋巴增殖性疾病與運(yùn)

7、動(dòng)神經(jīng)元病存在相關(guān)性副蛋白血癥：monoclonal gammopathy of unknown significance, Waldenstroms macroglobulinemia, osteosclerotic myeloma淋巴增殖性疾病：Hodgkins 和 non-Hodgkins lymphoma目前認(rèn)為其它腫瘤（lung, colon or thyroid and insulinoma）與運(yùn)動(dòng)神經(jīng)元病無(wú)因果關(guān)系單肢肌萎縮(Hirayamas disease) 成年早期，1525歲發(fā)病 80為男性 ，偶爾家族性發(fā)病 肌無(wú)力常限于上肢，C7, C8 & T1支配的肌群 ，肌束顫動(dòng)

8、在病側(cè)(66%) 病程進(jìn)展: 1至3年，然后停止 感覺喪失：輕微 肌電圖:慢性失神經(jīng)支配 HOPKINS 綜合癥發(fā)病年齡: 113 歲發(fā)病急性哮喘發(fā)作后發(fā)?。簼摲?18天 輕微疼痛：四肢，頸或假性腦膜炎 迅速發(fā)生肌無(wú)力 肌無(wú)力 單肢；不對(duì)稱；近端可能重于遠(yuǎn)端 感覺:正常 腦脊液細(xì)胞增多，蛋白升高 后脊髓灰質(zhì)炎綜合癥脊髓灰質(zhì)炎史，部分或完成恢復(fù)，15年后出現(xiàn)癥狀；重新出現(xiàn)疲勞、肌肉疼痛、肌萎縮和無(wú)力；電生理檢查：大運(yùn)動(dòng)單位電位，纖顫電位（可見于穩(wěn)定期）Summary of clinical course of postpolio syndrome, beginning with acute p

9、aralysis and continuing through partial recovery, 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 moto

10、r neurons in stable postpolio state can no longer maintain all distal axonal sprouts 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 MMN Electrophysiology Motor Conduction Block: Especially 50% Reduction of proximal vs distal CMAP amplitude Axonal LossEMG: No paraspinous denervation急性運(yùn)動(dòng)軸索性神經(jīng)病Prodrome Gastrointestinal: Diarrhea Positive Campylobacter jejuni titers in 67%U