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 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1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 運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病2總論（疾病分類）運(yùn)動(dòng)神經(jīng)元病 運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病2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)系統(tǒng)疾病3感染 總論（疾病分類）運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病3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

2、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 運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病4運(yùn)動(dòng)神經(jīng)元病上下運(yùn)動(dòng)神經(jīng)元受累運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病4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 sclerosis）遺傳性痙攣性截癱（Hereditary spastic paraparesis）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)元原發(fā)性側(cè)索硬化癥運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病5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 asia

3、lo-GM1 IgM 抗Hu抗體 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)元進(jìn)行性肌肉萎縮癥運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病6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 運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病7病變限于運(yùn)動(dòng)神經(jīng)免疫介導(dǎo)性運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病7嵌壓性神經(jīng)病正中神經(jīng)腕管綜合征（Carpal Tunnel 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)

4、Guyon氏管 橈神經(jīng) 后骨間神經(jīng)綜合癥（posterior interosseous nerve syndrome ）橈管綜合癥運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病8嵌壓性神經(jīng)病正中神經(jīng)運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病8遠(yuǎn)端型肌病welander肌?。ǔ扇送戆l(fā)型） markesbery-griggs/udd肌病（成人晚發(fā)型） nonaka肌?。ǔ扇嗽绨l(fā)型） miyoshi肌病（成人早發(fā)型） lang肌?。ǔ扇嗽绨l(fā)型） 運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病9遠(yuǎn)端型肌病welander肌病（成人晚發(fā)型） 運(yùn)動(dòng)神經(jīng)系統(tǒng)其它有遠(yuǎn)端肌無力的肌病 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-dreifuss肌營(yíng)養(yǎng)不良 炎性肌病 散發(fā)包涵體

5、肌炎 多發(fā)性肌炎 運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病10其它有遠(yuǎn)端肌無力的肌病 結(jié)蛋白肌病運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病10其它有遠(yuǎn)端肌無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運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病11其它有遠(yuǎn)端肌無力的肌病代謝性肌病 運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病11各論（重點(diǎn)討論疾病）ALS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)病運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病12各論（重點(diǎn)討論疾?。〢LS運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病12El Escorial criteria for

6、 diagnosis ALS運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病13El Escorial criteria for diagn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14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)肌無力：遠(yuǎn)近端均較顯著，不對(duì)稱，常累及脊旁肌和呼吸肌；肌痙攣常見于下肢，夜間多發(fā) ；肌束顫動(dòng)，無上運(yùn)動(dòng)神經(jīng)元征象 肌肉病理：群組萎縮 電生理檢查：無傳導(dǎo)阻滯 運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病15進(jìn)行性肌萎縮癥廣泛性下運(yùn)動(dòng)神經(jīng)元綜合癥 運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病15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AL

7、S和PMA慢以下肢開始的緩慢進(jìn)展性肢帶肌無力早期表現(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。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SMA IV型：70常染色體隱性遺Kennedys病symptoms generally begin in older age (after age 30 years). It affects only malesProminent fasciculation around the tongue and chin, muscle cramps, and bulbar and spinal LMN weakness, usually p

8、rominent 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運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病17Kennedys病symptoms generally b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)動(dòng)神經(jīng)元病存在相關(guān)性副蛋白血癥：monoclonal gammop

9、athy 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)元病無因果關(guān)系運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病18副腫瘤性運(yùn)動(dòng)神經(jīng)元病近年發(fā)現(xiàn)：?jiǎn)慰寺「钡鞍籽Y和淋巴增殖性疾單肢肌萎縮(Hirayamas disease) 成年早期，1525歲發(fā)病 80為男性 ，偶爾家族性發(fā)病 肌無力常限于上肢，C7, C8 & T1支配的肌

10、群 ，肌束顫動(dòng)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 運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病19單肢肌萎縮(Hirayamas disease) 成年早期HOPKINS 綜合癥發(fā)病年齡: 113 歲發(fā)病急性哮喘發(fā)作后發(fā)病：潛伏期118天 輕微疼痛：四肢，頸或假性腦膜炎 迅速發(fā)生肌無力 肌無力 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 運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病20HOPKINS 綜合癥發(fā)病年齡: 113 歲運(yùn)動(dòng)神經(jīng)系統(tǒng)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、肌萎縮和無力；電生理檢查：大運(yùn)動(dòng)單位電位，

11、纖顫電位（可見于穩(wěn)定期）運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病21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)Summary of clinical course of postpolio syndrome, beginning with acute paralysis and continuing through partial recovery, stability, and new weakness (postpolio syndrome). Overlapping boxes during periods of stability and postpolio syndrome indicat

12、e continuing subclinical instability of motor units despite clinically stable condition. Postpolio syndrome occurs when motor neurons in stable postpolio state can no longer maintain all distal axonal sprouts 運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病22Summary of clinical course of 多灶性運(yùn)動(dòng)神經(jīng)病Male Female: 2 to 1Onset Most between 30 an

13、d 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23多灶性運(yùn)動(dòng)神經(jīng)病Male Female: 2 to 1運(yùn)MMN Electrophysiology Motor Conduction Block: Especially 50% Reduction of proximal vs distal CMAP amplitude Axonal LossEMG: No paraspinous denervation運(yùn)動(dòng)神經(jīng)系統(tǒng)疾病24MMN Electrophysiology Motor Co急性運(yùn)動(dòng)軸索性神經(jīng)病Prodrome Gastrointestinal: Diarrhea Positiv