《神經(jīng)病學(xué)》（英文）課件10.Diseases of the spinal cord

1、脊 髓 疾 病(Diseases of the spinal cord)Department of Neurology, The 2nd affiliated hospital, Harbin Medical UniversityDiseases of the spinal cordOutlineAcute myelitisCompressive myelopathySyringomyeliaSubacute combined degeneration of the spinal cordMotor neuron diseaseThe anatomy of the spinal cordExt

2、ernal components:The upper and lower boundary of the cord The capsule The spinal nervesThe relation between the cord and spine The anatomy of the spinal cord1. External components:The upper and lower bourn of the cordLength: 4245cm, It is the continuation of medulla(occipital foramen). The lower end

3、 forms terminal cone. It occupies 2/3 length of the spine.Lumbar enlargement: L1S2, cervical enlargement: C5T2Terminal filament end on the periosteum of the 1st coccygeal vertebraNextThe anatomy of the spinal cord1. External components:Spinal nerves:31pairs of nerves were sent out: C8, T12, L5, S5Th

4、e segmental distribution of cutaneous sensory innervation 見(jiàn)圖1 圖2The anatomic base of the spinal cord1. External componentsThe capsule: 圖例Three layer capsule: spinal dura mater, arachnoid, tenuis materThree spaces: epidural space: venous plexus and adipose subdural space subarachnoid space: CSFThe an

5、atomy of the spinal cord1. External components:The cord of C18 is one segment higher than the correspond spine respectively, T18 is two segment higher, T912 is three higher, the count-part of lumbar is the 1012th thoracic vertebra , sacral cord lies in the 12th thoracic vertebra and the 1st lumbar.T

6、he anatomy of the spinal cord2. Internal components:Gray matter: shape like H. Anterior horn: motor neuron. The fibers are anterior root Posterior horn: Lateral horn in the myelon of C8L2 and S24White matter: anterior column, lateral column, posterior columnThe anatomy of the spinal cord3.Blood supp

7、ly Anterior spinal artery: which supplies the anterior two-thirds of the cord 供應(yīng)脊髓全長(zhǎng)和脊髓橫斷面前2/3區(qū)域。Posterior spinal arteries: 供應(yīng)脊髓全長(zhǎng)及脊髓后柱、后索，脊髓橫斷面后1/3區(qū)域。Radicular arteries: 根動(dòng)脈與脊髓前、后動(dòng)脈吻合，構(gòu)成冠狀動(dòng)脈(coronary artery)環(huán)圍繞脊髓表面，分出無(wú)數(shù)小支供應(yīng)脊髓表面結(jié)構(gòu)及脊髓實(shí)質(zhì)外周部分的供血。C6、T9和L2處最大，T4和L1為交界。見(jiàn)圖Cord impairments-Clinical features

8、Motor deficits: spastic or flaccid paralysisDisorders of somatic sensation:見(jiàn)圖 posterior horn & root: 節(jié)段性感覺(jué)障礙 posterior horn :節(jié)段性分離性感覺(jué)障礙 anterior white commissure: 感覺(jué)分離現(xiàn)象 spinalthalamic tract:傳導(dǎo)束性感覺(jué)障礙:Sphincter dysfunctions: paruria and dysporia incontinence or retention (including urination and defe

9、cation) Autonomic nervous imbalanceCord impairments-Clinical features脊髓半側(cè)損害 (hemi-transverse impairments): Brown-Sequard syndrome脊髓橫貫損害(transverse impairments): spinal shock 1) 高頸髓(C14): cervical myelon 2) 頸膨大(C5T2): cervical enlargement 3) 胸髓(T3T12): thoracic myelon 4) 腰膨大(L1S2): lumbar myelon 5) 脊

10、髓圓錐(S35和尾節(jié)): terminal cone 6) 馬尾(cauda equina)判定脊髓病灶上界依據(jù)根性癥狀：最上位根痛、根性感覺(jué)缺失、節(jié)段性肌無(wú)力或肌萎縮部位。傳導(dǎo)束性感覺(jué)缺失平面：皮膚感覺(jué)支配三根定律。見(jiàn)圖 Cutaneous sensation is in a segmenttal pattern because of overlap there is no loss of sensation unless three adjacent segments are affected.判定脊髓病灶上界依據(jù)Tendon reflex change： C56, biceps and

11、brachioradialis C78, triceps L24, knee jerk S12, ankle自主神經(jīng)征： 反射性皮膚劃紋癥(reflective dermatography) 頭頸部立毛反射(pilomotor reflex) 阿司匹林發(fā)汗試驗(yàn)(Aspirins weating test) 中斷處，均為脊髓病變上界。脊髓橫貫性損害推算脊柱節(jié)段分三步走：脊髓病變上界=皮節(jié)1， 如皮節(jié)即感覺(jué)障礙平面，T7脊髓病變，上界T6。病變上界脊柱節(jié)段=脊髓病變上界n。 (C14, n=0; C58, n=1；T18, n=2；T912, n=3)本例病變上界脊柱節(jié)段T62=T4 。病變中心層

12、面脊柱節(jié)段=病變上界脊柱節(jié)段+1 (向下1個(gè)層面)。本例為T(mén)5 。急性脊髓炎 (Acute myelitis) Concept Acute transverse impairments caused by demyelination or necrosis on white matter of the myeline. Such as: myelitis following infection, myelitis following vaccine inoculation, demyelinative myelitis, necrosis myelitis, para-tumor myelit

13、is.Acute myelitis- Pathogeny unclear auto-immune reaction in association with a viral infection or vaccine inoculation 14 weeks ago. But there were no detective virus in nervous tissues, also no antibodies in CSF detected.Acute myelitis-Pathology Every segment may be involved, Range: T35, cervical o

14、r lumbar segment. Focal or transverse lesions, Findings under naked eyes: 脊髓腫脹，質(zhì)地變軟，軟膜充血滲出，切面灰白質(zhì)不清，變性。Findings under microscope: 髓內(nèi)血管擴(kuò)張、充血，血管周?chē)仔约?xì)胞侵潤(rùn)，神經(jīng)細(xì)胞腫脹，破裂，消失，白質(zhì)脫髓鞘，軸突變性，膠質(zhì)增生。Acute myelitis-Clinical featuresCharacters: 1. The youth & post adolescent, No difference between the two sex. Infection

15、or vaccine inoculation history Inducement of cold, overfatigue, trauma Acute myelitis-Clinical features2. Acute onset, gets to the peak after several hours or 23 days. The initial symptoms: Numbness and weakness Backache and girdle sensation The most frequent sites: T3-5, Acute myelitis- Clinical fe

16、aturesDyskinesia: spinal shock 24weeks upper motor neuron paralysisSensory disturbance: 損傷以下所有感覺(jué)障礙，恢復(fù)較運(yùn)動(dòng)慢。Autonomic nerves dysfunction: 早期尿潴留(無(wú)張力性神經(jīng)原性膀胱)。膀胱充盈300400 ml即自動(dòng)排s尿 (反射性神經(jīng)原性膀胱)。損害平面以下無(wú)汗、少汗、皮膚脫殼、角化。Acute myelitis-Clinical features3. Acute ascending myelitis: 危重型，起病急感覺(jué)平面數(shù)h或12d天上升至高頸髓，癱瘓迅速波及上肢

17、及延髓支配肌群，出現(xiàn)吞咽困難、構(gòu)音不清和呼吸肌麻痹而死亡。4. Demyelinative myelitis: 脊髓內(nèi)有兩個(gè)以上散在病灶，橫貫性損害多不完全。Acute myelitis- investigation1. Blood routine test: 急性期周?chē)准?xì)胞計(jì)數(shù)正?；蛏愿?。2. Examination of the CSF: normal pressure, normal or increased white cell count, slightly increased protein concentration, normal glucose and chloride,

18、 壓頸通暢. Acute myelitis -Investigation3.Electro-physiologic examination: VEP、SEP、MEP。4. Iconographic examination: MRI脊髓增粗、病變區(qū)輕度斑點(diǎn)狀或條索狀長(zhǎng)T1、T2?；謴?fù)期可正常，但也有脊髓MRI始終未顯異常者。影像學(xué)Acute myelitis- Diagnosis and differential diagnosisDiagnose: Acute onsetThe history of infection and vaccine inoculation The symptoms

19、of cord transverse impairmentThe examination of CSF Acute myelitis- Diagnosis and differential diagnosisDifferential diagnosis:(1) Acute epidural abscess 1) 有原發(fā)性化膿或感染病灶。 有時(shí)原發(fā)病灶常被忽視。2) 脊膜及神經(jīng)根刺激癥狀明顯 3) 腦膜刺激癥及全身中毒癥狀明顯。4) 脊柱劇烈壓痛及叩擊痛。 Acute myelitis- Diagnosis and differential diagnosisDifferential diagn

20、osis:(1) Acute epidural abscess5) 外周血象白細(xì)胞增加；6) 腦脊液白細(xì)胞輕度增加及蛋白含量增高明顯，脊腔梗阻。 7) CT掃描和MRI可幫助診斷Acute myelitis- Diagnosis and differential diagnosisDifferential diagnosis: (2) 脊柱結(jié)核 （tabes dorsalis）結(jié)核中毒癥狀；病變椎體發(fā)生塌陷，椎旁寒性膿腫形成，可壓迫脊髓，可出現(xiàn)急性橫貫性脊髓損害 (secondary compression of the cord)病變脊柱畸形 脊柱X線片 Acute myelitis- Di

21、agnosis and differential diagnosisDifferential diagnosis:(3)脊柱轉(zhuǎn)移性腫瘤(carcinomatous metastases) 老年人多見(jiàn)；發(fā)病較快；早期出現(xiàn)根性疼痛；脊髓受壓癥狀，如截癱和尿儲(chǔ)留等；X線平片、CT、MRI檢查人員可見(jiàn)椎體破壞，但無(wú)寒性膿腫的陰影；原發(fā)病灶 Acute myelitis- Diagnosis and differential diagnosisDifferential diagnosis:(4) 視神經(jīng)脊髓炎(neuromyelitis optica) A subtype of multiple scl

22、erosis, neuritis optica: decline of eyesight signs reflecting multiple focus, such as: nystagmus, diplopia, ataxia.Acute myelitis- Diagnosis and differential diagnosisDifferential diagnosis:(5)脊髓出血 （spinal hemorrhage）多由外傷或血管畸形引起發(fā)病時(shí)有劇烈背痛迅速出現(xiàn)肢體癱瘓和括約肌障礙腦脊液多含血脊髓CT掃描顯示出血部位有高密度影像脊髓造影或脊髓血管造影可發(fā)現(xiàn)血管畸形Acute my

23、elitis-Treatment The principle of treatment in the acute stage: 支持療法和對(duì)癥措施。Corticosteroids are often prescribedProper antibiotics to prevent infectionEmphasis of nutrition, Emphasis of nursing, prevent complications.Acute myelitis-Treatment Treatment in recovery phase 康復(fù)醫(yī)療，加強(qiáng)肢體鍛煉，促進(jìn)肌力恢復(fù)。脊髓壓迫癥 (compre

24、ssive myelopathy)Concept: 椎管內(nèi)占位性病變引起的脊髓受壓表現(xiàn)的一組疾病，為呈進(jìn)行性發(fā)展，最后導(dǎo)致不同程度脊髓橫貫損害和椎管阻塞。Cord compression-PathogenesisThe lesions :the lesions of the spine 脊膜病變； myeline and nerve roots changesCauses: tumors inflammations trauma of the spine 脊柱退性變 congenital anomaliesCord compression- Pathology and physiologyTh

25、e mechanism of compensation and the patho-physiologic changes:cord shift, ejection of CSFand blood no symptomsbone resorption: positive symptomsCord compression- Pathology and physiologyFactors influencing the compensation： 1. Speed of compression: 急性受壓迫，慢性脊髓壓迫2. The relation between the cord and th

26、e lesions: Intramedullary lesions: 髓內(nèi)的占位性病變直接侵犯神經(jīng)組織，癥狀出現(xiàn)較早.Compressive myelopathy- Pathology and physiologyExtramedullary intradural lesions: 首先從一側(cè)壓迫脊髓, 癥狀進(jìn)展緩慢Extramedallary extradural lesions: 由于硬脊膜的阻擋，對(duì)脊髓的壓迫作用相對(duì)輕微，癥狀往往發(fā)生在脊腔明顯梗阻之后 3. 根動(dòng)脈受壓 可引起分布區(qū)脊髓缺血，靜脈高壓， 局部 脊髓組織水腫以及血漿蛋白滲出。Compressive myelopathy C

27、linical features (1)Irritating and deficit symptoms of nerve roots (2)Sensation disturbance ; (3)Dyskinasia:extension spastic paralysis or paraplegia in flexionCompressive myelopathy Clinical features (4) Reflex disorders: (5) Sympotoms of autonomic nerves：sphincter dysfunction (6) 脊膜刺激癥狀：Compressiv

28、e myelopathy-Examination (1) Lumbar puncture：對(duì)診斷有重要意義。 Froin綜合征。 壓頸試驗(yàn)(Queckenstedt試驗(yàn)): block (2) Plain X ray of the spine: (3) Myelography: (4) CT or MRI：清晰顯示脊髓受壓影像Compressive myelopathy-Diagnosis(1) Make decision of cord compression:The focus develops from one side Radicular pain cord hemisection d

29、eveloped total cord transection Progressive process, and the symptoms aggravate insidiously and gradually.Compressive myelopathy-Diagnosis Queckenstedt test at lumbar puncture may reveal block CSF show Protein-cell count dissociation. If CSF is xanthochromic and become autocoagulation, it is called

30、Froin syndrom. MRI or myelography may show the lesion accurately.Compressive myelopathy-Diagnosis(2) Localize the segment of the lesion(3)Localize the cross section of the lesion:(intra or extradural).Compressive myelopathy-Diagnosis(4) 定性診斷：A rapid onset and progress usually occur in extradural abc

31、ess, metastatic carcinoma of the spine, or spine tubercle.The extramedullary and intradural primary tumor may be a slow onset, the process begin as radicular pain on one side, and develops partial compress, and then transection.Compressive myelopathy-Diagnosis(4) Determine the nature:The symptoms be

32、gin insidiously, with a fluctuating and prolonged course it may be thought as adhesion due to spinal arachnoiditis or cyst.Intramedullar tumor exhibit urination and defecation disorders in the early stage; no radicular pain; paralysis and pyramidal signs emerging lately, no obvious upper bound of th

33、e sensation deficit; and positive disassociated sensation disorder or sparing of saddle. 髓外硬膜內(nèi)病變 髓內(nèi)病變根性痛 多見(jiàn),明顯,早期出現(xiàn),且部位固定 少見(jiàn),不明顯感覺(jué)障礙 病灶以下,呈上行性進(jìn)展, 由病變水平向下發(fā)展, 上界明顯, 無(wú)分離性感覺(jué)障礙, 可有分離性感覺(jué)障礙, 感覺(jué)正常 鞍 區(qū)鞍區(qū)感覺(jué)障礙錐體束征 常早期出現(xiàn),顯著 晚期出現(xiàn),不顯著營(yíng)養(yǎng)障礙 無(wú) 有肌肉萎縮 無(wú)或局限 明顯,廣泛尿便障礙 晚期出現(xiàn) 早期出現(xiàn),嚴(yán)重,圓錐病變尤多見(jiàn),半離斷征 由半離斷發(fā)展為全離斷 少見(jiàn)CSF沖擊征有 無(wú)椎管梗阻

34、早期出現(xiàn),腰穿后加重 無(wú)或晚期出現(xiàn)CSF 黃變 (+),蛋白含量增高 (-)脊柱平片常有改變,如椎間孔擴(kuò)大, 較少陽(yáng)性發(fā)現(xiàn) 椎弓根變扁,椎弓根根距變寬碘油造影 杯口型梗阻,可有脊髓移位 梭性缺損,無(wú)脊髓移位髓外硬膜內(nèi)病變與硬膜外病變鑒別要點(diǎn)髓外硬膜內(nèi)病變 硬膜外病變 發(fā)病率 較多見(jiàn) 較少見(jiàn) 病程發(fā)展 較緩慢 較快 病變性質(zhì) 良性腫瘤多見(jiàn) 轉(zhuǎn)移瘤和惡性瘤多見(jiàn) 根痛 單側(cè)多見(jiàn) 雙側(cè)多見(jiàn) 體征 多不對(duì)稱(chēng), 脊髓半離斷損害 多對(duì)稱(chēng),脊髓損害癥狀較晚發(fā)生 脊髓沖擊征 多有 多無(wú) 體位變化痛 多有 多無(wú) 椎骨壓痛,叩擊痛多無(wú)多有 腦脊液改變 明顯,蛋白細(xì)胞分離不明顯X線平片 可見(jiàn)椎間孔擴(kuò)大,椎弓根變扁,椎

35、弓根根距變寬 可有椎體破壞 碘油造影 多呈深杯口型完全梗阻,脊髓變細(xì)明顯梗阻平面邊緣不銳利,呈刷狀外觀,脊髓輕度移位 Compressive myelopathy-TreatmentTreatment to the primary diseases(1)Surgery(2）Radiotherapy and/or chemotherapy Symptomatic measures Physical therapy to recover the paralyzed limb after surgery, and prevent complication.脊髓空洞癥 (syringomyelia)C

36、oncept 是一種緩慢進(jìn)行性的脊髓變性疾病。Syringomyelia is cavitations in the center of the spinal cord caused by various reasons, and the patients appear typical clinical symptoms. Typically, there is dissociated sensory loss, muscular atrophy and dystrophy at the level of the lesion. If the cavitations involves the

37、brain stem, it is called syringobulbia.Syringomyelia-Etiology and Pathogenesis尚未明確，歸納起來(lái)有下列幾種學(xué)說(shuō)：1.Hydrodynamic disorder of the CSF pathways 2. Blood circulation disorders 3. Congenital development anomaliesSyringomyelia-Clinical featuresMore male patients than female. Commonly appear at 2030.Slow onset and progression.Sensory disorders: 最常起自一側(cè)（頸膨大后角基底），或雙側(cè)（擴(kuò)展至前連合）呈（對(duì)稱(chēng)性）、節(jié)段性、分離性痛、溫度覺(jué)障礙，其圖形分布似“短上衣”形。 The“ central pain” may be possible: continuous burning-like pain in the sensory loss region.Syringomyelia-Clinical featuresSegmental