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1、脊 髓 疾 病(Diseases of the spinal cord) Department of Neurology, The 2nd affiliated hospital, Harbin Medical UniversityDiseases of the spinal cord Outline Acute myelitis Compressive myelopathy Syringomyelia Subacute combined degeneration of the spinal cord Motor neuron diseaseThe anatomy of the spinal

2、cordExternal 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 cordlLength: 4245cm, It is the continuation of medulla(occipital foramen). The l

3、ower end forms terminal cone. It occupies 2/3 length of the spine.lLumbar enlargement: L1S2, cervical enlargement: C5T2lTerminal filament end on the periosteum of the 1st coccygeal vertebraThe anatomy of the spinal cord1. External components:Spinal nerves:l31pairs of nerves were sent out: C8, T12, L

4、5, S5The segmental distribution of cutaneous sensory innervation 見圖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: CS

5、FThe anatomy of the spinal cord1. External components:lThe cord of C18 is one segment higher than the correspond spine respectively, lT18 is two segment higher, lT912 is three higher, lthe count-part of lumbar is the 1012th thoracic vertebra ,l sacral cord lies in the 12th thoracic vertebra and the

6、1st lumbar.The 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 cord

7、3.Blood supply 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。見圖Cord impairments-Clini

8、cal featuresMotor deficits: spastic or flaccid paralysisDisorders of somatic sensation:見圖 posterior horn & root: 節(jié)段性感覺障礙 posterior horn :節(jié)段性分離性感覺障礙 anterior white commissure: 感覺分離現(xiàn)象 spinalthalamic tract:傳導(dǎo)束性感覺障礙:Sphincter dysfunctions: paruria and dysporia incontinence or retention (including urinat

9、ion and defecation) 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

10、 myelon 5) 脊髓圓錐(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、根性感覺缺失、節(jié)段性肌無力或肌萎縮部位。傳導(dǎo)束性感覺缺失平面：皮膚感覺支配三根定律。見圖 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,

11、 biceps and 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T

12、62=T4 。病變中心層面脊柱節(jié)段=病變上界脊柱節(jié)段+1 (向下1個(gè)層面)。本例為T5 。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-

13、tumor myelitis.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

14、, cervical or lumbar segment. Focal or transverse lesions, nFindings 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。nFindings under microscope: 髓內(nèi)血管擴(kuò)張、充血，血管周圍炎性細(xì)胞侵潤，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 s

15、ex. I n f e c t i o n o r v a c c i n e inoculation history I n d u c e m e n t o f c o l d , 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 freque

16、nt sites: T3-5, Acute myelitis- Clinical featuresDyskinesia: spinal shock 24weeks upper motor neuron paralysisSensory disturbance: 損傷以下所有感覺障礙，恢復(fù)較運(yùn)動(dòng)慢。Autonomic nerves dysfunction: 早期尿潴留(無張力性神經(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)。損害平面以下無汗、少汗、皮膚脫殼、角化。Acute myelitis-Clinical features3. Acute ascending

17、myelitis: 危重型，起病急感覺平面數(shù)h或12d天上升至高頸髓，癱瘓迅速波及上肢及延髓支配肌群，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: 急性期周圍血白細(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

18、concentration, normal glucose and chloride, 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 infe

19、ction and vaccine inoculation The symptoms 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 a

20、nd differential diagnosisDifferential diagnosis:(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 ( s e c o n d a r

21、y compression of the cord)病變脊柱畸形 脊柱X線片 Acute myelitis- Diagnosis and differential diagnosisDifferential diagnosis:(3)脊柱轉(zhuǎn)移性腫瘤(carcinomatous metastases) 老年人多見；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檢查人員可見椎體破壞，但無寒性膿腫的陰影；原發(fā)病灶 Acute myelitis-Acute myelitis- Diagnosis and differential Diagnosis and diff

22、erential diagnosisdiagnosisDifferential diagnosis:(4) 視神經(jīng)脊髓炎(neuromyelitis optica) A subtype of multiple sclerosis, neuritis optica: decline of eyesight signs reflecting multiple focus, such as: nystagmus, diplopia, ataxia.Acute myelitis-Acute myelitis- Diagnosis and differential Diagnosis and diffe

23、rential diagnosisdiagnosisDifferential diagnosis:(5)脊髓出血脊髓出血 （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 myelitis-Acute myelitis-TreatmentTreatment The principle of treatment in the acute stage: 支持療法和對(duì)癥措施。nCorticosteroids are often prescribednP

24、roper antibiotics to prevent infectionnEmphasis of nutrition, nEmphasis 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)。脊髓壓迫癥脊髓壓迫癥 (compressive (compressive myelopathy)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。Cor

25、d compression-Cord compression-PathogenesisPathogenesisThe 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 c o n g e n i t a l anomaliesCord compression- Cord compression- Pathology Pathology and physiologyand physiologyTh

26、e mechanism of compensation and the patho-physiologic changes:cord shift, ejection of CSFand blood no symptomsbone resorption: positive symptomsCord compression- Cord compression- Pathology Pathology and physiologyand physiologyFactors influencing the compensation： 1. Speed of compression: 急性受壓迫，慢性脊

27、髓壓迫2. The relation between the cord and the 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- Compressive myelopathy- Pathology Pathology and physiologyand 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

28、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 myelopathyCompressive myelopathy Clinical featuresClinical features (1)Irritating and deficit symptoms of nerve roots (2)Sensation disturbance ; (3)Dyskinasia:extension spastic paralysis or paraplegia in flexionCompressive

29、 myelopathyCompressive myelopathy Clinical featuresClinical features (4) Reflex disorders: (5) Sympotoms of autonomic nerves：sphincter dysfunction (6) 脊膜刺激癥狀：Compressive 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) Myelo

30、graphy: (4) CT or MRI：清晰顯示脊髓受壓影像Compressive myelopathy-Compressive myelopathy-DiagnosisDiagnosis(1) Make decision of cord compression:The focus develops from one side Radicular pain cord hemisection developed total cord transection Progressive process, and the symptoms aggravate insidiously and grad

31、ually.Compressive myelopathy-Compressive myelopathy-DiagnosisDiagnosis Queckenstedt test at lumbar puncture may reveal block CSF show Protein-cell count dissociation. If C S F i s x a n t h o c h r o m i c a n d b e c o m e autocoagulation, it is called Froin syndrom. MRI or myelography may show the

32、 lesion accurately.Compressive myelopathy-Compressive myelopathy-DiagnosisDiagnosis(2) Localize the segment of the lesion(3)Localize the cross section of the lesion:(intra or extradural).Compressive myelopathy-Compressive myelopathy-DiagnosisDiagnosis(4) 定性診斷：nA rapid onset and progress usually occu

33、r in extradural abcess, metastatic carcinoma of the spine, or spine tubercle.nThe 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-Compressive myelopathy-Diagno

34、sisDiagnosis(4) Determine the nature:The symptoms begin 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

35、 signs emerging lately, no obvious upper bound of the sensation deficit; and positive disassociated sensation disorder or sparing of saddle. 髓外硬膜內(nèi)病變髓外硬膜內(nèi)病變 髓內(nèi)病變髓內(nèi)病變根性痛根性痛 多見多見, ,明顯明顯, ,早期出現(xiàn)早期出現(xiàn), ,且部位固定且部位固定 少見少見, ,不明顯不明顯感覺障礙感覺障礙 病灶以下病灶以下, ,呈上行性進(jìn)展呈上行性進(jìn)展, , 由病變水平向下發(fā)由病變水平向下發(fā)展展, , 上界明顯上界明顯, , 無分離性感覺障礙無分

36、離性感覺障礙, , 可有可有分離性感覺障礙分離性感覺障礙, , 感覺正常感覺正常 鞍鞍 區(qū)鞍區(qū)感覺障礙區(qū)鞍區(qū)感覺障礙錐體束征錐體束征 常早期出現(xiàn)常早期出現(xiàn), ,顯著顯著 晚期出現(xiàn)晚期出現(xiàn), ,不不顯著顯著營養(yǎng)障礙營養(yǎng)障礙 無無 有有肌肉萎縮肌肉萎縮 無或局限無或局限 明顯明顯, ,廣泛廣泛尿便障礙尿便障礙 晚期出現(xiàn)晚期出現(xiàn) 早期出現(xiàn)早期出現(xiàn), ,嚴(yán)重嚴(yán)重, ,圓錐病變尤多見圓錐病變尤多見, ,半離斷征半離斷征 由半離斷發(fā)展為全離斷由半離斷發(fā)展為全離斷 少見少見CSFCSF沖擊征沖擊征有有 無無椎管梗阻椎管梗阻早期出現(xiàn)早期出現(xiàn), ,腰穿后加重腰穿后加重 無或晚期出現(xiàn)無或晚期出現(xiàn)CSF CSF 黃

37、變黃變 (+),(+),蛋白含量增高蛋白含量增高 (-)(-)脊柱平片脊柱平片常有改變常有改變, ,如椎間孔擴(kuò)大如椎間孔擴(kuò)大, , 較少陽性發(fā)現(xiàn)較少陽性發(fā)現(xiàn) 椎弓根變扁椎弓根變扁, ,椎弓根根距變寬椎弓根根距變寬碘油造影碘油造影 杯口型梗阻杯口型梗阻, ,可有脊髓移位可有脊髓移位 梭性缺損梭性缺損, ,無脊髓移位無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 較少見 病程發(fā)展 較緩慢 較快 病變性質(zhì) 良性腫瘤多見 轉(zhuǎn)移瘤和惡性瘤多見 根痛 單側(cè)多見 雙側(cè)多見 體征 多不對(duì)稱, 脊髓半離斷損害 多對(duì)稱,脊髓損害癥狀較晚發(fā)生 脊髓沖擊征 多有 多無 體位變化

38、痛 多有 多無 椎骨壓痛,叩擊痛多無多有 腦脊液改變 明顯,蛋白細(xì)胞分離不明顯X線平片 可見椎間孔擴(kuò)大,椎弓根變扁,椎弓根根距變寬 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-Compressive myelopathy-TreatmentTreatmentTreatment to the primary diseases(1)Surgery(2）Radiotherapy and/or chemotherapy Symptomatic measures Physical therapy t

39、o recover the paralyzed limb after surgery, and prevent complication.脊髓空洞癥脊髓空洞癥 (syringomyelia)(syringomyelia)Concept 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 disso

40、ciated sensory loss, muscular atrophy and dystrophy at the level of the lesion. If the cavitations involves the brain stem, it is called syringobulbia.Syringomyelia-Syringomyelia-Etiology and Etiology and PathogenesisPathogenesis尚未明確，歸納起來有下列幾種學(xué)說： 1.Hydrodynamic disorder of the CSF pathways 2. Blood

41、circulation disorders 3. Congenital development anomaliesSyringomyelia-Syringomyelia-Clinical Clinical featuresfeaturesMore 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)稱性）、節(jié)段性、分離性痛、溫度覺障礙，其圖形分布似“短上衣”形。 The“ central pai

42、n” may be possible: continuous burning-like pain in the sensory loss region.Syringomyelia-Clinical featuresSegmental muscle atrophy and fascicular twitching caused by involvement of anterior hornThe involvement of sympathetic center of the gray matter in the anterior-lateral column(The lateral horn

43、of (C8T2), leads to ipsilateral Horners sign.Syringomyelia-Clinical featuresDystrophy is also one of the main symptoms, such as Charcot joints.Presence of painless skin ulcers, scars, edema, hyperhidrosis; Resorption of the terminal phalanges is called Morvan sign: Bladder and rectum dysfunction in

44、the late stage.Syringobulbia is usually the continuation of syringomyelia.Other anomalies are also possible.Syringomyelia-examination The examination of CSF Myelography Delayed Myelin CT scan(DMCT） Magnetic Resonance Imaging（MRI)圖1 圖2 圖3 圖4Syringomyelia-ClassificationIntramedullary tumors Amyotrophi

45、c lateral sclerosis Others: cervical vertebra diseaseSyringomyelia-TreatmentNo specific treatment 1. Supporting treatment 2. 深部x線治療 3.Operation 總之，對(duì)較大的空洞，有主張用外科治療的趨勢(shì)，對(duì)較小空洞，仍采用保守療法。Subacute combined degeneration of the spine cord 由于維生素B12缺乏引起的神經(jīng)系統(tǒng)變性疾病主要累及脊髓后索、側(cè)索和周圍神經(jīng)，嚴(yán)重時(shí)大腦白質(zhì)及視神經(jīng)亦受累。 臨床表現(xiàn)為雙下肢深感覺障礙，感覺性共濟(jì)失調(diào)、痙攣性截癱及周圍神經(jīng)病變。Motor neuron disease(MND) 是一組病因未明，選擇性侵犯脊髓前角細(xì)胞、腦干運(yùn)動(dòng)神經(jīng)元、皮質(zhì)錐體細(xì)胞和錐體束的慢性進(jìn)行性變性疾病。臨床上兼有上和/或下運(yùn)動(dòng)神經(jīng)元受損體征，表現(xiàn)為肌無力、肌萎縮和錐體束征的不同組合，感覺和括約肌功能不受影響。 肌萎縮性側(cè)索硬化 進(jìn)行性脊肌萎縮癥 進(jìn)行性延髓麻痹 原發(fā)性側(cè)索硬化