脊 髓 疾 病ppt课件

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 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 lower 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, L5, S5The segmental distribution of cutaneous sensory innervation 见图1 图2The anatomic base of the spinal cord1. External componentsThe capsule: 图例Three layer capsule: spinal dura mater, arachnoid, tenuis materThree spaces: epidural space: venous plexus and adipose subdural space subarachnoid space: CSFThe 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 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 cord3.Blood supply Anterior spinal artery: which supplies the anterior two-thirds of the cord 供应脊髓全长和脊髓横断面前2/3区域。Posterior spinal arteries: 供应脊髓全长及脊髓后柱、后索，脊髓横断面后1/3区域。Radicular arteries: 根动脉与脊髓前、后动脉吻合，构成冠状动脉(coronary artery)环围绕脊髓表面，分出无数小支供应脊髓表面结构及脊髓实质外周部分的供血。C6、T9和L2处最大，T4和L1为交界。见图Cord impairments-Clinical featuresMotor deficits: spastic or flaccid paralysisDisorders of somatic sensation:见图 posterior horn & root: 节段性感觉障碍 posterior horn :节段性分离性感觉障碍 anterior white commissure: 感觉分离现象 spinalthalamic tract:传导束性感觉障碍:Sphincter dysfunctions: paruria and dysporia incontinence or retention (including urination and defecation) Autonomic nervous imbalanceCord impairments-Clinical features脊髓半侧损害 (hemi-transverse impairments): Brown-Sequard syndrome脊髓横贯损害(transverse impairments): spinal shock 1) 高颈髓(C14): cervical myelon 2) 颈膨大(C5T2): cervical enlargement 3) 胸髓(T3T12): thoracic myelon 4) 腰膨大(L1S2): lumbar myelon 5) 脊髓圆锥(S35和尾节): terminal cone 6) 马尾(cauda equina)判定脊髓病灶上界依据根性症状：最上位根痛、根性感觉缺失、节段性肌无力或肌萎缩部位。传导束性感觉缺失平面：皮肤感觉支配三根定律。见图 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据Tendon reflex change： C56, biceps and brachioradialis C78, triceps L24, knee jerk S12, ankle自主神经征： 反射性皮肤划纹症(reflective dermatography) 头颈部立毛反射(pilomotor reflex) 阿司匹林发汗试验(Aspirins weating test) 中断处，均为脊髓病变上界。脊髓横贯性损害推算脊柱节段分三步走：脊髓病变上界=皮节1， 如皮节即感觉障碍平面，T7脊髓病变，上界T6。病变上界脊柱节段=脊髓病变上界n。 (C14, n=0; C58, n=1；T18, n=2；T912, n=3)本例病变上界脊柱节段T62=T4 。病变中心层面脊柱节段=病变上界脊柱节段+1 (向下1个层面)。本例为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-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, cervical or lumbar segment. Focal or transverse lesions, nFindings under naked eyes: 脊髓肿胀，质地变软，软膜充血渗出，切面灰白质不清，变性。nFindings under microscope: 髓内血管扩张、充血，血管周围炎性细胞侵润，神经细胞肿胀，破裂，消失，白质脱髓鞘，轴突变性，胶质增生。Acute myelitis-Clinical featuresCharacters: 1. The youth & post adolescent, No difference between the two sex. 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 frequent sites: T3-5, Acute myelitis- Clinical featuresDyskinesia: spinal shock 24weeks upper motor neuron paralysisSensory disturbance: 损伤以下所有感觉障碍，恢复较运动慢。Autonomic nerves dysfunction: 早期尿潴留(无张力性神经原性膀胱)。膀胱充盈300400 ml即自动排s尿 (反射性神经原性膀胱)。损害平面以下无汗、少汗、皮肤脱壳、角化。Acute myelitis-Clinical features3. Acute ascending myelitis: 危重型，起病急感觉平面数h或12d天上升至高颈髓，瘫痪迅速波及上肢及延髓支配肌群，出现吞咽困难、构音不清和呼吸肌麻痹而死亡。4. Demyelinative myelitis: 脊髓内有两个以上散在病灶，横贯性损害多不完全。Acute myelitis- investigation1. Blood routine test: 急性期周围血白细胞计数正常或稍高。2. Examination of the CSF: normal pressure, normal or increased white cell count, slightly increased protein concentration, normal glucose and chloride, 压颈通畅. Acute myelitis -Investigation3.Electro-physiologic examination: VEP、SEP、MEP。4. Iconographic examination: MRI脊髓增粗、病变区轻度斑点状或条索状长T1、T2。恢复期可正常，但也有脊髓MRI始终未显异常者。影像学Acute myelitis- Diagnosis and differential diagnosisDiagnose: Acute onsetThe history of infection and vaccine inoculation The symptoms of cord transverse impairmentThe examination of CSF Acute myelitis- Diagnosis and differential diagnosisDifferential diagnosis:(1) Acute epidural abscess 1) 有原发性化脓或感染病灶。 有时原发病灶常被忽视。2) 脊膜及神经根刺激症状明显 3) 脑膜刺激症及全身中毒症状明显。4) 脊柱剧烈压痛及叩击痛。 Acute myelitis- Diagnosis and differential diagnosisDifferential diagnosis:(1) Acute epidural abscess5) 外周血象白细胞增加；6) 脑脊液白细胞轻度增加及蛋白含量增高明显，脊腔梗阻。 7) CT扫描和MRI可帮助诊断Acute myelitis- Diagnosis and differential diagnosisDifferential diagnosis: (2) 脊柱结核 （tabes dorsalis）结核中毒症状；病变椎体发生塌陷，椎旁寒性脓肿形成，可压迫脊髓，可出现急性横贯 性 脊 髓 损 害 ( s e c o n d a r y compression of the cord)病变脊柱畸形 脊柱X线片 Acute myelitis- Diagnosis and differential diagnosisDifferential diagnosis:(3)脊柱转移性肿瘤(carcinomatous metastases) 老年人多见；发病较快；早期出现根性疼痛；脊髓受压症状，如截瘫和尿储留等；X线平片、CT、MRI检查人员可见椎体破坏，但无寒性脓肿的阴影；原发病灶 Acute myelitis-Acute myelitis- Diagnosis and differential Diagnosis and differential diagnosisdiagnosisDifferential diagnosis:(4) 视神经脊髓炎(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 differential diagnosisdiagnosisDifferential diagnosis:(5)脊髓出血脊髓出血 （spinal hemorrhage）多由外伤或血管畸形引起发病时有剧烈背痛迅速出现肢体瘫痪和括约肌障碍脑脊液多含血脊髓CT扫描显示出血部位有高密度影像脊髓造影或脊髓血管造影可发现血管畸形Acute myelitis-Acute myelitis-TreatmentTreatment The principle of treatment in the acute stage: 支持疗法和对症措施。nCorticosteroids are often prescribednProper antibiotics to prevent infectionnEmphasis of nutrition, nEmphasis of nursing, prevent complications.Acute myelitis-Treatment Treatment in recovery phase 康复医疗，加强肢体锻炼，促进肌力恢复。脊髓压迫症脊髓压迫症 (compressive (compressive myelopathy)myelopathy)Concept: 椎管内占位性病变引起的脊髓受压表现的一组疾病，为呈进行性发展，最后导致不同程度脊髓横贯损害和椎管阻塞。Cord compression-Cord compression-PathogenesisPathogenesisThe lesions :the lesions of the spine 脊膜病变； myeline and nerve roots changesCauses: tumors inflammations trauma of the spine 脊柱退性变 c o n g e n i t a l anomaliesCord compression- Cord compression- Pathology Pathology and physiologyand physiologyThe 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: 急性受压迫，慢性脊髓压迫2. The relation between the cord and the lesions: Intramedullary lesions: 髓内的占位性病变直接侵犯神经组织，症状出现较早.Compressive myelopathy- Compressive myelopathy- Pathology Pathology and physiologyand physiologyExtramedullary intradural lesions: 首先从一侧压迫脊髓, 症状进展缓慢Extramedallary extradural lesions: 由于硬脊膜的阻挡，对脊髓的压迫作用相对轻微，症状往往发生在脊腔明显梗阻之后 3. 根动脉受压 可引起分布区脊髓缺血，静脉高压， 局部 脊髓组织水肿以及血浆蛋白渗出。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 myelopathyCompressive myelopathy Clinical featuresClinical features (4) Reflex disorders: (5) Sympotoms of autonomic nerves：sphincter dysfunction (6) 脊膜刺激症状：Compressive myelopathy-Examination (1) Lumbar puncture：对诊断有重要意义。 Froin综合征。 压颈试验(Queckenstedt试验): block (2) Plain X ray of the spine: (3) Myelography: (4) CT or MRI：清晰显示脊髓受压影像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 gradually.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 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) 定性诊断：nA rapid onset and progress usually occur 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-DiagnosisDiagnosis(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 signs emerging lately, no obvious upper bound of the sensation deficit; and positive disassociated sensation disorder or sparing of saddle. 髓外硬膜内病变髓外硬膜内病变 髓内病变髓内病变根性痛根性痛 多见多见, ,明显明显, ,早期出现早期出现, ,且部位固定且部位固定 少见少见, ,不明显不明显感觉障碍感觉障碍 病灶以下病灶以下, ,呈上行性进展呈上行性进展, , 由病变水平向下发由病变水平向下发展展, , 上界明显上界明显, , 无分离性感觉障碍无分离性感觉障碍, , 可有可有分离性感觉障碍分离性感觉障碍, , 感觉正常感觉正常 鞍鞍 区鞍区感觉障碍区鞍区感觉障碍锥体束征锥体束征 常早期出现常早期出现, ,显著显著 晚期出现晚期出现, ,不不显著显著营养障碍营养障碍 无无 有有肌肉萎缩肌肉萎缩 无或局限无或局限 明显明显, ,广泛广泛尿便障碍尿便障碍 晚期出现晚期出现 早期出现早期出现, ,严重严重, ,圆锥病变尤多见圆锥病变尤多见, ,半离断征半离断征 由半离断发展为全离断由半离断发展为全离断 少见少见CSFCSF冲击征冲击征有有 无无椎管梗阻椎管梗阻早期出现早期出现, ,腰穿后加重腰穿后加重 无或晚期出现无或晚期出现CSF CSF 黄变黄变 (+),(+),蛋白含量增高蛋白含量增高 (-)(-)脊柱平片脊柱平片常有改变常有改变, ,如椎间孔扩大如椎间孔扩大, , 较少阳性发现较少阳性发现 椎弓根变扁椎弓根变扁, ,椎弓根根距变宽椎弓根根距变宽碘油造影碘油造影 杯口型梗阻杯口型梗阻, ,可有脊髓移位可有脊髓移位 梭性缺损梭性缺损, ,无脊髓移位无脊髓移位髓外硬膜内病变与硬膜外病变鉴别要点髓外硬膜内病变 硬膜外病变 发病率 较多见 较少见 病程发展 较缓慢 较快 病变性质 良性肿瘤多见 转移瘤和恶性瘤多见 根痛 单侧多见 双侧多见 体征 多不对称, 脊髓半离断损害 多对称,脊髓损害症状较晚发生 脊髓冲击征 多有 多无 体位变化痛 多有 多无 椎骨压痛,叩击痛多无多有 脑脊液改变 明显,蛋白细胞分离不明显X线平片 可见椎间孔扩大,椎弓根变扁,椎弓根根距变宽 可有椎体破坏 碘油造影 多呈深杯口型完全梗阻,脊髓变细明显梗阻平面边缘不锐利,呈刷状外观,脊髓轻度移位 Compressive myelopathy-Compressive myelopathy-TreatmentTreatmentTreatment 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.脊髓空洞症脊髓空洞症 (syringomyelia)(syringomyelia)Concept 是一种缓慢进行性的脊髓变性疾病。 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 brain stem, it is called syringobulbia.Syringomyelia-Syringomyelia-Etiology and Etiology and PathogenesisPathogenesis尚未明确，归纳起来有下列几种学说： 1.Hydrodynamic disorder of the CSF pathways 2. Blood circulation disorders 3. Congenital development anomaliesSyringomyelia-Syringomyelia-Clinical Clinical featuresfeaturesMore male patients than female. Commonly appear at 2030.Slow onset and progression.Sensory disorders: 最常起自一侧（颈膨大后角基底），或双侧（扩展至前连合）呈（对称性）、节段性、分离性痛、温度觉障碍，其图形分布似“短上衣”形。 The“ central pain” may be possible: continuous burning-like pain in the sensory loss region.Syringomyelia-Clinical featuresSegmental 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 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 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)图1 图2 图3 图4Syringomyelia-ClassificationIntramedullary tumors Amyotrophic lateral sclerosis Others: cervical vertebra diseaseSyringomyelia-TreatmentNo specific treatment 1. Supporting treatment 2. 深部x线治疗 3.Operation 总之，对较大的空洞，有主张用外科治疗的趋势，对较小空洞，仍采用保守疗法。Subacute combined degeneration of the spine cord 由于维生素B12缺乏引起的神经系统变性疾病主要累及脊髓后索、侧索和周围神经，严重时大脑白质及视神经亦受累。 临床表现为双下肢深感觉障碍，感觉性共济失调、痉挛性截瘫及周围神经病变。Motor neuron disease(MND) 是一组病因未明，选择性侵犯脊髓前角细胞、脑干运动神经元、皮质锥体细胞和锥体束的慢性进行性变性疾病。临床上兼有上和/或下运动神经元受损体征，表现为肌无力、肌萎缩和锥体束征的不同组合，感觉和括约肌功能不受影响。 肌萎缩性侧索硬化 进行性脊肌萎缩症 进行性延髓麻痹 原发性侧索硬化