cord disorders脊髓疾病课件

Spinal cord disorders The department of Neurology, Xuanwu hospital, Capital University of Medical Sciences Words List（1） Vertebra 椎体 Cervical 颈的 Thoracic 胸的 Lumbar 腰的 Sacral 骶的 Coccygeal 尾骨的 Conus medullaris 脊髓圆锥 Cauda equina 脊髓马尾 Afferent fiber 传入纤维 Efferent fiber 传出纤维 Ganglion 神经节 Dermatome 皮区/皮节 Myotome 肌节 Gray matter 灰质 White matter 白质 Anterior white commissure 前连合 Fasciculus gracilis 薄束 Fasciculus cuneatus 楔束 Words List（2） Corticospinal tract 皮质脊髓束 Pyramidal tract 锥体束 Pyramidal decussation 锥体交叉 Axon 轴突 Synapse 突触 Dendrite 树突 Medial lemniscal system 内侧丘系 Proprioception 本体感觉 Lemniscal decussation 丘系交叉 Thalamus 丘脑 Vestibulospinal tract 前庭脊髓束 Rubrospinal tract 红核脊髓束 Reticulospinal system 网状脊髓系统 Spinothalamic tract 脊髓丘脑束 Spinocerebellar tract 脊髓小脑束 Ventral posterolateral nucleus 腹后外侧核（丘脑） Contents of The Lecture 一、Anatomy of spinal cord (SP) 二、Clinical manifestations of SP lesions 三、Diseases of the spinal cord 四、Questions for homework 五、Books recommended Spinal cord is part of central neural system. Begin at the end of the brainstem End at the first lumber vertibrate. Carry both incoming and outgoing messages between the brain and the rest of the body. It is also the center for reflexes. example Brain spinal cord hand station hot water Outline The anatomy of spinal cord The internal components External components The reflex of spinal cord The blood supply of spinal cord The clinical aspects of damaging spinal cord The anatomy of spinal cord External components: The upper and lower boundary of the cord The spinal nerves: C1-8, T1-12, L1-5, S1-5 The relation between the cord and spine How the Spinal cord Is Organized The spinal cord are contained in the spinal canal. Spinal canal is longer than the spinal cord. In most adults, the spine is composed of 26 vertebrae, which are the individual bones of the back. vertebrae protect the spinal cord. The upper and lower bound of the cord Length: 4245cm, It is the continuation of medulla(occipital foramen). The lower end forms terminal cone（圆锥）. It occupies 2/3 length of the spine. cervical enlargement: C5T2 Lumbar enlargement: L1S2, Terminal filament（终丝） end on the periosteum（骨膜） of the 1st coccygeal vertebra. External components Spinal nerves: 30pairs of nerves were sent out: C1-8, T1-12, L1-5, S1-5 The root in the front, the motor root, transmits impulses from the spinal cord to the muscles. The root in the back, the sensory root, carries sensory information (about touch, position, pain, and temperature) from the body to the spinal cord. External components The segmental distribution of cutaneous（皮肤的） sensory innervation Spinal segment Cervical cord C1-8 Thoracic cord T1-12 Lumbar cord L1-5 Sacral cord S1-5 Coccygeal 1 Cauda equina 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. Internal components Gray matter: shape like H. Anterior horn Posterior horn Lateral horn ： C8L2 and S24 Internal components White matter: anterior column, lateral column, posterior column Ascending transmit tracts Descending transmit tracts Ascending fibers Fasciculus gracilis（薄束） Fasciculus cuneatus（楔束） Spinothala mic tract（脊髓丘 脑束） Spinocereb ellar tract（脊髓小 脑束） Ascending fibers Fasciculus gracilis（薄束） contains fibers from sacral, lumbar, and lower thoracic dermatomes（皮肤感 觉分布区） Fasciculus cuneatus（楔束） carries fibers from upper thoracic and cervical dermatomes Function: convey well-localized sensation of fine touch, vibration, two-point discrimination, and proprioception (position sense) from the skin and joints. Ascending fibers Spinothalamic tract： lateral spinothalamic tract :carries information about pain and temperature anterior spinothalamic tract:carries information about crude touch Ascending fibers Spinocerebellar tract posterior spinocerebellar tract anterior spinocerebellar tract Coordinate the movement and posture of limbs Descending fibers corticospinal tract（皮质脊髓束） rubrospinal tract（红核脊髓束） vestibulospinal tract（前庭脊髓束 ） reticulospinal tract（网状脊髓束） tectospinal tract（顶盖脊髓束） medial longitudinal fasciculus（ 内侧纵束） Pyramidal system corticospinal tracts corticonuclear tracts Descending fibers corticospinal tract: lateral corticospinal tract anterior corticospinal tract Carries information for volitional movement （随 意运动）under direct cortical control Arteries of spinal cord Anterior spinal artery provide blood to anterior 2/3spinal cord Posterior spinal artery provide blood to posterior 1/3 spinal cord Radicular artery connect the arterior spinal artery with posterior spinal artery The reflex of spinal cord Reflexes: A reflex is an automatic response to a stimulus. For example, the lower leg jerks when the tendon below the kneecap is gently tapped with a small rubber hammer. The pathway that a reflex follows (reflex arc) is a complete circuit, without involvement of the brain. Cord impairments-Clinical features Motor deficits: Anterior horn or root damaging: low motor neuron (flaccid) paralysis, muscles fasciculations and fibrillations, no sense disturblance. Pyramidal tract damaging: spastic paralysis below the level of the lesion. Both anterior horn and Pyramidal tract damaging: combine damaging with upper and low motor neuron disturblances spinal shock（脊髓休克） flaccid paraplegia(迟缓性截瘫）, diminished or absent reflexes, without pyramidal sign, bladder and bowel are paralyzed, last 26 weeks. Cause: Loss of the tonic effect of corticospinal tract excitation on the the anterior horn cell. Lower-versus upper-motor-neuron lesions variableLower-Motor-Neuron Lesion Upper-Motor-Neuron Lesion WeaknessFlaccid paralysisSpastic paralysis Deep tendon reflexesDecreased or absentIncreased Babinskis reflexAbsentPresent AtrophyMay be markedAbsent or resulting from disuse Fasciculations and fibrillations May be presentabsent Sensation disturbances posterior horn But there were no detective virus in nervous tissues, also no antibodies in CSF detected. Pathology Findings under naked eyes: swelling（肿胀）, hyperemia（充血）, seeping（渗出）. Findings under microscope: vascular enlargement(血管扩张）and hyperemia, inflammated cell erosion（侵润） ; nerve cell swelling, disappearing， demyelinating（脱髓鞘） in white mater，axon degeneration（轴索变性） and gliosis（胶质细 胞增生） . Clinical features 1. Incidence have two peaks,1020, 3040 years. No difference between the gender. Infection or vaccine inoculation history ; Inducement of cold, overfatigue, trauma et al. Clinical features 2. Acute onset, gets to the peak after several hours or 23 days. The most frequent sites: T3-5. The initial symptoms: Numbness and weakness in the legs Backache and a belt-like tightness is felt around the chest or stomach at the level affected Clinical Manifestation Motor deficits: para- quadriparesis , spinal shock in acute phase spinal shock（脊髓休克） flaccid paraplegia(迟缓性截瘫）, diminished or absent reflexes, without pyramidal sign, bladder and bowel are paralyzed, last 26 weeks. Cause: Loss of the tonic effect of corticospinal tract excitation on the the anterior horn cell. Clinical Manifestation Sensation deficits: lose all of the sensation below the level of spinal cord Automatic nerve deficits: urinary, stool retention; dryness of skin; nails nutrition dysfunction Clinical features 3. Acute ascending myelitis: repaidly advanced, sensation level ascends to high cervical cord or medulla from several hours to 12d， paralysis in upper extremity and muscles contorlled by medulla ， Dysphagia（吞咽困难）、dysarthria（声音嘶哑） and dyspnea（呼吸困难）. Investigation 1. Blood routine test: wbc are normal or slightly increased . 2. CSF: normal pressure, Queckenstedt test is normal. white cell ： normal or increased, protein ： slightly increased glucose and chloride： normal Elevated IgG index Imaging Among the various imaging modalities, MR is the best technique due to its multiplanar capabilities and superior tissue sensitivity. It allows to answer the following questions: 1) is the spinal cord normal or not ? 2) is the lesion localized to the cord (focal or diffuse) or to the whole neuraxis (brain, nerve roots)?, 3) how is the signal, is there an enhancement or not? Imaging finding Acute lesions are hyperintense on T2-weighted images and hypointense on T1-weighted images and may demonstrate cord enlargement. Enhancement with gadolinium has been reported. Chronic lesions often show persistent hyperintense signal on T2 from gliosis. Severe cases may lead to cord atrophy. Acute myelitis Diagnose Acute onset The history of infection and vaccine inoculation The symptoms of cord transverse impairment The examination of CSF MRI Differential diagnosis: (1) Acute epidural abscess （急性硬膜外脓 肿） 1) Commonly have skin infection, severe fever; 2) Intense spinal root pain 3) Severe local tenderness in spine, 4)Rapidly advancing loss of neurological function below the lesion. 5) Blood wbc increased, 6) CSF wbc slight increased and protein increased, subaranoid space is blocked. 7) CT scan and MRI is helpful. Differential diagnosis (2) spine tuberclosis（脊柱结核） fever in afternoon, night sweat secondary compression of the cord malformation in spine X ray of spine showed destruction in exact segments of spine, abscess shadow Differential diagnosis (3) carcinomatous metastases（转移癌） more in old people； rapid course of disease ； nerve root pain in early stage； compression symptom, paraplegia , lossing sensation and urinating； X ray、CT、MRI: damaged in vertebrat，but no abscess shadow ； Idiocarcinoma Differential diagnosis (4) optica neuromyelitis（视神经脊髓炎） A subtype of multiple sclerosis, neuritis optica: decline of eyesight signs reflecting multiple focus, such as: nystagmus(眼球震颤）, diplopia（复视）, ataxia（共济 失调）. Differential diagnosis (5) spinal cord hemorrhage（脊髓出血 ） because of truma or AVM in spinal cord severe back pain paraplegia and difficulty urinating rapidly. secondary subarachnoid hemorrahge CT scan showed hyperdensitive in spinal cord DSA showed AVM Treatment Nursing is very important urethral catheterication for urinary retention artificial respiration for dyspnea Emphasis of nutrition, Emphasis of nursing, prevent complications. Drugs corticosteroid drugs antiviral drugs neurotrophic drugs Proper antibiotics to prevent infection Rehabilitative treatment passive activity, massage, intensive exercises. 脊髓压迫症 (compressive myelopathy) Concept: 椎管内占位性病变(occupy lesion)引 起的脊髓受压表现的一组疾病，呈进行 性发展，最后导致不同程度(in varying degree)脊髓横贯损害和椎管阻塞。 Pathogenesis Causes: tumors inflammations trauma of the spine 脊柱退行性变 (degeneration) congenital abnormality The warning signs of spinal cord compression Progressive feeling of stiffness or fatigue in the legs More or less rapidly progressive impairment of gait Urinary dysfunction Sensory disturbances in one or both legs Band-like abnormal sensation around the thorax or abdomen Back pain The distribution of the transmit tracts Pathology and physiology Factors influencing the compensation： 1. Speed of compression: acute or chronic 2. The relation between the cord and the lesions: Intramedullary or extramedullary Pathology and physiology Intramedullary lesions: 髓内的 占位性病变直接侵犯神经组织，症状出现 较早. Extramedullary 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。 Clinical features (1)Irritating and deficit symptoms of nerve roots(anterior/posterior root) (2)Sensation disturbance (spinalthalumas tract) ; (3)Dyskinasia(运动障碍）:extension spastic paralysis or paraplegia in flexion Clinical features (4) Reflex abnormal(anterior/posterior root,anterior horn,pyramidal tract, abdominal reflex (5)Sympotoms of autonomic nerves ： sphincter dysfunction (extramedullary, intramedullary) (6) 脊膜刺激症状：tendness, Examination (1) Lumbar puncture：对诊断有重要意义。 压颈试验(Queckenstedt试验): block (2) Plain X ray of the spine: bone destruction, widening of the spinal canal, destruction of the laminae or spinous processes, or a vertebral hemangioma（血管瘤） (3) CT or MRI：清晰显示脊髓受压影像， MRI is of crucial diagnostic value. Disk protrusion Disk protrusion Disk protrusion Compressive myelopathy Spinal tubercolosis Spinal cord tumour Spinal cord tumour Spinal cord tumour Diagnosis (1) Make decision of cord compression: The focus develops from one side Radicular pain， weakness, numbness in leg, cord hemisection developed total cord transection Progressive process, and the symptoms aggravate insidiously and gradually. Diagnosis Queckenstedt test at lumbar puncture may reveal block. CSF show Protein-cell count dissociation. MRI or myelography may show the lesion accurately. Diagnosis (2) Localize the segment of the lesion(extramedullary, intramedullary) . 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。 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) 阿司匹林发汗试验(Aspirins weating test) 中断处，均为脊髓病变上界。 Diagnosis (4) 定性诊断： A rapid onset and progress usually occur in extradural abcess, metastatic carcinoma of the spine, or spine tuberclosis. 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. Diagnosis The symptoms begin insidiously, with a fluctuating and prolonged course it may be thought as adhesion due to spinal arachnoiditis（ 蛛网膜炎） or cyst（囊肿）. Intramedullar tumor exhibit urination and defecation symptoms 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(鞍区回避） . 髓外硬膜内病变 髓内病变 根性痛 多见,明显,早期出现,且部位固定 少见,不明显 感觉障碍 病灶以下,呈上行性进展, 由病变水平向下发展, 上界明显, 无分离性感觉障碍, 可有分离性感觉障碍, 感觉正常 鞍 区鞍区感觉障碍 锥体束征 常早期出现,显著 晚期出现,不显著 营养障碍 无 有 肌肉萎缩 无或局限 明显,广泛 尿便障碍 晚期出现 早期出现,严重,圆锥病变尤多见, 半离断征 由半离断发展为全离断 少见 CSF冲击征有 无 椎管梗阻早期出现,腰穿后加重 无或晚期出现 CSF 黄变 (+),蛋白含量增高 (-) 脊柱平片常有改变,如椎间孔扩大, 较少阳性发现 椎弓根变扁,椎弓根根距变宽 碘油造影 杯口型梗阻,可有脊髓移位 梭性缺损,无脊髓移位 髓外硬膜内病变与硬膜外病变鉴别要点 髓外硬膜内病变变 硬膜外病变变 发发病率 较较多见见 较较少见见 病程发发展 较缓较缓 慢 较较快 病变变性质质 良性肿肿瘤多见见 转转移瘤和恶恶性瘤多见见 根痛 单侧单侧 多见见 双侧侧多见见 体征 多不对对称, 脊髓半离断损损害 多对对称,脊髓损损害症状较较晚发发 生 脊髓冲击击征 多有 多无 体位变变化痛 多有 多无 椎骨压压痛,叩击击痛多无多有 脑脑脊液改变变 明显显,蛋白细细胞分离不明显显 X线线平片 可见见椎间间孔扩扩大,椎弓根变变扁, 椎弓根根距变宽变宽 可有椎体破坏 碘油造影 多呈深杯口型完全梗阻,脊髓 变细变细 明显显 梗阻平面边缘边缘 不锐锐利,呈刷状 外观观,脊髓轻轻度移位 Treatment Treatment to the primary diseases (1)Surgery (2)Radiotherapyand/or chemotherapy Symptomatic measures Physical therapy to recover the paralyzed limb after surgery, and prevent complication. question What is the Brown-seguard