脊髓疾病ppt课件.ppt

脊 髓 疾 病(Diseases￿of￿the￿spinal￿cord)•Department of Neurology, • The 2nd affiliated hospital, • Harbin Medical UniversityDiseases￿of￿the￿spinal￿cord•Outline•Acute￿myelitis•Compressive￿myelopathy•Syringomyelia•Subacute￿combined￿degeneration￿of￿the￿spinal￿cord•Motor￿neuron￿diseaseThe￿anatomy￿of￿the￿spinal￿cord1.External￿components:•The￿upper￿and￿lower￿boundary￿of￿the￿cord￿•The￿capsule￿•The￿spinal￿nerves•The￿relation￿between￿the￿cord￿and￿spine￿The￿anatomy￿of￿the￿spinal￿cord1.￿External￿components:The￿upper￿and￿lower￿bourn￿of￿the￿cordlLength:￿42~45cm,￿It￿is￿the￿continuation￿of￿medulla(occipital￿foramen).￿The￿lower￿end￿forms￿terminal￿cone.￿It￿occupies￿2/3￿length￿of￿the￿spine.lLumbar￿enlargement:￿L1~S2,￿cervical￿enlargement:￿C5~T2lTerminal￿filament￿end￿on￿the￿periosteum￿of￿the￿1st￿coccygeal￿vertebraNextThe￿anatomy￿of￿the￿spinal￿cord1.￿External￿components:Spinal￿nerves:l31pairs￿of￿nerves￿were￿sent￿out:￿C8,￿T12,￿L5,￿S5•The￿segmental￿distribution￿of￿cutaneous￿sensory￿innervation￿见图1￿￿￿￿图2The￿anatomic￿base￿of￿the￿spinal￿cord1.￿External￿componentsThe￿capsule:￿￿￿￿￿￿￿图例•Three￿layer￿capsule:￿spinal￿dura￿mater,￿arachnoid,￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿tenuis￿mater•Three￿spaces:￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿epidural￿space:￿venous￿plexus￿and￿adipose￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿subdural￿space￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿subarachnoid￿space:￿CSFThe￿anatomy￿of￿the￿spinal￿cord1.￿External￿components:lThe￿cord￿of￿￿C1~8￿is￿one￿segment￿higher￿than￿the￿correspond￿spine￿respectively,￿lT1~8￿is￿two￿segment￿higher,￿lT9~12￿is￿three￿higher,￿lthe￿count-part￿of￿lumbar￿is￿the￿10~12th￿￿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￿C8~L2￿and￿S2~4•White￿matter:￿anterior￿column,￿lateral￿column,￿posterior￿columnThe￿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￿features•Motor￿deficits:￿spastic￿or￿flaccid￿paralysis•Disorders￿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￿imbalanceCord￿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(C1~4):￿cervical￿myelon￿￿￿2)￿颈膨大(C5~T2):￿cervical￿enlargement￿￿￿3)￿胸髓(T3~T12):￿thoracic￿myelon￿￿￿4)￿腰膨大(L1~S2):￿lumbar￿myelon￿￿￿5)￿脊髓圆锥(S3~5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：￿￿￿￿￿￿￿￿￿￿￿￿￿C5~6,￿biceps￿and￿brachioradialis￿￿￿￿￿￿￿￿￿￿￿￿￿￿C7~8,￿triceps￿￿￿￿￿￿￿￿￿￿￿￿￿￿L2~4,￿knee￿jerk￿￿￿￿￿￿￿￿￿￿￿￿￿￿S1~2,￿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￿￿￿￿￿(C1~4,￿ n=0;￿ C5~8,￿ n=1；T1~8,￿ n=2；T9~12,￿n=3)本例病变上界脊柱节段T62=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￿1~4￿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:￿ T3~5,￿ ￿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.￿￿￿￿￿￿￿￿Infection￿or￿vaccine￿inoculation￿history￿￿￿￿￿￿￿Inducement￿of￿cold,￿overfatigue,￿trauma￿Acute￿myelitis-Clinical￿features2.￿Acute￿onset,￿gets￿to￿the￿peak￿after￿several￿hours￿or￿2~3￿days.￿￿￿￿The￿initial￿symptoms:￿￿￿￿￿￿￿￿Numbness￿and￿weakness￿￿￿￿￿￿￿￿￿￿￿￿Backache￿and￿girdle￿sensation￿￿￿￿￿The￿most￿frequent￿sites:￿T3-5,￿Acute￿myelitis-￿Clinical￿features•Dyskinesia:￿spinal￿shock￿2~4weeks￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿upper￿motor￿neuron￿paralysis•Sensory￿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300~400￿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1~2d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￿onset•The￿ history￿ of￿ infection￿ and￿ vaccine￿inoculation￿•The￿ symptoms￿ of￿ cord￿ transverse￿impairment•The￿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）①结核中毒症状；②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 ￿(secondary￿compression￿of￿the￿cord)③病变脊柱畸形￿④脊柱X线片￿￿￿￿￿Acute￿myelitis-￿￿￿￿￿Diagnosis￿and￿differential￿diagnosisDifferential￿diagnosis:(3)脊柱转移性肿瘤(carcinomatous￿metastases)￿￿①老年人多见；②发病较快；③早期出现根性疼痛；④脊髓受压症状，如截瘫和尿储留等；⑤X线平片、CT、MRI检查人员可见椎体破坏，但无寒性脓肿的阴影；⑥原发病灶￿Acute￿myelitis-Acute￿myelitis-￿￿￿￿￿￿￿￿￿￿Diagnosis￿and￿differential￿diagnosisDiagnosis￿and￿differential￿diagnosisDifferential￿diagnosis:(4)￿视神经脊髓炎(neuromyelitis￿optica)￿￿￿￿①A￿subtype￿of￿multiple￿sclerosis,￿￿￿￿②neuritis￿optica:￿decline￿of￿eyesight￿￿￿￿③signs￿ reflecting￿ multiple￿ focus,￿ such￿as:￿￿￿￿￿￿￿￿￿￿￿nystagmus,￿diplopia,￿ataxia.Acute￿myelitis-Acute￿myelitis-￿￿￿￿￿￿￿￿￿￿Diagnosis￿and￿differential￿diagnosisDiagnosis￿and￿differential￿diagnosisDifferential￿diagnosis:(5)脊髓出血脊髓出血￿￿（（spinal￿￿hemorrhage）①多由外伤或血管畸形引起②发病时有剧烈背痛③迅速出现肢体瘫痪和括约肌障碍④脑脊液多含血⑤脊髓CT扫描显示出血部位有高密度影像⑥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脊髓脊髓压迫症迫症￿ ￿(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:￿￿￿￿￿①tumors￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿②inflammations￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿③trauma￿of￿the￿spine￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿④脊柱退性变￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿⑤congenital￿anomaliesCord￿compression-￿Cord￿compression-￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿Pathology￿and￿Pathology￿and￿physiologyphysiologyThe￿mechanism￿of￿compensation￿and￿the￿patho-physiologic￿changes:•cord￿shift,￿ejection￿of￿CSFand￿blood￿no￿symptoms•bone￿resorption:￿positive￿symptomsCord￿compression-￿Cord￿compression-￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿Pathology￿and￿Pathology￿and￿physiologyphysiology•Factors￿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￿and￿Pathology￿and￿physiologyphysiology•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。

Compressive￿myelopathyCompressive￿myelopathy￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿Clinical￿Clinical￿featuresfeatures￿￿￿(1)Irritating￿and￿deficit￿symptoms￿of￿nerve￿roots￿￿￿￿￿(2)Sensation￿disturbance￿;￿￿￿￿(3)Dyskinasia:extension￿ spastic￿ paralysis￿ or￿paraplegia￿in￿flexionCompressive￿myelopathyCompressive￿myelopathy￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿Clinical￿Clinical￿featuresfeatures￿￿￿￿￿(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￿CSF￿is￿xanthochromic￿and￿become￿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,￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿由病变水平向下发展由病变水平向下发展,￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿上界明显上界明显,￿无分离性感觉障碍无分离性感觉障碍,￿￿￿￿￿￿￿￿￿￿￿￿￿可有分离性感觉障碍可有分离性感觉障碍,￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿￿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,不显著不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CSF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￿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-TreatmentTreatment•Treatment￿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￿PathogenesisEtiology￿and￿Pathogenesis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￿anomaliesSyringomyelia-Syringomyelia-Clinical￿featuresClinical￿features•More￿male￿patients￿than￿female.￿•Commonly￿appear￿at￿20～30.•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￿features•Segmental￿ muscle￿ atrophy￿ and￿ fascicular￿ twitching￿caused￿by￿involvement￿of￿anterior￿horn•The￿involvement￿of￿sympathetic￿center￿of￿the￿gray￿matter￿in￿the￿anterior-lateral￿column(The￿lateral￿horn￿of￿(C8~T2),￿leads￿to￿ipsilateral￿Horner’s￿sign.Syringomyelia-Clinical￿features•Dystrophy￿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4Syringomyelia-Classification•Intramedullary￿tumors￿•Amyotrophic￿lateral￿sclerosis￿•Others:￿cervical￿vertebra￿diseaseSyringomyelia-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。