中英084进行性脊髓综合征原发性CNS淋

SECTION1第一部分

A51-year-oldCaucasianmanpresentedwithcervicalpain,righthandweakness,andprogressivelydeterioratinggait.Onsetofsymptomsoccurred1monthbeforeadmissionwithcervicalpainthatworsenedduringneckflexion.Afewdayslaterhenoticedreduceddexterityandnumbnessofhisrighthand.Duringthefollowing3weeks,hisgaitbecameincreasinglyunstable.Additionally,hereportederectiledysfunctionandurinaryhesitancy.Noprevioustraumawasrecalled.Hismedicalandfamilyhistorywasunremarkableexceptforhypertensionthatwastreatedwithangiotensinconvertingenzymeinhibitors.

患者为51岁白人男性，以“颈部疼痛、右手乏力、进行性步态异常”为主诉就诊。入院前1个月，患者渐感颈部疼痛，屈颈动作会加重疼痛。几天后，患者感右手活动欠灵活并且有麻木感；在接下来的3周里，其步态越发不稳。此外，患者还有勃起功能障碍和尿等待。发病前无创伤史。除了用血管紧张素转换酶抑制剂治疗高血压之外，其用药史和家族史无特殊。

Onadmission,thepatientwasabletowalkunaided.Neurologicexaminationrevealedmildrighthemiparesisthatmainlyinvolvedthetriceps,theintrinsichandmuscles,andtheiliopsoas,aswellasanipsilateralreductionofproprioceptiveandvibratorysensation.PainandtemperaturesenseweredecreasedbelowT1-T2levelprominentlyontheleft.Tendonreflexeswerehyperactivebilaterallyparticularlyontheright.Inaddition,apositiveBabinskisignwasnotedontherightside.Oncranialnerveexamination,rightlidptosiswasobserved;therightpupilwassmallanddilatedpoorlyinthedark,indicatingHornersyndrome.

入院时，患者能独立行走。神经系统检查：右侧肢体轻瘫，主要累及股三头肌、手部内侧肌群以及髂腰肌；右侧肢体本体感觉和振动觉减退，左侧T1-T2水平以下痛温觉减弱；双侧肌腱反射亢进，右侧更明显；右侧巴彬斯基征阳性；颅神经检查可见右睑下垂，右侧瞳孔相对较小，在黑暗中扩张不良，提示霍纳综合征。

Questionsforconsideration:

1.Whatisthedifferentialdiagnosis?Howdoesthesubacuteprogressionofsymptomsnarrowthedifferential?

2.Whatisthenextstepinmanagementforthispatient?Whatadditionaltestingwouldyouorder?

问题思考：

1.鉴别诊断有哪些？如何根据亚急性进展的症状缩小鉴别诊断范围？

2.该患者接下来该如何处理？该进一步进行哪些检查？

TheneurologicexaminationfindingsresemblethoseofaBrown-Séquardsyndromeinvolvingtherighthemicordinthesuperiorcervicalregion.Consideringthelackofsupraspinalsemiology,Hornersyndromemostlikelyisattributedtoimpairmentofthedescendingsympathetictractinthelateralcolumnofthecervicalcord.Thepresenceofbilateralhyperreflexiaaswellasthepainandtemperaturesensationdeficitthatisrecognizedonbothsidesatalowerlevelimpliesthatothermedullaryregionsmaybeaffected.Thedifferentialdiagnosisincludesspondyloticmyelopathy,demyelinatingprocessessuchasmultiplesclerosis(MS),acutedisseminatedencephalomyelitis,neuromyelitisoptica(NMO)spectrumdisorders,andidiopathictransversemyelitis,primaryormetastaticspinalcordtumors,connectivetissuediseases(e.g.,systemiclupuserythematosus,Sj?grensyndrome),neurosarcoidosis,infections(e.g.,herpessimplexvirus2,varicella-zostervirus,cytomegalovirus,syphilis,HIV),spinalvascularmalformations,fibrocartilaginousembolism,andparaneoplasticsyndromes(tablee-1ontheNeurology?Websiteat







































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