A48-year-oldwomanwasreferredforevaluationofprogressivegaitataxiaandstiffnessofbothlegsover6months.
48岁女性因进行性共济失调步态和双下肢僵硬感6月余来诊。
Shehadfirstnoticedgaitdisturbancewhilewalkingwithherhusbandandshereportedneitherpainnorsensorydeficits.Shehadahistoryofnon-Hodgkinlymphoma(NHL)treatedwithchemotherapyandradiotherapy20yearsago.Medicalhistoryfurtherrevealedosteoporosisand15pack-yearsofsmoking.
她最初跟丈夫散步时被注意到步态异常，无疼痛及感觉缺陷。非何杰金氏淋巴瘤史20年，曾行放疗和化疗。有骨质疏松病史，吸烟年包数15（年包=每天吸烟数/20*吸烟的年数）
Examinationrevealedsensorygaitataxiawithimpairedproprioceptionandslightlyreducedvibrationsenseonthelowerextremities(internalmalleolus5/8,patella7/8bilaterally,usingagraduatedRydel-Seiffertuningfork[Hz]withanarbitraryscalefrom0[minimumscore]to8[maximumscore],toquantifytheseverityofpallhypesthesia),spasticparaparesis(4/5forthehipflexorsontheMedicalResearchCouncilscale)withbriskreflexes,andbilateralBabinskisigns.Thepinpricksensationwasnormalinallextremitiesandbladderfunctionwasnotaffected.Theremainderofthegeneral,neurologic,andmentalexaminationwasunremarkable.Therewasnofamilyhistoryofneurologicdiseasesandthepatientdidnottakeanymedication.
体检发现双下肢感觉性共济失调，本体感觉障碍和音叉振动觉轻度减退（内踝分数5/8，双侧膝部分数7/8，使用HzRydel-Seiffer分级音叉，主观评分从0分至8分来定量振动觉减退的严重程度），痉挛性瘫痪（髋屈评分4/5，依据医学研究委员会量表），反射活跃，双巴氏征阳性。四肢针刺觉正常，膀胱功能未受累。余全身、神经系统和精神检查无异常。无神经系统疾病家族史，无服药史。
Questionforconsideration:
1.Whatisyourdifferentialdiagnosisatthisstage?
思考问题：
1.此时的鉴别诊断是什么？
SECTION2第二部分patientpresentedwithasubacuteonsetofaspasticataxicgaitwithimpairedproprioceptionofthelowerlimbs.TheconstellationofspasticparaparesisandbilateralBabinskisignssuggestsanuppermotorneuronsyndrome.Thetimecoursehintsatpossibleetiologies:neoplastic,metabolic,inflammatory,andneurodegenerativeprocessestypicallyhaveasubacuteonset.Therefore,ourdifferentialdiagnosisincludedchronicinflammatoryandneurodegenerativediseasesoftheCNS(e.g.,primaryprogressivemultiplesclerosis,myelitisinthecontextofsystemicinflammatorydiseaseslikesarcoidosisorSj?grensyndrome,hereditaryspasticparaplegia).CNSneoplasms,paraneoplasticsyndromes,infectiousdiseases(e.g.,humanT-lymphotropicvirus[HTLV],HIV,syphilis,Lymedisease),vascularmalformations(e.g.,arteriovenousfistula),andmetabolicdisordersoftheCNS(e.g.,vitaminB12deficiency)werealsoconsidered.
患者出现亚急性起病的痉挛性共济失调步态，伴下肢本体感觉障碍。痉挛性下肢瘫痪合并双侧巴氏征阳性提示上运动神经元受累。时间病程提示下列可能的病因：肿瘤、代谢、炎症和神经变性疾病通常亚急性起病。因此，鉴别诊断包括CNS慢性炎性和神经变性疾病（如原发性进行性多发性硬化、与系统性炎性疾病如结节病或干燥综合征相关的脊髓炎、遗传性痉挛性截瘫）。CNS肿瘤、副肿瘤综合征、感染性疾病（如人T-淋巴细胞病毒[HTLV]、HIV,梅毒，莱姆病），血管畸形（如动静脉瘘）和CNS代谢性疾病（如维生素B12缺乏）也要考虑。
Questionforconsideration:
1.Whatadditionaldiagnostictestswouldyouconsider?
思考问题：
1.还要做什么诊断性检查？
SECTION3第三部分Tonarrowdownthediagnosis,bloodandCSFexaminations,evokedpotentials,andMRIshouldbeperformed.
为缩小诊断范围，应行血和脑脊液检查、诱发电位和MRI检查。
Laboratorytestsincluding
