中英遗传性痉挛性截瘫HSP合并

A28-year-oldwomanwithamedicalhistoryofasthma,diabetes,andmorbidobesitybrokeherrightleg3yearspriortopresentationrelatedtoslippingonicyground.Sheunderwentsurgicalinterventionforatibialfracture,followedbyalengthyrehabilitationprocess.Sheneverregainedherpreviouswalkingabilityandinfactfeltthatherbalancewasworsening.Shestumbledfrequently,trippingoversmallobstaclesorunevenground,andhad3fallsover6monthspriortopresentation.Shehadlostaboutpoundsafteragastricbandingproceduredoneshortlybeforeheraccident.Shedeniedweaknessorclumsinessinupperextremities,headaches,vertigo,lightheadedness,orlossofconsciousness.Therewasnohistoryoffever,chills,autoimmunedisorders,skinrash,jointpainorswellings,bloodclots,ormiscarriages.Norelevantfamilyhistoryofneurologicdisorderswaspresent.Onexamination,shewasnotedtohavearightfootdrop,increasedtoneandhyperreflexiainlowerextremitieswithapositiveBabinskisign,andseveralbeatsofankleclonusbilaterally.Hergaitwasslightlywidebasedandunsteadyandshehaddifficultieswithtandemgait.Highercognitivefunctions,speech,oculomotorexamination,strengthinupperextremitiesandtheleftleg,andsensoryexaminationincludingpinprick,lighttouch,temperature,vibration,andproprioceptionwasnormal.Shehadnormalcoordinationinupperextremitiesandmilddifficultieswithheeltoshintestingbilaterallyrelatedtospasticity.

28岁女性，既往有哮喘、糖尿病和过度肥胖史，3年前由于在冰面上滑倒后摔断右腿，行胫骨骨折手术，其后经历了漫长的康复锻炼，行走能力未完全恢复，并且自觉平衡能力逐渐恶化。她经常跌跌撞撞地走，被小障碍物或地面不平而绊倒，就诊前6个月内摔倒3次。在外伤前不久刚行胃结扎手术，体重减了磅。无上肢无力和活动笨拙，无头痛、眩晕、头重脚轻和意识丧失病史。无发热、寒战、自身免疫性疾病、皮疹、关节痛及肿胀、血栓或流产史。无神经系统疾病相关家族史。查体：右足下垂，下肢肌张力增高，腱反射亢进，巴氏征阳性，双侧踝阵挛阳性。轻度宽基底，不稳，踵趾步态困难。高级认知功能、言语、眼动检查、上肢和左下肢肌力、感觉检查包括针刺觉、轻触觉、温度觉、振动觉和本体感觉均正常。上肢共济稳准，双侧跟膝胫试验由于痉挛轻度异常。

Questionsforconsideration

1.Whatisthelocalizationofthispatient’sexaminationfindings?

2.Whatdifferentialdiagnosiswouldyouconsideratthispoint?

思考问题：

根据查体如何定位？

需要与哪些疾病鉴别？

LowerextremityspasticityandhyperreflexiawithapathologicBabinskisignandnosensorydeficitsischaracteristicofuppermotorneuronpathology.Intheabsenceofabnormalfindingsintheupperextremities,lowcervicalorthoracicmyelopathyaffectingprimarilymotorpathwaysmustberuledout.Ataxicgaitcanbeduetoasensoryataxia,whichisruledoutbytheabsenceofsensorydeficitsinourpatient.Theabsenceofappendicularataxiaoroculomotororspeechabnormalitiesmakesacerebellarpathologyunlikely.Astructuralabnormalityleadingto







































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