SECTION1第一部分

A47-year-oldmanpresentedwitha5-yearhistoryofslowlyprogressivegaitdisorderwithclumsinessandunsteadinessduringwalking,aswellasstiffnessandcrampingpaininhislegs.Healsohaderectiledysfunctionandnocturia.Hedeniedsensorydeficitsandotherfocalneurologicorsystemicsymptoms.Hehadamedicalhistoryofhypogonadism,diagnosed1yearbeforetheonsetofthegaitdisorder,attributedtoabilateralorchiectomyduetoatesticulartumor,performedelsewherewhenhewas37.HewasreceivingIMtestosteroneinjectionsevery3weeks.Hisfamilymedicalhistoryincludedpescavusinhismotherandsiblings,otherwiseunremarkable.

患者，男性，47岁，行走时出现慢性进行性步态笨拙和行走不稳及下肢僵硬、痉挛性腿痛5年。他还有勃起功能障碍和夜尿症。他否认感觉障碍和其他局灶性神经系统或全身症状。当他37岁时在其他地方曾诊断“睾丸肿瘤”并行“双侧睾丸切除术”，下肢行走不稳前1年出现“性腺功能减退症”，接受每3周1次的睾酮注射治疗。他的家族史包括他的母亲和兄弟姐妹患有弓形足，其它无特殊意义。

Neurologicexaminationrevealedawide-basedspasticgaitwithpositiveRombergsign.Cognitionandcranialnerveexaminationwerenormal.Strengthwas4/5inbothiliopsoas,and4+/5intheremainingmusclesofthelowerlimbs,withincreasedmuscletone.Deeptendonreflexes(DTR)wereverybrisk,withbilateralAchillesclonus,andbilateralBabinskisigns.Vibrationsensationwasdecreasedinlowerlimbs,andjointpositionsensewaslostinthetoes.Therestoftheexaminationwasnormal.

神经系统检查发现患者宽基底痉挛步态，罗姆伯格征阳性，认知和颅神经检查正常。髂腰肌肌力为4/5级，其余下肢肌肉肌力为4/5+级，伴肌张力增高。深腱反射非常活跃，伴双侧踝阵挛，双侧巴氏征阳性。下肢振动觉减退，脚趾关节位置觉消失，其余的检查正常。

Questionsforconsideration:

1.Whatisthesyndromicdiagnosis?

2.Whatisthedifferentialdiagnosisatthisstage?

考虑的问题：

1.该症候群的诊断是什么？

2.现阶段的鉴别诊断是什么？

SECTION2第二部分

Thepatternofweaknessandgaitdisturbanceisconsistentwithachronicspasticparaparesissyndrome,beginningintheadulthood,andprogressingsteadily.Thesyndromeincludesuppermotorneuronsigns(increasedmuscletone,hyperactiveDTR,andBabinskisigns)anddeepsensorydisturbances(sensoryataxia)probablyinvolvingthedorsalascendingcolumns.TheinvolvementofsensoryperipheralnervesisunlikelybecauseofthehyperactiveDTR.Therefore,thesignsandsymptomssuggestadiseaseofthespinalcord.Althoughinfrequent,bilateraldamageofthefrontoparietalcortex(e.g.,parasagittalmeningioma)maycauseaslowlyprogressivespasticparaparesissyndromewithsensorysymptomsandsphincterdisturbances.

患者力弱和步态障碍的模式与成年起病的慢性痉挛性截瘫综合征一致，并且进行性加重。该综合征包括上运动神经元征象（肌张力增加，深腱反射活跃和巴氏征阳性）和可能累及到后索产生的深感觉障碍（感觉性共济失调）。因为腱反射活跃，感觉性周围神经的损害不明显。因此体征和症状提示是脊髓疾病。尽管不常见，双侧前额顶叶皮层（如窦旁脑膜瘤）损伤也可以引起伴随感觉症状和括约肌功能障碍的慢性进行性痉挛性截瘫综合征。

Asyndromeofthistypemaybeindicativeofhereditaryspinocerebellardegeneration(Friedrichataxia)oroneofitsvariants.1Inyoungadults,progressivemultiplesclerosis(MS)isa







































中科白癜风新年优惠享不停
中科白癜风新年优惠享不停