A45-year-oldmanwithepilepsyandimmunoglobulinG(IgG)kmultiplemyeloma(MM)diagnosedayearagopresentedtotheneurologyclinicwithrighthandweaknessandparesthesias,painlessproximalleftarmweakness,andparesthesiasintherightfoot.Symptomsbeganacutelyovera2-weekperiodinthesettingofadiffusenonpruriticrash.Thepatienthadundergoneanallogenichematopoieticstemcelltransplant(HSCT)amonthpriorduetosuboptimalresponsetobortezomib,cyclophosphamide,etoposide,cisplatin,carfilzomib,andautologousstemcelltransplant.Hedeniedheadaches,neckpain,radicularsymptoms,bladderorboweldysfunction,orgaitimbalance.
患者,45岁男性,1年前诊断为癫痫和IgGk链性多发性骨髓瘤,就诊于神经内科门诊,表现为右手无力、感觉异常,左臂近端无力、痛觉减退以及右足感觉异常。上述症状起病较急,发病前2周开始出现弥漫性非瘙痒性皮疹。由于对硼替佐米,环磷酰胺,依托泊苷,顺铂,卡菲偌米布,自体造血干细胞移植的治疗反应欠佳,患者已于1月前行同种异体造血干细胞移植。患者否认有既往头痛、颈部疼痛、神经根性症状、膀胱和肠道功能失调及步态失衡。
Examinationrevealednormalvitalsigns,mentalstatus,andcranialnerves.Amaculopapularrashwasseenoverthetrunkandextremities,withoutadermatomalpattern.Motorexaminationrevealednormaltoneandstrengthexceptforprofoundweakness(MedicalResearchCouncil[MRC]2/5)inthemusclesinnervatedbytheleftC5-6myotomesandweakness(MRC4/5)inthemusclesinnervatedbytherightulnarnerve.Reflexeswere1+throughoutexceptforabsentleftbicepsandbrachioradialis.Plantarreflexeswereflexor.Pinprickwasdiminishedintherightfourthandfifthdigits,andlateralaspectoftherightfoot.Therestofhisneurologicexaminationwasunremarkable.
各项检查示生命体征、精神状态、颅神经均为正常。躯干和四肢可见斑丘疹,未见节段性分布。运动检查示:(医学研究委员会[MRC]2/5)左侧颈5-6神经支配的肌肉,和右侧尺神经支配的肌肉肌无力(MRC4/5),其余肌肉肌力和肌张力均正常。除肱二头肌和肱桡肌腱反射消失外,其余反射均为1+。跖反射正常。右足外侧缘和第四、五趾针刺觉减弱。其它神经系统检查未见明显阳性体征。
Questionsforconsideration
1.Whatisthedifferentialdiagnosisformultifocalmotorandsensorydeficits?
2.Whataresomediagnosticconsiderationstoincludewiththepresenceoftheskinrash?
思考问题
1.多发运动和感觉缺失的鉴别诊断有哪些?
2.哪些疾病可能会在病程中出现皮疹?
SECTION2第二部分Multifocalsensorimotordeficitscanoccurfromcentralorperipheralprocesses.Centralcausescanincludedemyelination,metastaticdisease,multifocalinfarctions,abscesses,orgranulomas.Peripheralprocessescanresultininvolvementofmultiplenerveroots,brachialorlumbarplexus,orindividualnerves.Thepatient’sclinicalpresentationresultedfrominvolvementofrightulnarandsuralnerves,andleftC5–6nerverootsvsuppertrunkofthebrachialplexus.Interestingly,therewasnopainorsensoryfindingsintheleftarm.Ofnote,thepatientlackedsymptomsofneuropathyorriskfactorsforneuropathylikediabetes,vitamindeficiencies,oralcoholabusepriortothispresentation.Theasymmetricsensory-motordeficitsindiscretenervedistributions进展期白殿北京中科白殿疯眞棒