中英多发性骨髓瘤的神经系统并发症

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SECTION1第一部分

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？

Multifocalsensorimotordeficitscanoccurfromcentralorperipheralprocesses.Centralcausescanincludedemyelination,metastaticdisease,multifocalinfarctions,abscesses,orgranulomas.Peripheralprocessescanresultininvolvementofmultiplenerveroots,brachialorlumbarplexus,orindividualnerves.Thepatient’sclinicalpresentationresultedfrominvolvementofrightulnarandsuralnerves,andleftC5–6nerverootsvsuppertrunkofthebrachialplexus.Interestingly,therewasnopainorsensoryfindingsintheleftarm.Ofnote,thepatientlackedsymptomsofneuropathyorriskfactorsforneuropathylikediabetes,vitamindeficiencies,oralcoholabusepriortothispresentation.Theasymmetricsensory-motordeficitsindiscretenervedistributions