A28-year-oldmanwithsicklecelldiseasepresentedwith7monthsofdifficultywalking.Initialexamination3monthspriortoadmissiontoourhospitalwasthoughttobeconsistentwithapolyneuropathy.Hewasareflexic,wasunabletostandontoesorheelswithdecreasedsensationontheleftfoottolighttouchandvibration,haddifficultywithheeltoshin,andwasunabletoperformtandemgait.Laboratorytestsrevealedanemia(hemoglobin7.2g/dL)andelevatedcreatinine(1.49g/dL).HIV,antinuclearantibodies,antineutrophilcytoplasmicantibodies,hepatitisserologies,rapidplasmareagin,thyroid-stimulatinghormone,copper,SSA/B,andLymetiterswereunremarkable.B12deficiency(Bpg/mL)andamildlyelevatederythrocytesedimentationrate(ESR)at24mm/hourwerefound.InitialMRIbrainshowedmultiplefocioffluid-attenuatedinversionrecoveryhyperintenselesionsinthesubcorticalandperiventricularwhitematter.Lesionsdidnotrestrictondiffusion-weightedimaging(DWI);however,afewlesionsdemonstratedenhancementwithgadolinium.MRIofthespinewasunremarkable.Lumbarpuncture(LP)wasremarkableforaCSFproteinofmg/dL,leukocytes43/mL(92%lymphocytes),erythrocytes2,/mL,andglucose41mg/dL.Therewerenooligoclonalbands.ThepatientwastreatedwithIMinjectionsofB12,withoutimprovementinhisdeficits.
患者28岁男性，患有镰状细胞病，表现为行走困难7个月。入院前3个月初次就诊时考虑为多发性神经病。当时体格检查发现腱反射消失，不能垫脚尖或以脚跟站立，左脚轻触觉和振动觉减退，难以完成跟膝胫试验，足跟-足尖直线行走（踵趾步态）不能。实验室检查显示贫血（血红蛋白7.2g/dL）、肌酐升高（1.49g/dL）、B12缺乏（pg/mL）以及血沉（ESR）轻度升高（24mm/h）。HIV、抗核抗体、抗中性粒细胞胞浆抗体、肝炎血清学、梅毒快速血浆反应素试验、甲状腺刺激激素、血清铜、SSA/B以及莱姆病抗体均未见明显异常。初次头颅MRI检查提示皮层下和脑室周围白质多发Flair像高信号病灶，DWI像无弥散受限，但一些病灶有强化。脊髓MRI检查未见明显异常。腰椎穿刺检查提示脑脊液蛋白明显升高，为mg/dL，白细胞43个/mL（92％淋巴细胞），红细胞2,个/mL，葡萄糖水平41mg/dL，寡克隆带阴性。随后患者接受了肌注B12治疗，但症状无改善。
Questionsforconsideration:
1.Whatisthedifferentialdiagnosisofprogressivegaitdisorder?
2.HowdotheinitialCSFresultsargueagainsttheexclusivediagnosisofB12deficiency?
问题思考：
1.进行性步态异常的鉴别诊断有哪些？
2.如何根据初次脑脊液检查结果排除B12缺乏症的诊断？
SECTION2第二部分Gradualgaitataxiacanresultfromeitheraperipheraloracentralprocess.Peripheralnervoussystemprocessesthataffectlargefibernervesincludenutritional,inflammatory,orhereditaryneuropathies.CNSpathologiesincludeconditionsthataffectthedorsalcolumnsandcerebellum,suchasmultipleinfarctions,demyelination,orneoplasia.Thepatient’sexaminationlocalizedtobothaperipheralandcerebellarprocess.B12deficiencycouldhaveexplainedthelargefiberneuropathyonexamination,andpossiblyhisMRI;however,itwasinsufficienttoexplainhissubsequentcourseandCSFresults.ItisthoughtthatpatientswithsicklecelldiseasearemorepronetoB12deficiencyduetoincreasedrequirementordecreasedabsorptionintheterminalileumsecondarytorecurrentsicklingcrisis.1TheCSFpleocytosiswasinconsistentwithB12deficiency.TheMRIlesionswerenonspecific.T2hyperintensitiescanbeobservedinB12deficiency,butdemyelinatingandvascularconditionsshouldalsobeincludedinthedifferential.Somelesionswerenotinclassiclocationsfordemyelinatingdiseaseandcouldbeconsistentwitholdinfarctionsfromsicklecelldisease,orfromaninflammatoryprocesslikevasculitis.
渐进性步态共济失调可由周围性和中枢性神经系统病变引起。影响大纤维神经的周围神经疾病病因包括营养缺乏、炎症性或遗传性神经病。中枢性病因包括影响脊髓后索和小脑的病变如多发性梗死、脱髓鞘或肿瘤。此患者的检查定位于周围神经和小脑。B12缺乏可以解释检查所见大纤维神经病变的表现，也有可能解释其MRI表现，但不足以解释脑脊液结果和后续病程特点。研究认为镰状细胞病患者更容易发生B12缺乏，因为这些患者对B12需求增加或因反复镰状细胞危象导致回肠末端对B12吸收减少所致[1]。但脑脊液细胞数增多也与B12缺乏不一致。MRI病灶无特异性，T2高信号可见于B12缺乏症，但脱髓鞘和血管性疾病也应包括于鉴别中。有些病灶不在脱髓鞘疾病的典型部位，但能符合镰状细胞病引起的梗死或像血管炎引起炎症过程。
Threemonthsafterhisinitialevaluation,thepatientwasadmittedforworseningfatigue.MRIbrainhadintervaldevelopmentofmultiplenewfociofT2prolongation,manyofwhichenhancedandrestrictedonDWI(figure1).LPwasremarkableforaproteinofmg/dL,leukocytes6/mL,erythrocytes/mL,glucose45mg/dL,negativecytology,andnegativeEpstein-Barrvirus,varicella-zostervirus(VZV),JCvirus,andherpessimplexvirusstudies.
在他初次就诊检查的3个月后，患者因疲惫渐重入院。此时段间隔后头颅MRI出现新的多灶性长T2信号，其中很多有强化和弥散受限（图1）。腰椎穿刺脑脊液蛋白明显高，为mg/dL，白细胞为6个/mL，红细胞为个/mL，葡萄糖45mg/dL，细胞学检查阴性，E-B病毒、水痘-带状疱疹病毒（VZV）、JC病毒和单纯疱疹病毒检测阴性。
Questionforconsideration:
1.WhatisthedifferentialofrestricteddiffusiononMRIbrain?
问题思考：
1.头颅MRI弥散受限的疾病有哪些鉴别诊断？
图1大脑横切位MRI液体衰减反转恢复序列，T1加权钆增强序列，DWI及ADC序列
（A，B）大脑横切位MRI液体衰减反转恢复（FLAIR）序列可见皮质下白质包括双侧放射冠和半卵圆中心T2高信号。（C）T1加权钆对比增强序列可见左侧额叶有强化。（D，E）小脑蚓部扩散加权成像和表观扩散系数序列，可见变化一致的弥散受限病灶。（F）FLAIR序列可见小脑蚓部T2高信号。
SECTION3第三部分ThedifferentialofrestricteddiffusiononMRIbrainincludesinfarction,statusepilepticus,abscess,Creutzfeldt-Jakobdisease(CJD),neoplasm/lymphoma,andrarelyacutedemyelination.2Diffusionrestrictionincreasesthelikelihoodofavascularprocess.Sicklecelldiseasecancausevaso-occlusivecrisisandamultifocalvasculopathywithrecurrentinfarction.Clinicalandlaboratorydataruledoutstatusepilepticus,abscess,andCJD.Despitethesefindings,thepatienthaddisseminationinspaceandtimeofT2lesionsonhisMRIscanandaworkingdiagnosiswasmultiplesclerosis(MS).HewastreatedempiricallywithIVsteroids;however,hismentalstatusrapidlydeclined,andhewassubsequentlytransferredtoourhospitalforfurthercare.
头颅MRI弥散受限的鉴别诊断包括梗死、癫痫持续状态、脓肿、克雅氏病（CJD）、肿瘤/淋巴瘤和少见的急性脱髓鞘病变[2]。弥散受限增加了血管性疾病过程的可能性。镰状细胞病可引起血管闭塞危象及伴反复梗死的多灶性血管病。临床和实验室结果排除了癫痫持续状态、脓肿和CJD的可能性。尽管有这些发现，患者MRIT2像上病灶有时间和空间的多发性，拟治疗性诊断为多发性硬化症。给予经验性静脉类固醇治疗。但其精神状态迅速恶化，随后被转到我院进一步治疗。
Onexaminationhewaslethargic,butarousabletolighttouch.Hewasorientedtopersonandtime,andhadaverypoorattentionspan.Hewasperseverativeandabletofollowsimplemidline北京治疗白癜风的中药皮肤白癜风能治的好吗
转载请注明：http://www.ebsaw.com/jbjc/8529.html
