A19-year-oldmanwithnosignificantmedicalhistorynoted3weeksofrightfacialnumbnessandslurredspeech.Onexamination,hehaddecreasedsensationintherightmiddleandlowertrigeminalnervedistributions,righttonguedeviation,andbilateralfacialweakness.AlumbarpunctureyieldedCSFwithalymphocyticpredominantpleocytosis(50leukocytes/mm3,95%lymphocytes),elevatedprotein(mg/dL),andnormalglucose(49mg/dL),withoutotherevidenceofinflammationorinfection,whileserumstudieswerenormal.BrainMRIwithgadoliniumdemonstratedasubcentimeterleftfrontalsubcorticalwhitematterlesiononfluid-attenuatedinversionrecoveryimagesandanonenhancingpinealcyst.
19岁男性，既往体键，主述右侧面部麻木及言语不清3周。体格检查示：右侧三叉神经第二第三支分布区感觉减退，伸舌右偏，双侧面肌无力。腰穿脑脊液示淋巴细胞增多为主（50个白细胞/mm3,95%淋巴细胞），蛋白升高（mg/dL）,葡萄糖正常（49mg/dL），没有炎症或感染的其他证据，血液检测无异常。头部增强MRI显示左额叶皮质下白质几毫米大的FLAIR异常信号，松果体囊肿，无增强。
Questionforconsideration:
1.Whatisthedifferentialdiagnosis?
思考问题：
1.鉴别诊断是什么？
Inanotherwisehealthyyoungperson,diagnosticconsiderationswouldincludetumors,demyelinatingconditions,infectiousprocessessuchasCNSborreliosisorfungalmeningitis,andsystemicinflammatoryconditionsincludingneurosarcoidosis.Anonenhancingpinealcystraisesconcernforagermcelltumor,pinealparenchymaltumor,andlow-gradeglioma.
对于既往体健的青年人，诊断可以考虑：肿瘤、脱髓鞘、感染如中枢神经系统莱姆病或真菌性脑膜炎、系统性炎症包括神经系统结节病。松果体区无强化的囊肿需要考虑到生殖细胞肿瘤、松果体实质肿瘤、低级别胶质瘤。
SECTION2第二部分Over6months,thepatientdevelopedrighthorizontaljerkingnystagmus,bilateralhearingloss,dysarthria,anddysphagia.Twolumbarpuncturesdemonstratedapersistentlymphocyticpleocytosis(37leukocytes/mm3,87%lymphocytes,and60leukocytes/mm3,77%lymphocytes)withelevatedprotein(andmg/dL)andelevatedglucose(69and70mg/dL)intheCSFwithnegativeinfectiousstudiesandatypicallymphocytesonflowcytometrics.Serumstudieswereunremarkable.
6个月后，患者出现了右侧水平性急动性眼震、双侧听力丧失、构音障碍和吞咽困难。2次腰穿示持续的淋巴细胞增多（分别为：37个白细胞/mm3,87%淋巴细胞；60个白细胞/mm3,77%淋巴细胞），蛋白升高（和mg/dL）,葡萄糖升高（69和70mg/dL），没有感染征象，流式细胞分析见不典型淋巴细胞。血液学检查无明显异常。
Questionforconsideration:
1.Whatadditionaltests/studiesshouldbeconsidered?
思考问题：
1.进一步安排什么化验或检查？
RepeatbrainMRIwithgadoliniumanddetailedimagingofthemidbrain,pons,andmedulla;wholebodyCT;CSFfungalcultures;CSFacid-fastbacillistainandculture;3largevolume(30mL)CSFcollectionsforcytopathology;serumandCSFa-fetoprotein(aFP);andβ-humanchorionicgonadotropin(bHCG).
复查头部增强MRI扫描，特别是中脑、脑桥及延髓区；全身CT；脑脊液真菌培养；脑脊液抗酸杆菌涂片及培养；细胞学检查（收集3次大剂量的脑脊液，30ml）；血清和脑脊液α甲胎蛋白检查；β-HCG检查。
sECTION3第三部分RepeatMRIofthebrainwithgadoliniumdemonstratedenhancementofcranialnervesV,VII,IX,X,XI,andthepinealglandcyst.CervicalandthoracicspinalMRIwasnormal.PETdemonstratedhyperplasticmarrowbutnometastaticdisease.Hehadbeentreatedwithasloworaltaperofprednisone,IVglucocorticoids,andIVimmunoglobulin(IVIg).
复查头增强MRI显示第V,VII,IX,X,XI颅神经增强和松果体囊肿。颈胸椎MRI正常。PET显示骨髓增生但没有转移性疾病。治疗上给予静脉糖皮质激素、静脉免疫球蛋白、口服强的松缓慢减量。
Ontransfertoatertiaryfacility,heendorseddyspnea,weakcough,difficultymanagingsecretions,progressiveweaknessinthelegs,diminishedsensationintheleftarmandleg,and1monthofurinaryincontinenceandconstipation.Hismedicationsontransferwereoralprednisoneaswellasantibioticsforanaspirationpneumonia,gastriculcerprophylaxis,andsymptommanagementfornauseaandoralsecretions.Therewerenonotablemedicalconditionsinhisfamilyhistory.Hewasahighschoolgraduatewithnosignificantsubstanceuseortravelhistory.
转移到三级医疗机构之后，患者出现了呼吸困难、咳嗽无力、流涎、双下肢进行性无力，左侧肢体感觉减退，伴尿失禁及便秘1月。转诊时的治疗包括口服强的松，针对吸入性肺炎的抗生素，预防胃溃疡以及对恶心、流涎的对症处理。家族史无特殊。高中毕业、没有药物滥用史及旅游史。
Hisneurologicexaminationwasremarkableforvisualsensationonlyforlightandmovement,fixeddilatedpupils,downgazeinprimaryposition,upwardandlateraleyemovementparesis,bilateralptosis,diminishedbilateralfacialsensation,bilateralfacialweakness,diminishedhearing,poorsoftpalateelevation,weakcough,andabsentgagreflex.Hisleftlegwasexternallyrotatedwithotherwiseantigravitystrengththroughoutthebilaterallegs.Hedemonstratedresistancetomovementwhichcouldbeover