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图A,磁敏感加权MRI示多发脑实质内低信号（红色箭头）。B，T1加权MRI示颈5椎体水平脊髓右前部高信号（红色箭头）。C，T2加权MRI示颈5至胸7椎体水平（未显示）之间脊髓中央低密度（红色箭头）。
Amaninhis50spresentedwithweaknessinthedistalrighthandandlegandparesthesiasoftherighthand.Fourdaysbeforepresentation,heexperiencedsudden,severeneckpainthatswiftlyprogressedtoneckstiffnessandbilateralshoulderpain.Thenextday,henoticedweaknessintherightarmandleg.Thedaybeforehospitalization,theweaknessprogressed,andhelosttheabilitytoambulate.Until18monthsago,hehadworkedasaclerk,butheretiredbecauseofahistoryofhypertensionanddiabetes.Hisfamilyhistoryrevealednoneurologicaldisorders,exceptforabrotherwhohadabrainlesionexcised.Onexamination,hewasafebrile,andhisvitalsignswerenormal.Hewasfullyalertbuthadweaknessintherightdistalarm(medicalresearchcouncil[MRC]grade3/5)andtherightleg(MRCgrade4/5)withsensoryimpairmenttopinprickandtemperatureintheleftleg.Laboratoryvaluesrevealedanelevatedγ-glutamyltransferaselevelofU/L(referencerange,10-71U/L).Analysisofthecerebrospinalfluidindicatedxanthochromia;normalwhitebloodcellcount,1cell/μL;redbloodcellcount,2.19×10/μL;increasedglucoselevelatmg/dL(referencerange,40-80mg/dL);increasedtotalproteinlevelat0.10g/dL(referencerange,0.03-0.05mg/dL);andincreasedlactatelevelat28.8mg/dL(referencerange,9.9-21.6mg/dL).Magneticresonanceimaging(MRI)ofthebrainandtheentirespinalcordrevealedpatencyoftheintracranialvesselsandmultipleabnormalsignalsthroughoutthebrainonT1-,T2-,andsusceptibility-weightedsequences(SWIs)and1lesionatthelevelofthefifthcervicalvertebra(Figure).
50岁男性，因右侧肢体远端无力及右手感觉异常入院。入院前4天，患者突然出现颈部剧烈疼痛，迅速进展为颈部僵硬伴双肩疼痛。第二天，出现右臂及右腿无力。入院前1天，患者肢体无力加重，不能行走。患者之前为一名职员，18个月前因患高血压和糖尿病而退休。除了一个兄弟有脑病变切除史外，其家族中无神经系统疾病患者。体检示，患者无发热，生命体征正常。神清，右前臂远端肌力3级，右腿肌力4级，左腿针刺觉及温度觉障碍。实验室检查示，γ-谷氨酰转移酶水平升高：U/L（参考值：10-71U/L）。脑脊液分析提示黄变;白细胞计数正常：1个细胞/μL;红细胞计数：2.19×10^6/μL;葡萄糖水平升高：mg/dL（40-80mg/dL）;总蛋白水平升高：0.10g/dL（0.03-0.05mg/dL）;乳酸水平升高：28.8mg/dL（9.9-21.6mg/dL）。脑及全脊髓磁共振成像（MRI）示颅内血管通畅，T1，T2及SWI序列示全脑内多发异常信号，在第五颈椎水平有一病灶（图）。
WHATISYOURDIAGNOSIS?
A.Amyloidangiopathy
B.Multiplecavernousmalformations
C.Metastaticmelanoma
D.Cerebralautosomaldominantarteriopathywithsubcorticalinfarctsandleukoencephalopathy
诊断是什么？
A.淀粉样血管病
B.多发海绵状血管畸形
C.转移性黑色素瘤
D.伴皮质下梗死和白质脑病的常染色体显性遗传性脑动脉病
Diagnosis
B.Multiplecavernousmalformations
诊断
B.多发海绵状血管畸形
Discussion讨论BrainandspinalcordMRIshowedmultiplehemorrhagesthrough-outthebrainashypointensitiesonSWI.T1andT2sequencesshowedvariousstagesofbleedingthroughoutthebrain(notshown)andoneacutehemorrhageinthespinalcord(Figure).
脑和脊髓MRI示全脑多发出血灶（SWI呈低信号）。T1和T2序列示全脑多发的各种阶段出血（未提供），以及脊髓急性出血（图）。
Becauseofspinalcordinvolvement,anamyloidangiopathywashighlyunlikely.Theotherconsiderationwasmetastaticmelanoma;however,therewasnoevidenceofamasseffectonMRI.Cerebralautosomaldominantarteriopathywithsubcorticalinfarctsandleukoencephalopathywasanotherpossibility;however,ourpatienthadnoevidenceofleukoencephalopathy.
由于有脊髓受累，淀粉样血管病不太可能。也需考虑转移性黑色素瘤，但MRI未发现占位效应。此例患者没有脑白质病的证据，也不考虑伴皮质下梗死和白质脑病的常染色体显性遗传性脑动脉病。
ThespinalT1hyperintensityatthecervicallevelwasconsistentwithacutebleedingintherightventralportionofthespinalcordandthepatient’ssymptoms.TherewasalargesyrinxcavityunderneaththespinalcavernomathatwashypointenseonT2MRIandwasconsistentwithahematosyrinx,arare鐧界櫆椋庤兘涓嶈兘褰诲簳娌诲ソ鐧界櫆椋庤兘濂藉悧
