语义性痴呆患者的临床、影像及神经心理学特点
어의성치태환자적림상、영상급신경심이학특점
Clinical, neuroimage, and neuropsychological profiles of semantic dementia
  • 万方数据
    中华神经科杂志 중화신경과잡지
    Chinese Journal of Neurology
    2012年 2期 84-89 ,共6页

    周爱红%魏翠柏%张亚旭%唐毅%宋海庆%闵宝权%黄丽%杨延辉%贾建平

    주애홍%위취백%장아욱%당의%송해경%민보권%황려%양연휘%가건평

    痴呆%磁共振成像%神经心理学测验 癡呆%磁共振成像%神經心理學測驗
    치태%자공진성상%신경심이학측험
    Dementia%Magnetic resonance imaging%Neuropsychological tests
    目的 探讨语义性痴呆( semantic dementia,SD)患者的临床、影像和神经心理学特点.方法 SD患者18例,详细收集患者的临床资料,进行头MRI检查和神经心理评估,神经心理评估包括语义记忆(物体命名任务)、总体认知功能评估[简易精神状态检查(Mini-mental State Examination,MMSE)]、视空间能力[画钟测验(Clock Drawing Test,CDT)]、日常生活能力[日常生活能力量表( Activities of Daily Living,ADL)]、精神行为症状[神经精神问卷(Neuropsychiatric Inventory,NPI)]及总体严重程度[改良额颞叶变性临床痴呆评定量表( Frontotemporal Lobar Degeneration Modified Clinical Dementia Rating Scale,FTLD-CDR)]的评估.结果 患者发病年龄(60.6±8.5)岁,5例于65岁后发病,均以找词困难、命名障碍为首发症状,10例主诉有记忆障碍,14例出现人格行为改变.5例曾被诊断为阿尔茨海默病( Alzheimer's disease,AD),1例诊断为精神分裂症,无一例患者此前被明确诊断为SD.语义记忆评估显示所有患者对生命类和非生命类物体的名称及功能均有明显遗忘.MMSE(10.94±8.86)分,16例患者命名完全错误,定向力、记忆力、计算力、阅读和书写能力亦有损害,但与命名比较相对较好.CDT临摹(4.61±0.85)分,14例完全正常.ADL (29.72±8.75)分,病程5年的患者日常生活能力全面下降.NPI(8.00±7.22)分,14例患者出现异常.FTLD-CDR显示所有患者的语言障碍得分最高.头MRI检查显示多数患者以左侧颞叶萎缩为主,但有1例患者右侧颞叶萎缩重于左侧.早期萎缩局限于左侧颞极,随病情进展,累及右侧颞极、左侧额叶和顶叶皮质.结论 SD多为老年前期发病,但1/3的患者发生于老年期.语义记忆障碍最突出,行为和人格异常亦常见.临床上存在较高的误诊率和漏诊率,最常被误诊为AD.在病程早期,患者的视空间及其他能力相对保留,病程5年的患者功能全面衰退.头颅MRI可反映疾病的进展过程和临床特征,但个别患者以右侧颞叶萎缩为主.
    목적 탐토어의성치태( semantic dementia,SD)환자적림상、영상화신경심이학특점.방법 SD환자18례,상세수집환자적림상자료,진행두MRI검사화신경심리평고,신경심리평고포괄어의기억(물체명명임무)、총체인지공능평고[간역정신상태검사(Mini-mental State Examination,MMSE)]、시공간능력[화종측험(Clock Drawing Test,CDT)]、일상생활능력[일상생활능역량표( Activities of Daily Living,ADL)]、정신행위증상[신경정신문권(Neuropsychiatric Inventory,NPI)]급총체엄중정도[개량액섭협변성림상치태평정량표( Frontotemporal Lobar Degeneration Modified Clinical Dementia Rating Scale,FTLD-CDR)]적평고.결과 환자발병년령(60.6±8.5)세,5례우65세후발병,균이조사곤난、명명장애위수발증상,10례주소유기억장애,14례출현인격행위개변.5례증피진단위아이자해묵병( Alzheimer's disease,AD),1례진단위정신분렬증,무일례환자차전피명학진단위SD.어의기억평고현시소유환자대생명류화비생명류물체적명칭급공능균유명현유망.MMSE(10.94±8.86)분,16례환자명명완전착오,정향력、기억력、계산력、열독화서사능력역유손해,단여명명비교상대교호.CDT림모(4.61±0.85)분,14례완전정상.ADL (29.72±8.75)분,병정5년적환자일상생활능력전면하강.NPI(8.00±7.22)분,14례환자출현이상.FTLD-CDR현시소유환자적어언장애득분최고.두MRI검사현시다수환자이좌측섭협위축위주,단유1례환자우측섭협위축중우좌측.조기위축국한우좌측섭겁,수병정진전,루급우측섭겁、좌측액협화정협피질.결론 SD다위노년전기발병,단1/3적환자발생우노년기.어의기억장애최돌출,행위화인격이상역상견.림상상존재교고적오진솔화루진솔,최상피오진위AD.재병정조기,환자적시공간급기타능력상대보류,병정5년적환자공능전면쇠퇴.두로MRI가반영질병적진전과정화림상특정,단개별환자이우측섭협위축위주.
    Objective To explore the clinical,neuroimage,and neuropsychological profiles of semantic dementia (SD).Methods Detailed medical history were collected on 18 SD patients.Brain MRI scans were administered.Neuropsychological evaluation taping semantic memory (things naming),overall cognitive function ( Mini-mental State Examination,MMSE),visuoconstructive skill ( Clock Drawing Test,CDT), daily functional ability (Activities of Daily Living, ADL), neuropsychiatric symptoms (Neuropsychiatric Inventory,NPI),and disease severity (Frontotemporal Lobar Degeneration Modified Clinical Dementia Rating Scale,FTLD-CDR) were performed on all patients.Results The mean age at onset was (60.6 ± 8.5 ) years,with 5 cases over 65. All patients developed progressive word-finding difficulty and anomic speech.Ten patients complained significant memory impairment and 14 experienced behavioral disturbance.Five patients were previously diagnosed as Alzheimer' s disease (AD) and 1 as schizophrenia.All patients developed marked semantic memory impairment both for living things and nonliving things.The mean score on MMSE was 10.94 ± 8.86,with 16 cases performed incorrectly on naming.Mean score on CDT copy was 4.61 ±0.85,with 14 cases scored normally.Mean score on ADL was 29.72 ± 8.75.Cases with a 5-year course showed a significant overall decline.Fourteen cases presented behavior symptoms and scored 8.00 ± 7.22 on NPI.All patients scored worst on language domain among all the domains evaluated in FTLD-CDR.Atrophy,typically involving the left anterior temporal was shown on MRI scans.However,predominantly right temporal atrophy was observed in one patient.Atrophy confined to the temporal lobe in patients with early stage and extended to the contralateral temporal,frontal lobe,and parietal lobe as disease progression. Conclusions Current study suggested that SD tend to develop in presenile age.However,about 1/3 cases develop the disease after 65 years. Deficit in language is the earliest and most prominent symptom. Behavior change is prevalent as well. Patients are commonly misdiagnosed as AD or lack a definite diagnosis.Visuoconstructive skill and other abilities are relatively preserved in the early stage.With progression into the 5th years,overall decline comes inevitably.Brain scans can reflect the disease characteristics and progression. Of note,there exists individual with right dominant atrophy.
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