语义性痴呆患者的临床、影像及神经心理学特点

摘要

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.%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.