CAJ | 학술논문

目的探讨肌萎缩侧索硬化(ALS)患者认知功能状态、ALS轻度认知功能损害(ALS-MCI)的受累领域和各种亚型及其可能的危险因素.方法收集ALS患者29例,健康对照者58名,选用改良Norris量表评价ALS患者的延髓功能及肢体功能.根据美国精神病学会精神障碍诊断和统计手册(DSM-Ⅳ-R)痴呆的诊断标准,将ALS患者分为痴呆和非痴呆;对于非痴呆的ALS患者,通过常用的认知功能(包括精神状态、记忆力、执行功能、注意力、视空间功能)量表、汉密尔顿焦虑量表(HAMA)、汉密尔顿抑郁量表(HAMD)进行评分,按照Petersen等修订的MCI诊断标准,将其分为认知功能正常(ALS-CogNL)组和ALS-MCI组,分析ALS-MCI受累的领域及其亚型;比较2组在年龄、起病年龄、病程、起病部位、延髓性麻痹症状、肢体运动功能损害等方面的差异,分析ALS患者出现MCI的相关危险因素.结果 29例ALS患者中,认知功能正常14例(48.3%),MCI有15例(51.7%),未发现痴呆患者.15例ALS-MCI患者中,执行功能损害12例,注意力损害9例,记忆力损害8例,未发现视空间功能损害;其中遗忘型(ALS-aMCI)1例,非记忆单一领域损害型(ALS-sdMCI)6例,多领域受损型(ALS-mdMCI)8例.ALS-MCI组与ALS-CogNL组的教育年限[(8.7±2.8)年与(11.3 ±3.0)年]、Norris量表延髓功能评分[(28.4±7.7)分与(34.0±3.4)分]差异有统计学意义(t=-2.435、-2.576,均P＜0.05),性别、年龄、起病年龄、病程、起病部位、HAMA及HAMD评分、Norris量表肢体功能评分差异无统计学意义.结论 ALS患者常出现MCI,其中以执行功能损害最常见,记忆力、注意力亦有损害,未发现视空间功能损害,ALS-mdMCI是最常见的亚型.文化程度低、严重延髓性麻痹症状是ALS患者出现认知功能损害的危险因素.
목적 탐토기위축측색경화(ALS)환자인지공능상태、ALS경도인지공능손해(ALS-MCI)적수루영역화각충아형급기가능적위험인소.방법 수집ALS환자29례,건강대조자58명,선용개량Norris량표평개ALS환자적연수공능급지체공능.근거미국정신병학회정신장애진단화통계수책(DSM-Ⅳ-R)치태적진단표준,장ALS환자분위치태화비치태;대우비치태적ALS환자,통과상용적인지공능(포괄정신상태、기억력、집행공능、주의력、시공간공능)량표、한밀이돈초필량표(HAMA)、한밀이돈억욱량표(HAMD)진행평분,안조Petersen등수정적MCI진단표준,장기분위인지공능정상(ALS-CogNL)조화ALS-MCI조,분석ALS-MCI수루적영역급기아형;비교2조재년령、기병년령、병정、기병부위、연수성마비증상、지체운동공능손해등방면적차이,분석ALS환자출현MCI적상관위험인소.결과 29례ALS환자중,인지공능정상14례(48.3%),MCI유15례(51.7%),미발현치태환자.15례ALS-MCI환자중,집행공능손해12례,주의력손해9례,기억력손해8례,미발현시공간공능손해;기중유망형(ALS-aMCI)1례,비기억단일영역손해형(ALS-sdMCI)6례,다영역수손형(ALS-mdMCI)8례.ALS-MCI조여ALS-CogNL조적교육년한[(8.7±2.8)년여(11.3 ±3.0)년]、Norris량표연수공능평분[(28.4±7.7)분여(34.0±3.4)분]차이유통계학의의(t=-2.435、-2.576,균P＜0.05),성별、년령、기병년령、병정、기병부위、HAMA급HAMD평분、Norris량표지체공능평분차이무통계학의의.결론 ALS환자상출현MCI,기중이집행공능손해최상견,기억력、주의력역유손해,미발현시공간공능손해,ALS-mdMCI시최상견적아형.문화정도저、엄중연수성마비증상시ALS환자출현인지공능손해적위험인소.
Objective To explore the cognitive status of amyotrophic lateral sclerosis (ALS) patients, and to explore the involved cognitive domains, subtypes and risk factors of mild cognitive impairment in ALS ( ALS-MCI).Methods Twenty-nine cases of ALS and 58 healthy volunteers were included.The severity of the bulbar and spinal functions of the patients was evaluated by the Improved Norris Scale.According to the Diagnostic and Statistical Manual of Mental Disorders 4th Edition-Revised( DSM-Ⅳ-R) criteria of dementia, ALS cases were classified as demented and non-demented.For non-demented ALS cases, the common cognitive batteries evaluating mental state, verbal memory, executive, attentional and visuospatial abilities were performed.Hamilton Anxiety Scale ( HAMA) and Hamilton Depression Scale (HAMD) were evaluated too.They were further classified into ALS-cognitively normal (ALS-CogNL) and ALS-MCI groups according to Petersen criteria of MCI.Risk factors possibly correlated with ALS-MCI were analyzed by comparing the differences in age, age of onset, duration of the disease, sites of onset, symptoms of bulbar and limb function between ALS-CogNL and ALS-MCI groups.Results Among 29 ALS cases, 14 (48.3% ) cases with cognitively normal( ALS-CogNL), 15 cases (51.7% ) with ALS-MCI,and none with dementia were identified.Among 15 ALS-MCI cases, 12 cases with executive dysfunction, 8 cases with memory deficits,9 cases with attention impairment and none with visuospatial impairment were found.ALSMCI cases could be further classified into three subtypes; 1 case with amnestic MCI (aMCI) ,6 cases with single domain non-memory MCI ( sdMCI), and 8 cases with multiple domains slightly impaired MCI (mdMCI).Between ALS-MCI and ALS-CogNL groups, there were significant differences (t = -2.435,- 2.576, both P ＜ 0.05) in education ((8.7 ± 2.8) years vs (11.3 ± 3.0) years) and Improved Norrisscale (bulbar score: (28.4 ± 7.7) scores vs ( 34.0 ± 3.4) scores) , however, no significant differences in sex, age, age of onset, duration,site of onset,HAMA or HAMD scores,and Improved Norris scale( spinal score) were found.Conclusions Cognitive deficits commonly exist in ALS patients.For the involved domains, executive dysfunction is the most common, deficits of attention and memory are also common, and deficit in visuospatial function is not found.The most common subtype of ALS-MCI is mdMCI.Severe bulbar symptoms and lower education may be the risk factors of ALS-MCI.