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This article aims to comprehensively review the concept, etiology, classification, classical cortical mapping, assessment, a proposed flowchart for the diagnosis and differential diagnosis, treatment, rehabilitation, mechanisms of development and recovery, prognosis, and influencing factors for post-stroke aphasia(PSA)types in the Chinese language.We emphasize the necessity and significance of neuroimaging assessment of the brain and blood vessels and neuropsychological assessment in the diagnosis and differential diagnosis of PSA in Chinese.We also recommend and encourage the use of the dichotomies of internal vs.external and anterior vs.posterior as a starting point, based on the association of anatomical locations of the brain and blood vessels with brain language areas and language disorders.A classification system of PSA in Chinese developed from this approach in the form of a flowchart is well-suited for guiding the clinical treatment of cerebral stroke.Incorporating the "four elements" , the flowchart enables convenient diagnosis, classification and differential diagnosis of PSA in Chinese and facilitates targeted and personalized rehabilitation planning to benefit the patient.This article introduces the use of memantine, piracetam, donepezil and other drugs for PSA treatment, evaluates clinical trials on memantine conducted in China and abroad and its mechanisms of action for the treatment of PSA, and discusses how rehabilitation therapy achieves therapeutic effects.For the treatment of PSA, clinical research and practice using drugs such as memantine, piracetam and donepezil in combination with non-pharmacotherapy and rehabilitation training should be promoted.
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Objective:To evaluate the value of Facial Emotion Recognition Test (FERT) in the early diagnosis of behavioral variant frontotemporal dementia (bvFTD).Methods:A total of 27 patients with mild bvFTD, 27 patients with mild Alzheimer′s disease (AD) and 54 normal controls were successively collected in the Memory Clinic of Department of Neurology,Xuanwu Hospital, Capital Medical University from January 2018 to July 2019. The subjects were assessed by the FERT and a battery of neuropsychological background tests including Mini-Mental State Examination, Frontal Assessment Battery (FAB), and Auditory Verbal Learning Test (AVLT), etc. The discriminatory power of the FERT was evaluated using the receiver operating characteristic curve (ROC).Results:The average FERT scores of bvFTD, AD and control groups were 18.00 (14.00, 21.00), 25.00 (21.00, 28.00) and 28.00 (26.00, 30.00) respectively. The FERT scores of bvFTD group were significantly lower than those of control groups ( H=-55.278, P<0.001) and AD groups ( H=-28.407, P=0.002). ROC results showed that the FERT had a high discriminatory power for differentiating bvFTD from the controls, with an area under the curve (AUC) value of 0.969 (95% CI 0.931-1.000, P<0.001). Both the sensitivity and specificity were 92.6% with a cut-off value at 24. For differentiating bvFTD group from AD group, the AUC value for the FERT was 0.850 (95% CI 0.749-0.951, P<0.001), with sensitivity of 81.5% and specificity of 71.4% with a cut-off value of 22. Compared with the AUC values for the FAB and AVLT-Delayed Recall (0.776 and 0.714), the AUC value for the FERT was slightly higher, though the differences among them were not statistically significant. Conclusions:Disturbance of emotion processing is presented in the early stage of bvFTD. FERT is one of the sensitive and specific neuropsychological indicators for the early diagnosis of bvFTD.
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Objective To explore the features of connected speech produced by Chinese mild Alzheimer's disease (AD) patients.Methods Thirty Chinese mild AD patients (eight males and 22 females,and the age was (72.73 ± 7.78) years) and 30 age and education matched normal controls from three communities were recruited in Xuanwu Hospital from January 2018 to May 2018.All subjects completed tasks of picture naming,semantic fluency (animal),and Cookie Theft picture description.Picture description was analyzed for speech production (total word output,speech rate,sound errors),lexical content (number of nouns and verbs,proportion of pronouns and verbs,semantic errors),information content (information unit,idea density,efficiency),syntactic structure and complexity (mean length of utterance,words in sentences,syntactic errors).Differences between groups were calculated.Association between picture naming,semantic fluency,and measures in picture descriptions was analyzed.Results Compared with the controls,mild AD patients achieved lower scores on the picture naming task (74.45±8.04 vs 79.60± 1.73,t=-2.800,P=0.010) as well as on the semantic fluency task (10.35±3.08 vs 18.50±4.66,t=-6.520,P＜0.01) and produced lower speech rate (131.80±36.40 vs 149.64±26.69,t=-2.095,P=0.041),less number of nouns (7.52±2.55 vs 9.67±3.58,t=-2.509,P=-0.015) and verbs (4.40±2.24 vs 5.73±1.92,t=-2.375,P=0.021),increased proportion of pronouns (0.286 (0.319) vs 0.039 (0.122),Z=3.602,P＜0.01) and semantic errors (0.629 (1.396) vs 0 (0.779),Z=2.386,P=0.017),less information units (11.00±3.63 vs 13.70±3.53,t=-2.787,P=0.007),decreased idea density (0.11±0.04 vs 0.16±0.05,t=-3.946,P＜0.01),decreased efficiency (14.23±7.56 vs 22.95±7.14,t=-4.738,P＜0.01),shorter mean length of utterance (6.64± 1.27 vs 8.25± 1.70,t=-3.928,P＜0.01),and less words in sentence (0.84±0.10 vs 0.95±0.06,t=-4.625,P＜0.01).Group differences in total word output,sound errors,proportion of verbs,and syntactic errors did not reach statistical significance.Scores on picture naming task were negatively associated with the proportion of pronouns (r=-0.737,P=0.003),and positively associated with the idea density (r=0.540,P=0.046) and efficiency (r=0.592,P=0.026) in the AD group.Scores on semantic fluency task were negatively associated with the proportion of pronouns (r=-0.554,P=0.011).Conclusions Connected speech in mild AD patients was characterized by decreased naming accuracy,decreased sematic fluency,decreased content words,information units,conciseness,efficiency,and syntactic complexity,but motoric aspects of speech and sentence structure remained relatively preserved.Decreased content words and increased pronouns in mild AD may relate to semantic impairment.
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Objective To investigate the clinical features,polysomnography,imaging examination,genetic analysis and laboratory examination of eight patients with familial fatal insomnia (FFI).Methods The clinical data,neuropsychological examination,results of cerebrospinal fluid analysis,imaging examination and polysomnography of eight patients with FFI in Xuanwu Hospital,Capital Medical University from 2009 to 2018 were retrospectively analyzed and summarized.Results Among the eight FFI patients,there were 3 males and 5 females,the onset age being (49.8+14.3) years (19 to 64 years) and the course of disease being eight to 18 months.D178N mutation in the PRNP gene of chromosome 20 and 129 amino acid polymorphisms of M/M were found in genetic examination in all the eight patients,of which five patients had family history.All the patients had sleep disorders,sleep-related involuntary movement,sleep-related dyspnea,laryngeal stridor.All the patients showed rapid progressive dementia with or without symptoms or signs of psychosis,ataxia,pyramidal and extrapyramidal.All the eight patients had progressive sympathetic symptoms,including hypertension,sweating,tachycardia,irregular breathing,and dysarthria.Cerebrospinal fluid 14-3-3 protein was found positive in one patient,and negative in seven patients.Electroencephalograph showed diffuse slow wave and non periodic synchronous discharge.Single-photon emission computed tomography or 18F fluorodeoxyglucose positron emission tomography showed decreased thalamic glucose metabolism in three patients.Seven patients showed decreased total sleep time,sleep awakening cycle disorder,especially the reduction or loss of rapid eye movement,laryngeal stridor and involuntary movement in polysomnography.Conclusions FFI is characterized by sleep disorder,sleep-related involuntary movement,dyspnea,laryngosis,rapid progressive dementia and sympathetic symptoms.The family history,polysomnography and positron emission tomography are helpful for the diagnosis of FFI.PRNP gene detection can confirm the diagnosis of FFI.
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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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Objective To determine the risk factors and clinical features of mild vascular cognitive impairment due to subcortical small vessel disease (mVCI-SSVD).Methods Detailed demographic data,vascular risk factors, past and present history were collected and carefully neurological examination, National Institutes of Health Stroke Scale (NIHSS), as well as Hachinski ischemic score (HIS) were performed on 56 mVCI-SSVD patients.Further, the demographic data and vascular risk factors of mVCI-SSVD patients were compared with those of 80 normal control subjects.Results Proportions of smoking (39.3% (22/56)), hypertension (67.9% (38/56)), and diabetes (25.0% (14/56)) were higher in the patient group than in the normal control group (21.3% (17/80) , 47.5% (39/80), 11.3% (9/80)).Odds ratio (2.32(95% CI 1.05-5.13),2.15 (95% CI 1.02-4.54),2.26(95% CI 0.86-5.92)) between the two groups was statistically significant (P value: 0.039, 0.045, 0.047).There was no difference in terms of hyperlipidemia and cardiac disease between groups.Fifty percent (28/56) mVCI-SSVD patients had a clear stroke history.Twenty-six point eight percent (15/56) patients developed the cognitive impairment with an acute onset.Neurological focal signs presented in 20 patients (35.7%).Twenty four (42.9%) patients with HIS ≤ 4 points.Thirty eight cases (67.9%) scored 0 on NIHSS.Conclusions Current study suggested that smoking, hypertension, and diabetes may be risk factors for mVCI-SSVD.While hyperlipidemia and cardiac disease do not increase the risk of mVCI-SSVD.Unlike mVCI caused by large vessel disease, about half mVCI-SSVD patients lack of stroke history.Most patients show a relatively insidious onset and free of significant neurological focal signs.
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Objective To investigate the mechanisms of decreasing insulin degrading enzyme (IDE) level by mutation V97L in the gene presenilin 1 (PS-1).Methods Transcription factor GATA binding protein 3 (GATA-3) activity was assessed by protein/DNA array and verified by Western blot in SH-SY5Y cells transfected by PS-1 mutation V97L.Results Protein/DNA array and Western blot revealed that there was increased transcript factor activity (5.5 times high) and protein level of GATA-3 in V97L-PS-1 transfected SH-SY5Y cells.Transcription factor GATA-3 can bind to the IDE promoter and negatively control the IDE transcription level.Conclusion PS-1 mutation V97L may regulate the transcription of IDE via GATA-3, and subsequently involve in deposition of Aβ42 and development of Alzheimer's disease.
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Objective To investigate the correlation of the vascular endothelial growth factor (VEGF) gene variations in the promoter region with the sporadic Alzheimer' s disease (SAD) in Chinese Han population for better understanding the mechanism of SAD. MethodsThe polymorphisms of 279 SAD Chinese Han patients from Northern China were analyzed by comparing with those from 317 healthy individuals using the method of polymeraee chain reaction-restriction fragment length polymorphism ( PCR-RFLP) or direct sequencing.The commercial statistics package SPSS 11.5 was used to compare the distribution of the allele and the genotype, and to analyze their correlations with SAD. ResultsThree polymorphism sites were found for the VEGF promoters in the Chinese Han sample group including -2578C/A,- 2549I/D and- 1154G/A.- 2549I/D and- 2578C/A exhibiting strong linkage disequilibrium. Individuals with the A allele at position -2578 had an insertion of 18 nucleotides at -2459I/D, whereas CC homozygotes did not contain th es were found between the SAD patients and the controls in the 3 VEGF polymorphisms. After adjusting the data for gender, age and the ApoE ε4 allele using Logistic regression, the - 1154G/G genotype of the VEGF promoter might increase the risk of SAD in Chinese Han population.Among the subgroup without the ApoE ε4 allele, -2549D/-1154G haplotype might increase the risk for SAD (OR = 1.325, 95％ CI 1.023--1.716, P=0.033). ConclusionsThree polymorphism sites ( -2578C/A, -254911D, and -1154G/A) are found in the VEGF promoter regions in Chinese Han population. The-1154G/G genotype of the VEGF promote appears to increase the risk of SAD in Chinese Han population.In the absence of ApoE ε4, the -2549D/-1154G haplotype of the VEGF promoter appears to affect the risk for SAD.
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Objective To determine the cognitive profile of mild cognitive impairment due to subcortieal small vessel disease(MCI-SSVD)and mild cognitive impairment caused by Alzheimer's disease(MCI-AD)and to establish the best way of differentiating.Methods Extensive neuropsychological tests covenng 5 cognitive domains were performed on 45 MCI-SSVD patients,30 MCI-AD patients,and 61healthy controls.The impaired domains in patient groups were determined.Tests valuable in discriminating MCI-SSVD and MCI-AD were established using logistie regression analysis.Results Both patient groups showed impairments in multiple cognitive domains.The auditory verbal learning test immediate recall(control group 55.48±5.33;MCI-SSVD group 38.55±8.04;MCI-AD group 34.93±8.79;F=113.407,P=0.000),short time delayed recall(control group 13.34±1.38;MCI-SSVD group 8.47±2.18;MCI-AD group 4.06±2.87;F=216.284,P=0.000),and long time delayed recall(control group13.18±1.19;MCI-SSVD group 8.58±2.02;MCI-AD group 3.93±2.84;F=239.394,P=0.000)impaired most.Compared with MCI-SSVD,MCI-AD patients did worse in memory assessments(P=0.000),but better in mental processing and visuoconstruction(P=0.000-0.023).Two tests tapping memory and processing speed in combination could identify 93.3%MCI-SSVD patients and 93.3% MCI-AD patients correctly.Conclusions Current study indicates that both MCI-SSVD and MCI-AD,varying significantly in memory and mental processing speed,have a multiple-domain cognitive deficit,with memory impaired most seriously.Tests involving these 2 domains might be useful in differentiating MCI-SSVD from MCI-AD.
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@#Vascular cognitive impairment(VCI)is a continuum including early cognitive impairment to dementia caused by cerebral vascular disease.VCI was introduced to identify cognitive decline in early stage for valid treatment.There is inconsistence about the cognitive impairment profile in VCI.This paper reviewed the neuropsychological features of VCI.The problems existed in researches were also discussed.
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BACKGROUND: Memory impairment is the main manifestation of Alzheimer disease (AD) and mild cognitive impairment (MCI) conspicuous in the early stage. However, such patients very often develop attentional dysfunction, which may affect their normal work and daily life.OBJECTIVE: To investigate the attentional function, including continuous attention, selective attention and divided attention, of patients with mild AD and of those with MCI.DESIGN: A case-control study.SETTING: Department of neurology of a university hospital and department of psychology in a university.PARTICIPANTS: The research was completed in the Laboratory of Neuropsychology, the First Hospital of Peking University from November 2003 to January 2004. Totally 18 patients with mild AD and 19 with MCI were enrolled in this study, with 20 normal elderly subjects also included to serve as the normal control group.INTERVENTIONS: Several tests were designed to assess the attentional functions of all the subjects, including continuous performance task (CPT) test for assessing continuous attention, simplified Stroop test for selective attention, and dual task test for divided attention.MAIN OUTCOME MEASURES: The reaction time (RT) and the percentage of false response were recorded in CPT test and Stroop test, and performance decrement in dual task test was observed.RESULTS: In CPT test, the RT of mild AD patients was much longer, and the rates of missing and false responses were higher than those of normal control group [(539.29±103.86) vs (458.47±40.87) ms, 4.08% (3. 13, 13.64) vs0.91% (0.60, 1.90), and 1.51% (0.64, 2.54) vs 0.43% (0.37, 0. 84), respectively, P ＜ 0. 05]. The difference between MCI group and normal control group failed to reach statistical significance. The rate of missing response of all the three groups grow higher with the prolongation of time, with the highest occurring in the third 5 minutes of the test.More AD patients showed sustained attention deficiency after 10 minutes of test (33.33% within 10 minutes and 77.8% within 20 minutes). In Stroop test, all the three groups tended to make more false responses under incongruous condition[11.25% (7.50, 22.50), 2.50% ( -2.50, 5.00), 2.50% (0.00, 4. 38) ], exhibiting significant interference effect, which was the most obvious in mild AD group ( P ＜ 0.05). Patients with mild AD responded more slowly than MCI patients and normal control subjects [ (579. 19 ± 89.93) ms,(524.28 ± 68. 96) ms, (486. 75 ± 51.58) ms, respectively, P ＜ 0.05 ]. MCI group made more false responses than normal control group during the whole course of the test as well as under coincident and neutral conditions[5.83% (4. 17, 8.33) vs2.92% (1.67,3.96); 5.00% (2.50, 7.50) vs0.00% (0.00, 2.50); 5.00% (2.50, 10. 00) vs 2.50% (0.00, 5.00), P ＜ 0. 05]. In dual-task test, a significant decrement in mild AD patients was noted without statistical difference between MCI and normal control group[ 1.03 (0.49,3.75),0.46 (0.08, 1.02),0. 10 ( -0.25, 0. 64), P ＜0.05]. Within mild AD group, more patients showed deficiency in Stroop test and CPT test than in dual-task test (81.25%, 77.8%, and 29.41%, respectively).CONCLUSION: Patients with mild AD suffer impairment in continuous,selective and divided attentions, especially obvious in the former two attentional functions. MCI also impairs selective attention as compared with normal aged subjects, with the continuous and divided attention remaining normal. Mild AD patients, MCI patients and normal elderly subjects all show decreased continuous attention, and longer time (than 10 ninutes) of CPT may more sensitively identify continuous attention deficit.
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Objective To determine whether the sustained,selective and diverted attention functions are affected in patients with mild Alzheimer's disease(AD).Methods Several tasks designed to test the attention functions on the computer were performed in patients with mild AD as well as elderly controls: Continuous Performance Task(CPT) for assessing sustained attention function,simplified Stroop task for selective attention function,dual task for diverted attention function.Results In CPT test,mild AD patients responded more slowly((539.29?103.86)ms vs(458.47?40.87)ms,P