Cortical thinning associated with mild cognitive impairment in Parkinson's disease

The aim of this study was to investigate patterns of cortical atrophy associated with mild cognitive impairment in a large sample of nondemented Parkinson's disease (PD) patients, and its relation with specific neuropsychological deficits. Magnetic resonance imaging (MRI) and neuropsychological assessment were performed in a sample of 90 nondemented PD patients and 32 healthy controls. All underwent a neuropsychological battery including tests that assess different cognitive domains: attention and working memory, executive functions, memory, language, and visuoperceptual‐visuospatial functions. Patients were classified according to their cognitive status as PD patients without mild cognitive impairment (MCI; n = 43) and PD patients with MCI (n = 47). Freesurfer software was used to obtain maps of cortical thickness for group comparisons and correlation with neuropsychological performance. Patients with MCI showed regional cortical thinning in parietotemporal regions, increased global atrophy (global cortical thinning, total gray matter volume reduction, and ventricular enlargement), as well as significant cognitive impairment in memory, executive, and visuospatial and visuoperceptual domains. Correlation analyses showed that all neuropsychological tests were associated with cortical thinning in parietotemporal regions and to a lesser extent in frontal regions. These results provide neuroanatomic support to the concept of MCI classified according to Movement Disorders Society criteria. The posterior pattern of atrophy in temporoparietal regions could be a structural neuroimaging marker of cognitive impairment in nondemented PD patients. All of the neuropsychological tests reflected regional brain atrophy, but no specific patterns were seen corresponding to impairment in distinct cognitive domains. © 2014 International Parkinson and Movement Disorder Society     

Cognitive impairment 3 years after neurological Varicella-zoster virus infection: a long-term case control study

Varicella-zoster virus (VZV) is one of the most common viruses causing central nervous system (CNS) infection, sometimes with severe neurological complications and sequelae despite appropriate antiviral treatment. Whether the neurological sequelae of VZV CNS infections include long-term cognitive impairment and how this impairment might affect the patients is still largely unknown. In this study, 14 patients with predominant CNS manifestations caused by VZV infection underwent cognitive testing 3 years (median 39.5 months, range 31–52 months) after acute disease. The results were compared with those for 28 controls, matched for age and gender. The tests covered the cognitive domains of speed and attention, memory and learning, visuospatial function, language and executive function. To further assess the cognitive dysfunction caused by neurological VZV infection, patients were classified into the concept of mild cognitive impairment (MCI), which is associated with development of dementia in other pathologies. The VZV patients performed significantly worse than controls on four tests covering the domains of speed and attention, memory and learning and executive function. The cut-off was set at 1.5 SD below mean age. In addition, a greater proportion of VZV patients were classified with MCI as compared with controls. In conclusion, patients with previous VZV infection affecting the brain had signs of long-term cognitive impairment in the domains of speed and attention, memory and learning and executive function. However, larger study populations are needed to confirm these results.     

Multiple cognitive deficits in amnestic mild cognitive impairment

OBJECTIVE: To determine if more widespread cognitive deficits are present in a narrowly defined group of patients with the amnestic form of mild cognitive impairment (MCI). METHODS: From a larger sample of patients clinically diagnosed as meeting the criteria of Petersen et al. for amnestic MCI, we selected 22 subjects who had Clinical Dementia Rating scores of zero on all domains besides memory and orientation. These MCI subjects with presumably isolated memory impairments were compared to 35 age-matched normal controls and 33 very mild Alzheimer's disease (AD) patients on a battery of neuropsychological tests. RESULT: In addition to the expected deficits in episodic memory, the amnestic MCI group performed less well than the controls but better than the AD group on design fluency, category fluency, a set shifting task and the Stroop interference condition. Over half the amnestic MCI group (vs. none of the normal controls) scored at least 1 standard deviation below control means on 4 or more of the nonmemory cognitive tasks. CONCLUSIONS: Isolated memory impairment may be fairly uncommon in clinically diagnosed amnestic MCI patients, even when the criteria for amnestic MCI are fairly narrow. Additional cognitive impairments are likely to include fluency and executive functioning. These more diffuse deficits argue for comprehensive cognitive assessments, even when the patient and family are reporting only memory decline, and are consistent with the increase in attention paid to the heterogeneity of MCI.     

Early detection of cognitive disturbances in mild cognitive impairment: a systematic review of observational studies

Mild cognitive impairment (MCI) is an intermediate state between normal cognition and early dementia and is not considered as a typical outcome of brain aging. It has been estimated that 10% to 20% of individuals above 65 years of age will be diagnosed as having MCI. The increased rate of dementia and the importance of early detection of its forerunners have encouraged researchers to focus on detecting MCI and modifiable risk factors with the hope of developing better ways of managing dementia and its consequences. The main aim of this study was to systematically review the related literature concerning the cognitive changes in the spectrum of cognitive aging to cognitive impairment. Articles included in this review were identified through searching the databases of PubMed, Psych Info, Embase, ProQuest, and Scopus. Many domains like verbal memory, language, executive function, visual memory, attentional skills, and working memory showed acceptable predictive power. Testing subdomains such as executive function, speed of processing, working memory and semantic language are critical and others may indicate some suggestions for further clinical deteriorations in normal individuals. Although various cognitive instruments have been used for evaluation of impaired cognitive domains, it remains challenging to select the most appropriate ones having high‐level accuracy and their related cognitive subdomains. It also revealed that none of the identified cognitive domains solely fulfilled the criteria for MCI screening; in clinical settings, multiple neuropsychological batteries may be used for one single cognitive domain, while longitudinal studies prefer the use of at least two cognitive measures for each domain to improve accuracy and research settings might focus on only a single neuropsychological test. However, along with episodic memory, testing for amnestic MCI, executive function could increase the chance of early detection of MCI. Executive control has been found to deteriorate the earliest in MCI patients.     

Neuropsychological characteristics of mild cognitive impairment subgroups

OBJECTIVE: To describe the neuropsychological characteristics of mild cognitive impairment (MCI) subgroups identified in the Cardiovascular Health Study (CHS) cognition study. METHODS: MCI was classified as MCI-amnestic type (MCI-AT): patients with documented memory deficits but otherwise normal cognitive function; and MCI-multiple cognitive deficits type (MCI-MCDT): impairment of at least one cognitive domain (not including memory), or one abnormal test in at least two other domains, but who had not crossed the dementia threshold. The MCI subjects did not have systemic, neurological, or psychiatric disorders likely to affect cognition. RESULTS: MCI-AT (n = 10) had worse verbal and non-verbal memory performance than MCI-MCDT (n = 28) or normal controls (n = 374). By contrast, MCI-MCDT had worse language, psychomotor speed, fine motor control, and visuoconstructional function than MCI-AT or normal controls. MCI-MCDT subjects had memory deficits, though they were less pronounced than in MCI-AT. Of the MCI-MCDT cases, 22 (78.5%) had memory deficits, and 6 (21.5%) did not. MCI-MCDT with memory disorders had more language deficits than MCI-MCDT without memory disorders. By contrast, MCI-MCDT without memory deficits had more fine motor control deficits than MCI-MCDT with memory deficits. CONCLUSIONS: The most frequent form of MCI was the MCI-MCDT with memory deficits. However, the identification of memory impaired MCI groups did not reflect the true prevalence of MCI in a population, as 16% of all MCI cases and 21.5% of the MCI-MCDT cases did not have memory impairment. Study of idiopathic amnestic and non-amnestic forms of MCI is essential for an understanding of the aetiology of MCI.     

轻度认知功能障碍患者的神经心理学研究

目的 探讨轻度认知功能障碍(MCI)患者神经心理学的特点. 方法 对42例MCI患者和55例健康对照者进行多项神经心理学检查,包括简易精神状态检查量表(MMSE)、蒙特利尔认知评估量表(MoCA)、临床痴呆评定量表(CDR)、语言流畅性测验(RVR)、韦氏智力测验(WAIS-RC)[包括数字广度测验(DS)、积木测验(BD)、相似性测验]、韦氏记忆测验(WMS-R)(包括逻辑记忆、联想学习、视觉再认、图片回忆)、日常生活能力量表(ADL),比较2组患者上述量表评分和MMSE、MoCA量表各亚项评分的差异.结果 与对照者比较,MCI患者MMSE、MoCA总分和RVR、WAIS-RC、WMS-R分测验,MoCA量表各亚项(地点定向力除外),MMSE量表中计算与注意、延迟回忆两亚项评分较低,差异均有统计学意义(P<0.05).结论 MCI患者不仅记忆受损,其计算与注意力、命名、视空间结构能力、执行功能也可受损,尤以延迟回忆、计算与注意力受损明显.MoCA涵盖了重要的认知领域,能较全面评估MCI患者的认知功能,值的临床推广应用.     

EEG correlates in the spectrum of cognitive decline.

OBJECTIVE: To investigate relations between EEG measures and performance on tests of global cognition, memory, language and executive functioning. METHODS: Twenty-two controls, 18 patients with mild cognitive impairment (MCI) and 16 with probable Alzheimer's disease (AD) underwent neuropsychological and EEG investigations. We used the following EEG measures: theta relative power during eyes closed, alpha reactivity during memory activation (i.e. the percentual decrease in alpha power as compared to eyes closed) and alpha coherence during eyes closed and memory activation. RESULTS: Theta relative power was increased in AD patients as compared with controls (p<0.001) and MCI patients (p<0.01) and related to decreased performance in all cognitive domains. Alpha reactivity was decreased in AD patients as compared with controls (p<0.005) and related to decreased performance on tests of global cognition, memory and executive functioning. Alpha coherence did not differ between groups and was unrelated to cognition. CONCLUSIONS: EEG power measures were associated with decreased performance on tests of global cognition, memory, language and executive functioning, while coherence measures were not. SIGNIFICANCE: The EEG yielded several power measures related to cognitive functions. These EEG power measures might prove useful in prospective studies aimed at predicting longitudinal cognitive decline and dementia.     

Longitudinal change in neuropsychological performance using latent growth models: a study of mild cognitive impairment

The goal of the current study was to examine cognitive change in both healthy controls (n?=?229) and individuals with mild cognitive impairment (MCI) (n?=?397) from the Alzheimer’s Disease Neuroimaging Initiative (ADNI). We applied latent growth modeling to examine baseline and longitudinal change over 36 months in five cognitive factors derived from the ADNI neuropsychological test battery (memory, executive function/processing speed, language, attention and visuospatial). At baseline, MCI patients demonstrated lower performance on all of the five cognitive factors when compared to controls. Both controls and MCI patients declined on memory over 36 months; however, the MCI patients declined at a significantly faster rate than controls. The MCI patients also declined over 36 months on the remaining four cognitive factors. In contrast, the controls did not exhibit significant change over 36 months on the non-memory cognitive factors. Within the MCI group, executive function declined faster than memory, while the other factor scores changed slower than memory over time. These findings suggest different patterns of cognitive change in healthy older adults and MCI patients. The findings also suggest that, when compared with memory, executive function declines faster than other cognitive factors in patients with MCI. Thus, decline in non-memory domains may be an important feature for distinguishing healthy older adults and persons with MCI.     

Cognitive impairment in amyotrophic lateral sclerosis: evidence from neuropsychological investigation and event-related potentials

The presence of subclinical cognitive impairment in patients with amyotrophic lateral sclerosis (ALS) is investigated using neuropsychological assessment and event-related potential recordings (ERP). An extensive battery of neuropsychological tests assessing the domains of attention, memory, language, visuo-spatial and executive functions were administered to 20 non-demented patients with sporadic ALS and 13 age- and education-matched healthy control subjects. Mismatch negativity (MMN), P3b, P3a (novelty P300) and contingent negative variation (CNV) were recorded. ALS patients were significantly impaired in tests of working memory, sustained attention, response inhibition, naming, verbal fluency and complex visuo-spatial processing. The memory impairment seemed to be secondary to deficits in forming learning strategies and retrieval. In ERP recordings, P3a and P3b amplitudes of ALS patients were lower compared with the controls, P3a latencies were significantly longer and mean CNV amplitudes were higher. These results indicate subclinical impairment of cognitive functions in patients with ALS. The pattern of cognitive impairment suggests the dysfunction of the frontal network.     

Neuropsychological profiles in MCI and in depression: Differential cognitive dysfunction patterns or similar final common pathway disorder?

The concept of “mild cognitive impairment” (MCI) refers to alterations in cognition in the transition between normal aging and dementia. However, from a neuropsychological point of view the conventional diagnostic criteria appear not sufficiently valid. In particular, it is still difficult to differentiate between subjects with MCI and subjects with depression plus cognitive deficits on the basis of their neuropsychological profiles. The aim of this study is to compare cognitive deficit patterns of subjects with MCI and with depression. 24 subjects with MCI, 50 subjects with depression (DEP) and 20 healthy control subjects were included (age: 55-74 years). The neuropsychological assessment consisted of standardized tests to assess attention, memory, and executive functions. Compared to healthy controls both subject groups showed significantly lower performance in all cognitive domains. However, we did not find significant differences in cognitive performance between MCI and DEP subjects, neither at baseline nor at follow-up. In addition, preliminary results of follow-up assessments after 2 (DEP) and 6 months (MCI), respectively, revealed no significant changes in cognition in subjects with depression, regardless of whether depressive symptoms had improved. Subjects with MCI also showed no changes in cognition at follow-up. The comparable neuropsychological patterns identified in the two subject groups may be understood as a consequence of similar alterations in cognitive systems, supporting the idea of a final common pathway disorder. Thus, the cognitive deficits present in a subgroup of subjects with depression may possibly better be understood in the context of MCI.     

Executive dysfunction in mild cognitive impairment is associated with changes in frontal and cingulate white matter tracts

Mild cognitive impairment (MCI) may affect multiple neuropsychological domains. While amnestic MCI is associated with Alzheimer's disease, patterns of brain pathology in non-amnestic subtypes have been less studied. Twenty-three patients with attention/executive MCI (a/e MCI), seen at a university-based memory clinic, and 23 normal controls, matched according to age, gender, and education, were included in this study. All subjects were assessed with a neuropsychological test battery, including tests of memory, attention and executive function, and underwent magnetic resonance imaging. Diffusion tensor imaging derived white matter (WM) tract radial and mean diffusivity (DR and MD) were assessed using Tract-Based Spatial Statistics, and cortical thickness (CTH) was assessed using FreeSurfer. This study investigated changes of WM DR/MD and CTH in subjects with a/e MCI, and associations between these changes and different a/e subfunctions. WM DR/MD underlying rostral middle frontal, medial orbitofrontal, caudal anterior cingulate, posterior cingulate, retrosplenial and entorhinal cortices was higher for the a/e MCI than the control group, but CTH was not different from controls in any of the regions. WM DR/MD underlying superior frontal, rostral middle frontal, lateral/medial orbitofrontal and retrosplenial cortices were significantly associated with inhibition/switching performance, while caudal middle frontal CTH was significantly associated with attention and divided attention in the patient group. We conclude that increased WM DR/MD in frontal and cingulate regions and cortical thinning in caudal middle frontal region are both associated with executive dysfunction in MCI.     

Neurocognitive function in clinically stable men with bipolar I disorder or schizophrenia and normal control subjects.

BACKGROUND: Patients with bipolar disorder and schizophrenia have been shown to have neurocognitive deficits when compared with control subjects. The degree and pattern of impairment between psychiatric groups have rarely been compared, especially when subjects are psychiatrically stable. METHODS: Using a standard neurocognitive battery, we compared euthymic outpatients with bipolar disorder (n = 40), stable patients with schizophrenia (n = 20), and subjects with no psychiatric disorder (n = 22). The neurocognitive domains assessed included executive functioning, verbal memory, visual memory, procedural learning, visuoconstructive ability, and language functions. Effect sizes were calculated for each cognitive domain across groups. RESULTS: Stable schizophrenic subjects demonstrated a generalized cognitive impairment across most domains compared with control subjects, with average effect sizes of .9. Euthymic bipolar subjects were significantly impaired compared with control subjects only in executive functioning (Wisconsin Card Sorting Task) and verbal memory (California Verbal Learning Test) domains (effect sizes in the .8-.9 range). Performance on the executive function measures was bimodal among bipolar subjects, suggesting two subgroups: one with relatively normal and one with impaired executive functioning. No significant differences between the bipolar patient group and control subjects were observed in visuoconstructive ability, procedural learning, or language function. CONCLUSIONS: Both euthymic bipolar subjects and relatively stable schizophrenic subjects differed from control subjects in neurocognitive function. Among schizophrenic subjects, a generalized cognitive impairment was observed, and the degree of impairment was greater in the schizophrenic compared with the bipolar subjects. Subjects with bipolar disorder were impaired in two specific domains (verbal memory and executive function). Furthermore, within the bipolar group there was a subset with relatively normal executive functioning and a subset with significant impairment. Possible reasons for the persistence of these neurocognitive deficits in some subjects with bipolar disorder during periods of euthymia are reviewed.     

老年轻度认知障碍记忆损害特点的研究

目的探讨老年轻度认知障碍患者的记忆损害特点,比较不同神经心理学测验对诊断和发现轻度认知障碍的敏感性,筛选临床实用的简捷敏感的神经心理学测验工具。方法轻度认知障碍患者20例,轻度阿尔茨海默病患者26例,健康老年人30名。应用词语回忆测验、图片回忆测验、数字广度测验、本顿视觉保持测验C式A法及D式D法进行测试。结果轻度认知障碍组在图片回忆分、视觉保持测验D式D法正确分及持续错误分上的成绩较正常对照组差(P<0．05);轻度认知障碍组视觉保持测验D法成绩明显差于A法(P<0．01)。结论轻度认知障碍患者对具体视觉形象的记忆能力及对无意义图形的视觉记忆能力受损,存在即刻记忆和延迟记忆障碍,且延迟回忆较即刻回忆损害明显,短时记忆更新的可塑性差;图片回忆测试、本顿视觉保持测验的延迟回忆测试或与即刻回忆联合测试有助于轻度认知障碍的早期发现。     

Prefrontal hypoperfusion and cognitive dysfunction correlates in spinocerebellar ataxia type 6

OBJECTIVE: The aim of this study is to evaluate the correlation between brain perfusion and cognitive dysfunction in spinocerebellar ataxia type 6 (SCA6) patients. METHODS: Thirteen genetically confirmed SCA6 patients and 21 age- and education-matched control subjects were subjected to single photon emission computed tomography (SPECT) and neuropsychological tests. Brain perfusion was examined with SPECT analysis, while general cognition, verbal and visual memory, attention, visuospatial ability, language, executive function, depression, and anxiety were examined with the neuropsychological tests. RESULTS: SCA6 patients showed prefrontal hypoperfusion, and impairments of visual memory, verbal fluency, and executive function compared to control subjects. These neuropsychological impairments in SCA6 patients were significantly correlated with a decrease in prefrontal perfusion. This relation was not correlated to other factors, such as age, education and severity of cerebellar ataxia, which are possible relevant factors associated with cognitive performance. CONCLUSIONS: SCA6 patients have mild cognitive impairment, and correlating prefrontal hypoperfusion. These results indicate cognitive impairment in SCA6 patients resulting from prefrontal hypoperfusion.     

An investigation of PreMCI: subtypes and longitudinal outcomes

Background/AimsTo investigate the clinical features and rates of progression of conditions that are not considered to be normal, but do not fulfill criteria for mild cognitive impairment (MCI).MethodsWe longitudinally evaluated 269 elderly subjects who did not meet formal criteria for MCI at baseline but had: (1) a clinical history suggesting MCI without neuropsychological deficits (PreMCI-Clinical); or (2) neuropsychological deficits on one or more memory measures in conjunction with a negative clinical examination (amnestic PreMCI-NP) or were normal on both neuropsychological and clinical examination.ResultsThe rate of progression to MCI or dementia over an average of 2- to 3 years was 3.7% for no cognitive impairment subjects, whereas it was significantly greater for all PreMCI subtypes (22.0% for PreMCI-Clinical, 38.9% for amnestic PreMCI-NP subjects with two or more memory impairments). Among PreMCI subjects as a whole, lower baseline scores on object memory and category fluency tests were the best predictors of progression to MCI or dementia. Cardiovascular risk factors, Parkinsonian symptoms, and hippocampal atrophy were not associated with progression.ConclusionDistinct PreMCI subtypes defined on the basis of clinical and neuropsychological evaluations were found to have distinct characteristics, but both subtypes demonstrated elevated risk for progression to MCI or dementia. Despite the lack of evidence of clinical impairment, subjects with neuropsychological deficits in two memory domains were particularly at increased risk for progression of their deficits.     

Cognitive deficits in beta-thalassemia major

OBJECTIVES: To assess cognitive functioning in patients affected by beta-thalassemia major (beta-th) by using a neuropsychological battery, and to identify clinical correlates. MATERIAL AND METHODS: Forty-six beta-th patients and 46 controls similar for age, sex, and education participated in the study. All subjects performed a comprehensive neuropsychological battery including tests of abstract reasoning, attention, executive functions, language, constructional/visuospatial skills, and memory. RESULTS: Compared to controls beta-th patients, in particular those showing signs of hemosiderosis, were significantly impaired on all neuropsychological tests. There was no relationship between cognitive performances and signs of deferoxamine toxicity, deferoxamine dosage, and levels of hemoglobin and ferritin, while duration of transfusional therapy and time interval between onset of blood transfusions and onset of chelating treatment correlated with performances of tests assessing abstract reasoning, attention, constructional/visuospatial skills, memory and with the scores of the Mini Mental State Examination. CONCLUSION: Our findings suggest that beta-th is associated with neuropsychological impairment involving multiple cognitive domains and argue for a potential role of hemosiderosis on cognitive functioning.     

轻度认知功能障碍患者的执行功能和工作记忆研究

目的 了解轻度认知功能障碍(MO)患者的执行功能和工作记忆是否损害及工作记忆损害特点,探讨执行功能对MCI患者的日常生活活动能力(ADL)的影响.方法 运用神经心理学测试的方法对30例MCI患者进行执行功能、工作记忆及其他认知功能检查,同时进行ADL评定,另外选择30名健康老人作为对照组.结果 MCI组的执行功能和工作记忆成绩(分)显著低于对照组,其中数字颜色连线干扰(130.8±58.2)、数字颜色连线B(210.2±81.8)、词汇流畅性测试(8.9±5.4)、视觉客体工作记忆(0.73±0.12)和数字广度(3.4±0.9),除视觉空间工作记忆外,与对照组比较(52.0±13.5、121.0±33.4、16.4±5.4、0.85±9.18、4.2±1.1)差异均有统计学意义(t=7.108、5.159、-4.879、-4.351、-2.544,均P<0.01或P<0.05).用多元逐步回归方法分析执行功能对ADL的影响,结果 客体工作记忆、空间工作记忆和词汇流畅性与ADL的影响相关,而客体工作记忆与ADL的影响有显著相关性(β=-0.720,t=-3.571,P=0.001).结论 MCI患者具有明显的执行功能障碍和工作记忆损害,工作记忆与MCI患者的ADL有良好相关性,而且视觉客体工作记忆与MCI患者的ADL测查具有显著相关性,因此MCI患者的执行功能障碍可能是导致其ADL下降和客体工作记忆损害的主要因素.     

帕金森病患者的轻度认知功能障碍

目的评估帕金森病(Parkinson’s disease,PD)患者的认知功能状态,分析帕金森病合并轻度认知功能障碍(Parkinson’s Disease with Mild Cognitive Impairment,PD-MCI)的特点。方法纳入PD患者64例,采用神经心理测试组评估其注意、视空间、执行和记忆等认知域的功能。结果本组PD患者中,认知功能正常24例(37.5%),PD-MCI 30例(46.9%),帕金森病痴呆10例(15.6%)。在PD-MCI患者中,单个认知领域轻度损害(60.0%)较多个认知域型轻度认知损害(40.0%)更常见,累及较多的认知域依次为记忆(20例,66.7%),执行(13例,43.3%),视空间(12例,36.7%)。早期PD患者PD-MCI发生率为36.4%,痴呆发生率为3.0%;中晚期PD患者PD-MCI发生率为58.1%,痴呆发生率达29.0%,两组差异有显著性(χ2=18.222,P<0.001)。PD患者的病情程度与认知功能状态呈负相关(Spearman相关系数=-0.553,P<0.001)。结论轻度认知功能障碍(mild cogni-tive impairment,MCI)在PD患者中常见,即使在疾病早期,其发生率也较高,其中单个认知领域轻度损害较多个认知域型轻度认知损害更常见。PD患者的病情越严重,认知功能状态越差。     

Subjective cognitive complaints, neuropsychological performance, affective and behavioural symptoms in non-demented patients

OBJECTIVE: Subjective cognitive complaints (SCC) have been previously investigated to establish whether they are risk factors for dementia, but no clear-cut conclusions have emerged. In this study non-demented patients with SCC were studied and the neuropsychological findings, affective and behavioural aspects and parameters with the highest correct classifications in discriminating patients who had only SCC but no objective clinical and neuropsychological impairment, i.e. no cognitive impairment (NCI) patients and those with objective neuropsychological deficits, namely patients with mild cognitive (MCI) were analyzed. METHODS: Consecutive non-demented outpatients with SCC were enrolled of over 9 months and examined using neuropsychological tests and scales for depression, anxiety and behaviour. Clinical criteria and neuropsychological test results were used to classify patients into groups of NCI, MCI and subtypes of MCI. RESULTS: Ninety-two patients with SCC were included; 49 of them had objective deficits (MCI patients), whereas 43 were without any clinical and cognitive impairment (NCI patients). These patients had lower age, higher education and better general cognitive indices than MCI patients who had higher caregiver distress, depression and irritability. The combination of a battery for mental deterioration and for behavioural memory assessment were the most discriminative in differentiating the two groups. CONCLUSIONS: An objective cognitive impairment, reaching the criteria for a MCI diagnosis, was present in almost half of patients having SCC. MCI patients have more behavioural disturbances than NCI subjects. SCC should not be underestimated and appropriate neuropsychological assessment is required to reassure subjects with normal results and to identify patients with MCI.     

The Neuropsychological Profile of Infantile Duchenne Muscular Dystrophy

It has been shown that children with Duchenne muscular dystrophy (DMD) exhibit specific cognitive deficits. However, the neuropsychological profile has not yet been fully characterized. In order to control for the contribution of motor impairments as a confounding variable that is usually present when assessing children with muscular pathologies, we compared children with DMD to a group of children with an autoimmune pathology that does not entail either brain or cognitive dysfunction but does imply motor impairment: juvenile rheumatoid arthritis (JRA). An extensive neuropsychological evaluation was administered, including intelligence, perception, language, memory and learning, attention, executive function, praxis and reasoning. As the main result, in children with DMD we found significantly lower performances in verbal IQ, verbal short-term memory and phonological abilities, as well as in praxis and executive functioning domains. A wide range of cognitive domains, including verbal abilities, executive functioning, and praxis, must be taken into account when assessing neuropsychological functioning in children with DMD. In particular, subtle executive function impairment could be considered as a subclinical marker of cognitive impairment in developmental disorders.