Neurological Examination via Telemedicine: An Updated Review Focusing on Movement Disorders.

Patients with movement disorders such as Parkinson's disease (PD) living in remote and underserved areas often have limited access to specialized healthcare, while the feasibility and reliability of the video-based examination remains unclear. The aim of this narrative review is to examine which parts of remote neurological assessment are feasible and reliable in movement disorders. Clinical studies have demonstrated that most parts of the video-based neurological examination are feasible, even in the absence of a third party, including stance and gait-if an assistive device is not required-bradykinesia, tremor, dystonia, some ocular mobility parts, coordination, and gross muscle power and sensation assessment. Technical issues (video quality, internet connection, camera placement) might affect bradykinesia and tremor evaluation, especially in mild cases, possibly due to their rhythmic nature. Rigidity, postural instability and deep tendon reflexes cannot be remotely performed unless a trained healthcare professional is present. A modified version of incomplete Unified Parkinson's Disease Rating Scale (UPDRS)-III and a related equation lacking rigidity and pull testing items can reliably predict total UPDRS-III. UPDRS-II, -IV, Timed "Up and Go", and non-motor and quality of life scales can be administered remotely, while the remote Movement Disorder Society (MDS)-UPDRS-III requires further investigation. In conclusion, most parts of neurological examination can be performed virtually in PD, except for rigidity and postural instability, while technical issues might affect the assessment of mild bradykinesia and tremor. The combined use of wearable devices may at least partially compensate for these challenges in the future.

Double Trouble: Association of Malignant Melanoma with Sporadic and Genetic Forms of Parkinson's Disease and Asymptomatic Carriers of Related Genes: A Brief Report.

Introduction: Previous epidemiological evidence has established the co-occurrence of malignant melanoma (MM) and Parkinson's disease (PD). Shared molecular mechanisms have been proposed to be implicated in this relationship. The aim of the present study was to assess the prevalence of MM in patients with sporadic and genetic types of PD, as well as in asymptomatic carriers of PD-related genes. Methods: Data regarding past medical history and concomitant disease of 1416 patients with PD (including 20 participants with prodromal disease who phenoconverted to PD), 275 healthy controls (HCs) and 670 asymptomatic carriers of PD-related genes were obtained from the database of the Parkinson's Progression Markers Initiative (PPMI). Focus was placed on information about a medical record of MM. We also retrieved data regarding the genetic status of selected PPMI participants with a positive MM history. Results: In total, 46 patients with PD reported a positive MM history. Concerning the genetic forms of PD, nine of these PD patients (2.47%) carried a Leucine Rich Repeat Kinase 2 (LRRK2) gene mutation (mainly the G2019S), while eight (4.49%) harbored a Glucocerebrosidase (GBA) gene mutation (mainly the N370S). No alpha-synuclein (SNCA) gene mutation was identified in patients with an MM history. The remaining 29 PD patients (3.5%) were genetically undetermined. In total, 18 asymptomatic carriers of PD-related genes had a positive medical history for MM: among them, 10 carried an LRRK2 gene mutation (2.69%) and 10 a GBA gene mutation (3.51%) (2 were dual carriers). MM history was identified for seven HCs (2.5%). Conclusions: We replicated the previously reported association between genetically undetermined PD (GU-PD) and MM. A correlation of LRRK2 mutations with the development of MM could not be verified in either symptomatic PD patients or asymptomatic carriers, implicating distinct pathogenetic mechanisms as compared to GU-PD. Importantly, despite the limited literature evidence on Gaucher disease, this study highlights for the first time the relatively high prevalence of MM among asymptomatic and symptomatic PD GBA mutation carriers, with potential clinical implications.

Pharmacological and Non-Pharmacological Treatments for Depression in Parkinson's Disease: An Updated Review.

Depression represents one of the most common non-motor disorders in Parkinson's disease (PD) and it has been related to worse life quality, higher levels of disability, and cognitive impairment, thereby majorly affecting not only the patients but also their caregivers. Available pharmacological therapeutic options for depression in PD mainly include selective serotonin reuptake inhibitors, serotonin and norepinephrine reuptake inhibitors, and tricyclic antidepressants; meanwhile, agents acting on dopaminergic pathways used for motor symptoms, such as levodopa, dopaminergic agonists, and monoamine oxidase B (MAO-B) inhibitors, may also provide beneficial antidepressant effects. Recently, there is a growing interest in non-pharmacological interventions, including cognitive behavioral therapy; physical exercise, including dance and mind-body exercises, such as yoga, tai chi, and qigong; acupuncture; therapeutic massage; music therapy; active therapy; repetitive transcranial magnetic stimulation (rTMS); and electroconvulsive therapy (ECT) for refractory cases. However, the optimal treatment approach for PD depression is uncertain, its management may be challenging, and definite guidelines are also lacking. It is still unclear which of these interventions is the most appropriate and for which PD stage under which circumstances. Herein, we aim to provide an updated comprehensive review of both pharmacological and non-pharmacological treatments for depression in PD, focusing on recent clinical trials, systematic reviews, and meta-analyses. Finally, we discuss the pharmacological agents that are currently under investigation at a clinical level, as well as future approaches based on the pathophysiological mechanisms underlying the onset of depression in PD.

How Well Did the Healthcare System Respond to the Healthcare Needs of Older People with and without Dementia during the COVID-19 Pandemic? The Perception of Healthcare Providers and Older People from the SI4CARE Project in the ADRION Region.

One major challenge during the COVID-19 pandemic was the limited accessibility to healthcare facilities, especially for the older population. The aim of the current study was the exploration of the extent to which the healthcare systems responded to the healthcare needs of the older people with or without cognitive impairment and their caregivers in the Adrion/Ionian region. Data were collected through e-questionnaires regarding the adequacy of the healthcare system and were anonymously administered to older individuals and stakeholder providers in the following countries: Slovenia, Italy (Calabria), Croatia, Bosnia and Herzegovina, Greece, Montenegro, and Serbia. Overall, 722 older people and 267 healthcare stakeholders participated in the study. During the COVID-19 pandemic, both healthcare stakeholders and the older population claimed that the healthcare needs of the older people and their caregivers increased dramatically in all countries, especially in Italy (Calabria), Croatia and BiH. According to our results, countries from the Adrion/Ionian regions faced significant challenges to adjust to the special needs of the older people during the COVID-19 pandemic, which was possibly due to limited accessibility opportunities to healthcare facilities. These results highlight the need for the development of alternative ways of providing medical assistance and supervision when in-person care is not possible.

Neuropsychology in Greece: Results from a survey of practicing professionals.

Neuropsychology is a fast-growing specialty in Greece. This study surveyed the status of neuropsychologists in Greece investigating several aspects of the profession. An online-based questionnaire collected data from December 2019 to February 2020. A total of 133 participants specialized in neuropsychology were included in the final sample: 81% of the participants were women with a mean age of 35 years. In the total sample, 25.8% of the participants reported working in the hospital system, 18.5% in the university or college, and 17.7% in a private practice job. Greek professionals cited to engage actively in assessment (87.9%), in research (65.1%), in rehabilitation (47.7%), and teaching (30.2%). Professionals primarily declared to assess individuals with dementia (80.3%), depression (47.7%), and stroke (44.0%), and they reported neurologists, psychiatrists and psychologists as their leading sources of referrals. The top five perceived barriers to the field include the lack of recognized specialty (75.9%), the lack of clinical training opportunities (63.9%), the lack of strong professional associations (57.9%), the lack of access to neuropsychological instruments (57.9%) and the lack of willingness to collaborate between professionals (48.9%). The average monthly income of professionals represents a ratio of 0.76 in comparison to that of other scientists in the country and is the lowest reported among other countries. Despite the significant development of the profession, it is essential to create more clinical training opportunities, apply practices systematically to diverse populations, redefine the specialty of neuropsychology in the national health system of the country, and advocate for the profession.

Perceptions of Patients, Caregivers, and Healthcare Professionals toward Telemedicine Use for Cognitive and Movement Disorders in the Aegean Islands, Greece: A Pilot Study of the SI4CARE European Project.

BACKGROUND: Patients with neurodegenerative diseases who live in remote areas often have limited access to specialized healthcare, and telemedicine represents a useful solution. The aim of this study was to investigate the perceptions toward the use of a specialized-tertiary telemedicine service of patients with cognitive and movement disorders, caregivers, and local healthcare professionals (HPs) in the Aegean Islands. METHODS: Data were derived from the "Specialized Outpatient Clinic of Memory, Dementia and Parkinson's disease through the National Telemedicine Network", March 2021-March 2023. The survey included 10 questions (5-point Likert scale). RESULTS: We received 64 questionnaires (25 patients, 18 caregivers, 21 HPs). Most participants positively perceived all aspects of telemedicine, including comfort (mean ± standard deviation: patients 4.5 ± 0.9, caregivers: 4.8 ± 0.5, HPs: 4.6 ± 0.7), access to specialized care (4.7 ± 0.6, 4.7 ± 0.5, 4.9 ± 0.4), number of transportations (4.6 ± 0.8, 4.6 ± 0.9, 4.8 ± 0.5), adequacy of follow-up (4.6 ± 0.7, 4.4 ± 0.8, 4.2 ± 0.7), future telemedicine selection (4.8 ± 0.4, 4.8 ± 0.4, 4.6 ± 0.6), perceived reliable medical assessment (4.7 ± 0.5, 4.6 ± 0.6, 4.3 ± 0.6), information delivery (4.7 ± 0.6, 4.6 ± 0.5, 4.4 ± 0.9), health status improvement (4.6 ± 0.7, 4.6 ± 0.6, 4.0 ± 0.7), cost (4.6 ± 1, 4.6 ± 1, 5.0 ± 0.2), and general satisfaction (4.8 ± 0.4, 4.7 ± 0.5, 4.5 ± 0.6). The commonest recommendations were more frequent visits, medical specialties, and dissemination of information. CONCLUSIONS: The positive perception of participants highlights the value of telemedicine for specialized healthcare for neurodegenerative disorders, especially in remote areas.

How Telemedicine Can Improve the Quality of Care for Patients with Alzheimer's Disease and Related Dementias? A Narrative Review.

Background and Objectives: Dementia affects more than 55 million patients worldwide, with a significant societal, economic, and psychological impact. However, many patients with Alzheimer's disease (AD) and other related dementias have limited access to effective and individualized treatment. Care provision for dementia is often unequal, fragmented, and inefficient. The COVID-19 pandemic accelerated telemedicine use, which holds promising potential for addressing this important gap. In this narrative review, we aim to analyze and discuss how telemedicine can improve the quality of healthcare for AD and related dementias in a structured manner, based on the seven dimensions of healthcare quality defined by the World Health Organization (WHO), 2018: effectiveness, safety, people-centeredness, timeliness, equitability, integrated care, and efficiency. Materials and Methods: MEDLINE and Scopus databases were searched for peer-reviewed articles investigating the role of telemedicine in the quality of care for patients with dementia. A narrative synthesis was based on the seven WHO dimensions. Results: Most studies indicate that telemedicine is a valuable tool for AD and related dementias: it can improve effectiveness (better access to specialized care, accurate diagnosis, evidence-based treatment, avoidance of preventable hospitalizations), timeliness (reduction of waiting times and unnecessary transportation), patient-centeredness (personalized care for needs and values), safety (appropriate treatment, reduction of infection risk),integrated care (interdisciplinary approach through several dementia-related services), efficiency (mainly cost-effectiveness) and equitability (overcoming geographical barriers, cultural diversities). However, digital illiteracy, legal and organizational issues, as well as limited awareness, are significant potential barriers. Conclusions: Telemedicine may significantly improve all aspects of the quality of care for patients with dementia. However, future longitudinal studies with control groups including participants of a wide educational level spectrum will aid in our deeper understanding of the real impact of telemedicine in quality care for this population.

Effect of Apolipoprotein E4 on the Driving Behavior of Patients with Amnestic Mild Cognitive Impairment or Mild Alzheimer's Disease Dementia.

BACKGROUND: The driving behavior of patients with mild Alzheimer's disease dementia (ADD) and patients with mild cognitive impairment (MCI) is frequently characterized by errors. A genetic factor affecting cognition is apolipoprotein E4 (APOE4), with carriers of APOE4 showing greater episodic memory impairment than non-carriers. However, differences in the driving performance of the two groups have not been investigated. OBJECTIVE: To compare driving performance in APOE4 carriers and matched non-carriers. METHODS: Fourteen APOE4 carriers and 14 non-carriers with amnestic MCI or mild ADD underwent detailed medical and neuropsychological assessment and participated in a driving simulation experiment, involving driving in moderate and high traffic volume in a rural environment. Driving measures were speed, lateral position, headway distance and their SDs, and reaction time. APOE was genotyped through plasma samples. RESULTS: Mixed two-way ANOVAs examining traffic volume and APOE4 status showed a significant effect of traffic volume on all driving variables, but a significant effect of APOE4 on speed variability only. APOE4 carriers were less variable in their speed than non-carriers; this remained significant after a Bonferroni correction. To further examine variability in the driving performance, coefficients of variation (COV) were computed. Larger headway distance COV and smaller lateral position COV were observed in high compared to moderate traffic. APOE4 carriers had smaller speed COV compared to non-carriers. CONCLUSION: The lower speed variability of APOE4 carriers in the absence of neuropsychological test differences indicates reduced speed adaptations, possibly as a compensatory strategy. Simulated driving may be a sensitive method for detecting performance differences in the absence of cognitive differences.

Frontotemporal dementia spectrum: first genetic screen in a Greek cohort.

Frontotemporal dementia (FTD) is a heterogeneous group of neurodegenerative syndromes associated with several causative and susceptibility genes. Herein, we aimed to determine the incidence of the most common causative dementia genes in a cohort of 118 unrelated Greek FTD spectrum patients. We also screened for novel possible disease-associated variants in additional 21 genes associated with FTD or amyotrophic lateral sclerosis. Pathogenic or likely pathogenic variants were identified in 16 cases (13.6%). These included repeat expansions in C9orf72 and loss-of-function GRN variants, and likely pathogenic variants in TARDBP, MAPT, and PSEN1. We also identified 14 variants of unknown significance in other rarer FTD or amyotrophic lateral sclerosis genes that require further segregation and functional analysis. Our genetic screen revealed a high genetic burden in familial Greek FTD cases (30.4%), whereas only two of the sporadic cases (3.5%) carried a likely pathogenic variant. A substantial number of familial cases still remain without an obvious causal variant, suggesting the existence of other FTD genetic causes besides those currently screened in clinical routine.

123I-FP-CIT SPECT [(123) I-2ß-carbomethoxy-3ß-(4-iodophenyl)-N-(3-fluoropropyl) nortropane single photon emission computed tomography] Imaging in a p.A53T α-synuclein Parkinson's disease cohort versus Parkinson's disease.

BACKGROUND: The p.A53T point mutation in the α-synuclein gene (SNCA) is a rare but highly relevant cause of autosomal dominant Parkinson's disease (PD). OBJECTIVES: The objective of this study was to assess striatal dopaminergic denervation in a cohort of symptomatic carriers of the p.A53T SNCA mutation as compared to PD patients. METHODS: Data from the Parkinson's Progression Markers Initiative database of 11 symptomatic p.A53T SNCA mutation carriers who underwent 123I-FP-CIT SPECT [(123) I-2ß-carbomethoxy-3ß-(4-iodophenyl)-N-(3-fluoropropyl) nortropane single photon emission computed tomography] imaging at our site were compared with those of 33 age-, sex-, and disease duration-matched PD patients. RESULTS: The p.A53T mutation carriers had significantly lower caudate nucleus binding ratio both contralaterally and ipsilaterally to the most affected side (P = .002 and P = .006) and a decreased contralateral caudate/putamen signal ratio (P = .007) as compared to PD. A similar degree of striatal asymmetry was observed in both subgroups. No correlation between scores in neuropsychological tests and caudate nucleus dopaminergic denervation could be demonstrated. CONCLUSIONS: PD patients harboring the p.A53T SNCA mutation show evidence of a more severe nigrostriatal denervation, especially evident in the caudate nucleus. The lack of significant differences in the putaminal binding ratios may reflect a floor effect or a true preferential targeting of the caudate terminals in p.A53T SNCA-associated PD. © 2018 International Parkinson and Movement Disorder Society.

Exploring the Profile of Incidental Memory in Patients with Amnestic Mild Cognitive Impairment and Mild Alzheimer's Disease.

Incidental memory can be defined as the ability to acquire information unintentionally. The present study investigated incidental memory performance in amnestic mild cognitive impairment (aMCI) and mild Alzheimer's disease (AD) patients; additionally, hippocampal atrophy between groupswas examined. Twenty-nine aMCI patients (14 with hippocampal atrophy, measured by the Medial Temporal Lobe Atrophy scale), 15 mild AD patients, and 20 cognitively intact individuals underwent a detailed medical and neuropsychological assessment examining intentional memory, using the Hopkins Verbal Learning Test-Revised and the Brief Visuospatial Memory Test. Participants first took part in a driving simulator experiment, followed by an unexpected incidental memory questionnaire referring to elements related to the driving simulation. The mild AD group performed worse than the aMCI group and the control group both in incidental and intentional memory tasks, whereas the aMCI group differed significantly from the control group only in the intentional memory tasks. The incidental recognition memory task was the only measure that differed between aMCI patients with and without hippocampal atrophy. Moreover, incidental memory tasks were the only measures that correlated significantly with both left and right hippocampal atrophy. The current findings indicate that incidental memory testing may provide potentially useful information for detecting aMCI patients with greater hippocampal atrophy, who may be considered at higher risk of developing dementia due to AD.

Self-awareness of Driving Ability in the Healthy Elderly and Patients With Mild Cognitive Impairment (MCI).

INTRODUCTION: According to latest research, a percentage of cognitively impaired drivers fail to recognize their areas of weakness and overestimate their driving abilities. METHODS: Twenty-seven individuals with amnestic mild cognitive impairment (MCI) and 26 healthy elderly drivers participated in a driving simulator study. After the driving assessment, participants were asked to self-evaluate their performance in comparison with what they considered as average for people of similar age and educational level. RESULTS: According to the applied mixed analysis of variance model, the MCI patients presented increased difficulties in estimating their driving performance to a greater extent in the rural environment in comparison with the urban condition. DISCUSSION: Our findings suggest that the ability of MCI patients to evaluate their driving performance accurately seems to be enhanced or compromised, depending on the number of cues available in their environment, suggesting that providing feedback may improve their metacognitive abilities.

Selective cognitive impairment and hyposmia in p.A53T SNCA PD vs typical PD.

OBJECTIVE: To evaluate nonmotor symptoms in early SNCA/p.A53T Parkinson disease (PD) (A53T PD) compared to typical PD (tPD). METHODS: The presence of hyposmia, neuropsychiatric, dysautonomic, and sleep disturbances was assessed by standardized questionnaires and validated scales in 18 patients with A53T PD and 18 patients with tPD, matched for age, sex, and disease duration. All patients were enrolled into the Parkinson's Progression Markers Initiative study. RESULTS: The levodopa equivalent daily dose was higher in the A53T PD (p = 0.018) group vs the tPD group. Scores on the University of Pennsylvania Smell Identification Test (p = 0.001), Benton Judgement of Line Orientation test (p = 0.001), Letter Number Sequencing Test (p = 0.002), and phonemic verbal fluency (p = 0.002) were lower in the A53T PD group vs the tPD group. In contrast, overall cognition, verbal memory, and semantic fluency were similar between groups. CONCLUSION: The observed selective cognitive impairment reflecting frontal-parietal network dysfunction, together with impaired olfaction, define a set of nonmotor dysfunctions related to A53T PD. These results have implications for the prognosis of patients with A53T PD. Moreover, as the archetypal α-synucleinopathy, such results may give insights into tPD.

Mild Cognitive Impairment and driving: Does in-vehicle distraction affect driving performance?

OBJECTIVES: In-vehicle distraction is considered to be an important cause of road accidents. Drivers with Mild Cognitive Impairment (MCI), because of their attenuated cognitive resources, may be vulnerable to the effects of distraction; however, previous relevant research is lacking. The main objective of the current study was to explore the effect of in-vehicle distraction on the driving performance of MCI patients, by assessing their reaction time at unexpected incidents and accident probability. METHODS: Thirteen patients with MCI (age: 64.5±7.2) and 12 cognitively intact individuals (age: 60.0±7.7), all active drivers were introduced in the study. The driving simulator experiment included three distraction conditions: (a) undistracted driving, (b) conversing with passenger and (c) conversing through a hand-held mobile phone. RESULTS: The mixed ANOVA models revealed a greater effect of distraction on MCI patients. Specifically, the use of mobile phone induced a more pronounced impact on reaction time and accident probability in the group of patients, as compared to healthy controls. On the other hand, in the driving condition "conversing with passenger" the interaction effects regarding reaction time and accident probability were not significant. Notably, the aforementioned findings concerning the MCI patients in the case of the mobile phone were observed despite the effort of the drivers to apply a compensatory strategy by reducing significantly their speed in this driving condition. CONCLUSION: Overall, the current findings indicate, for the first time, that a common driving practice, such as the use of mobile phone, may have a detrimental impact on the driving performance of individuals with MCI.

Incidental and Intentional Memory: Their Relation with Attention and Executive Functions.

OBJECTIVE: The aim of the current study was to investigate the impact of gender and age on incidental and intentional memory in healthy participants and to explore the strength of the association of incidental and intentional memory with attentional and executive functioning. METHOD: A total number of 47 participants underwent a driving simulation experiment and went through detailed neuropsychological testing. Incidental memory was assessed with a questionnaire that evaluated the memorization of information related to the driving simulator task while intentional memory was assessed using the Hopkins Verbal Learning Test-Revised and the Brief Visuospatial Memory Test-Revised. RESULTS: The analysis revealed a greater impact of age on incidental as compared to intentional memory. Gender did not appear to have such an effect on either incidental or intentional memory. Finally, attentional and executive functioning were more strongly associated with incidental memory than the intentional memory measures that were utilized in the current study. CONCLUSIONS: Ageing appears to affect incidental rather than intentional memory to a greater extent. In addition, attentional and executive functioning seem to play a more important role in incidental than intentional encoding and consolidation processes.

Driving in mild cognitive impairment: The role of depressive symptoms.

OBJECTIVES: Previous studies indicate a negative association between depression and driving fitness in the general population. Our goal was to cover a gap in the literature and to explore the link between depressive symptoms and driving behavior in individuals with mild cognitive impairment (MCI) through the use of a driving simulator experiment. METHODS: Twenty-four individuals with MCI (mean age = 67.42, SD = 7.13) and 23 cognitively healthy individuals (mean age = 65.13, SD = 7.21) were introduced in the study. A valid driving license and regular car use served as main inclusion criteria. Data collection included a neurological/neuropsychological assessment and a driving simulator evaluation. Depressive symptomatology was assessed with the Patient Health Questionnaire (PHQ-9). RESULTS: Significant interaction effects indicating a greater negative impact of depressive symptoms in drivers with MCI than in cognitively healthy drivers were observed in the case of various driving indexes, namely, average speed, accident risk, side bar hits, headway distance, headway distance variation, and lateral position variation. The associations between depressive symptoms and driving behavior remained significant after controlling for daytime sleepiness and cognition. CONCLUSIONS: Depressive symptoms could be a factor explaining why certain patients with MCI present altered driving skills. Therefore, interventions for treating the depressive symptoms of individuals with MCI could prove to be beneficial regarding their driving performance.

Frontotemporal dementia as the presenting phenotype of p.A53T mutation carriers in the alpha-synuclein gene.

INTRODUCTION: The p.A53T point mutation in SNCA, the alpha-synuclein gene, has been linked to a rare dominant form of Parkinson's disease (PD). METHODS: Here, we describe two apparently unrelated cases of p.A53T (G209A) SNCA mutation carriers with an atypical initial manifestation and disease course. Moreover, cerebrospinal fluid (CSF) levels of tau, p-tau and amyloid Aß42 were measured in these patients and in an additional cohort of 5 symptomatic and 2 asymptomatic p.A53T carriers without an initial manifestation of dementia. RESULTS: Both patients exhibited an early onset frontal-dysexecutive dysfunction with apathy and emotional blunting resembling frontotemporal dementia (FTD). Motor symptoms typical of Parkinson's disease appeared only later in the disease course and were less prominent than cognitive ones, which included language impairment. Autonomic dysfunction and myoclonus also emerged in a more advanced disease stage. In both patients, Brain Magnetic Resonance Imaging showed fronto-temporo-parietal atrophy, and CSF analysis showed elevated tau protein levels. In contrast, tau protein levels were normal in a cohort of 7 other p.A53T mutation carriers (5 symptomatic/2 asymptomatic). A screen of Greek patients presenting with frontotemporal dementia failed to identify any additional subjects with the p.A53T SNCA mutation. CONCLUSION: Although cognitive decline has been recognized as a feature of the full-blown clinical picture of p.A53T related parkinsonism, a predominant frontotemporal dementia-like phenotype at presentation has not been previously described. This may represent a subtype of this disorder, with distinctive clinical, imaging and CSF biochemical characteristics, in which additional genetic or epigenetic factors may play a role.

Self-awareness of cognitive efficiency: Differences between healthy elderly and patients with mild cognitive impairment (MCI).

INTRODUCTION: Self-estimation of performance implies the ability to understand one's own performance with relatively objective terms. Up to date, few studies have addressed this topic in mild cognitive impairment (MCI) patients. The aim of the present study was to compare objective measures of performance with subjective perception of specific performance on cognitive tests and investigate differences in assessment between MCI patients and healthy elderly. METHOD: Thirty-five participants diagnosed with MCI (women = 16, men = 19, mean age = 65.09 years ±SD = 7.81, mean education = 12.83 years ±SD = 4.32) and 35 control subjects similar in terms of age and education (women = 20, men = 15, mean age = 62.46 years ± SD = 9.35, mean education = 14.26 ± SD = 2.84) were examined with an extended battery of neuropsychological tests. After every test they were asked to self-evaluate their performance by comparing it to what they considered as average for people of their age and educational level. This self-evaluation was reported on a scale ranging from -100 to +100. RESULTS: Significant differences were found in the self-assessment patterns of the two groups in memory measures of verbal and visual delayed recall, visuospatial perception, and tests of attention. MCI patients overestimated their performance on every cognitive domain while control participants underestimated their performance on measures of verbal memory. CONCLUSIONS: The present results indicate that accuracy of self-report is not uniform across groups and functional areas. The discrepancies in the MCI patients indicate unawareness of their memory deficits, which is contradictory to subjective memory complaints as being an important component for clinical diagnosis.