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This study aimed at providing standardized regression-based (SRB) reliable change indices (RCIs) for the Italian Edinburgh Cognitive and Behavioral ALS Screen (ECAS). Thirty-one consecutive ALS patients undergoing the ECAS were followed-up (T1) at 6.5 ± 1 months ( range  = 5-8). Ceiling/floor effects, practice effect, and test-retest reliability were assessed. Each ECAS measure was regressed by stepwise-entering as predictors demographics, respective T0 scores, T0 disease duration and ALSFRS-R, retest interval, and progression rate (ΔFS) - i.e., (48 - ALSFRS-R T0 )/disease duration T0 in months. Ceiling effects were infrequently detected, no practice effect emerged and all ECAS measures were reliable at retest (except for Language and Visuo-spatial subscales). T0 scores predicted all ECAS measures except for the Visuo-spatial subscale. The availability of RCIs for the Italian ECAS will aid ALS-related clinical practice and research within the longitudinal dimension.

Background: The present study aimed at (1) providing further validity and reliability evidence for the Italian version of the cognitive section of the ALS Cognitive Behavioral Screen (ALS-CBS™) and (2) testing its diagnostics within an Italian ALS cohort, as well as at (3) exploring its capability to discriminate patients from healthy controls (HCs)., Methods: N = 293 non-demented ALS patients were administered the cognitive sections of the ALS-CBS™ and Edinburgh Cognitive and Behavioural ALS Screen (ECAS). N = 96 HCs demographically matched with N = 96 patients were also administered the cognitive section of the ALS-CBS™. In patients, factorial and construct validity, internal reliability, and diagnostics against a defective score on the cognitive section of the ECAS were tested. Case-control discrimination was assessed via a logistic regression., Results: ALS-CBS™ cognitive subscales were underpinned by a simple, unidimensional structure, internally reliable (McDonald's ω = 0.74), and mostly related with ECAS executive and fluency scores (r s  = 0.54-0.71). Both raw and age- and education-adjusted scores on the cognitive section of the ALS-CBS™ accurately detected ECAS-defined cognitive impairment (AUC = 0.80 and .88, respectively), yielding optimal error-based, information-based and unitary diagnostics. A cut-off of < 15.374 was identified on adjusted scores. The test was able to discriminate patients from HCs (p < 0.001)., Discussion: The cognitive section of the Italian ALS-CBS™ is a valid, reliable, and diagnostically sound ALS-specific screener for detecting frontotemporal, executive-/attentive-based cognitive inefficiency in non-demented ALS patients, being also able to discriminate them from normotypical individuals., (© 2022. Fondazione Società Italiana di Neurologia.)

The Multiple Sclerosis Neuropsychological Questionnaire (MSNQ) is a brief questionnaire useful for screening patients with multiple sclerosis (MS) at risk for cognitive impairment. It includes a patient self-assessment (MSNQ-p) and a section for the caregiver (informant) (MSNQ-i). This study's aim was to validate the Italian version of MSNQ and to compare MSNQ scores with Symbol Digit Modality Test (SDMT), Beck Depression Inventory (BDI), and Expanded Disability Status Scale (EDSS) score, measuring cognitive skills, mood status, and physical disability respectively. We enrolled 122 MS patients (and related caregivers) at MS center of Tor Vergata University Hospital of Rome. The final study sample consisted of 122 patients with MS (90 relapsing-remitting, 24 secondary progressive, and 8 primary progressive). Our results highlighted that MSNQ has a unidimensional factor structure. Correlational analyses found a good correlation between both versions (MSNQ-p and MSNQ-i) of the questionnaire. Both MSNQ-p and MSNQ-i were correlated with clinical variables, specifically with cognitive impairment, mood disorder, and with disability. The Italian version of MSNQ is reliable and useful as screening tool to identify MS patients at high risk of cognitive impairment., (© 2021. Fondazione Società Italiana di Neurologia.)

Context: Cushing's syndrome frequently causes mental health impairment. Data in patients with adrenal incidentaloma (AI) are lacking., Objective: We aimed to evaluate psychiatric and neurocognitive functions in AI patients, in relation to the presence of subclinical hypercortisolism (SH), and the effect of adrenalectomy on mental health., Design: We enrolled 62 AI patients (64.8 ± 8.9 years) referred to our centers. Subclinical hypercortisolism was diagnosed when cortisol after 1mg-dexamethasone suppression test was >50 nmol/L, in the absence of signs of overt hypercortisolism, in 43 patients (SH+)., Interventions: The structured clinical interview for the Diagnostic and Statistical Manual of Mental Disorders-5, and 5 psychiatric scales were performed. The Brief Assessment of Cognition in Schizophrenia (Verbal and Working Memory, Token and Symbol Task, Verbal Fluency, Tower of London) was explored in 26 patients (≤65 years)., Results: The prevalence of psychiatric disorders was 27.4% (SH+ 30.2% vs SH- 21.1%, P = 0.45). SH+ showed a higher prevalence of middle insomnia (by the Hamilton Depression Rating Scale) compared with SH- (51% vs 22%, P = 0.039). Considering the Sheehan Disability Scale, SH+ showed a higher disability score (7 vs 3, P = 0.019), higher perceived stress (4.2 ± 1.9 vs 2.9 ± 1.9, P = 0.015), and lower perceived social support (75 vs 80, P = 0.036) than SH-. High perceived stress was independently associated with SH (odds ratio [OR] = 5.46, confidence interval 95% 1.4-21.8, P = 0.016). Interestingly, SH+ performed better in verbal fluency (49.5 ± 38.9 vs 38.9 ± 9.0, P = 0.012), symbol coding (54.1 ± 6.7 vs 42.3 ± 15.5, P = 0.013), and Tower of London (15.1 vs 10.9, P = 0.009) than SH-. In 8 operated SH+, no significant changes were found., Conclusions: Subclinical hypercortisolism may influence patients' mental health and cognitive performances, requiring an integrated treatment., (© The Author(s) 2020. Published by Oxford University Press on behalf of the Endocrine Society.)

Objective: Cognitive inflexibility and over-attention to detail are two cognitive styles common to eating disorders (ED) and other psychopathologies characterized by rigid perfectionism. Despite many options to assess the above styles, the only self-report tool that simultaneously permits their specific assessment is the Detail and Flexibility Questionnaire (DFlex), originally developed to investigate the ED domain. The aim of this study was to validate the Italian version of the DFlex., Method: 170 patients (149 females) with ED and 148 controls (100 females) completed the Italian version of the DFlex., Results: A Confirmatory Factor Analysis showed that the two-factor model originally proposed in the literature was a good fit to the data. Item factor loadings were largely equivalent across clinical and control groups, with intercept distributions showing higher values for the clinical group compared to controls. Both DFlex subscales showed good internal reliability and were able to predict membership to the group condition., Conclusions: The DFlex is a reliable tool for investigation in the ED population. However, because DFlex items do not explicitly assess food and body shape domains, it is possible to extend its use to other psychopathologies characterized by rigid perfectionism. We propose the use of the DFlex in research and clinical practice as a useful tool for better understanding patients' cognitive functioning and in order to support treatment planning., (© 2020 Eating Disorders Association and John Wiley & Sons Ltd.)

Background and Purpose: Heterozygous mutations in the STUB1 gene have recently been associated with an autosomal dominant form of spinocerebellar ataxia (SCA) associated with cerebellar cognitive-affective syndrome (CCAS), named SCA48., Methods: Molecular screening was performed in a cohort of 235 unrelated patients with adult-onset, autosomal dominant (17) or sporadic (218) cerebellar ataxia, negative for pathological trinucleotide expansions in the common SCAs, FRDA and FXTAS loci, by using targeted multigene panels or whole-exome sequencing. Bioinformatics analyses, detailed neurological phenotyping and family segregation studies corroborated the pathogenicity of the novel STUB1 mutations. Clinico-diagnostic findings were reviewed to define the phenotypic spectrum., Results: Eight heterozygous STUB1 mutations were identified, six of which were novel in 11 patients from eight index families, giving an estimated overall frequency of 3.4% (8/235) for SCA48 in our study cohort, rising to 23.5% (4/17) when considering only familial cases. All our SCA48 patients had cerebellar ataxia and dysarthria associated with cerebellar atrophy on brain magnetic resonance imaging; of note, many cases were also associated with parkinsonism, chorea and dystonia. CCAS also occurred frequently, whereas definite signs of pyramidal tract dysfunction and peripheral nervous system involvement were absent. One SCA48 patient presented with hypogonadism, associated with other autoimmune endocrine dysfunctions., Conclusions: Our results support SCA48 as a significant cause of adult-onset SCA. Besides CCAS, our SCA48 patients often showed movement disorders and other clinical manifestations previously described in SCAR16, linked to biallelic variants in the same gene, thus suggesting a continuous clinical spectrum and significant overlap amongst recessive and dominantly inherited mutations in STUB1., (© 2019 European Academy of Neurology.)

The symptoms related to insular ischemia have been the object of several studies in patients affected by stroke, although they are often accompanied by other ischemic alteration of adjacent brain structures supplied by the middle cerebral artery (MCA). The insula is vulnerable because of an ischemia due to thromboembolic vascular occlusion of the M1 MCA segment and the 2 main MCA branches (M2), mainly when they abruptly arise from the principal stem at a right angle. This topographical and anatomical peculiarity could enable an embolic formation, especially due to atrial fibrillation (AF), to occlude the transition pathway between M1 and M2, while the proximal origin of vascular supply protects the insula from ischemia due to hemodynamic factors. The aim of the study is to characterize the clinical aspects of acute ischemic strokes as a first event in the insular territory with specific attention to atypical manifestation. We have considered 233 patients with a first event stroke involving the insular territory and 13 cases of isolated insular stroke (IIS), from the stroke registry of the Policlinico "G.Martino", University of Messina, between the February 10, 2014 and the February 7, 2018. IIS patients showed CT/MRI lesions restricted to the insular region. Exclusion criteria were coexisting neurological diseases, structural brain lesions, extension to the subinsular area >50% of the total infarct volume. We identified 13 IIS patients (mean age 74 years), with an isolated symptom or a combination of typical and atypical aspects. Furthermore, we observed high frequency detection of cardiac disturbances. To our knowledge, just a few previous studies have described IIS; their incidence is still not well defined. IIS manifested with a combination of deficits including motor, somatosensory, speaking, coordination, autonomic and cognitive disturbances. After an ischemic stroke, AF manifestation could follow briefly the major event and its duration could be very short, as an autonomic dysfunction due to an insular infarction. This clinical condition requires a continuous cardiac monitoring for this dangerous occurrence., (© 2020 S. Karger AG, Basel.)

Background: Neurocognitive deficits at school starting age may affect as many as 50% of children who underwent cardiac surgery for complex congenital heart disease (CHD). The aim of this study was to identify which phases of cardiopulmonary bypass (CPB) are associated with an increased risk of impaired neurodevelopmental skills in children with complex CHD. This was assessed by means of glial fibrillary acidic protein (GFAP) plasma levels during CPB for CHD surgery, as a marker of neurologic insult. We correlated GFAP levels with clinical parameters and neurodevelopmental outcome., Methods: We studied 45 children undergoing surgery for complex CHD. We measured plasma GFAP levels by enzyme-linked immunosorbent assay at the following steps: anesthesia induction, CPB start, end of hypothermia, end of rewarming, and end of CPB. Neurologic assessment and Vineland Adaptive Behavior Scales (VABS-I) were administered to patients at least 18 months after surgery., Results: GFAP was undetectable before surgery and it peaked at the end of hypothermia or rewarming. Multiple regression analyses showed that GFAP peak level and preoperative neurologic comorbidity were significant independent predictors of neurologic impairment, as showed by VABS-I communication domain intelligence quotient (IQ). Receiver operating characteristic curve showed that the model was highly significant., Conclusions: Impaired neurodevelopment was associated with increase of GFAP plasma levels during cardiac surgery in infants. The identification of the neurologic high-risk phases of CPB run could support the application of new neuroprotective strategies for CHD repair., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objective: The study presents data on the longitudinal administration of the Italian Edinburgh Cognitive and Behavioral ALS Screen (ECAS). We investigated cognitive-behavioral performance in a group of ALS patients over time and the feasibility of repeating the ECAS longitudinally compared with standard neuropsychological tests. Finally, correlations between clinical/genetic and cognitive/behavioral data were considered., Methods: One hundred and sixty-eight ALS patients were tested at baseline (T 0 ). Among these, 48 patients performed the ECAS after 6 months (T 1 ), 18 patients performed it at T 2 (12 months), and five patients were assessed after 24 months (T 3 ). Participants were also administered two cognitive test (FAB; MoCA) and psychological questionnaires (BDI; STAI/Y). The FBI was carried out with caregivers., Results: No cognitive deterioration was found across follow-ups. In contrast, although scores did not change between T 0 and T 1 , scores improved significantly for ECAS Total/ALS Non-specific and Memory domains when the ECAS was repeated on three occasions (T 0 , T 1 , T 2 ). Apathy/Inertia was the most common behavioral symptom, but no worsening of behavioral scores was detected over time. After 12-24 months, patients were still able to perform the ECAS in total, in contrast to FAB and MoCA, which were only partially administrable., Conclusions: The significant improvement of some ECAS scores over time supports the presence of possible practice effects, particularly in the memory domain, highlighting the need to accommodate for these in longitudinal assessments, through healthy controls groups or alternate versions. This work represents the first Italian ECAS follow-up study and confirms ECAS feasibility in patients with increasing physical disability.

We conducted a cross-sectional study in 448 HIV positive patients attending five European outpatient clinics to determine prevalence of and factors associated with neurocognitive impairment (NCI) using computerized and pen-and-paper neuropsychological tests. NCI was defined as a normalized Z score ≤-1 in at least 2 out of 5 cognitive domains. Participants' mean age was 45.8 years; 84% male; 87% white; 56% university educated; median CD4 count 550 cells/mm 3 ; 89% on antiretroviral therapy. 156 (35%) participants had NCI, among whom 26 (17%; 5.8% overall) reported a decline in activities of daily living. Prevalence of NCI was lower in those always able to afford basic needs (adjusted prevalence ratio [aPR] 0.71, 95% confidence interval [CI] 0.54-0.94) or with a university education (aPR 0.72, 95% CI 0.54-0.97) and higher in those with severe depressive symptoms (aPR 1.53, 95% CI 1.09-2.14) or a significant comorbid condition (aPR 1.40, 95% CI 1.03-1.90).

Background: Alternating Hemiplegia of Childhood (AHC) is a severe disorder. Several drugs have been administered as prophylaxis for paroxysmal attacks, however, no therapy is completely effective., Methods: Our aim is to review the pharmacological data related to the prophylactic and acute treatment of a cohort of 30 patients (16M, 14F, age range 5-42years) and to correlate them with the clinical and genetic data collected through the Italian Biobank and Clinical Registry for AHC., Results: Flunarizine was the most commonly used long-term treatment in the cohort; it reduced duration and frequency of attacks in 50% of patients and decreased intensity in 32.1%. In younger patients, flunarizine seemed significantly more effective in reducing intensity. We found no correlation between the effectiveness of flunarizine and genotype, or between developmental outcome and duration of treatment. In particular, 3 of our patients affected by E815K mutation presented rapid neurological deterioration despite ongoing treatment. Among the other administered prophylactic therapies, few proved to be effective (benzodiazepines, niaprazine, acetazolamide, melatonin, olanzapine, ketogenic diet). No clear rationale exists regarding their use, but these therapies may work by reducing the triggering factors., Conclusions: The presented data are retrospective, but they are aimed at filling a gap given the rarity of the disease and the lack of randomized and controlled studies. Besides their usefulness in clarifying the pathophysiology of the disease, prospective studies involving larger cohorts of ATP1A3 mutated AHC patients are needed to provide a rationale for testing other molecules., (Copyright © 2017 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.)

Introduction: The prevalence of cognitive impairment may be increased in adults with end-stage kidney disease compared with the general population. However, the specific patterns of cognitive impairment and association of cognitive dysfunction with activities of daily living and clinical outcomes (including withdrawal from treatment) among haemodialysis patients remain incompletely understood. The COGNITIVE impairment in adults with end-stage kidney disease treated with HemoDialysis (COGNITIVE-HD) study aims to characterise the age-adjusted and education-adjusted patterns of cognitive impairment (using comprehensive testing for executive function, perceptual-motor function, language, learning and memory, and complex attention) in patients on haemodialysis and association with clinical outcomes., Methods and Analysis: A prospective, longitudinal, cohort study of 750 adults with end-stage kidney disease treated with long-term haemodialysis has been recruited within haemodialysis centres in Italy (July 2013 to April 2014). Testing for neurocognitive function was carried out by a trained psychologist at baseline to assess cognitive functioning. The primary study factor is cognitive impairment and secondary study factors will be specific domains of cognitive function. The primary outcome will be total mortality. Secondary outcomes will be cause-specific mortality, major cardiovascular events, fatal and non-fatal myocardial infarction and stroke, institutionalisation, and withdrawal from treatment at 12 months., Ethics and Dissemination: This protocol was approved before study conduct by the following responsible ethics committees: Catania (approval reference 186/BE; 26/09/2013), Agrigento (protocol numbers 61-62; 28/6/2013), USL Roma C (CE 39217; 24/6/2013), USL Roma F (protocol number 0041708; 23/7/2013), USL Latina (protocol number 20090/A001/2011; 12/7/2013), Trapani (protocol number 3413; 16/7/2013) and Brindisi (protocol number 40259; 6/6/2013). All participants have provided written and informed consent and can withdraw from the study at any time. The findings of the study will be disseminated through peer-reviewed journals and national and international conference presentations and to the participants through communication within the dialysis network in which this study is conducted., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/)

Dull and hypoxic brains have been a frequent subject in the medical and mountaineering literature. Deterioration of cognitive and other neurological function occurs at high altitude, with or without high altitude cerebral edema. This historical essay explores a 2014 first-ever English translation of cerebral blood flow studies by nineteenth century physiologist Angelo Mosso. Some of the medical history and physiology of brain function is discussed, but much of the style focuses on quotations from the writings of mountaineers and mountaineering physicians to provide color commentary about dull brains at high altitude.

Objective: Cognitive dysfunction is a common sequela of traumatic brain injury (TBI); indeed, patients show a heterogeneous pattern of cognitive deficits. This study was aimed at investigating whether patients who show selective cognitive dysfunction after TBI present a selective pattern of cerebral damage., Setting: Post-Coma Unit, IRCCS Santa Lucia Foundation, Rome, Italy., Participants: We collected data from 8 TBI patients with episodic memory disorder and without executive deficits, 7 patients with executive function impairment and preserved episodic memory capacities, and 16 healthy controls., Design: We used 2 complementary analyses: (1) an exploratory and qualitative approach in which we investigated the distribution of lesions in the TBI groups, and (2) a hypothesis-driven and quantitative approach in which we calculated the volume of hippocampi of individuals in the TBI and control groups., Main Measures: Neuropsychological scores and hippocampal volumes., Results: We found that patients with TBI and executive functions impairment presented focal lesions involving the frontal lobes, whereas patients with TBI and episodic memory disorders showed atrophic changes of the mesial temporal structure (hippocampus)., Conclusion: The complexity of TBI is due to several heterogeneous factors. Indeed, studying patients with TBI and selective cognitive dysfunction should lead to a better understanding of correlations with specific brain impairment and damage, better follow-up of long-term outcome scenarios, and better planning of selective and focused rehabilitation programs.

Introduction: this study aimed to evaluate physical and psychological stress in a group of italian caregivers., Methods: 20 subjects were enrolled in the study and divided into 2 groups: 10 healthy controls and 10 caregivers. Every subject underwent an cognitive, clinical and instrumental assessment., Results: the statistical analysis showed an increased presence of pathological alterations in caregivers group such as decreased ejection fraction, cognitive impairment and depressed mood., Conclusions: the study shows the figure of a elderly caregiver, predominantly women, who spend many hours in patient care. The caregiver-type takes about four drugs a day and is not involved in screening programs offered by the health service for his age group. Results a high risk of social isolation, emotional fragility and an increased risk of cardiovascular events and cognitive impairment.

Aims: Cognitive impairment, anaemia and chronic kidney disease (CKD) are associated with mortality and disability in chronic heart failure patients. We hypothesized that anaemia and CKD are independent predictors of cognitive impairment in older patients with heart failure., Methods: One hundred and ninety community-living elderly patients aged at least 70 years, treated with optimized therapy for heart failure in stable clinical conditions, were prospectively studied. They underwent clinical and multidimensional assessment. Cognitive status was assessed by the Mini Mental State Examination. Cognitive impairment was defined as the Mini Mental State Examination score adjusted by age and educational level below 24. CKD was defined as the Cockcroft-Gault glomerular filtration rate below 60  ml/min and anaemia as haemoglobin below 12  g/dl., Results: Cognitive impairment was diagnosed in 38.9% of patients, CKD in 85.7% and anaemia in 42.6%. Age, female sex, BMI, education less than 5 years, depressive symptoms, anaemia, CKD, disability and worse quality of life were significantly associated with cognitive impairment. Cognitive impairment involved primarily global cognitive deficit, memory, mental speed, attention, calculation and language. A significant relationship between haemoglobin levels and cognitive impairment was found, with the range of 15-16.5  g/dl having the lower prevalence of cognitive impairment (19.4%). At multivariate analysis, advanced age, low education level, anaemia and CKD were independently associated with cognitive impairment. Cox analysis showed that cognitive impairment was an independent predictor of hospitalization for worsening heart failure alone and combined with all-cause death., Conclusion: Cognitive impairment is common in elderly heart failure patients and is independently associated with anaemia and renal dysfunction. Further studies are needed to assess whether optimal treatment of anaemia and CKD may prevent the development of cognitive impairment in heart failure patients.

Objectives: This study was designed to provide further insights into the effects of dyslipidemia (Dys-y) and use of statins (St-y) on cognitive functions and mood in older people., Methods: Three hundred and twenty-nine subjects aged > or = 65 years were screened for cognitive dysfunction using mini mental state examination (MMSE). The geriatric depression scale (GDS) was used to detect depression. Interview questionnaires surveyed activities of daily living (ADL) and instrumental ADL (IADL), as well as other functional disabilities. The presence of neutral lipids (NLs) in cytoplasm of peripheral blood mononuclear cells (PBMCs) was determined with the Oil red O (ORO) staining., Results: There was no significant difference in MMSE and GDS scores between normal (Dys-n) and Dys-y. However, when Dys-y subjects were divided into St-y and non-statin users (St-n), significant differences emerged in the scores of MMSE and GDS: St-y had lower MMSE and higher GDS than St-n. Multiple correspondence analysis and logistic regression provided further evidence that elderly St-y were much more likely to suffer of cognitive impairment and depression than St-n. Another interesting finding was that the intensity of NL-PBMCs measured by ORO staining was greater in subjects with altered MMSE compared with cognitively normal subjects. In addition St-y had higher ORO score than St-n., Discussion: This is an observational study and cannot, therefore, prove a causal relationship between St-y in the elderly and a higher cognitive decline, nevertheless it provides substantial indications that caution should be exercised in the provision of statins in elderly subjects to avoid accelerated memory loss.

Background and Objective: Services dedicated to patients with cognitive and behavioral consequences of cerebrovascular diseases are not well established. In this paper, we report on the general organization of such a service (the Florence VAS-COG Clinic) after 9 years of activity, updating a previous work related to the first 5 years., Methods: The Florence VAS-COG clinic, started in 2006, is an outpatient service dedicated to the assessment and follow-up of patients with cerebrovascular diseases and related cognitive, psychiatric, and behavioral disturbances. The staff involved in the clinic is composed of certified neurologists, one neuropsychologist, and neurology residents. The diagnostic protocol includes detailed personal and family history, general and neurologic examinations, and functional, neuropsychological, and neuroimaging assessment. After this work-up, comprehensive diagnoses are made., Results: From January 2006 to March 2014, 600 patients (mean age 67.3 years ± 13.9; 52% females) have been evaluated in the clinic. Cognitive impairment, including mild cognitive impairment and dementia, mainly of vascular origin, was the most common (36.4%) diagnostic category, followed by suspected or confirmed familial micro-angiopathy (35.8%). Compared to the first years of activity, we are now facing the need of augmenting the number of visits due to increasing request and to better implement the multidisciplinarity of the team. Efforts are currently directed towards the definition of management protocols in pharmacological and non-pharmacological strategies., Conclusions: The establishment of a VAS-COG clinic represents an important step for the appreciation of the patient clinical needs and for the implementation of screening, diagnostic, and treatment options in the field of the neuropsychiatric consequences of cerebrovascular diseases.

The mini mental Parkinson (MMP) is a test built to overcome the limits of the mini mental state examination (MMSE) in the short-time screening of cognitive disorders in individuals with Parkinson's disease (PD). In fact, in this scale, items tapping executive functioning are included to better capture PD-related cognitive changes. Some data sustain the sensitivity and validity of the MMP in the short neuropsychological screening of these individuals. Here, we report normative data on the MMP we collected on a sample of 307 Italian healthy subjects ranging from 40 to 91 years. The results document a detrimental effect of age and an ameliorative effect of education on the MMP total performance score. We provide for correction grids for age and literacy that derive from results of the regression analyses. Moreover, we also computed equivalent scores in order to allow a direct and fast comparison between the performance on the MMP and on other psychometric measures that can be administered to the subjects.

Introduction: No information exists, to our knowledge, about the possible role of cardiovascular drug administration in the acute phase of ischemic stroke and possible effects on stroke outcome. The aim of our study was to evaluate the relationship between in-hospital treatment with cardiovascular drugs in patients with acute ischemic stroke and some outcome indicators., Methods and Results: 1096 subjects enrolled in the GIFA study, who had a main discharge diagnosis of ischemic stroke represent the final sample. Drugs considered for the analysis were the following: ACE-inhibitors (ACEI), angiotensin II receptor blockers (ARBs), statins, calcium-channel-blockers (CCBs), antiplatelet (APL) drugs, antivitamin-k (VKAs), and heparins. As outcome indicators we choose in-hospital mortality, cognitive function evaluated by Hodkinson Abbreviated Mental Test (HAMT), and functional status evaluated by activity daily living (ADL). Indicators of a good outcome were: no in-hospital mortality, HAMT >6 and 0 ADL impaired. Patients with a good outcome showed a higher rate of in-hospital treatment with ACE-inhibitors, calcium-channel blockers and a lower rate of pre-treatment with heparin., Conclusions: Our study suggests that if a patient with acute ischemic stroke has higher SBP at admission, higher total cholesterol plasma levels, a lower Charlson index and is treated with ACE-inhibitors, calcium channel blockers and antiplatelet drugs, the short term outcome is better in terms of in-hospital mortality and functional indicators such as cognitive and functional performance at discharge., (Copyright © 2012 Elsevier B.V. All rights reserved.)

Background: Sickle Cell Disease (SCD) is the most common genetic disease worldwide. Neurological events are among the most worrisome clinical complications of SCD and are frequently accompanied by cognitive impairment. Intellectual function in SCD may vary according to genetic and environmental factors. Immigrant children with SCD are increasing at a global level and display specific health care needs. The aim of our multicenter study was to describe the intellectual function of first generation African immigrants with SCD and the influence of sociodemographic factors on its characteristics., Methods: The Wechsler Intelligence Scales were administered to evaluate broad intellectual functions in children with SCD and in age-matched healthy siblings. Patients' clinical, socio-demographic, Magnetic Resonance Imaging (MRI) and Angiography (MRA) data were correlated to intellectual function scores., Results: 68 children, mean age 8.95 years were evaluated. 72% spoke three languages, 21% two. FSIQ was <75 in 25% of the children. Mean VIQ was lower than PIQ in 75%. Mean verbal subtest scores were lower than performance scores. Female gender, number of languages spoken at home and mother's employment were associated with single subtest performances (p < 0.05). MRA was abnormal in 73.4% and MRI in 35.9%. No significant correlation was established between silent lesions and intellectual function, even if patients with lesions performed worse. Fifteen siblings performed better than patients on cognitive domains, including language (p < 0.05)., Conclusions: Immigrant bilingual children with SCD seem to display a rate of cognitive impairment similar to their monolingual counterparts but a more pronounced and precocious onset of language difficulties. Adjunctive tests need to be considered in this group of patients to better define their specific deficits.

Objective: The objective was to examine whether preexisting cognitive function rather than cognitive decline associated with intraoperative procedures may predict change in behavioral functional capacity in patients 1 year after cardiac surgery., Method: Forty-five patients completed a cognitive evaluation, including the Trail Making Test part B (TMT-B) for attention and psychomotor speed, the Memory with 10-s interference for working memory, the Digit Span test for short-term memory and the Instrumental Activities of Daily Living (IADLs) questionnaire for behavioral functional capacity, before surgery and 1 year after cardiac surgery., Results: Sixteen patients (36%) exhibited cognitive decline after cardiac surgery. Preoperative scores on TMT-B significantly predicted change in behavioral functional capacity as measured by IADLs (beta = 0.371, P < .05), whereas the postoperative cognitive decline and intraoperative variables were unrelated to residualized change scores in IADLs (all Ps > .08)., Conclusions: Preexisting cognitive dysfunctions as assessed by TMT-B can be a marker of preoperative brain dysfunction, which, in turn, in addition to brain damage caused by cardiac surgery procedures, may further predispose patients to poor behavioral functional capacity and outcome 1 year after surgery. Impaired cognitive functions before surgery should be considered when evaluating the effects of cardiac surgery procedures on long-term behavioral functional status of patients., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Objective: Persistent cognitive and behavioural deficits have been documented in children suffering severe TBI. The aim of the present study was to examine the cognitive and adaptive profile of children of school age with severe TBI., Methods: This study selected 118 patients and divided them into three groups according to the severity of their clinical-functional picture. All the patients received a functional assessment using the Wee-FIM. Subjects with reduced responsiveness were evaluated by LOCFAS. Last, the cognitive profile children with a better recovery were described with WISC-III and Leiter-R and their adaptive behaviour with VABS., Results: Group 1 (n = 77) showed a borderline cognitive level with a disharmonious profile between VIQ and PIQ, significant deficits in the Processing Speed and Perceptual Organization Indices, lastly specific adaptive behavioural deficits. Length of coma correlated with their cognitive and adaptive profile. Group 2 (n = 14) included subjects with severe language and/or motor disabilities presenting with a partial cognitive functioning level moderately impaired. Group 3 (n = 27) included patients with reduced responsiveness (LOCFAS ≤ 3)., Conclusions: In the first 12 months following severe TBI, 22.9% children stayed in minimal responsiveness, 11.9% showed debilitating language and motor deficits and 65.2% showed a more favourable cognitive recovery and could be assessed by WISC-III.

Background: Parkinson's disease (PD) occurs more frequently in men than in women and a higher risk for PD development in males compared with females has been hypothesized, suggesting gender may be a significant factor in the development and progression of parkinsonism. To date, gender differences in non-motor symptoms are under-reported., Objective: To assess gender differences in motor and non-motor symptoms among Sardinian PD patients., Methods: One hundred fifty-six (91 male and 65 female) consecutive Sardinian PD outpatients were included in this analysis. Modified Hoehn and Yahr scale and UPDRS were used to assess motor symptoms, while non-motor disturbances were evaluated with the non-motor symptoms scale (NMSS). Presence of depression, anxiety and other iatrogenic behavioral disorders was also investigated. In order to determine how gender differences could be specific to PD, 132 age-matched normal controls were assessed with the NMSS., Results: Women were more likely than men to present with tremor as initial symptom (p<.025) and worse UPDRS instability score (p<.02). NMSS score in females was significantly higher than that in males (p<.018). A significantly higher severity in cardiovascular (p<0.002), sleep/fatigue (p<.018) and mood/apathy (p<.001) domains was observed in female PD patients, while the sexual dysfunction domain was reported with a significantly higher score in male patients (p<.017). Fatigue (p<.03), lack of motivation (p<.015) and sadness (p<.009) were observed significantly more frequent in females, while altered interest in sex was noted as more common in males (p<.001). Frequency of depression (p<.011) and anxiety (p<.001) was significantly higher in females, while male patients had increased frequency of compulsive sexual behaviors (p<.05). There was a significantly higher frequency of non-motor symptoms in eight domains in both male and female PD patients compared with controls (p<.001, for all comparisons, with the exception of urinary disturbances in females: p<.004). Only sexual dysfunctions were not significantly higher in male and female PD patients compared with controls., Discussion: The present study highlights the role of gender differences associated with the occurrence of motor and non-motor disorders and our findings indicate that spectrum and severity of non-motor symptoms may present with different gender distribution in PD patients, suggesting a possible sex-related effect., (Copyright © 2012 Elsevier B.V. All rights reserved.)

While overt hypothyroidism is associated with reversible dementia in the elderly, the relationship of subclinical hypothyroidism with cognition remains a controversial issue. Our aim was to investigate the correlation between subclinical hypothyroidism and cognition in the elderly, with particular reference to long term memory and selective attention. We selected 337 outpatients (177 men and 160 women), mean age 74.3 years, excluding the subjects with thyroid dysfunction and those treated with drugs influencing thyroid function. The score of Mini Mental State Examination (MMSE) was significantly lower in the group of patients with subclinical hypothyroidism than in euthyroid subjects (p<0.03). It was observed that patients with subclinical hypothyroidism had a probability about 2 times greater (RR = 2.028, p<0.05) of developing cognitive impairment. Prose Memory Test (PMT) score resulted significantly lower in subjects with subclinical hypothyroidism (p<0.04). Considering the Matrix Test (MT) score, the performance was slightly reduced in subclinical hypothyroidism (NS). Furthermore, TSH was negatively correlated with MMSE (p<0.04), PMT (p<0.05) and MT score (NS). No correlation was found between FT4 and FT3 and MMSE, PMT and MT score. In the elderly, subclinical hypothyroidism is associated with cognitive impairment, and its impact on specific aspects of cognition (long term memory and selective attention) is less evident.

Acute inflammation is associated with cognitive deficits and alterations of cortical plasticity in multiple sclerosis (MS). We tested whether early treatment with high-dose interferon (IFN) beta-1a, known to reduce inflammatory activity, improves cortical function and cognitive deficits in MS. Eighty treatment-naïve relapsing-remitting MS (RRMS)patients received IFN beta-1a (44 mcg) subcutaneously three times per week. Cognitive performance and cortical plasticity were measured through the paced auditory serial addition test (PASAT) and intermittent theta burst stimulation (iTBS) before and up to two years af-ter IFN beta-1a initiation. Before treatment, patients with gadolinium-enhancing lesions (Gd+) on MRI performed worse on the PASAT,and showed lower iTBS-induced plasticity, compared with Gd- patients. Six months after treatment initiation both PASAT and iTBS-induced plasticity improved in Gd+ and remained stable in Gd- patients. These results suggest that cognitive and synaptic plasticity deficits may be rescued during high-doseIFN beta-1a treatment in newly-diagnosed RRMS patients with Gd+ lesions.

Up to now, controversy still exists regarding the role of secondhand smoking (SHS) in developing cognitive impairment. This study aimed to evaluate the prevalence of SHS in hospitalized older patients with cognitive deficit, particularly in those with mild cognitive impairment (MCI). Smoking history was classified into four groups: never smokers, former-active smokers/no SHS, active smokers, and secondhand smokers, and cognitive function into three levels: normal cognition (C), MCI, and dementia. A total of 933 older subjects with diagnoses of MCI (n = 98), dementia (n = 124), or C (n = 711) were enrolled in this cross-sectional study. As expected, patients with dementia had significantly higher frequency of former-active smokers than cognitively normal. Moreover, patients with MCI showed a significantly higher frequency of active and secondhand smokers than patients with dementia or C. A smoking history is very frequent in older patients with dementia. Patients with MCI had even higher rate of exposure to active or secondhand smoking.

Introduction: The body mass index (BMI) is commonly used to assess nutritional status and the Mini Mental State Examination (MMSE) is a validated tool for assessing cognitive status in elderly people. Nutritional and cognitive aspects are closely related in dementia., Objectives: To establish whether BMI predicts cognitive decline in demented patients and whether an "alarm" BMI cut-off exists for declining MMSE scores., Subjects and Methods: 82 elderly demented patients underwent clinical, bio-chemical and functional assessment., Design: Transversal study., Results: The mean BMI was 26.08±4.48 kg/m² and the mean MMSE 18.68±5.38. Patients with BMI<25 kg/m² had significantly lower MMSE scores (16.5±5.53 vs 20.38±4.64; p 0.001), fat-free mass (FFM; 27.76±8.99 vs 37.38±10.58 kg; p<0.001), fat-free mass index (FFMI; 11.52±3.03 vs 14.67±2.89 kg/m²; p<0.001), and fat mass (FM; 24.90±6.89 vs 36.86±6.77 kg; p<0.001), as well as lower Mini Nutritional Assessment (MNA) scores (23.80±2.50 vs 25.00±2.29; p=0.03) and higher vitamin B12 levels (460.95±289.80 vs 332.43±82.07 pg/ml; p=0.01). In the sample as a whole, MMSE scores significantly correlated with scores for MNA (r=0.27, p=0.01), FFM (r=0.27, p=0.01), BMI (r=0.19, p=0.05), ADL (r=0.28, p=0.01) and instrumental activities of daily living (IADL; r=0.34, p=0.002). On multiple logistic regression, BMI<25 kg/m² was independently associated with the risk of moderate-severe cognitive impairment (OR=2.96; 95% CI; 1.16-7.55) and female gender was independently associated with severity of dementia (OR=3.14; 95% CI; 1.09-9.03)., Conclusion: BMI seems to indicate global health status in elderly demented people and a BMI of 25 kg/m² can be considered an "alarm" cut-off, lower values coinciding with a worse cognitive status based on MMSE scores.

Background: Despite being the fastest growing and the most cognitively impaired age group, the oldest olds are under-represented in clinical research. The purpose of this study was to describe the design, methods, and baseline characteristics of the survey population and investigate possible differences in demographic, cognitive, functional, and behavioral characteristics between oldest old with and without any performance on cognitive tests and between oldest old alive and those deceased prior to the interview., Methods: The Monzino 80-plus Study is a prospective door-to-door population-based survey among 80 years or older residents in the municipalities in the province of Varese, Italy. Dementia cases were identified with a one-phase design. Trained psychologists interviewed both the subject and a proxy informant. The interview included a comprehensive standardized questionnaire together with an array of rating scales and a multidomain cognitive battery to assess cognitive and functional ability, behavioral disturbances and mood., Results: Information was available for 2,139 of the 2,428 registered individuals aged 80 years or older. Main baseline characteristics of the population are reported and discussed. In comparison with those living, elderly persons who had died before the first visit were older, had twice the rate of institutionalization, poorer cognitive performance and competence, and significantly greater instrumental and basic functional disability. The percentage of elderly persons, alive at baseline, without Mini-Mental State Examination rose rather evenly with age. Moreover, they had significantly worse cognitive competence and functional ability, and reported higher prevalences of depressive symptoms and problem behaviors than those with Mini-Mental State Examination., Conclusions: Prospective investigation of a large population of oldest old can contribute significantly to understanding the relations between age, cognitive decline, and dementia occurrence. Use of informant-based instruments in surveys in the oldest old is crucial in assessing everyday functioning and changes, especially in participants with no cognitive test performance available. Failure to include information on deceased elderly would underestimate, increasingly with age, the prevalence of cognitive and functional disability in the elderly population.

Background: Extremely low and very low gestational age (ELGA and VLGA) constitutes a risk factor for development even in absence of cerebral damage, as an immature central nervous system is exposed to invasive and inadequate stimulation. We tested the hypothesis that GA impacts developmental outcomes and trajectories of preterms without major cerebral damage in the first 2 years of life, expecting poorer developmental outcomes and higher rate of impairment with the decreasing of GA. We also evaluated whether GA, together with developmental outcomes in the first year of life, was related to developmental outcomes at 24 months., Methods: Eighty-eight infants, divided into three GA groups (ELGA: ≤28 weeks; VLGA: 29-32 weeks; full term: >37 weeks) were assessed longitudinally at 6, 12, 18 and 24 months using the Griffiths Mental Development Scales., Results: Use of a repeated measure multivariate analysis of variance resulted in several significant findings. GA was associated with the developmental quotient (DQ) scores (P= 0.006); and locomotor (P < 0.001), eye and hand co-ordination (P= 0.016) and performance (P= 0.040) sub-scale quotient (SQ) scores; age of evaluation was also associated with DQ scores (P= 0.002), and locomotor (P < 0.001) and performance (P < 0.001) SQ scores. In particular, ELGAs exhibited lower DQ and SQ scores compared with the VLGA and full-term groups; some ELGAs showed mild, moderate or severe cognitive impairments, while few VLGAs mild impairments. Linear regression analysis showed that GA (P= 0.034) and 12-month developmental outcome (P < 0.001) were related to 24-month developmental outcome., Conclusions: Different developmental trajectories emerged in relation to GA, with poorer developmental outcomes and higher rates of impairment in ELGAs and few mild impairments in VLGAs. The relevance of taking into account both GA and repeated assessments in the first 2 years of life was shown., (© 2010 Blackwell Publishing Ltd.)

Objective: To assess the evolution of cognitive and psychosocial functioning in a cohort of childhood and juvenile multiple sclerosis (MS) cases after a mean period of 2 years had elapsed since baseline evaluation., Methods: In this cohort study, we used the same extensive neuropsychological battery with alternative versions of the tests assessing memory, attention/concentration, executive functions, and language. Fatigue and depression were also measured. An interview on school and daily living activities was obtained from the parents. The cognitive performance of the patients was compared with that of demographically matched healthy controls (HC)., Results: Fifty-six patients and 50 HC were assessed. At follow-up, criteria for cognitive impairment (failure on at least 3 tests) were fulfilled in 39 patients (70%) and 75% of the cases were classified as having a deteriorating cognitive performance. Changes were prominent in tests of verbal memory, complex attention, verbal fluency, and receptive language. In the regression analysis, the only significant predictor of cognitive deterioration was older age of the subject (odds ratio 1.9, 95% confidence interval 1.2-2.9, p = 0.003). Psychiatric disorders, most frequently depression, were diagnosed in 12 patients (30.5%). Fatigue was reported by 21% of the patients. MS negatively affected school and everyday activities in 30% to 40% of the subjects., Conclusions: Our findings confirm the importance of systematic assessment of cognitive and psychosocial issues in children and teens with MS. The progressive nature of the cognitive difficulties emphasizes the need for developing effective treatment strategies.

Objective: Much research on copper-dependent neurodegeneration has focused on the study of total copper levels in the organism. However, recent evidence suggests that the portion of copper that does not bind to ceruloplasmin and is loosely transported by micronutrients (free copper) may play a more significant role than copper as a whole. In this paper, we measured markers of copper metabolism in the sera of a group of cognitively normal women to test whether abnormal amounts of free copper have detectable effects on the mental state of clinically normal people., Methods: We measured serum levels of free and ceruloplasmin-bound copper in 64 women whose normal mental state had been assessed via a battery of neuropsychological tests representing the major cognitive domains., Results: Results show a significant inverse correlation of the serum levels of free copper with both Mini-Mental State Examination (MMSE) and attention-related neuropsychological tests scores. Bound copper, instead, did not correlate with either MMSE scores or any cognitive domain., Conclusions: Free copper appears to be a player in cognitive decline., Significance: This evidence suggests the need for a shift of focus from total to free copper levels in the study of mental decline and sustains the notion that free copper may be a risk factor in the development of impaired cognition., (2010. Published by Elsevier Ireland Ltd.)

The aim of the study is to evaluate whether depression or apathy in patients with amnestic-mild cognitive impairment (MCI) increases the risk of progressing to Alzheimer's disease (AD). We investigated 131 consecutive memory-clinic outpatients with newly-diagnosed amnestic-MCI (mean age 70.8, SD=6.5). Psychiatric disorders were diagnosed at baseline according to the criteria for depression and apathy in AD. Neuropsychiatric symptoms were assessed with the Neuropsychiatric Inventory (NPI). Follow-up examinations were conducted after six months and annually for four years. Neurologists diagnosed AD at follow-up using NINCDS-ADRDA criteria. Cox proportional hazard models with 95% confidence intervals were used to test the hypothesis that apathy or depression increases the risk of developing AD. At baseline, 36.6% amnestic-MCI patients had a diagnosis of depression and 10.7% had apathy. Patients with both amnestic-MCI and an apathy diagnosis had an almost sevenfold risk of AD progression compared to amnestic-MCI patients without apathy (HR=6.9; 2.3-20.6), after adjustment for age, gender, education, baseline global cognitive and functional status, and depression. Furthermore, the risk of developing AD increased 30% per point on the NPI apathy item (HR=1.3; 1.1-1.4). There was no increased risk of developing AD in amnestic-MCI patients with either a diagnosis or symptoms of depression. In conclusion, apathy, but not depression, predicts which patients with amnestic-MCI will progress to AD. Thus, apathy has an important impact on amnestic-MCI and should be considered a mixed cognitive/psychiatric disturbance related to ongoing AD neurodegeneration.

Alzheimer's disease (AD) is characterized by accumulation of toxic amyloid-beta (Abeta) in the brain, with neuronal death, and an associated increased Abeta(42/40) ratio. Several mutations in presenilin 1 (PSEN1), presenilin 2 (PSEN2), and amyloid-beta precursor protein are involved in the etiology of familial AD (FAD); these mutations alter the Abeta(42/40) ratio and promote apoptosis. We describe an Italian pedigree linked to a novel mutation (S175C) at the third transmembrane domain of PSEN2. Clinical phenotype in these individuals is characterized by fast cognitive decline with progressive memory impairment, early involvement of executive functions, behavioral disturbances, and extrapyramidal signs.

A total of 7,389 dementia-free elderly (60-102 years old) enrolled in the "Faenza Project" (Northern Italy) were clinically evaluated by nurses and physicians with the aim of detecting the independent and combined association of medical and social factors with cognitive status. Cognitive Impairment No Dementia (CIND) was defined for MMSE scores ≤ 2 standard deviations than the age- and education-corrected mean score obtained by the nondemented persons of the Faenza cohort. Logistic Regression analysis was used to estimate Odds Ratios and 95% Confidence Intervals (OR, 95%CI) for CIND. The diagnostic procedure identified 402 (5.4%) CIND cases. Diabetes (OR, 95%CI = 1.6, 1.2-2.2), stroke (OR, 95%CI = 1.9, 1.4-2.6), and depressive symptoms (OR, 95%CI = 1.9, 1.4-2.7) emerged as the most relevant medical comorbidities of CIND. Low education (OR, 95%CI = 1.8, 1.1-2.9), low Socio Economic Status (SES) (OR, 95%CI = 1.5, 1.1-2.1), and unmarried status (OR, 95%CI = 1.7, 1.2-2.5) were associated with CIND. Medical and social factors were independently related to CIND occurrence. In comparison to subjects without any of the above mentioned conditions, subjects with one medical and one social factor had an OR, 95%CI for CIND equal to 6.0, 2.9-12.4. The strength of the association increased when more of those conditions occurred in combination, suggesting a synergistic effect. Despite some methodological limitations, data from this cross-sectional population-based Italian study show that low education, low SES, unmarried status together with diabetes, stroke, and depressive symptoms are related to cognitive impairment in the general population. The interaction of medical and social factors further increases the probability of CIND.

We performed a multicenter survey using a semistructured interview in 1,072 consecutive patients with Parkinson's disease (PD) enrolled during 12 months in 55 Italian centers to assess the prevalence of nonmotor symptoms (NMSs), their association with cognitive impairment, and the impact on patients' quality of life (QoL). We found that 98.6% of patients with PD reported the presence of NMSs. The most common were as follows: fatigue (58%), anxiety (56%), leg pain (38%), insomnia (37%), urgency and nocturia (35%), drooling of saliva and difficulties in maintaining concentration (31%). The mean number of NMS per patient was 7.8 (range, 0-32). NMS in the psychiatric domain were the most frequent (67%). Frequency of NMS increased along with the disease duration and severity. Patients with cognitive impairment reported more frequently apathy, attention/memory deficit, and psychiatric symptoms. Apathy was the symptom associated with worse PDQ-39 score but also presence of fatigue, attention/memory, and psychiatric symptoms had a negative impact on QoL. These findings further support a key role for NMS in the clinical frame of PD and the need to address them specifically in clinical trials using dedicated scales., (2009 Movement Disorder Society.)

Background: Cognitive impairment is a common symptom of multiple sclerosis (MS), but the association between cognitive impairment and magnetic resonance imaging (MRI) disease measures in patients with relapsing-remitting (RR) MS is unclear., Objectives: To study the prevalence of cognitive impairment and its relation with MRI disease measures in mildly disabled patients with RRMS., Methods: Patients aged 18-50 years with RRMS (McDonald criteria) and an Expanded Disability Status Scale (EDSS) score or=3 cognitive tests) was present in approximately 20% of all patients and in the subgroup who underwent MRI. T2 hyperintense and T1 hypointense lesion volumes were significantly higher in patients with cognitive impairment (defined as impaired performance on at least three tests of the Rao's battery) than those without. EDSS score was also significantly higher in cognitively impaired than in cognitively preserved patients. Disease duration, depression, and years in formal education did not differ significantly between cognitively impaired and cognitively preserved patients. T2 lesion volume, performance intelligence quotient, and age were significant predictors of cognitive impairment in this population. Weak correlations were found between performance on individual cognitive tests and specific MRI measures, with T1 and T2 lesion volumes correlating with performance on most cognitive tests., Conclusions: Cognitive impairment occurs in approximately one-fifth of mildly disabled patients with MS and is associated with specific MRI disease measures. Assessment of cognitive function at diagnosis could facilitate the identification of patients who may benefit from therapeutic intervention with disease-modifying therapies to prevent further lesion development.

Parkinson's disease (PD), Dementia with Lewy Bodies (DLB), Progressive Supranuclear Palsy (PSP) and Corticobasal Degeneration Syndrome (CBDS) are the most common neurodegenerative extrapyramidal syndromes. Beyond motor symptoms, cognitive dysfunctions and behavioral disturbances are reported. Neuropsychological and neuropsychiatry features in the early stages, however, are under-investigated, and few comparison studies are available yet. The aim of the present study was to evaluate the cognitive and behavioral profile in the early stages of neurodegenerative extrapyramidal syndromes. Thirty-nine PD, 27 DLB, 16 CBDS, and 24 PSP were recruited. Groups were matched for global cognitive and motor impairment. The overall sample showed a common neuropsychological core characterized by visuospatial deficits. Although in the early stage of the disease, a high presence of behavioral disturbances was detected, depression and anxiety were the most common disorders, followed by apathy and sleep disturbances. The observation of overlapping clinical entities points the attention on the need of adjunctive diagnostic markers for early differential diagnosis.

Currently available epidemiological evidence suggested that an increase of saturated fatty acids (SFA) could have negative effects on cognitive functions, while increased polyunsaturated fatty acids (PUFA) and monounsaturated fatty acids (MUFA) may be protective against cognitive decline. In a Southern Italian elderly population from the Italian Longitudinal Study on Aging (ILSA), a clear reduction of risk of age-related cognitive decline (ARCD) has been found with elevated intake of PUFA and MUFA. Furthermore, in the ILSA, while dietary fatty acids intakes were not associated with incident mild cognitive impairment (MCI), high PUFA intake appeared to have borderline non-significant trend for a protective effect against the development of MCI. These epidemiological findings on predementia syndromes, i.e. MCI or ARCD, together with a recent randomised controlled trial on a possible effect on cognitive and depressive symptoms of omega-3 PUFA supplementation in patients with very mild AD, suggested a possible role of fatty acids intake in maintaining adequate cognitive functioning and possibly in preventing or delaying the onset of dementia.

Objective: To provide normative values for the Italian population for the Brief Assessment of Cognition in Schizophrenia (BACS), a recent brief neuropsychological instrument for the assessment of cognition in patients with schizophrenia., Participants: Data were collected from 204 healthy adult Italian subjects, stratified by gender, education and age., Measurements and Results: Tests included in the BACS are the following: list learning, digit sequencing, verbal fluency, token motor task, symbol-coding and Tower of London. Normative values were established using the Equivalent Scores method in order to enable comparison with other neuropsychological tasks commonly used in the assessment of the Italian population. Performance on the BACS was influenced by the commonest demographic variables such as age and education., Conclusions: The availability of normative data for the Italian population will increase the usefulness of this test for both clinical and experimental purposes.

Aim of the Study: To determine the Health Related Quality of Life (HRQL) in a population-based cohort of long-term survivors of childhood cancer in Piedmont, northwestern Italy., Patients and Methods: During 2003, a 15-item Health Utilities Index questionnaire was mailed to 1005 5-year survivors, identified from the population-based Childhood Cancer Registry of Piedmont, to derive scores for overall HRQL and for eight single attributes of health. Score differences were estimated as adjusted prevalence odds ratios., Results: A large majority of long-term survivors had moderately high scores for overall HRQL and for each of the single attributes. Males reported better overall HRQL and less morbidity with respect to dexterity, emotion and pain than females. Survivors diagnosed when they were 10-14 years of age had better overall HRQL and less morbidity with respect to emotion, cognition and pain than younger persons. Long-term survivors of central nervous system (CNS) tumours, retinoblastoma and bone tumours had greater impairment of overall HRQL, vision, ambulation, dexterity, cognition and pain than survivors of other forms of cancer., Conclusion: Many survivors of childhood cancer in Piedmont had fairly good overall HRQL. Greater probability of impaired HRQL was seen for females, survivors of CNS tumours, retinoblastoma and bone tumours, and persons diagnosed before 10 years of age.

Background: Data on the potential behavioral effects of music therapy in autism are scarce., Objective: The aim of this study was to investigate whether a musical training program based on interactive music therapy sessions could enhance the behavioral profile and the musical skills of young adults affected by severe autism., Methodology: Young adults (N = 8) with severe (Childhood Autism Rating Scale >30) autism took part in a total of 52 weekly active music therapy sessions lasting 60 minutes. Each session consisted of a wide range of different musical activities including singing, piano playing, and drumming. Clinical rating scales included the Clinical Global Impression (CGI) scale and the Brief Psychiatric Rating Scale (BPRS). Musical skills-including singing a short or long melody, playing the C scale on a keyboard, music absorption, rhythm reproduction, and execution of complex rhythmic patterns-were rated on a 5-point Likert-type scale ranging from "completely/entirely absent" to "completely/entirely present.", Results: At the end of the 52-week training period, significant improvements were found on both the CGI and BPRS scales. Similarly, the patients' musical skills significantly ameliorated as compared to baseline ratings., Conclusions: Our pilot data seem to suggest that active music therapy sessions could be of aid in improving autistic symptoms, as well as personal musical skills in young adults with severe autism.

Objective: To investigate whether coffee consumption is associated with 10-year cognitive decline in elderly men, as results of previous studies obtained hitherto have been controversial and prospective information on this association has been lacking., Design, Subjects and Setting: Six hundred and seventy six healthy men born between 1900 and 1920 from Finland, Italy and the Netherlands participated in a 10-year prospective cohort study. Cognitive functioning was assessed using the Mini-Mental State Examination (0-30 points, with a higher score indicating better cognitive performance). Coffee consumption was estimated in cups per day. A mixed longitudinal model was used to investigate the association between baseline coffee consumption and 10-year cognitive decline. Multiple adjustments were made., Results: Men who consumed coffee had a 10-year cognitive decline of 1.2 points (4%). Non-consumers had an additional decline of 1.4 points (P<0.001). An inverse and J-shaped association was observed between the number of cups of coffee consumed and cognitive decline, with the least cognitive decline for three cups of coffee per day (0.6 points). This decline was 4.3 times smaller than the decline of non-consumers (P<0.001)., Conclusions: Findings suggest that consuming coffee reduces cognitive decline in elderly men. An inverse and J-shaped association may exist between the number of cups of coffee consumed and cognitive decline, with the least cognitive decline for men consuming three cups of coffee per day.

In this study, a series of tests exploring long-term verbal memory (the Short Story Test), attention (a modified version of Attentional Matrices and the Trail Making Test) and frontal functions (a modified version of the Frontal Assessment Battery) have been standardised on an Italian population of 283 children aged 5-14. Raw scores for each test have been adjusted for a series of variables (child's age, years of parents' education, handedness, gender) and transformed in equivalent scores enabling direct comparison across measures. This study was promoted by LICE (the Italian League Against Epilepsy) in order to provide Italian instruments standardised on the developmental age population and to study some of the most frequently impaired cognitive functions in epilepsy.

The aim of the present paper was to describe some formative projects on work integration of patients with epilepsy and mild cognitive deficits, so that similar initiatives may be promoted. The different phases of the formative projects were described: the inclusion criteria, the selection of participants, the assessment instruments, the courses themselves and the final results in terms of working experience. Thirteen participants were selected for each course. Patients were treated in different Lombardy Epilepsy departments, which addressed them to the Epilepsy Center, S. Paolo Hospital, Milan, where the selection took place. Seventy percent of the participants in the formative courses found a job in 1 year, even though most of the positions were temporary. The work integration seemed to be mainly related to the level of intelligence and to verbal memory. We stress the importance of organising similar courses in the future, in the context of wider networks. This will help more patients in acquiring both economic and psychological autonomy from their families.

Cognitive dysfunction is considered one of the clinical markers of multiple sclerosis (MS). However, in the literature there are inconsistent reports on the prevalence of cognitive dysfunction, and separate data for the relapsing-remitting (RR) type of the disease are not always presented. In this study, we submitted 461 RRMS patients to a battery of neuropsychological tests to investigate their impairment in various cognitive domains. As a consequence of the exclusion criteria, the sample is not fully representative of the entire population of RRMS patients. In this selected sample, when only the eight scores of a core battery (Mental Deterioration Battery) were considered (with respective cutoffs), it emerged that 31% of the patients were affected by some degree of cognitive deficit. In particular, 15% had mild, 11.2% moderate and 4.8% had severe impairment. Information processing speed was the most frequently impaired area, followed by memory. When two other tests (SDMT and MCST) were added and cognitive domains were considered, it emerged that 39.3% of the patients were impaired in two or more domains. When four subgroups were obtained by means of cluster analysis and then compared, it emerged that information processing speed and memory deficits differentiated the still cognitively unimpaired from the mildly impaired MS patients. Significant associations were found between cognitive and clinical characteristics. However, due to the large sample size, clinically irrelevant relationships may also have emerged. Even with the limitations imposed by the sample selection and the possible underestimation of the prevalence and severity of cognitive dysfunction, these results seem to provide further evidence that information processing speed deficit may be an early and important marker of cognitive impairment in MS patients.

Increased levels of alpha-1-antichymotrypsin (ACT), a protease inhibitor and an acute phase protein, have been found in the brain and peripheral blood of patients with Alzheimer's disease (AD). Patients from northern Italy with a clinical diagnosis of probable AD, and patients with early onset AD (EOAD) from UK with AD neuropathological diagnosis were genotyped for a new polymorphism in the promoter region of the ACT gene which has been shown to affect ACT expression. A subset of patients with clinical AD from northern Italy was also followed up for 2 years and monitored for cognitive decline. The ACT TT promoter genotype was associated with an increased risk of EOAD independently from the presence of the apolipoprotein E (APOE) epsilon 4 allele. After manifestation of the disease the ACT TT genotype was also associated with faster cognitive decline in patients with the APOE allele epsilon 4. The ACT gene appears to influence the early clinical development of the disease, and the interaction of the ACT and APOE genes affects clinical progression of AD.

Objective: This study was designed to establish whether, in growth-retarded fetuses, absent or reverse end-diastolic (ARED) flow velocity in the umbilical artery can be predictive of an increased incidence of long-term neurological and intellectual impairment., Study Design: A total of 14 children with intra-uterine growth retardation (IUGR) and ARED flow in the umbilical artery and 11 children without this velocimetric pattern were examined by pediatric neuropsychiatrists at a median age of 8.7 years to evaluate and compare their neurological and intellectual development., Results: The incidence of major neurological sequelae was higher in the children with ARED velocity in the umbilical artery (21%) than in those without this velocimetric pattern (9%), as was the incidence of mild neurological sequelae (35% versus 27%). No differences in mean intelligence quotient (IQ) as evaluated by mean of Intelligence Scale for Children-Revised (WISC-R) scale were found between the two groups of children at school age., Conclusions: Our data demonstrate that Doppler velocimetry in the umbilical artery is a reliable predictor for neurological sequelae when ARED flow is present but cannot be considered a good predictor of intellectual performance at school.

The increase of the GUCH Community requires a new definition of cardiac surgical success, which has to be considered either in terms of survival or quality of life and quality of health. Clinical and psychological problems are present in this population secondary to native cardiac disease, surgical correction, and social environment. Cognitive impairment after cardiac surgery is not rare, but an exhaustive evaluation of the psychological and neurodevelopmental outcome of patients operated on for total correction of congenital heart disease is complex and its clinical significance can be obtained by the integration of cardiological data, psychological status, and sociodemographic variables.