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Context Caring for a child who will die from a life-limiting illness is one of the most difficult experiences a parent may face. Pediatric palliative care (PPC) has grown as a specialty service to address the unique needs of children and families with serious illness. However, gaps remain between the needs of families in PPC and the support received. Objectives The objective of this study was to explore the concerns of parents who have a child in home-based PPC. Methods Semistructured interviews were conducted with 25 mothers and 10 fathers from 25 families shortly after their child's referral to home-based PPC. Children (57% male, Mage = 10.5 years, SD = 3.95, range = 4–18 years) had a range of diagnoses. Data were analyzed using inductive content analysis. Results Parents' concerns clustered into four main themes: 1) ensuring that their child's remaining days were spent living well physically, emotionally, and socially; 2) uncertainty regarding their child's diagnosis, prognosis, and treatments; 3) their child's death (e.g., the process of dying and when it will occur); and 4) the family, including the impact of the child's illness and death on siblings and wanting to cherish as much time together with family as possible. Conclusion Parents of children receiving home-based PPC expressed concerns across a range of domains, both about their seriously ill child and the broader family. These results highlight salient worries among parents of children in PPC and point to critical areas for intervention for seriously ill children and the broader family.

Survivors of childhood cancer may be at risk of experiencing pain, and a systematic review would advance our understanding of pain in this population. The objective of this study was to describe: 1) the prevalence of pain in survivors of childhood cancer, 2) methods of pain measurement, 3) associations between pain and biopsychosocial factors, and 4) recommendations for future research. Data sources for the study were articles published from January 1990 to August 2019 identified in the PubMed, PsycINFO, EMBASE, and Web of Science data bases. Eligible studies included: 1) original research, 2) quantitative assessments of pain, 3) articles published in English, 4) cancers diagnosed between birth and age 21 years, 5) survivors at 5 years from diagnosis and/or at 2 years after therapy completion, and 6) a sample size >20. Seventy-three articles were included in the final review. Risk of bias was considered using the Cochrane risk of bias tool. The quality of evidence was evaluated according to Grading of Recommendations Assessment Development and Evaluation (GRADE) criteria. Common measures of pain were items created by the authors for the purpose of the study (45.2%) or health-related quality-of-life/health status questionnaires (42.5%). Pain was present in from 4.3% to 75% of survivors across studies. Three studies investigated chronic pain according the definition in the International Classification of Diseases. The findings indicated that survivors of childhood cancer are at higher risk of experiencing pain compared with controls. Fatigue was consistently associated with pain, females reported more pain than males, and other factors related to pain will require stronger evidence. Theoretically grounded, multidimensional measurements of pain are absent from the literature.

Cancer and its treatment can have lasting consequences on somatosensation, including pain, which is often underrecognized and undertreated. Research characterizing the impact of cancer on pain and sensory processing in survivors of childhood cancer is scarce. This study aimed to quantify generalized differences in pain and sensory processing in survivors of childhood cancer compared with reference data using a standardized thermal and mechanical quantitative sensory testing (QST) protocol. The association between demographic, clinical (eg, leukemia vs other cancers and treatment exposures), and psychosocial (eg, anxiety and pain catastrophizing) variables and sensitivity to pain and sensory stimuli were also evaluated. Participants were 56 survivors of various types of childhood cancer (52% male, Mage = 13.5 years, SD = 3.2, range = 8-17 years). On average, children were 7 years (SD = 4.1, range = 1.2-16.5) post treatment. Almost all participants (86%) had at least 1 abnormal QST parameter compared with age- and sex-matched reference data; however, few participants self-reported the presence of sensory abnormalities. Generally, participants exhibited reduced sensitivity across the QST parameters examined (Ps < 0.05, ds = 0.40-3.45). A significant minority (45%) also exhibited pain sensitization (P

IntroductionChronic pain, defined as persistent or recurring pain or pain lasting longer than 3 months, is a common childhood problem and can profoundly impact children’s physical, psychological and social functioning. The last comprehensive systematic review estimating the prevalence of chronic pain in children and adolescents was published in 2011. Since then, the literature on paediatric chronic pain has grown substantially. This manuscript outlines a protocol for an updated systematic review to provide updated estimates of the prevalence of various forms of chronic pain in children and adolescence. The review will also examine the relationship between sociodemographic and psychosocial factors related to chronic pain prevalence.Methods and analysisThis review will follow Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. We will search EMBASE, PubMed, CINAHL and PsycINFO for observational studies published in English between 2009 and 2020 reporting population-based estimates of chronic non-disease-related pain prevalence in children or adolescents (age ≤19 years). Two independent reviewers will screen the titles and abstracts retrieved from the search based on predefined eligibility criteria. The full texts of relevant studies will then be assessed by two reviewers. Studies meeting inclusion criteria will be categorised according to the type of pain investigated: headache only, abdominal pain only, back pain only, musculoskeletal pain, combined pain, general pain and other pain. Data will be extracted using customised forms and studies will be assessed for risk of bias using a 10-item tool developed by Hoy et al (2012). A narrative synthesis will summarise the prevalence estimates of paediatric chronic pain and associated sociodemographic and psychosocial correlates. Meta-analyses and meta-regressions will be performed if the data permit.Ethics and disseminationEthical approval is not required. Findings will be disseminated through publication in an academic journal, presentations at conferences and in various media.PROSPERO registration numberCRD42020198690.

Acute pain is a common childhood issue, from routine procedures (e.g., vaccinations, venipuncture) to more specialized medical procedures and treatments (e.g., lumbar punctures, surgery). This chapter provides an overview of best practices in acute pain management for pediatric psychology consultations, including evidence-based approaches to effective assessment and management. The chapter outlines developmental considerations to guide decision-making for pain assessment and management. Practical issues pediatric psychologists need to address when working with pediatric patients are discussed, including family, medical, and treatment-related factors. Key considerations for acute pain assessment and management in special populations are discussed, including children with intellectual disabilities, developmental disorders, and needle phobias. Recommendations for post-procedure pain management and support are discussed. The pediatric psychologist plays a critical role in acute pain management and should be armed with the evidence and expertise summarized in this chapter in order to ensure children with pain and their families receive the pain management they deserve.

Background: Children with cancer often undergo long treatment trajectories involving repeated needle procedures that potentially cause pain and distress. As part of a comprehensive effort to develop clinical practice guidelines (CPGs) to address pain prevention and management in children with cancer, we aimed to provide recommendations on the pharmacological and psychological management of procedure-related pain and distress. Methods: Of the international inter-disciplinary CPG development panel (44 individuals), two working groups including 13 healthcare professionals focused on procedural pain and distress. Grading of Recommendations Assessment, Development and Evaluation methodology was used, including the use of systematic literature reviews to inform recommendations and the use of evidence to decision frameworks. At an in-person meeting in February 2018, the guideline panel discussed these frameworks and formulated recommendations which were then discussed with a patient-parent panel consisting of 4 survivors and 5 parents. Results: The systematic reviews led to the inclusion of 48 randomised controlled trials (total number of participants = 2271). Quality of evidence supporting the recommendations ranged from very low to moderate. Strong recommendations were made for the use of topical anesthetics in all needle procedures, for offering deep sedation (DS)/general anesthesia (GA) to all children undergoing lumbar puncture, for the use of DS/GA in major procedures in children of all ages, for the use of hypnosis in all needle procedures and for the use of active distraction in all needle procedures. Conclusion: In this CPG, an evidence-based approach to manage procedure-related pain and distress in children with cancer is presented. As children with cancer often undergo repeated needle procedures during treatment, prevention and alleviation of procedure-related pain and distress is of the utmost importance to increase quality of life in these children and their families. (C) 2020 The Authors. Published by Elsevier Ltd.

Pain is a common and distressing symptom of pediatric cancer, as reported by both children and their parents. Increasingly, children with cancer are cared for as outpatients, yet little is known about how parents manage their cancer-related pain. The aim of the current study was to examine pain prevalence and characteristics, and the pharmacological, physical, and psychological pain management strategies used by parents to manage their child's cancer pain.In total, 230 parents and caregivers (89% mothers) of children (mean age=8.93 y, SD=4.50) with cancer currently in treatment or who are survivors completed an online survey about their child's pain in the preceding month.Results indicated that children with cancer who were on active treatment and who were posttreatment experienced clinically significant levels of pain. Parents reported using more physical and psychological strategies than pharmacological strategies to manage their child's pain. The most frequently used physical/psychological strategy was distraction and acetaminophen was the most frequently administered pain medication. Parents' confidence in managing their child's pain was inversely associated with both how much pain they perceived their child had, and also whether they had given any pain medication.The results of this study suggest that despite parents' use of pain management strategies, management of cancer-related pain continues to be a problem for children during treatment and into survivorship.

Purpose: Adolescents and young adults (AYAs) with terminal cancer are a marginalized population with unique medical and psychosocial needs. AYAs commonly report challenges with their health care experiences, however, little is known about the experiences of the health care providers (HCPs) who deliver this specialized care. The purpose of the current study was to understand HCPs' experiences caring for AYAs with terminal cancer. Methods: Nine HCPs (four nurses and five physicians) took part in in-depth semistructured interviews. Participants were eligible if they were a nurse or physician in Atlantic Canada; cared for at least one AYA patient with terminal cancer in the past 3 years; and were able to speak and understand English. Data were analyzed using interpretive phenomenological analysis. Results: Analyses revealed four superordinate themes present in the data: (1) many unknowns and uncertainties associated with providing care for AYAs compounded by minimal or no training specifically concerning this population; (2) an intense emotional experience compared with caring for patients with terminal cancer of other ages; (3) personal identification with patients and their families; and (4) attempts to make sense of the circumstance thwarted by feelings of injustice and unfairness. Conclusions: HCPs experienced unique emotional and logistical challenges when caring for AYAs with terminal cancer, which can influence the care they provide. HCPs' experiences highlight the need for training to support clinicians in caring for AYAs with terminal cancer to optimize their own well-being and delivery of health care services to this population.

Although pain is one of the most prevalent and bothersome symptoms children with cancer experience, evidence-based guidance regarding assessment and management is lacking. With 44 international, multidisciplinary healthcare professionals and nine patient representatives, we aimed to develop a clinical practice guideline (following GRADE methodology), addressing assessment and pharmacological, psychological, and physical management of tumor-, treatment-, and procedure-related pain in children with cancer. In this paper, we present our thorough methodology for this development, including the challenges we faced and how we approached these. This lays the foundation for our clinical practice guideline, for which there is a high clinical demand.

Introduction/Aim: Juvenile Idiopathic Arthritis (JIA) affects approximately 24,000 children throughout Canada, many of whom report pain as the predominant symptom. Caregivers play an important role in managing JIA-related pain; not only are they the primary support, many are also tasked with administering potentially painful treatments at home. This study explored caregivers’ confidence and information needs in managing their child’s pain. Methods: Recruitment took place worldwide by engaging partner organizations and sharing across online and social media platforms. 216 caregivers of children with JIA aged 0–17 participated in an online survey, completing questions about their child’s arthritis and pain, and their own information needs. Participants were predominantly mothers (96%) residing in North America (77%). Results: Caregivers reported lacking confidence in alleviating their child’s arthritis-related pain (M = 4.11, SD = 2.60, range = 0–10). Although all participants reported wanting some information, regressions demonstrate that lower caregiver confidence related to a need for more information [t = −2.91, β = −0.34 (−0.57, −0.12)]. The most frequently identified needs included managing emotions related to painful experiences (60%) and differentiating arthritis pain from normal pain (56%). Caregivers strongly valued developing partnerships between researchers and parent organizations wherein evidence-based information related to arthritis pain could be shared (M = 4.25, SD = 1.05, range = 1–5). Discussion/Conclusions: As caregivers of children with JIA are tasked with helping to manage their child’s pain, it is important that they have the necessary tools to do so. This research demonstrates that although parents’ lack confidence in pain management, they are keen to learn more. Implications will be discussed for knowledge translation within this community.

Background: Pain Squad is an evidence-based, freely available iOS app designed to assess pain in children with cancer. Once research-based technologies such as Pain Squad are validated, it is important to evaluate their performance in natural settings to optimize their real-world clinical use. Objective: The objective of this study was to evaluate the implementation effectiveness of Pain Squad in a natural setting. Methods: Parents of 149 children with cancer (aged 8-18 years) were contacted to invite their child to participate. Participating children downloaded Pain Squad on their own iOS devices from the Apple App Store and reported their pain using the app twice daily for 1 week. Participants then emailed their pain reports from the app to the research team and completed an online survey on their experiences. Key implementation outcomes included acceptability, appropriateness, cost, feasibility, fidelity, penetration, and sustainability. Results: Of the 149 parents contacted, 16 of their children agreed to participate. More than a third (6/16, 37.5%) of participating children returned their pain reports to the research team. Adherence to the pain assessments was 62.1% (mean 8.7/14 assessments). The 6 children who returned reports rated the app as highly feasible to download and use and rated their overall experience as acceptable. They also reported that they would be willing to sustain their Pain Squad use over several weeks and that they would recommend it to other children with cancer, which suggests that it may have potential for penetration. Conclusions: While Pain Squad was well received by the small number of children who completed the study, user uptake, engagement, and adherence were significant barriers to the implementation of Pain Squad in a natural setting. Implementation studies such as this highlight important challenges and opportunities for promoting the use and uptake of evidence-based technologies by the intended end-users.

Supplemental Digital Content is Available in the Text. The relevance of KT tools, and parents' confidence in using them, was related to uptake of vaccination pain management strategies for children., Introduction: Although several evidence-based strategies for managing children's vaccination pain exist, many parents report being unaware of them. Knowledge translation (KT) tools present evidence-based information in plain language. Objectives: This two-phase study assessed parents/caregivers' uptake of evidence-based pain management strategies via a KT tool and considered factors related to parents' planned, actual, and future use of these strategies. Methods: In phase 1, parents were exposed to an online KT tool on physical, psychological, and pharmacological vaccination pain management strategies, and their impressions were assessed by questionnaires including the Information Assessment Method for Parents. In phase 2, after vaccination, parents completed a follow-up survey on their uptake and experiences using the information. Results: A total of 312 participants reported their plans for KT tool use. Parents who found the KT tool relevant were more likely to plan to use it at their child's upcoming vaccination. A total of 128 parents (93% mothers) completed both surveys. Nearly all parents who planned to use the information did so during their child's subsequent vaccination (90%). When the KT tool was relevant to their needs, parents were more likely to use the information during their child's vaccination. Parents who felt confident using the tool were significantly more likely to report plans for future tool use. Discussion: This study demonstrates the effectiveness of a KT tool that was relevant to parents' needs and built confidence to increase parent-reported uptake of evidence-based strategies. Proper pain management could positively impact parents' uptake of vaccinations for children.

Dementia with Lewy bodies (DLB) causes elevated outlays for the National Health Systems due to high institutionalization rate and patients' reduced quality of life and high mortality. Furthermore, DLB is often misdiagnosed as Alzheimer's disease. These data motivate harmonized multicenter longitudinal cohort studies to improve clinical management and therapy monitoring. The Italian DLB study group of the Italian Neurological Society for dementia (SINdem) developed and emailed a semi-structured questionnaire to 572 national dementia centers (from primary to tertiary) to prepare an Italian large longitudinal cohort. The questionnaire surveyed: (1) prevalence and incidence of DLB; (2) clinical assessment; (3) relevance and availability of diagnostic tools; (4) pharmacological management of cognitive, motor, and behavioural disturbances; (5) causes of hospitalization, with specific focus on delirium and its treatment. Overall, 135 centers (23.6 %) contributed to the survey. Overall, 5624 patients with DLB are currently followed by the 135 centers in a year (2042 of them are new patients). The percentage of DLB patients was lower (27 ± 8 %) than that of Alzheimer's disease and frontotemporal dementia (56 ± 27 %) patients. The majority of the centers (91 %) considered the clinical and neuropsychological assessments as the most relevant procedure for a DLB diagnosis. Nonetheless, most of the centers has availability of magnetic resonance imaging (MRI; 95 %), electroencephalography (EEG; 93 %), and FP-CIT single photon emission-computerized tomography (SPECT; 75 %) scan for clinical applications. It will be, therefore, possible to recruit a large harmonized Italian cohort of DLB patients for future cross-sectional and longitudinal multicenter studies.

Background A strict, lifelong, gluten-free diet (GFD) remains the sole treatment for celiac disease (CD). The assessment of adherence to the GFD in pediatric studies is often based on self-report and visual analogue scales which lack proven validity. We sought to compare parental-report of GFD adherence to expert registered dietitian (RD) assessments, the best available standard. Methods Parents of children with biopsy-proven CD scored their adherence to the GFD on a five-point Likert scale similar to that used in previous pediatric CD studies. Each family was then evaluated by an RD expert in CD management who conducted a comprehensive and standardized assessment and scored the family’s adherence. The agreement between parents and the RD was assessed using paired t test and intraclass correlation coefficient (ICC) based on their scores. Results One hundred twenty-two children and their families participated in the study, with a median of 32 months on a GFD. Excellent adherence (score 5 out of 5) was attributed to 60.5% of the sample by the RD. The parents scored adherence higher than the RD by an average difference of 0.41 scale points (95% CI, 0.28–0.54; P < 0.001). The agreement between parents and the registered dietitian was poor (ICC = 0.21). Conclusion Reliance on self-report through Likert scales for GFD adherence overestimates adherence and misses opportunities for patient and family education. Approximately 40% of children with CD have ongoing gluten exposure, highlighting the need for regular assessment by an RD expert in the GFD to identify education and counselling needs for children with CD.

147 Background: Symptom monitoring plays an important role in both the physical and psychological challenges of surviving cancer. Anecdotally, cancer survival is characterized by uncertainty, symptom-related fear, and the interpretation of normal bodily sensations as symptomatic of cancer recurrence. This fear may lead to over-vigilance of benign bodily sensations, increasing anxiety and decreasing quality of life. Yet, there are few studies investigating how cancer survivors interpret and make sense of post-cancer symptoms. These studies are needed to guide considerations for clinical practice and the development of supportive interventions. Methods: We conducted in-depth semi-structured interviews with 18 AYA cancer survivors about how they interpret, manage, and respond to physical symptoms during survivorship. Participants were 15-25 years old. The sample was diverse in terms of disease history, ethnicity (8 Hispanic), and gender (9 females, 1 nonbinary). We conducted thematic analysis using a deductive coding scheme that was developed using our Cancer Threat Interpretation (CTI) theoretical model of cognitive, affective, and behavioral processes in post-cancer symptom perception. Results: AYA cancer survivors reported experiencing anxiety in the face of common physical sensations. These sensations were often interpreted as potential signs of recurrence or as late effects of treatment. Survivors most commonly reported worries about pain and fatigue, but also other sensations such as breathlessness. We coded participant transcripts into the following themes: biased attending towards symptoms, biased interpretations of symptoms as threatening, fear and worry about symptoms, and behavioral response to symptoms. In addition, we generated a new theme that was not previously captured in the CTI model: trust in the body. Conclusions: Knowing how to appraise and respond to symptoms such as pain is a challenge for AYA cancer survivors. This rich qualitative data provides support for the CTI model and suggests future directions. The results of this study can inform how clinicians talk to their patients about symptom monitoring after treatment ends.

Different aspects of episodic long-term, short-term and implicit long-term memory were investigated in subjects who strictly fulfilled the criteria for the amnestic form of Mild Cognitive Impairment (a-MCI). Results showed normal short-term memory abilities in these subjects, while each of the episodic long-term memory indices explored showed poorer results in a-MCI subjects with respect to normal controls. Although some episodic memory functions were relatively well preserved, others appeared to have deteriorated to a level comparable to that of mild AD patients. The finding of an extensive impairment of all memory functions depending on hippocampal structures in a population with a high risk of developing dementia is strongly supportive of the hypothesis that a pure amnesic syndrome characterizes the preclinical phase of AD. Copyright © Taylor & Francis Ltd.

BACKGROUND/OBJECTIVES: Subjects affected by mild cognitive impairment (MCI) may improve during the observation period. This is the first study investigating qualitative features of memory deficits in subjects affected by reversible MCI [reversible cognitive impairment (RCI)]. METHODS: Baseline cognitive and memory performances of 18 subjects affected by amnestic MCI who had normalized cognitive performances at follow-ups were compared with those of 76 amnestic MCI subjects who still showed impaired cognitive performances at the 24-month follow-up (MCI) and with those of a group of 87 matched control subjects (normal controls). RESULTS: Compared with normal controls the memory deficit in the MCI group affected all aspects of explicit long-term memory functioning; in the RCI group, instead, the memory deficit only affected the free recall of verbal material, particularly when the encoding could be improved by the use of semantic strategies. CONCLUSIONS: These results are consistent with the view that the memory deficit in the MCI group is due to a very early degenerative pathology; in the RCI group, instead, a transitory reduction of processing resources, resulting a poor encoding of incoming material, is likely at the origin of the reversible memory disorder.

Background: Different rates and cognitive predictors of conversion to dementia have been reported in subjects with different kinds of mild cognitive impairment (MCI). Methods: A prospective, 24-month follow-up study, involving 269 subjects who strictly fulfilled criteria for the amnestic MCI. Results: Conversion rate to dementia was 21.4% per year. Seventy-nine out of the 83 individuals who developed dementia were affected by probable Alzheimer’s disease (AD). Among others, at the 24-month follow-up 24.1% were still affected by amnestic MCI, 13.3% had changed their neuropsychological profile of impairment and 17.2% were cognitively normalised. Compared to subjects who did not convert to AD, those who did convert showed poorer immediate and delayed recall and recognition of verbal and visual material at baseline as well as reduced executive abilities. A combination of age, Clinical Dementia Rating boxes and scores on delayed recall and recognition of verbal and visual material accurately identified 86% of the subjects who developed AD. Conclusions: Elderly subjects affected by an isolated memory disorder have a high probability of developing AD. The ability of verbal and visual measures to predict incipient dementia of memory impairment may be increased by the simultaneous assessment of individual features, such as age or rate of functional impairment.

A door-to-door prevalence survey of epilepsy was conducted in 3 Sicilian municipalities, as of November 1, 1987. In phase 1, the screening by questionnaire of 24,496 eligible subjects (participation = 92%) identified 544 suspected to have epilepsy. In phase 2, neurological evaluation of the 544 subjects yielded 111 with epilepsy. Of the 111 subjects, 103 (93%) had been previously diagnosed, 68 (61%) were taking antiepileptic medication, and 81 (73%) had active epilepsy. Referring to the 81 subjects with active epilepsy, the seizure type was generalized in 60 (74%), partial in 19 (23%) and undetermined in 2 (3%). The prevalence of active epilepsy (per 1,000 population) was 3.3 overall, 3.5 for men and 3.2 for women. The age-specific patterns for active epilepsy differed by sex, with higher figures for men at younger ages (5-19 years) and older ages (50-99 years). The age-specific prevalence figures for active epilepsy were lower than those from previous Italian surveys. Copyright © 2001 S. Karger AG, Basel.

Aim: To determine the applicability and psychometric properties of the Italian version of the Disability Assessment of Dementia scale (DAD-I) in a community-residing population with early-stage Alzheimer’s disease (AD). Methods: The DAD-I was administered to the primary caregivers of 159 patients (mean age ± SD 77.1 ± 5.2) with mild AD (mean Mini Mental State Examination, MMSE, ± SD 23.1 ± 2.2). Results: The DAD-I showed excellent internal consistency reliability (Cronbach’s α = 0.92) and good construct validity. The DAD-I score was not significantly associated with gender, education and presumed duration of the illness, and had a low negative correlation with age. The DAD-I score correlated moderately with the traditional Instrumental Activities of Daily Living and Activities of Daily Living scales, respectively, with r = 0.53 and r = 0.54 (p < 0.0001). Relatively low, but statistically significant correlations (r ranging between 0.21 and 0.31) with the MMSE were also found. Conclusion: The DAD-I was found to be a reliable and valid instrument to assess functional disability in community-dwelling Italian subjects with early-stage AD.

Background: Executive dysfunction in Alzheimer’s disease (AD) has been recently recognized as an early and prominent clinical sign. The Tower of London (ToL), a task specifically devised to test executive functions of visuospatial planning and problem solving, has frequently been used in neuropsychological experiments, but rarely in the clinical ground. Methods: One hundred and sixty-one AD patients and 212 nondemented healthy controls were administered a simplified ToL version. Results: AD patients were significantly impaired (p < 0.0001) in all ToL scores and in the total execution time. The ‘accuracy’ score of ToL at a cut off of ≤29/36 yielded a sensitivity of 71.2% and a specificity of 76.4% (AUC 0.79) for the diagnosis of AD versus controls. Conclusions: Visuospatial planning and problem solving are significantly impaired in early dementia of the Alzheimer’s type. A successful sensitivity/specificity ratio, the independence of education and the simplicity of this version of ToL make it a useful executive functioning screening test for early AD.

We assessed the validity of an Italian language version of the Epworth sleepiness scale (ESS). The translated ESS was compared to the multiple sleep latency test (MSLT), considered the gold standard for the diagnosis of excessive daytime sleepiness (EDS). Within the context of a multicentric national study on narcolepsy (Gruppo Italiano Narcolessia Studio Epidemiologico Nazionale, GINSEN) involving 17 Italian sleep centres, we compared the two diagnostic tests on 91 prospectively recruited subjects with suspected EDS (34 with narcolepsy, 16 with obstructive sleep apnea syndrome, 19 with idiopathic hypersomnia, and 22 with other sleep, neurologic or psychiatric disorders). ESS scores were inversely correlated with mean sleep latency values, as measured with MSLT (rho = −0.31, p

Diurnal variation in dipropylacetic acid (DPA) plasma levels was investigated in 47 and 42 epileptic patients, chronically treated with sodium dipropylacetate and dipropylacetamide (DPM), respectively, taken alone or in addition to other antiepilepic drugs. Fluctuation in DPA plasma levels was significantly less in patients receiving dipropylacetamide. The slow absorption and the more prolonged plasma half-life of dipropylacetamide accounted for these findings. Although the importance of diurnal fluctuations in DPA levels has not yet been established, possible clinical implications are discussed.

The objective of this study was to verify the adherence of Italian family physicians and neurologists to the Guidelines on Diagnosis of Dementia of the Italian Society of Neurology. A multicentre survey was carried out, in 72 neurological centres. The centres included at least 15 consecutive subjects suspected of having a dementia. The adherence of family physicians to the guidelines was poor. Neurologists performed a complete neuropsychological evaluation in a minority of the cases. Patients who had a decrease of Mini Mental Status Examination scores after six months higher than or equal to 4 were more represented among those patients for whom one or more recommendations were not respected. In Italy the adherence to the Guidelines on Diagnosis of Dementia and AlzheimerValidation studys Disease of the Italian Society of Neurology is very poor for family physicians (GPs) and satisfactory, albeit improvable, on the part of neurologists. Respect for the guidelines might improve the outcome of patients with dementia.

Background: Motor and inhibitory control rely on frontal cortex activity, which is known to reach full maturation only in late adolescence. The development of inhibitory control has been studied using event-related potentials (ERP), focusing on reactive processing (i.e. the N2 and the P3 components). Scarce information exists concerning pre-stimulus activity as that represented by the Bereinshafstpotential (BP) and by the prefrontal negativity (pN). Further, no literature exists concerning the post-stimulus components originating within the anterior insula (pN1, pP1, pP2). This study aims at associating children performance with these motor-cognitive processing in frontal brain areas. Methods: High-resolution EEG recordings were employed to measure ERPs from 18 children (12 years old) and 18 adults (28 years old) during a visuo-motor discriminative response task. Response time (RT), commission (CE) and omission errors, and RT variability were compared between groups. At brain level, two pre-stimulus (BP and pN) and seven post-stimulus (P1; pN1; N1; pP1; N2; pP2; P3) ERP components were compared between groups. Results: Children showed slower and more variable RTs and poorer inhibition (higher CEs) than adults. At electrophysiological level, children presented smaller BP and pN. After stimulus onset, children showed lower amplitude of N1, pP1, P3, and pP2 components. The P1, pP1, N2 and P3 were delayed compared to adults. Conclusions: Our results demonstrate that children are characterized by less intense task-related proactive activities in frontal cortex, which may account for subsequent poor and delayed reactive processing and, thus, for inaccurate and slow performance.

Objective: As the literature on conventional cri- teria for discriminating early-onset (EO) from late-onset (LO) Alzheimer’s disease (AD) is sparse and controversial, the aim of this study was to establish a precise age at onset (AAO) criterion, by using a specific statistical proce- dure, and to describe the clinical characteristics of the two sub-groups. Methods: Admixture analysis was performed to establish the AAO cut-off in a multi-center study including 2000 AD patients consecutively recruited in eight Italian Memory Clinics. None of the patients were tak- ing acetylcholinesterase inhibitors, antipsychotic or anti-depressant drugs. At the first diagnostic visit, they were administered the Mini Mental State Examination, the Basic and Instrumental Activi- ties of Daily Living and the Neuropsychiatric Inventory to assess clinical phenomenology. Results: Using a specific statistical procedure, we established that AAO that discriminated EO- from LO-AD was 66. Compared with the LO-AD group, the EO-AD group showed longer duration of illness and a higher educational level as well as less severe functional impairment and delu- sions. Conclusions: Differences in sociodemo- graphic and clinical characteristics, such as duration of illness, education and delusion se-verity, suggested the involvement of different pathogenic processes. Additional studies are needed to further investigate the mechanisms underlying the disorder in the two sub-groups of AD patients.

Clinical assessment and management of sleep disturbances in patients with mild cognitive impairment and dementia has important clinical and social implications. Poor sleep results in an increased risk of morbidities and mortality in demented patients and is a source of stress for caregivers. Sleep disturbances show high prevalence in mild cognitive impairment and dementia patients and they are often associated one to another in the same patient. A careful clinical evaluation of sleep disorders should be performed routinely in the clinical setting of individuals with cognitive decline. The Sleep Study Group of the Italian Dementia Research Association (SINDem) reviewed evidence from original research articles, meta-analyses and systematic reviews published up to December 2013. The evidence was classified in quality levels (I, II, III) and strength of recommendations (A, B, C, D, E). Where there was a lack of evidence, but clear consensus, good practice points were provided. These recommendations may not be appropriate for all circumstances and should therefore be adopted only after a patient's individual characteristics have been carefully evaluated.

Background/Aims: Sleep disturbances are common in the elderly and in persons with cognitive decline. The aim of this study was to describe frequency and characteristics of insomnia, excessive daytime sleepiness, sleep-disordered breathing, REM behavior disorder and restless legs syndrome in a large cohort of persons with mild cognitive impairment or dementia. Methods: 431 consecutive patients were enrolled in 10 Italian neurological centers: 204 had Alzheimer’s disease, 138 mild cognitive impairment, 43 vascular dementia, 25 frontotemporal dementia and 21 Lewy body dementia or Parkinson’s disease dementia. Sleep disorders were investigated with a battery of standardized questions and questionnaires. Results: Over 60% of persons had one or more sleep disturbances almost invariably associated one to another without any evident and specific pattern of co-occurrence. Persons with Alzheimer’s disease and those with mild cognitive impairment had the same frequency of any sleep disorder. Sleep-disordered breathing was more frequent in vascular dementia. REM behavior disorder was more represented in Lewy body or Parkinson’s disease dementia. Conclusion: A careful clinical evaluation of sleep disorders should be performed routinely in the clinical setting of persons with cognitive decline. Instrumental supports should be used only in selected patients.

Objective To evaluate whether the survival of patients with Parkinson disease (PD) is shorter than that of the general population. Design Survival was investigated in a cohort of patients with PD previously identified during a population-based prevalence study (prevalence day, November 1, 1987; reference follow-up date, October 31, 1995). The survival of patients with PD was compared with that of a control sample randomly selected from the same population (2 controls for each case, matched for age, sex, and study municipality). The causes of death in the 2 groups were also compared. Both univariate and multivariate survival analyses were performed to investigate the association with disease-related variables. Setting A door-to-door 2-phase prevalence survey performed in 3 Sicilian municipalities. Patients Fifty-nine patients with PD and 118 controls. Results Patients with PD showed a high risk of death (relative risk, 2.3; 95% confidence interval, 1.60-3.39). Greater age at November 1, 1987, high Hoehn-Yahr score, and lack of levodopa therapy were associated with a lower survival on univariate analysis. Multivariate analysis confirmed the association between shorter survival among patients with PD and greater age on November 1, 1987. One-way analysis of variance indicated a different effect of levodopa therapy according to age. Multivariate analysis did not confirm this finding. Pneumonia was the cause of death most frequently associated with PD. Conclusion This study indicates that patients with PD have a shorter survival time than the general population.

We investigated the incidence of first-ever stroke in three Sicilian municipalities over the years 1984–1987. Case ascertainment involved two approaches: a doorto-door two-phase prevalence survey and a study of death certificates. Only first-ever strokes occurring within the study time interval were included, and diagnoses were based on specified criteria. We found 138 subjects who had experienced a first stroke over 73 488 person-years; the average incidence rate (new cases per 1000 population per year) was 1.9 overall, 1.7 in men, and 2.1 in women. Incidence increased steeply with age and was similar in men and women. Incidence was similar in the three study municipalities. The overall case-fatality rate was 35% at 30 days and 22% at one week. Survival after stroke was similar in men and women but better in younger compared to older stroke patients. Comparisons with previous studies suggest the absence of major geographic variations in stroke incidence within Italy.

Introduction - In a door-to-door two-phase survey of common neurologic diseases conducted in two Sicilian municipalities, we investigated the incidence and the lifetime prevalence of Bell's palsy (BP). Material & methods - During phase 1, we administered a screening instrument for facial palsy to 11,901 adult persons. During phase 2, study neurologists using specified diagnostic criteria evaluated those subjects who screened positive. Results - We found 73 subjects who had experienced BP during their life. The lifetime prevalence as of November 1, 1987, was 642.8 cases per 100,000 population age 15 years and above. The prevalence increased with age and was similar in men and women. Eighteen episodes of BP occurred in the three years preceding the prevalence day. The average annual incidence rate was 52.8 new episodes per 100,000 population age 15 years and above. Incidence increased with age. Conclusion - Comparison with other incidence studies suggests some geographic variability.

Objectives - As part of a door-to-door survey of neurologic diseases, we investigated the prevalence of lumbosacral radiculopathy (LR) in two Sicilian municipalities (N = 14,540, as of November 1, 1987). Material & methods - During phase 1, we administered a brief screening instrument to subjects in the study population. In phase 2, study neurologists using specified diagnostic criteria investigated those subjects who screened positive. Our case finding was restricted to subjects who manifested symptoms of LR in the six months preceding the prevalence day. Results - We found 143 subjects affected by LR (112 definite, 31 possible) yielding a crude prevalence of 9.8 cases per 1,000 population. Age-specific prevalence was generally higher in men ; it increased to a peak at age 50-59 years and decreased steadily thereafter. At the peak, prevalence was higher in factory workers, housewives, and clerks compared to workers in other occupations. Conclusions - LR is a common neurologic disease in the general population.

Introduction - Because of the limited information on cervical spondylotic radiculopathy, we conducted a door-to-door two-phase survey in a Sicilian municipality. Material and methods - We first screened for cervical spondylotic radiculopathy among the inhabitants of the municipality : (N= 7653, as of the prevalence day, November 1, 1987). Study neurologists then investigated those subjects suspected to have had a cervical spondylotic radiculopathy. Diagnoses were based on specified criteria. Results - We found 27 subjects affected by CSR (17 definite, 10 possible). Prevalence (cases per 1000 population) was 3.5 in the total population ; it increased to a peak at age 50-59 years and decreased thereafter. The age-specific prevalence was consistently higher in women. Conclusions - Comparison with other prevalence studies shows similar age-specific patterns, but different magnitudes, which may partly reflect methodologic differences across studies.