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https://www.readbyqxmd.com/read/28335910/management-strategies-for-cln2-disease
#1
REVIEW
Ruth E Williams, Heather R Adams, Martin Blohm, Jessica L Cohen-Pfeffer, Emily de Los Reyes, Jonas Denecke, Kristen Drago, Charlie Fairhurst, Margie Frazier, Norberto Guelbert, Szilárd Kiss, Annamaria Kofler, John A Lawson, Lenora Lehwald, Mary-Anne Leung, Svetlana Mikhaylova, Jonathan W Mink, Miriam Nickel, Renée Shediac, Katherine Sims, Nicola Specchio, Meral Topcu, Ina von Löbbecke, Andrea West, Boris Zernikow, Angela Schulz
CLN2 disease (neuronal ceroid lipofuscinosis type 2) is a rare, autosomal recessive, pediatric-onset, rapidly progressive neurodegenerative lysosomal storage disorder caused by tripeptidyl peptidase 1 (TPP1) enzyme deficiency, and is characterized by language delay, seizures, rapid cognitive and motor decline, blindness, and early death. No management guidelines exist and there is a paucity of published disease-specific evidence to inform clinical practice, which currently draws upon experience from the field of childhood neurodisability...
April 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/28325154/the-behavioral-profile-of-methylenedioxypyrovalerone-mdpv-and-%C3%AE-pyrrolidinopentiophenone-pvp-a-systematic-review
#2
Cornel N Stanciu, Thomas M Penders, Samantha A Gnanasegaram, Elvin Pirapakaran, Juvraj S Padda, Jeeven S Padda
BACKGROUND: New psychoactive substances are emerging at an alarming rate. With novel structures and properties they produce unique behavioral characteristics during intoxication. Synthetic cathinones are some of the most common. More and more are presenting to emergency departments due to consequences of use. Aside from isolated reports, little is known about these compounds. OBJECTIVE: Here we review literature pertaining to two common synthetic cathinones, MDPV and alphaPVP, and correlate clinically with guidance on management of intoxications...
March 21, 2017: Current Drug Abuse Reviews
https://www.readbyqxmd.com/read/28325104/communication-and-well-being-outcomes-of-a-hybrid-service-delivery-model-of-intensive-impairment-based-treatment-for-aphasia-in-the-hospital-setting-a-pilot-study
#3
Rachel Wenke, Elizabeth Cardell, Melissa Lawrie, Dana Gunning
PURPOSE: This pilot study aimed to evaluate the effects of an intensive hybrid service delivery model (i.e., combining face-to-face individual, computer and group therapy) on communication and well-being for people with aphasia (PWA) in the hospital setting. MATERIALS AND METHODS: The study explored two different intensities of the hybrid model, 4 h/week (Hybrid-4) and 8 h/week (Hybrid-8) both for 8 weeks. Participants ranging from 1 month to 5 years post-onset were allocated using matched-pair randomisation to receive either Hybrid-4 (n = 5) or Hybrid-8 (n = 4) and assessed using a comprehensive language battery by a blinded assessor, as well as selected activity, participation and well-being measures before, immediately after and 4-week post-treatment...
March 21, 2017: Disability and Rehabilitation
https://www.readbyqxmd.com/read/28323747/supporting-a-youth-with-cerebellar-ataxia-into-adolescence
#4
Veronica Meneses, Zurisadai Gonzalez-Castillo, Veronica B Edgar, Marilyn Augustyn
Zoe, a 13-year-old white girl, presents as a new patient to your pediatric clinic with complaints of frequent emesis, anxiety, and learning problems, and previous diagnosis of cerebellar ataxia. Parents accompany Zoe and state, "it is really hard for her to go out, she gets sick and falls easily." She was born full term by vaginal delivery without complications. Given globally delayed milestones, she received early intervention services. Feeding problems began at infancy, including gastroesophageal reflux and aspiration pneumonia...
March 17, 2017: Journal of Developmental and Behavioral Pediatrics: JDBP
https://www.readbyqxmd.com/read/28319481/application-of-telepractice-for-head-and-neck-cancer-management-a-review-of-speech-language-pathology-service-models
#5
Elizabeth C Ward, Laurelie R Wall, Clare L Burns, Bena Cartmill, Anne J Hill
PURPOSE OF REVIEW: Head and neck cancer (HNC) is a complex and heterogeneous disease, requiring specialist intervention from a multidisciplinary team including speech language pathology (SLP). Unfortunately, multiple patient and service-related challenges exist which currently limit equitable access to SLP support for all individuals. This review highlights the existing evidence for different telepractice models designed to help patients and services optimize management of swallowing and communication disorders arising from HNC...
March 17, 2017: Current Opinion in Otolaryngology & Head and Neck Surgery
https://www.readbyqxmd.com/read/28315852/characteristics-and-management-of-mirror-syndrome-a-systematic-review-1956-2016
#6
Sabah Allarakia, Hassan A Khayat, Moyassar M Karami, Abdulaziz M Aldakhil, Ahmed M Kashi, Abdulrahman H Algain, Mohammad A Khan, Loai S Alghifees, Raed E Alsulami
OBJECTIVES: To describe the clinical features of mirror syndrome and to correlate the effects of different treatments with the fetal outcomes. DATA SOURCES: Online search up to May 2016 was conducted in the PubMed, Embase (Ovid platform) and clinicalTrials.gov without restrictions of language, date or journal. Only papers providing both fetal and maternal presentations and outcomes were included. RESULTS: The study included 74 papers (n=111), with an additional two patients diagnosed at our center (n=113)...
March 20, 2017: Journal of Perinatal Medicine
https://www.readbyqxmd.com/read/28301848/predictors-of-reversion-from-mild-cognitive-impairment-to-normal-cognition
#7
Seema Y Pandya, Laura H Lacritz, Myron F Weiner, Martin Deschner, Fu L Woon
BACKGROUND/AIMS: Few studies have examined predictors of reversion from mild cognitive impairment (MCI) to normal cognition. We sought to identify baseline predictors of reversion, using the National Alzheimer's Coordinating Center Uniform Data Set, by comparing MCI individuals who reverted to normal cognition to those who progressed to dementia. METHODS: Participants (n = 1,208) meeting MCI criteria were evaluated at the baseline visit and 3 subsequent annual visits...
March 17, 2017: Dementia and Geriatric Cognitive Disorders
https://www.readbyqxmd.com/read/28299159/autism-spectrum-disorder-in-a-community-based-sample-with-neurodevelopmental-problems-in-lagos-nigeria
#8
Yewande O Oshodi, Andrew T Olagunju, Motunrayo A Oyelohunnu, Elizabeth A Campbell, Charles S Umeh, Olatunji F Aina, Wellington Oyibo, Folusho E A Lesi, Joseph D Adeyemi
Autism Spectrum Disorder (ASD) is a globally prevalent neurodevelopmental disorder for which early diagnosis and intervention is the mainstay of management. In the African continent, limited data is available regarding the non-clinic based samples. Lack of information available to caregivers and inadequate skilled manpower often limit early detection and access to the few available though under resourced services in the community. Community based screening can be an important drive to create awareness and improve information dissemination regarding services available for those living with this disorder...
December 31, 2016: Journal of Public Health in Africa
https://www.readbyqxmd.com/read/28292606/speech-language-and-reading-skills-in-10-year-old-children-with-palatal-clefts-the-impact-of-additional-conditions
#9
Kristin Billaud Feragen, Ragnhild Aukner, Tone K Særvold, Øydis Hide
BACKGROUND: This study examined speech (hypernasality and intelligibility), language, and reading skills in children with a cleft palate, specifically investigating additional conditions to the cleft, in order to differentiate challenges related to a cleft only, and challenges associated with an additional condition. DESIGN: Cross-sectional data collected during routine assessments of speech and language in a centralised treatment setting. PARTICIPANTS: Children born with cleft with palatal involvement from four birth cohorts (n=184), aged 10...
March 7, 2017: Journal of Communication Disorders
https://www.readbyqxmd.com/read/28292268/social-inclusion-for-children-with-hearing-loss-in-listening-and-spoken-language-early-intervention-an-exploratory-study
#10
Gabriella Constantinescu-Sharpe, Rebecca L Phillips, Aleisha Davis, Dimity Dornan, Anthony Hogan
BACKGROUND: Social inclusion is a common focus of listening and spoken language (LSL) early intervention for children with hearing loss. This exploratory study compared the social inclusion of young children with hearing loss educated using a listening and spoken language approach with population data. METHODS: A framework for understanding the scope of social inclusion is presented in the Background. This framework guided the use of a shortened, modified version of the Longitudinal Study of Australian Children (LSAC) to measure two of the five facets of social inclusion ('education' and 'interacting with society and fulfilling social goals')...
March 14, 2017: BMC Pediatrics
https://www.readbyqxmd.com/read/28288547/neuropsychological-correlates-of-schizotypy-a-systematic-review-and-meta-analysis-of-cross-sectional-studies
#11
Sara Siddi, Donatella Rita Petretto, Antonio Preti
INTRODUCTION: Cognitive deficits can precede the onset of psychotic episodes and predict the onset of the illness in individuals with schizotypy traits. In some studies, high levels of schizotypy were associated with impairments in memory, attention, executive functions, and verbal fluency. This review provides a more comprehensive understanding of cognitive impairments related to schizoytpy. METHODS:  A systematic review of "schizotypy and neuropsychological measures" was conducted, and it retrieved 67 studies...
March 13, 2017: Cognitive Neuropsychiatry
https://www.readbyqxmd.com/read/28288010/functional-connectivity-is-reduced-in-early-stage-primary-progressive-aphasia-when-atrophy-is-not-prominent
#12
Borna Bonakdarpour, Emily J Rogalski, Allan Wang, Jaiashre Sridhar, M M Mesulam, Robert S Hurley
Primary progressive aphasia (PPA) is a clinical syndrome of language decline caused by neurodegenerative pathology. Although language impairments in PPA are typically localized via the morphometric assessment of atrophy, functional changes may accompany or even precede detectable structural alterations, in which case resting state functional connectivity (RSFC) could provide an alternative approach. The goal of this study was to determine whether language network RSFC is reduced in early-stage PPA when atrophy is not prominent...
March 10, 2017: Alzheimer Disease and Associated Disorders
https://www.readbyqxmd.com/read/28286963/rehabilitation-and-education-are-underutilized-for-mild-stroke-and-tia-sufferers
#13
Steven G Faux, Pooja Arora, Christine T Shiner, Angelica G Thompson-Butel, Linda A Klein
PURPOSE: Transient ischemic attack (TIA) and mild stroke represent a large proportion of cerebrovascular events, at high risk of being followed by recurrent, serious events. The importance of early education addressing risk management, secondary prevention and lifestyle modifications is the centerpiece of further stroke prevention. However, delivering education and rehabilitation to this population can be complex and challenging. METHODS: Via synthesis of a narrative review and clinical experience, we explore the unique and inherent complexities of rehabilitation management and education provision for patients following mild stroke and TIA...
March 12, 2017: Disability and Rehabilitation
https://www.readbyqxmd.com/read/28286431/input-subject-diversity-enhances-early-grammatical-growth-evidence-from-a-parent-implemented-intervention
#14
Pamela A Hadley, Matthew Rispoli, Janet K Holt, Theodora Papastratakos, Ning Hsu, Mary Kubalanza, Megan M McKenna
PURPOSE: The current study used an intervention design to test the hypothesis that parent input sentences with diverse lexical noun phrase (NP) subjects would accelerate growth in children's sentence diversity. METHOD: Child growth in third person sentence diversity was modeled from 21 to 30 months (n = 38) in conversational language samples obtained at 21, 24, 27, and 30 months. Treatment parents (n = 19) received instruction on strategies designed to increase lexical NP subjects (e...
2017: Language Learning and Development
https://www.readbyqxmd.com/read/28285893/motor-and-verbal-perspective-taking-in-children-with-autism-spectrum-disorder-changes-in-social-interaction-with-people-and-tools
#15
Breanna E Studenka, Sandra L Gillam, Daphne Hartzheim, Ronald B Gillam
BACKGROUND: Children with Autism Spectrum Disorder (ASD) have difficulty communicating with others nonverbally, via mechanisms such as hand gestures, eye contact and facial expression. Individuals with ASD also have marked deficits in planning future actions (Hughes, 1996), which might contribute to impairments in non-verbal communication. Perspective taking is typically assessed using verbal scenarios whereby the participant imagines how an actor would interact in a social situation (e...
March 9, 2017: Research in Developmental Disabilities
https://www.readbyqxmd.com/read/28283312/language-disorders-in-victims-of-domestic-violence-in-children-s-homes
#16
Martha Cobos-Cali, Valentina Ladera, María Victoria Perea, Ricardo García
Studies that deal with child maltreatment have become relevant during these past years. One important aspect to consider is the impact of maltreatment on the cognitive functioning and more precisely on language. Our objective is to analyze the different components in the comprehension and production of language in children victims of domestic abuse in Childreńs Homes. PARTICIPANTS: The sample consists of 104 participants divided in two groups. A group of children who have just been institutionalized due to domestic abuse (VG) (Age: 8 years 2 months with a standard deviation of 1, 5 years) without previous treatment; a group of comparison (CG) made up by children who have not been victim of domestic violence (Age: 8 years 6 months with a standard deviation of 2 years and a month), with similar characteristics of gender, age and schooling...
March 7, 2017: Child Abuse & Neglect
https://www.readbyqxmd.com/read/28256376/-autism-an-early-neurodevelopmental-disorder
#17
F Bonnet-Brilhault
With approximately 67 million individuals affected worldwide, autism spectrum disorder (ASD) is the fastest growing neurodevelopmental disorder (United Nations, 2011), with a prevalence estimated to be 1/100. In France ASD affects approximately 600,000 individuals (from childhood to adulthood, half of whom are also mentally retarded), who thus have a major handicap in communication and in adapting to daily life, which leads autism to be recognized as a national public health priority. ASD is a neurodevelopmental disorder that affects several domains (i...
April 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28256009/adaptation-of-macarthur-communicative-development-inventory-in-rural-pakistan-useful-tool-for-early-childhood-studies
#18
M A Rasheed, G Tabussum, Z H Habib, A K Yousafzai
BACKGROUND: Given the significance of early language for later academic achievement, language development can be an important outcome measure in evaluation of early childhood intervention programmes. Language development may be challenging to assess in settings where trained personnel are hard to find; therefore, maternal report can be a reliable alternative measure. AIM: The aim of this study was to adapt a maternal report measure, the MacArthur Communicative Development Inventory II-Short Form, in the Sindhi language and to examine its psychometric properties as a measure of language outcome in 2-year-old children in rural Pakistan...
March 3, 2017: Child: Care, Health and Development
https://www.readbyqxmd.com/read/28255576/systematic-review-of-screening-instruments-for-psychosocial-problems-in-children-and-adolescents-with-long-term-physical-conditions
#19
Hiran Thabrew, Heather McDowell, Katherine Given, Kathryn Murrell
Children and adolescents with long-term physical conditions (LTPCs) are at greater risk of developing psychosocial problems. Screening for such problems may be undertaken using validated psychometric instruments to facilitate early intervention. A systematic review was undertaken to identify clinically utilized and psychometrically validated instruments for identifying depression, anxiety, behavior problems, substance use problems, family problems, and multiple problems in children and adolescents with LTPCs...
2017: Global Pediatric Health
https://www.readbyqxmd.com/read/28253013/acculturation-and-post-migration-psychological-symptoms-among-iraqi-refugees-a-path-analysis
#20
Joseph W LeMaster, Carissa L Broadbridge, Mark A Lumley, Judith E Arnetz, Cynthia Arfken, Michael D Fetters, Hikmet Jamil, Nnamdi Pole, Bengt B Arnetz
Refugees frequently experience symptoms of posttraumatic stress and depression, which impede their acculturation in the new host country where they are resettling. There are few longitudinal studies investigating predictors of mental health and acculturation during the early postmigration period. We conducted a longitudinal study of 298 Iraqi refugees, assessing them upon arrival to the U.S. and 1 year after migration. Premigration trauma was associated with increased PTSD and depressive symptoms at baseline, and with decreased acculturation 1 year later...
March 2, 2017: American Journal of Orthopsychiatry
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