Parent-Reported Quality of Life of Children With Cerebral Palsy in Europe

OBJECTIVE. The goal was to determine whether the type and severity of the child's impairments and the family's psychosocial, social, and economic characteristics influence parent-reported child quality of life across the spectrum of severity of cerebral palsy.

METHODS. Our population-based, cross-sectional survey conducted in 2004 to 2005 involved 818 children with cerebral palsy, 8 to 12 years of age, from 7 countries (9 regions) in Europe. Child quality of life was assessed through parent reports by using the Kidscreen questionnaire, and data were analyzed separately for each of its 10 domains.

RESULTS. The parental response rates were >93% for all domains except one. Gross motor function and IQ level were found to be associated independently with quality of life in most domains. However, greater severity of impairment was not always associated with poorer quality of life; in the moods and emotions, self-perception, social acceptance, and school environment domains, less severely impaired children were more likely to have poor quality of life. Pain was associated with poor quality of life in the physical and psychological well-being and self-perception domains. Parents with higher levels of stress were more likely to report poor quality of life in all domains, which suggests that factors other than the severity of the child's impairment may influence the way in which parents report quality of life.

CONCLUSIONS. The parent-reported quality of life for children with cerebral palsy is associated strongly with impairment. However, depending on the areas of life, the most severely impaired children (in terms of motor functioning or intellectual ability) do not always have the poorest quality of life.

Health status of children with cerebral palsy living in Europe: a multi-centre study

Aim The aim of this report is to describe the health status of 8–12-year-old children with cerebral palsy (CP) of all severities in Europe using the Child Health Questionnaire (CHQ).

Method A total of 818 children with CP from nine centres in defined geographical areas participated. CP type, gross and fine motor function, additional impairments were classified and family data were obtained. The CHQ was used to measure the parent's perception of their child's physical (PHY) and psychosocial (PSY) health.

Results PHY scores were lower than the reference samples with a median of 46. The severity of gross motor function influenced the CHQ scores significantly in the PHY scale with the lowest scores for children with least gross motor function. There were significant differences between the CP types in PHY with the higher scores for children with unilateral spastic and the lowest scores for children with bilateral spastic and dyskinetic CP type. Fine motor function severity significantly affected both the PHY and PSY scales. The severity of intellectual impairment was significantly associated with CHQ scores in most dimensions with higher scores for higher IQ level in PHY and PSY. Children with seizures during the last year had a significantly lower health compared with children without seizures. The results of the multivariate regression analyses (forward stepwise regression) of CHQ scores on CP subtype, gross and fine motor function, cognitive function, additional impairments, seizures, parental education and employment revealed gross motor function, cognitive level and type of school attended were significant prognostic factors.

Conclusion This report is based on the largest sample to date of children with CP. Health status as measured using the CHQ was affected in all children and was highly variable. Gross motor function level correlates with health from the PHY well-being perspective but the PSY and emotional aspects do not appear to follow the same pattern.

Reliability and Validity of the Child Health QuestionnairePF-50 for European Children with Cerebral Palsy

Objective: To evaluate the psychometric performance of the Child Health Questionnaire (CHQ) in children with cerebral palsy (CP).
Method: 818 parents of children with CP, aged 8–12 from nine regions of Europe completed the CHQ (parent form 50 items). Functional abilities were classified using the five-level Gross Motor Function Classification Scheme (Levels I–III as ambulant; Level IV–V as nonambulant CP).
Results: Ceiling effects were observed for a number of subscales and summary scores across all Gross Motor Function Classification System levels, whilst floor effects occurred only in the physical functioning scale (Level V CP). Reliability was satisfactory overall. Confirmatory factor analysis (CFA) revealed a seven-factor structure for the total sample of children with CP but with different factor structures for ambulant and nonambulant children.
Conclusion: The CHQ has limited applicability in children with CP, although with judicious use of certain domains for ambulant and nonambulant children can provide useful and comparable data about child health status for descriptive purposes.

Validity of a 1 minute walk test for children with cerebral palsy.

The concurrent validity of a 1 minute walk test at a child's maximum walking speed was assessed in children with bilateral spastic cerebral palsy (BSCP). The distance covered during the 1 minute walk test was compared with the children's gross motor function as assessed by the Gross Motor Function Measure (GMFM). Twenty-four male and 10 female children with CP (mean age 11y, range 4 to 16y) participated in the study. Gross Motor Function Classification System (GMFCS) levels were; level I (n=3), level II (n=17), level III (n=10), and level IV (n=4). Participants had clinical diagnoses of symmetrical diplegia (n=19), asymmetrical diplegia (n=14), and quadriplegia (n=1). Results showed a significant correlation between GMFM score and the distance covered during the 1 minute walk (r=0.92; p<0.001). There was also a significant decrease in the distance walked with increasing GMFCS level (p<0.001). We concluded that the 1 minute walk test is a valid measure for assessing functional ability in children with ambulatory BSCP. Its cost-effectiveness and user friendliness make it a potentially useful tool in the clinical setting. Further study needs to address its reliability and ability to detect change over time.

Age-related changes in energy efficiency of gait, activity, and participation in children with cerebral palsy

Aim
The aim of this study was to use a prospective longitudinal study to describe age-related trends in energy efficiency during gait, activity, and participation in ambulatory children with cerebral palsy (CP).

Method
Gross Motor Function Measure (GMFM), Paediatric Evaluation of Disability Inventory (PEDI), and Lifestyle Assessment Questionnaire-Cerebral Palsy (LAQ-CP) scores, and energy efficiency (oxygen cost) during gait were assessed in representative sample of 184 children (112 male; 72 female; mean age 10y 9mo; range 4–16y) with CP. Ninety-four children had unilateral spastic CP, 84 bilateral spastic CP, and six had other forms of CP. Fifty-seven were classified as Gross Motor Function Classification System (GMFCS) level I, 91 as level II, 22 as level III, and 14 as level IV). Assessments were carried out on two occasions (visit 1 and visit 2) separated by an interval of 2 years and 7 months. A total of 157 participants returned for reassessment.

Results
Significant improvements in mean raw scores for GMFM, PEDI, and LAQ-CP were recorded; however, mean raw oxygen cost deteriorated over time. Age-related trends revealed gait to be most inefficient at the age of 12 years, but GMFM scores continued to improve until the age of 13 years, and two PEDI subscales to age 14 years, before deteriorating (p<0.05). Baseline score was consistently the single greatest predictor of visit 2 score. Substantial agreement in GMFCS ratings over time was achieved (?lw=0.74–0.76).

Interpretation
These findings have implications in terms of optimal provision and delivery of services for young people with CP to maximize physical capabilities and maintain functional skills into adulthood.

Oromotor dysfunction and communication impairments in children with cerebral palsy: a Register study

Aim  To report the prevalence, clinical associations, and trends over time of oromotor dysfunction and communication impairments in children with cerebral palsy (CP).

Method  Multiple sources of ascertainment were used and children followed up with a standardized assessment including motor speech problems, swallowing/chewing difficulties, excessive drooling, and communication impairments at age 5 years.

Results  A total of 1357 children born between 1980 and 2001 were studied (781 males, 576 females; median age 5y 11mo, interquartile range 3–9y; unilateral spastic CP, n=447; bilateral spastic CP, n=496; other, n=112; Gross Motor Function Classification System [GMFCS] level: I, 181; II, 563; III, 123; IV, 82; IV, 276). Of those with ‘early-onset’ CP (n=1268), 36% had motor speech problems, 21% had swallowing/chewing difficulties, 22% had excessive drooling, and 42% had communication impairments (excluding articulation defects). All impairments were significantly related to poorer gross motor function and intellectual impairment. In addition, motor speech problems were related to clinical subtype; swallowing/chewing problems and communication impairments to early mortality; and communication impairments to the presence of seizures. Of those with CP in GMFCS levels IV to V, a significant proportion showed a decline in the rate of motor speech impairment (p=0.008) and excessive drooling (p=0.009) over time.

Interpretation  These impairments are common in children with CP and are associated with poorer gross motor function and intellectual impairment.

A nonsynonymous LNK polymorphism associated with idiopathic erythrocytosis

Anemia is a symptom associated with cognitive dysfunction and is diagnosed if the hemoglobin level of a blood sample is too low. The clinical impact of chronically low hemoglobin level may be insuf?cient
brain oxygenation, which may result in a decline in cognitive functioning. Previous studies have provided evidence of decrements in cognitive functioning associated with anemia across various disease processes, but few have investigated the association between cognitive dysfunction and hemoglobin level in patients with acute myelogenous leukemia (AML) and myelodysplastic syndrome (MDS). As this population is inherently anemic, studying these patients allowed for an exploration of cognitive changes at mild, moderate, and severe levels of anemia. This investigation explored cutoff points for hemoglobin at which cognitive decline may occur. Findings showed decrements in cognitive functioning occurring at hemoglobin levels of 10 g/dL or below. Performance on measures of word retrieval, attention, and ?ne motor function was most affected which suggests fronto-temporal lobe dysfunction. Results provided evidence as to a hemoglobin cutoff point below which cognitive function may be affected in patients with AML and MDS. This cutoff value may provide a clinical marker at which cognitive testing and therapeutic interventions could be utilized to improve patients’ cognitive function, level of fatigue and overall quality of life.

Neuropeptides in Helminths: Occurrence and Distribution

Nematode neuropeptide systems comprise an exceptionally complex array of similar to 250 peptidic signaling molecules that operate within a structurally simple nervous system of similar to 300 neurons. A relatively complete picture of the neuropeptide complement is available for Caenorhabditis elegans, with 30 flp, 38 ins and 43 nlp genes having been documented; accumulating evidence indicates similar complexity in parasitic nematodes from clades I, III, IV and V. In contrast, the picture for parasitic platyhelminths is less clear, with the limited peptide sequence data available providing concrete evidence for only FMRFamide-like peptide (FLP) and neuropeptide F (NPF) signaling systems, each of which only comprises one or two peptides. With the completion of the Schmidtea meditteranea and Schistosoma mansoni genome projects and expressed sequence tag datasets for other flatworm parasites becoming available, the time is ripe for a detailed reanalysis of neuropeptide signaling in flatworms. Although the actual neuropeptides provide limited obvious value as targets for chemotherapeutic-based control strategies, they do highlight the signaling systems present in these helminths and provide tools for the discovery of more amenable targets such as neuropeptide receptors or neuropeptide processing enzymes. Also, they offer opportunities to evaluate the potential of their associated signaling pathways as targets through RNA interference (RNAi)-based, target validation strategies. Currently, within both helminth phyla, the flp signaling systems appear to merit further investigation as they are intrinsically linked with motor function, a proven target for successful anti-parasitics; it is clear that some nematode NLPs also play a role in motor function and could have similar appeal. At this time, it is unclear if flatworm NPF and nematode INS peptides operate in pathways that have utility for parasite control. Clearly, RNAi-based validation could be a starting point for scoring potential target pathways within neuropeptide signaling for parasiticide discovery programs. Also, recent successes in the application of in planta-based RNAi control strategies for plant parasitic nematodes reveal a strategy whereby neuropeptide encoding genes could become targets for parasite control. The possibility of developing these approaches for the control of animal and human parasites is intriguing, but will require significant advances in the delivery of RNAi-triggers.

Neuromusculature of Gyrodactylus rysavyi, a monogenean gill and skin parasite of the catfish Clarias gariepinus

Phalloidin fluorescence technique, enzyme cytochemistry and immunocytochemistry in conjunction with confocal scanning laser microscopy were used for the first time to describe the nervous and muscle systems of the viviparous monogenean parasite, Gyrodactylus rysavyi inhabiting the gills and skin of the Nile catfish Clarias gariepinus. The body wall muscles are composed of an outer layer of circular fibres, an intermediate layer of paired longitudinal fibres and an inner layer of well-spaced bands of diagonal fibres arranged in two crossed directions. The musculature of the pharynx, intestine, reproductive tract and the most prominent muscles of the haptor were also described. Two characteristic muscular pads were found lying in the anterior region of the haptor in close contact with the hamuli. To each one of these pads, a group of ventral extrinsic muscles was connected. The role of this ventral extrinsic muscle in the body movement was discussed. The mechanism operating the marginal hooklets was also discussed. The central nervous system (CNS) consists of paired cerebral ganglia from which three pairs of longitudinal ventral, lateral and dorsal nerve cords arise. The nerve cords are connected at intervals by many transverse connectives. The CNS is better developed ventrally than dorsally or laterally and it has the highest reactivity for all neuroactive substances examined. Both the central and the peripheral nervous system (PNS) are bilaterally symmetrical. Structural and functional correlates of the neuromusculature of the pharynx, haptor and reproductive tracts were explained. The results implicated acetylcholine, FMRFamide-related peptides (FaRPs) and serotonin in sensory and motor function. The results were compared with those of the monogeneans Macrogyrodactylus clarii and M. congolensis inhabiting the gills and skin respectively of the same host fish C. gariepinus.

Neuromusculature of Macrogyrodactylus congolensis, a monogenean skin parasite of the Nile catfish Clarias gariepinus

Phalloidin fluorescence technique, enzyme cytochemistry and immunocytochemistry, in conjunction with confocal scanning laser microscopy, were used to describe the neuromusculature of the monogenean skin parasite Macrogyrodactylus congolensis from the Nile catfish Clarias gariepinus. The body wall muscles are composed of an outer layer of compactly arranged circular fibres, an intermediate layer of paired longitudinal fibres and an inner layer of well-spaced bands of diagonal fibres arranged in two crossed directions. The central nervous system consists of paired cerebral ganglia from which three pairs of longitudinal ventral, lateral and dorsal nerve cords arise. The nerve cords are connected at intervals by many transverse connectives. Both central and peripheral nervous systems are bilaterally symmetrical and better developed ventrally than laterally and dorsally. Structural and functional correlates of the neuromusculature of the pharynx, haptor and reproductive tracts were examined. Results implicate acetylcholine, FMRFamide-related peptides and serotonin in sensory and motor function. The results were compared with those of Macrogyrodactylus clarii, a gill parasite of the same host fish C. gariepinus.

Neuromusculature of Macrogyrodactylus clarii, a monogenean gill parasite of the Nile catfish Clarias gariepinus in Egypt

Phalloidin fluorescence technique, enzyme cytochemistry and immunocytochemistry in conjunction with confocal scanning laser microscopy have been used for the first time to describe the nervous and muscle systems of the viviparous monogenean gill parasite, Macrogyrodactylus clarii. The gross spatial arrangement of muscle and associated cholinergic, peptidergic and aminergic innervations has been examined. The central nervous system (CNS) consists of paired cerebral ganglia from which emanate three pairs of longitudinal ventral, lateral and dorsal nerve cords, connected at intervals by transverse connectives. The CNS is better developed ventrally than dorsally or laterally, and has the strongest reactivity for all neuroactive substances examined. Structural and functional correlates of the neuromusculature of the pharynx, haptor and reproductive tracts have been examined. Results implicate acetylcholine, FMRFamide-related peptides (FaRPs) and serotonin in sensory and motor function in this monogenean, although confirmatory physiological data are obviously required.

A systematic review of evidence-based assessment practices by allied health practitioners for children with cerebral palsy

AIM: The routine use of psychometrically robust assessment tools is integral to best practice. This systematic review aims to determine the extent to which evidence-based assessment tools were used by allied health practitioners for children with cerebral palsy (CP).

METHOD: The Preferred Reporting Items for Systematic Reviews and Meta-Analysis protocols 2015 was employed. A search strategy applied the free text terms: 'allied health practitioner', 'assessment', and 'cerebral palsy', and related subject headings to seven databases. Included articles reported assessment practices of occupational therapists, physiotherapists, or speech pathologists working with children with CP aged 0 to 18 years, published from the year 2000.

RESULTS: Fourteen articles met the inclusion criteria. Eighty-eight assessment tools were reported, of which 23 were in high use. Of these, three tools focused on gross motor function and had acceptable validity for use with children with CP: Gross Motor Function Measure, Gross Motor Function Classification System, and goniometry. Validated tools to assess other activity components, participation, quality of life, and pain were used infrequently or not at all.

INTERPRETATION: Allied health practitioners used only a few of the available evidence-based assessment tools. Assessment findings in many areas considered important by children and families were rarely documented using validated assessment tools.

Classifying cerebral palsy: are we nearly there?

BACKGROUND: Cerebral palsy (CP) is the most common cause of physical disability in childhood in developed countries and encompasses a wide range of clinical phenotypes. Classification of CP according to movement disorder or topographical distribution is widely used. However, these classifications are not reliable nor do they accurately predict musculoskeletal pathology. More recently, the Gross Motor Function Classification System (GMFCS) has been introduced and its validity, reliability, and clinical utility have been confirmed. In 2005 it was suggested that children should be described and classified according to the GMFCS in all outcome studies involving children with CP, in the Journal of Pediatric Orthopaedics (JPO). This study aimed to describe utilization of the GMFCS in 3 journals: Journal of Bone and Joint Surgery (JBJS Am), JPO, and Developmental Medicine and Child Neurology (DMCN), over a 7-year period (2005 to 2011), and any relationship to the journal's impact factor. A secondary aim was to establish if differences in methodological quality existed between those studies utilizing GMFCS and those that did not.

METHODS: A targeted literature search of the 3 selected journals using the term "cerebral palsy" was conducted using the Medline database. Utilization of the GMFCS was assessed using report of these data in the methods or results section of the retrieved papers. The Methodological Index for Non-Randomized Studies (MINORS) was employed to evaluate the quality of papers published in JPO.

RESULTS: One hundred and fifty-four studies met the inclusion criteria and in 85 (68%) the GMFCS was used. Of these, 112 were published in JPO, of which 51 (46%) utilized the GMFCS, compared with 72% for JBJS Am, and 88% for DMCN. In the JPO, utilization of the GMFCS improved from 13% to 80%, over the 7-year study period.

CONCLUSIONS: Utilization of the GMFCS has increased rapidly over the past 7 years in the JPO but there is room for further improvement.

LEVEL OF EVIDENCE: Not applicable.

SWORD: an intelligent vibratory wearable device to improve rehabilitation in stroke patients

Anualmente ocorrem cerca de 16 milhões AVCs em todo o mundo. Cerca de metade dos sobreviventes irá apresentar défice motor que necessitará de reabilitação na janela dos 3 aos 6 meses depois do AVC. Nos países desenvolvidos, é estimado que os custos com AVCs representem cerca de 0.27% do Produto Interno Bruto de cada País. Esta situação implica um enorme peso social e financeiro. Paradoxalmente a esta situação, é aceite na comunidade médica a necessidade de serviços de reabilitação motora mais intensivos e centrados no doente. Na revisão do estado da arte, demonstra-se o arquétipo que relaciona metodologias terapêuticas mais intensivas com uma mais proficiente reabilitação motora do doente. Revelam-se também as falhas nas soluções tecnológicas existentes que apresentam uma elevada complexidade e custo associado de aquisição e manutenção. Desta forma, a pergunta que suporta o trabalho de doutoramento seguido inquire a possibilidade de criar um novo dispositivo de simples utilização e de baixo custo, capaz de apoiar uma recuperação motora mais eficiente de um doente após AVC, aliando intensidade com determinação da correcção dos movimentos realizados relativamente aos prescritos. Propondo o uso do estímulo vibratório como uma ferramenta proprioceptiva de intervenção terapêutica a usar no novo dispositivo, demonstra-se a tolerabilidade a este tipo de estímulos através do teste duma primeira versão do sistema apenas com a componente de estimulação num primeiro grupo de 5 doentes. Esta fase validará o subsequente desenvolvimento do sistema SWORD. Projectando o sistema SWORD como uma ferramenta complementar que integra as componentes de avaliação motora e intervenção proprioceptiva por estimulação, é descrito o desenvolvimento da componente de quantificação de movimento que o integra. São apresentadas as diversas soluções estudadas e o algoritmo que representa a implementação final baseada na fusão sensorial das medidas provenientes de três sensores: acelerómetro, giroscópio e magnetómetro. O teste ao sistema SWORD, quando comparado com o método de reabilitação tradicional, mostrou um ganho considerável de intensidade e qualidade na execução motora para 4 dos 5 doentes testados num segundo grupo experimental. É mostrada a versatilidade do sistema SWORD através do desenvolvimento do módulo de Tele-Reabilitação que complementa a componente de quantificação de movimento com uma interface gráfica de feedback e uma ferramenta de análise remota da evolução motora do doente. Finalmente, a partir da componente de quantificação de movimento, foi ainda desenvolvida uma versão para avaliação motora automatizada, implementada a partir da escala WMFT, que visa retirar o factor subjectivo da avaliação humana presente nas escalas de avaliação motora usadas em Neurologia. Esta versão do sistema foi testada num terceiro grupo experimental de cinco doentes.

Patient classification for intelligent wheelchair adaptation

A importância e preocupação dedicadas à autonomia e independência das pessoas idosas e dos pacientes que sofrem de algum tipo de deficiência tem vindo a aumentar significativamente ao longo das últimas décadas. As cadeiras de rodas inteligentes (CRI) são tecnologias que podem ajudar este tipo de população a aumentar a sua autonomia, sendo atualmente uma área de investigação bastante ativa. Contudo, a adaptação das CRIs a pacientes específicos e a realização de experiências com utilizadores reais são assuntos de estudo ainda muito pouco aprofundados. A cadeira de rodas inteligente, desenvolvida no âmbito do Projeto IntellWheels, é controlada a alto nível utilizando uma interface multimodal flexível, recorrendo a comandos de voz, expressões faciais, movimentos de cabeça e através de joystick. Este trabalho teve como finalidade a adaptação automática da CRI atendendo às características dos potenciais utilizadores. Foi desenvolvida uma metodologia capaz de criar um modelo do utilizador. A investigação foi baseada num sistema de recolha de dados que permite obter e armazenar dados de voz, expressões faciais, movimentos de cabeça e do corpo dos pacientes. A utilização da CRI pode ser efetuada em diferentes situações em ambiente real e simulado e um jogo sério foi desenvolvido permitindo especificar um conjunto de tarefas a ser realizado pelos utilizadores. Os dados foram analisados recorrendo a métodos de extração de conhecimento, de modo a obter o modelo dos utilizadores. Usando os resultados obtidos pelo sistema de classificação, foi criada uma metodologia que permite selecionar a melhor interface e linguagem de comando da cadeira para cada utilizador. A avaliação para validação da abordagem foi realizada no âmbito do Projeto FCT/RIPD/ADA/109636/2009 - "IntellWheels - Intelligent Wheelchair with Flexible Multimodal Interface". As experiências envolveram um vasto conjunto de indivíduos que sofrem de diversos níveis de deficiência, em estreita colaboração com a Escola Superior de Tecnologia de Saúde do Porto e a Associação do Porto de Paralisia Cerebral. Os dados recolhidos através das experiências de navegação na CRI foram acompanhados por questionários preenchidos pelos utilizadores. Estes dados foram analisados estatisticamente, a fim de provar a eficácia e usabilidade na adequação da interface da CRI ao utilizador. Os resultados mostraram, em ambiente simulado, um valor de usabilidade do sistema de 67, baseado na opinião de uma amostra de pacientes que apresentam os graus IV e V (os mais severos) de Paralisia Cerebral. Foi também demonstrado estatisticamente que a interface atribuída automaticamente pela ferramenta tem uma avaliação superior à sugerida pelos técnicos de Terapia Ocupacional, mostrando a possibilidade de atribuir automaticamente uma linguagem de comando adaptada a cada utilizador. Experiências realizadas com distintos modos de controlo revelaram a preferência dos utilizadores por um controlo compartilhado com um nível de ajuda associado ao nível de constrangimento do paciente. Em conclusão, este trabalho demonstra que é possível adaptar automaticamente uma CRI ao utilizador com claros benefícios a nível de usabilidade e segurança.