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Although the name of Andreas Rett is familiar to many from his eponymous neurogenetic syndrome, his other achievements involving the care of disabled children deserve special attention. His tireless advocacy helped to bring fundamental changes in the medical and societal attitude toward disabled individuals in a city that had recently seen more than 7500 disabled children and inmates of psychiatric hospitals actively euthanized by National Socialist (Nazi) decree. Most notably, this study demonstrates the remarkable changes that can be achieved single-handedly by a vocal and energetic physician. Yet at the same time, several instances are recorded in which Rett appeared to prioritize his own professional advancement at the expense of truthful disclosure of his own past, as well as that of some of his close associates. Dr Rett's professional life and contributions, now 10 years after his death, presents a compelling object lesson for neurologists and others involved in the care of the disabled. [ABSTRACT FROM AUTHOR]

Childhood epilepsy is one of the most important and prevalent neurological conditions in the developing years. Persons with childhood onset epilepsy are at a high risk for poor psychosocial outcomes, even without experiencing co-morbidities. The goal of management of children with epilepsy should be to enable the child and the family to lead a life as free as possible from the medical and psychosocial complications of epilepsy. This comprehensive care needs to go beyond simply trying to control seizures with minimal adverse drug reactions. Seizure frequency and severity is only one important outcome variable. Other factors such as social, psychological, behavioural, educational, and cultural dimensions of their lives affect children with epilepsy, their families and their close social networks. A number of epilepsy-specific health-related quality of life (HRQL) scales for children have been developed with the aim to include and measure accurately the impact and burden of epilepsy. Their target populations, details of the origin of the items, and psychometric properties vary significantly. Their strengths and weaknesses will be identified more clearly through their continued use in the clinical setting and in research studies. Only a few studies to date have utilized these or generic HRQL measures to assess the HRQL of specific populations with epilepsy. Future research needs to develop theory driven models of HRQL and identify measurable factors that have important correlations with outcomes. Since biomedical variables like seizure frequency and severity have only moderate correlations with HRQL, other independent factors including the child's resilience, co-morbid conditions, parental well-being, family factors and societal/cultural variables may play a major role. We also need to learn what encompasses comprehensive patient care, define the goals of management and evaluate the impact of different interventions. Future studies need to include the children's own perspectives of their HRQL in addition to parental reports. Finally, clinicians need to familiarize themselves with outcome measures, be able to evaluate them, and use them routinely in their day-to-day practice. [ABSTRACT FROM AUTHOR]

At present, there is no internationally accepted set of core outcomes or measurement methods for epilepsy clinical practice. The International Consortium for Health Outcomes Measurement (ICHOM) convened an international working group of experts in epilepsy, people with epilepsy, and their representatives to develop minimum sets of standardized outcomes and outcome measurement methods for clinical practice. Using modified Delphi consensus methods with consecutive rounds of online voting over 12 months, a core set of outcomes and corresponding measurement tool packages to capture the outcomes were identified for infants, children, and adolescents with epilepsy. Consensus methods identified 20 core outcomes. In addition to the outcomes identified for the ICHOM Epilepsy adult standard set, behavioral, motor, and cognitive/language development outcomes were voted as essential for all infants and children with epilepsy. The proposed set of outcomes and measurement methods will facilitate the implementation of the use of patient-centered outcomes in daily practice., (© 2024 The Authors. Epilepsia published by Wiley Periodicals LLC on behalf of International League Against Epilepsy.)

In this chapter we argue that modern thinking about chronic neurological and developmental conditions requires that we recognize all aspects of functioning and quality of life in addition to the biomedical dimensions of these disorders. We find the International Classification of Functioning, Disability, and Health (ICF) a useful framework by which to think about and understand the many dimensions of health; and we appreciate its heuristic value as a stimulus to consider a range of outcomes of these individuals. Quality of Life (QOL) and Health-Related Quality of Life (HRQL) cover the additional perceived dimensions that are so important to patients' personal valuations, aspirations, and satisfaction about health throughout their lifespan, of which we see QOL being the broader construct. In order to measure outcomes at either the individual clinical or research level it is essential to ask clear and specific questions as a prelude to selecting measures that are appropriate in terms of both their content and their measurement properties. We provide some brief guidelines that we hope will be helpful to readers who wish to expand their activities in measuring clinical outcomes., (Copyright © 2013 Elsevier B.V. All rights reserved.)

The practice of pediatric neurology demands a high level of responsibility at multiple levels. These include listening carefully to people's stories in order to assess each situation, planning and implementing investigations and therapies, individual and family counseling, longitudinal follow-up from fetal life throughout childhood and adolescence, organization of transition to adult care, and societal advocacy. In the 21st century these activities must be carried out in the context of major societal and technological changes which have brought about many new challenges for pediatric neurologists. In this chapter, we address ethical and moral issues that may help guide pediatric neurologists with regard to a number of specific challenges. These include physician-patient relationships that are based on benign paternalism with respect for autonomy and promoting quality of life, practicing evidence-based medicine, and the technological imperative. In addition we discuss the tension between clinical practice and research, relationships between physicians and industry, and the public role of pediatric neurologists to advocate for children with neurological and developmental conditions. We also illustrate some challenges in selected situations such as prenatal counseling (fetal neurology), neonatal encephalopathy, and persistent vegetative state., (Copyright © 2013 Elsevier B.V. All rights reserved.)

Objectives: To explore, identify and understand the contribution that biomedical and psychosocial factors make to the assessment of health-related quality of life (HRQL) of children and youth with epilepsy using the CHEQOL-25., Methods: We identified and measured variables that may influence HRQL; grouped the variables into four conceptual categories; and used simple and hierarchical linear regressions to model CHEQOL-25 as a function of these variable groupings. Participants were 8-15year olds with epilepsy and their parents., Results: 131 child and parent pairs participated. Overall, the unique variances associated with the biomedical and psychosocial variables are R(2)=0.22 and 0.29, respectively, for child self-reported HRQL and R(2)=0.11 and 0.13, respectively, for parent-proxy report., Conclusions: This study increases our understanding of factors that contribute to the expression of HRQL in this population. These results should be interpreted with caution due to the limited sample size and large number of variables., (Copyright (c) 2010 Elsevier Inc. All rights reserved.)

Children and adolescents with epilepsy are at increased risk for poor health-related quality of life (HRQL) even in the absence of active seizures. Clinicians who aim to achieve optimal seizure control also need to focus on improving HRQL. This can only be accomplished by recognizing how different features of the epilepsy itself, co-morbid conditions, as well as psychosocial factors can all make a difference to HRQL. These psychosocial factors include child, family and community/society variables that play a role in the well-being of these children. Conceptual models are relevant insofar as they provide us with a basis for hypothesizing the causal processes that lead to improved HRQL. They delineate what is meant by HRQL and highlight factors relevant to this important outcome. Researchers can use such models to test statistically and clinically relevant relationships. Clinicians can use this knowledge to direct the planning and implementation of services that are informed by these relationships, critically evaluate and then choose HRQL measures for use in clinical practice that most closely approximate what they understand HRQL is. The purpose of this review is to identify what is meant by HRQL as well as outline the theoretical and empirical basis for what it is about epilepsy, co-morbidity, and its treatment that we believe make a difference to HRQL.