Martin Bax

A report: the definition and classification of cerebral palsy April 2006.

For a variety of reasons, the definition and the clawification of cerebral palsy (CP) need to be reconsidered. Modern brain imaging techniques have shed new light on the nature of the underlying brain injury and studies on the neurobiology of and pathology associated with brain development have further explored etiologic mechanisms. It is now recognized that assessing the extent of activity restriction is part of CP evaluation and that people without activity restriction should not be included in the CP rubric. Also, previous definitions have not given sufficient prominence to the non‐motor neurodevelopmental disabilities of performance and behaviour that commonly accompany CP, nor to the progression of musculoskeletal difficulties that often occurs with advancing age. In order to explore this information, pertinent material was reviewed on July 11–13,2004 at an international workshop in Bethesda, MD (USA) organized by an Executive Committee and participated in by selected leaders in the preclinical and clinical sciences. At the workshop, it was agreed that the concept ‘cerebral palsy’ should be retained. Suggestions were made about the content of a revised definition and classification of CP that would meet the needs of clinicians, investigators, health officials, families and the public and would provide a common language for improved communication. Panels organized by the Executive Committee used this information and additional comments from the international community to generate a report on the Definition and Classification of Cerebral Palsy, April 2006. The Executive Committee presents this report with the intent of providing a common conceptualization of CP for use by a broad international audience.

Proposed definition and classification of cerebral palsy, April 2005

Because of the availability of new knowledge about the neurobiology of developmental brain injury, information that epidemiology and modern brain imaging is providing, the availability of more precise measuring instruments of patient performance, and the increase in studies evaluating the efficacy of therapy for the consequences of injury, the need for reconsideration of the definition and classification of cerebral palsy (CP) has become evident. Pertinent material was reviewed at an international symposium participated in by selected leaders in the preclinical and clinical sciences. Suggestions were made about the content of a revised definition and classification of CP that would meet the needs of clinicians, investigators, and health officials, and provide a common language for improved communication. With leadership and direction from an Executive Committee, panels utilized this information and have generated a revised Definition and Classification of Cerebral Palsy. The Executive Committee presents this revision and welcomes substantive comments about it.

TERMINOLOGY AND CLASSIFICATION OF CEREBRAL PALSY

A SMALL group” met recently in Edinburgh to discuss the terminology and classification of cerebral palsy. At the meeting, a good deal of thinking and discussion went into the basis of medical classification in general, and attention was drawn to the extremely loose way in which, in the cerebral palsy field, we have used terms in the past and continue to do so today. The group reached agreement on a definition of cerebral palsy, but ran into difficulties after this. Cerebral palsy, they agreed, is one of a group of conditions generally described as ‘Syndromes of Cerebral Dysfunction’. This fitted into the proposals put forward by DENHOFF and ROBINAULT,~ and the only serious criticism was that the term ‘cerebral’ does not strictly include all parts of the brain, although most people would accept this extension of its meaning. Cerebral palsy is therefore defined as : ‘A disorder of movement and posture due to a defect or lesion of the immature brain.’ For practical purposes it is usual to exclude from cerebral palsy those disorders of posture and movement which are (1) of short duration, (2) due to progressive disease, or (3) due solely to mental deficiency. The group felt that this simple sentence could be readily translated into other languages and hoped that it might be universally accepted. They felt that it is wiser at the present time not to define precisely what they meant by ‘immature brain’, as any such definition might lead to administrative difficulties. The individual clinician dealing with the few cases arising in older children could decide whether it was reasonable to describe a certain child as having cerebral palsy. Translators do not always find the word ‘palsy’ easy to cope with, as the more exactminded expect ‘palsy’ to mean the same as ‘paralysis’ and by ‘paralysis’ understand what others would term ‘complete paralysis’. It might be well in English and American neurological and medical writing to make greater use of the word ‘paresis’ where power is present but impaired. The group ran into considerable difficulty when they went on to discuss the classification of various types of cerebral paresis. Indeed, they barely got beyond discussing the word ‘spastic’. It was not only that the word is used by lay people to describe anybody with cerebral palsy, but that there are various medical uses of the term. Sometimes it is used to describe a particular clinical syndrome rather common in the whole group of cerebral palsy, whatever the stage of evolution of the clinical picture. Sometimes its use is limited

Long-term sleep disturbances in children: A cause of neuronal loss

Short-term sleep loss is known to cause temporary difficulties in cognition, behaviour and health but the effects of persistent sleep deprivation on brain development have received little or no attention. Yet, severe sleep disorders that last for years are common in children especially when they have neurodevelopmental disabilities. There is increasing evidence that chronic sleep loss can lead to neuronal and cognitive loss in children although this is generally unrecognized by the medical profession and the public. Without the restorative functions of sleep due to total sleep deprivation, death is inevitable within a few weeks. Chronic sleep disturbances at any age deprive children of healthy environmental exposure which is a prerequisite for cognitive growth more so during critical developmental periods. Sleep loss adversely effects pineal melatonin production which causes disturbance of circadian physiology of cells, organs, neurochemicals, neuroprotective and other metabolic functions. Through various mechanisms sleep loss causes widespread deterioration of neuronal functions, memory and learning, gene expression, neurogenesis and numerous other changes which cause decline in cognition, behaviour and health. When these changes are long-standing, excessive cellular stress develops which may result in widespread neuronal loss. In this review, for the first time, recent research advances obtained from various fields of sleep medicine are integrated in order to show that untreated chronic sleep disorders may lead to impaired brain development, neuronal damage and permanent loss of developmental potentials. Further research is urgently needed because these findings have major implications for the treatment of sleep disorders.

The role of the thalamus in sleep, pineal melatonin production, and circadian rhythm sleep disorders

Abstract:  The thalamus has a strong nonphotic influence on sleep, circadian rhythmicity, pineal melatonin production, and secretion. The opening of the sleep gate for nonrapid eye movement sleep is a thalamic function but it is assisted by melatonin which acts by promoting spindle formation. Thus, melatonin has a modulatory influence on sleep onset and maintenance. A remarkable similarity exists between spindle behavior, circadian rhythmicity, and pineal melatonin production throughout life. Together, the thalamic and chronobiological control of sleep leads to a new and improved understanding of the pathophysiology of circadian rhythm sleep disorders and also of the principles of sleep hygiene interventions.

SLEEP PROBLEMS IN CHILDREN WITH SANFILIPPO SYNDROME

Sanfilippo syndrome is a rare degenerative disorder which has severe intellectual and behavioural sequelae, commonly including sleep problems. A parental questionnaire was used to gather information on the sleep patterns of 80 children with Sanfilippo syndrome (mean age 10 years 2 months). The majority were found to have sleep problems (78%). Many also exhibited other distressing and unusual night time behaviours (staying up all night, chewing the bedclothes or crying out suddenly), and a few laughed or sang. Such problems may have been more severe in‐those with Sanfilippo syndrome type B. In four of the families offered individually tailored behaviour‐management advice there was immediate improvement, which was maintained at follow‐up in two cases. These results demonstrate the usefulness of even such a minimal intervention, even in a very difficult population such as this.

Evidence supporting the use of melatonin in short gestation infants

Abstract:  Pineal melatonin regulates circadian rhythms and influences sleep. Melatonin also has protective actions against tissue damage from free‐radicals and other toxins. Evidence is presented that this indoleamine is involved in pre‐ and postnatal brain (and ocular) development and intrauterine growth. In the absence of maternal melatonin, short gestation infants have a prolonged period of melatonin deficiency. Melatonin supplementation, which has a benign safety profile, may help reduce complications in the neonatal period that are associated with short gestation. We believe that this treatment might result in a wide range of health benefits, improved quality of life and reduced healthcare costs.

Autism spectrum disorder in children with and without epilepsy: impact on social functioning and communication.

Aim: To compare developmental and psychological functioning in two groups of children with autism spectrum disorder (asd), one with epilepsy and one without.

Melatonin has membrane receptor-independent hypnotic action on neurons: An hypothesis

Abstract:  Melatonin, which is known to have sleep‐promoting properties, has no morpho‐physiological barriers and readily enters neurons and their subcellular compartments from both the blood and cerebrospinal fluid. It has multiple receptor‐dependent and receptor‐independent functions. Sleep is a neuronal function, and it can no longer be postulated that one or more anatomical structures fully control sleep. Neurons require sleep for metabolically driven restorative purposes, and as a result, the process of sleep is modulated by peripheral and central mechanisms. This is an important finding because it suggests that melatonin should have intracellular sleep‐inducing properties. Based on recent evidence, it is proposed that melatonin induces sleep at the neuronal level independently of its membrane receptors. Thus, the hypnotic action of melatonin and the mechanisms involving the circadian rhythms are separate neurological functions. This is contrary to the presently accepted view.

Sleep Health Issues for Children with FASD: Clinical Considerations

This article describes the combined clinical experience of a multidisciplinary group of professionals on the sleep disturbances of children with fetal alcohol spectrum disorders (FASD) focusing on sleep hygiene interventions. Such practical and comprehensive information is not available in the literature. Severe, persistent sleep difficulties are frequently associated with this condition but few health professionals are familiar with both FASD and sleep disorders. The sleep promotion techniques used for typical children are less suitable for children with FASD who need individually designed interventions. The types, causes, and adverse effects of sleep disorders, the modification of environment, scheduling and preparation for sleep, and sleep health for their caregivers are discussed. It is our hope that parents and also researchers, who are interested in the sleep disorders of children with FASD, will benefit from this presentation and that this discussion will stimulate much needed evidence-based research.