For a variety of reasons, the definition and the classification 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.

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

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.

/pdf/proposed-definition-and-classification-of-cerebral-palsy-3khmsd9sde.pdf

Proposed definition and classification of cerebral palsy, April 2005.

Aneuploidy (trisomy or monosomy) is the most commonly identified chromosome abnormality in humans, occurring in at least 5% of all clinically recognized pregnancies. Most aneuploid conceptuses perish in utero, which makes this the leading genetic cause of pregnancy loss. However, some aneuploid fetuses survive to term and, as a class, aneuploidy is the most common known cause of mental retardation. Despite the devastating clinical consequences of aneuploidy, relatively little is known of how trisomy and monosomy originate in humans. However, recent molecular and cytogenetic approaches are now beginning to shed light on the non-disjunctional processes that lead to aneuploidy.

To err (meiotically) is human: the genesis of human aneuploidy

Poor children confront widespread environmental inequities. Compared with their economically advantaged counterparts, they are exposed to more family turmoil, violence, separation from their families, instability, and chaotic households. Poor children experience less social support, and their parents are less responsive and more authoritarian. Low-income children are read to relatively infrequently, watch more TV, and have less access to books and computers. Low-income parents are less involved in their children's school activities. The air and water poor children consume are more polluted. Their homes are more crowded, noisier, and of lower quality. Low-income neighborhoods are more dangerous, offer poorer municipal services, and suffer greater physical deterioration. Predominantly low-income schools and day care are inferior. The accumulation of multiple environmental risks rather than singular risk exposure may be an especially pathogenic aspect of childhood poverty.

/pdf/the-environment-of-childhood-poverty-26w5pgnxr6.pdf

The environment of childhood poverty.

The Manual Ability Classification System (MACS) has been developed to classify how children with cerebral palsy (CP) use their hands when handling objects in daily activities. The classification is designed to reflect the child's typical manual performance, not the child's maximal capacity. It classifies the collaborative use of both hands together. Validation was based on the experience within an expert group, a review of the literature, and thorough analysis of children across a spectrum of function. Discussions continued until consensus was reached, first about the constructs, then about the content of the five levels. Parents and therapists were interviewed about the content and the description of levels. Reliability was tested between pairs of therapists for 168 children (70 females, 98 males; with hemiplegia [n=52], diplegia [n=70], tetraplegia [n=19], ataxia [n=6], dyskinesia [n=19], and unspecified CP [n=2]) between 4 and 18 years and between 25 parents and their children's therapists. The results demonstrated that MACS has good validity and reliability. The intraclass correlation coefficient between therapists was 0.97 (95% confidence interval 0.96-0.98), and between parents and therapist was 0.96 (0.89-0.98), indicating excellent agreement.

https://onlinelibrary.wiley.com/doi/pdf/10.1111/j.1469-8749.2006.tb01313.x

The Manual Ability Classification System (MACS) for children with cerebral palsy: scale development and evidence of validity and reliability

OVER the past decade, regular international meetings devoted to the state of the art in the epidemiology of cerebral palsy have been held: in California in 1987, in Cambridge in 1989 and in Brioni, Yugoslavia, in 1990. Among the longstanding problems considered have been those of case definition, case-finding, reported changes in prevalence, the bases on which distinctions between different clinical types and meaningful classification can be achieved, and the crucial question of the timing and nature of the original insult. Reports of the meetings have not been published, however, so this annotation is intended to document some of the recurrent questions that have been considered.

Cerebral Palsy Epidemiology: Where are We Now and Where are We Going?

From birth to seven

https://www.thelancet.com/pdfs/journals/lancet/PIIS0140-6736(05)73917-9.pdf

Adult cardiovascular risk factors in premature babies

Objectives: To revise the estimates of maternal age specific live birth prevalence of Down's syndrome in the absence of antenatal screening and selective termination using newly available data. Set...

http://prenatest.com.tr/pdf/JMedScreen-2002-Morris-2-6.pdf

Revised estimates of the maternal age specific live birth prevalence of Down's syndrome.

Objectives To describe trends in the numbers of Down’s syndrome live births and antenatal diagnoses in England and Wales from 1989 to 2008.

Design and setting The National Down Syndrome Cytogenetic Register holds details of 26488 antenatal and postnatal diagnoses of Down’s syndrome made by all cytogenetic laboratories in England and Wales since 1989.

Interventions Antenatal screening, diagnosis, and subsequent termination of Down’s syndrome pregnancies.

Main outcome measures The number of live births with Down’s syndrome.

Results Despite the number of births in 1989/90 being similar to that in 2007/8, antenatal and postnatal diagnoses of Down’s syndrome increased by 71% (from 1075 in 1989/90 to 1843 in 2007/8). However, numbers of live births with Down’s syndrome fell by 1% (752 to 743; 1.10 to 1.08 per 1000 births) because of antenatal screening and subsequent terminations. In the absence of such screening, numbers of live births with Down’s syndrome would have increased by 48% (from 959 to 1422), since couples are starting families at an older age. Among mothers aged 37 years and older, a consistent 70% of affected pregnancies were diagnosed antenatally. In younger mothers, the proportions of pregnancies diagnosed antenatally increased from 3% to 43% owing to improvements in the availability and sensitivity of screening tests.

Conclusions Since 1989, expansion of and improvements in antenatal screening have offset an increase in Down’s syndrome resulting from rising maternal age. The proportion of antenatal diagnoses has increased most strikingly in younger women, whereas that in older women has stayed relatively constant. This trend suggests that, even with future improvements in screening, a large number of births with Down’s syndrome are still likely, and that monitoring of the numbers of babies born with Down’s syndrome is essential to ensure adequate provision for their needs.

https://www.bmj.com/content/bmj/339/bmj.b3794.full.pdf

Trends in Down’s syndrome live births and antenatal diagnoses in England and Wales from 1989 to 2008: analysis of data from the National Down Syndrome Cytogenetic Register

Eva Alberman

Papers

Cerebral Palsy Epidemiology: Where are We Now and Where are We Going?

From birth to seven

Adult cardiovascular risk factors in premature babies

Revised estimates of the maternal age specific live birth prevalence of Down's syndrome.

Trends in Down’s syndrome live births and antenatal diagnoses in England and Wales from 1989 to 2008: analysis of data from the National Down Syndrome Cytogenetic Register