Cerebal Palsy: Etiology

Cerebral palsy sufferers can be classified according to several different organizational schemes. On the most basic level, cerebral palsied people can be classified into four types of cerebral palsy: spastic (the most common form), ataxic (much less common), dyskinetic (or athetoid), and mixed (having a combination of two or more of the types aforementioned). Within those categories, patients may be further classified by how much or which parts of their bodies are affected (hemiplegic, biplegic, quadrapelgic). Cerebral palsy patients can yet still be classified more specifically according to things such as the extent of their gross motor functioning (e.g., ambulatory or not), or the extent of their fine motor functioning.

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Article Abstract
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1. An algorithm for identifying and classifying cerebral palsy in young children

OBJECTIVE: To develop an algorithm on the basis of data obtained with a reliable, standardized neurological examination and report the prevalence of cerebral palsy (CP) subtypes (diparesis, hemiparesis, and quadriparesis) in a cohort of 2-year-old children born before 28 weeks gestation. STUDY DESIGN: We compared children with CP subtypes on extent of handicap and frequency of microcephaly, cognitive impairment, and screening positive for autism. RESULTS: Of the 1056 children examined, 11.4% (120) were given an algorithm-based classification of CP. Of these children, 31% had diparesis, 17% had hemiparesis, and 52% had quadriparesis. Children with quadriparesis were 9 times more likely than children with diparesis (76% versus 8%) to be more highly impaired and 5 times more likely than children with diparesis to be microcephalic (43% versus 8%). They were more than twice as likely as children with diparesis to have a score <70 on the mental scale of the BSID-II (75% versus 34%) and had the highest rate of the Modified Checklist for Autism in Toddlers positivity (76%) compared with children with diparesis (30%) and children without CP (18%). CONCLUSION: We developed an algorithm that classifies CP subtypes, which should permit comparison among studies. Extent of gross motor dysfunction and rates of co-morbidities are highest in children with quadriparesis and lowest in children with diparesis.
[Kuban, K. C., Allred, E. N., O’Shea, M., Paneth, N., Pagano, M., Leviton, A. & ELGAN Study Cerebral Palsy-Algorithm Group. (2008). An algorithm for identifying and classifying cerebral palsy in young children. The Journal of Pediatrics, 153(4), 466-72.]

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2. A classification system for hip disease in cerebral palsy

In population-based studies, hip displacement affects approximately one-third of children with cerebral palsy (CP). Given the extreme range of clinical phenotypes in the CP spectrum, it is unsurprising that hip development varies from normality, to dislocation and degenerative arthritis. Numerous radiological indices are available to measure hip displacement in children with CP; however, there is no grading system for assessing hip status in broad categorical terms. This makes it difficult to audit the incidence of hip displacement, determine the relationship between hip displacement and CP subtypes, assess the outcome of intervention studies, and to communicate hip status between health care professionals. We developed a categorical, radiographic classification of hip morphology based on qualitative indices and measurement of the key continuous variable, the migration percentage of Reimers. One hundred and thirty-four radiographs were reviewed of 52 female and 82 male adolescents with CP who were at, or close to, skeletal maturity (mean age 16y 1mo [SD 1y 4mo] range 14y to 19y 1mo). Twenty-nine were classified at Gross Motor Function Classification System (GMFCS) Level I, 25 at Level II, 27 at Level III, 24 at Level IV, and 29 at Level V. A classification system was developed to encapsulate the full spectrum of hip morphology in CP, with and without intervention.
[Robin, J., Graham, H. K., Baker, R., Selber, P., Simpson, P., Symons, S. & Thomason, P. (2008). A classification system for hip disease in cerebral palsy. Developmental Medicine and Child Neurology [epublication ahead of print].]

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3. Classification of spastic hemiplegic cerebral palsy in children

BACKGROUND: The Winter classification of spastic hemiplegic cerebral palsy (CP) is based on sagittal kinematic data from 3-dimensional gait analysis used in preoperative decision making and postoperative evaluation. Our goal was to investigate how well children with spastic hemiplegic CP can be classified using Winter criteria. Second, we assessed if patients move between groups over time and/or with surgical intervention. METHODS: One hundred twelve patients with spastic hemiplegic CP with a mean age of 8.1 years were included. Medical records and the full gait analysis data were reviewed. Patients were classified using Winter criteria, and an independent sample t test was used to compare groups. RESULTS: We found 26 patients (23%) that could not be classified according to Winter criteria. We defined these patients as group 0. This group showed the least deviation from normal values. Each of the 5 groups in our study showed a higher mean velocity of gait and were younger than any of the groups from the Winter study. In regard to rotational alignment, kinetic variables, and, to a certain extent, muscle tone, group 0 showed the least deviation from normal values; however, most differences were subtle. When reclassifying patients after a mean of 3 years, 8 of 15 had deteriorated in the nonsurgical group, moving to a higher numbered group, whereas 19 of 31 surgically treated patients had improved. CONCLUSIONS: The Winter classification failed to classify 23% (26/112) of our spastic hemiplegic CP children. We suggest that the classification be complemented with the less involved group 0. In this way, all patients can be classified, and thus, treatment plans can be established for all patients. The classification can be divided into ankle, knee, and hip joint involvement. The ankle involvement can be further divided into 3 separate groups. Treating physicians should be aware of the possibility that patients may move into another classification group over time. LEVEL OF EVIDENCE: Diagnostic level 4. See instructions to authors for a complete description of levels of evidence.
[Riad, J., Haglund-Akerlind, Y. & Miller, F. (2007). Classification of spastic hemiplegic cerebral palsy in children. Journal of Pediatric Orthopedics, 27(7), 758-64.]

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

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.
[Eliasson, A. C., Krumlinde-Sundholm, L., Rosblad, B., Beckung, E., Arner, M., Ohrvall, A. M. & Rosenbaum, P. (2006). The Manual Ability Classification System (MACS) for children with cerebral palsy: Scale development and evidence of validity and reliability. Developmental Medicine and Child Neurology, 48(7), 549-54.]

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5. Gross Motor Function Classification System: Impact and utility

In summary, the GMFCS has had, and continues to have, a major effect on the health care of children with CP. The number of citations of the GMFCS has been increasing every year, and the classification system has had good uptake internationally and across the spectrum of health professionals for use in research design and clinical practice by providing a system for clearly communicating about children's gross motor function. The utility of diagnostic labels such as diplegia has been questioned. However, although by definition CP is a disorder of posture and movement, the movement disability is often only one of the neurodevelopmental problems for many children with CP. When a complete description of a child's clinical presentation is required we recommend that the GMFCS be used together with the Surveillance of Cerebral Palsy in Europe classification indicating the type and topography of movement impairment. When appropriate the clinical profile will similarly be enhanced with details of other impairments and disabilities such as epilepsy or sensory, learning, feeding, or emotional disturbance. The observations in this annotation are constrained by the amount of information in the public domain. Although these sources adequately represent the effect of the GMFCS on research design, they are less likely to inform us of how the GMFCS is being used in administration, clinical practice, or education. It is not yet clear whether information is being used for these purposes or in assisting with case load management, as intended by the developers. By its localized nature, such information might remain difficult to gauge. We would therefore be interested to hear from others who are using the system for these or any other purposes.
[Morris, C. & Bartlett, D. (2004). Gross Motor Function Classification System: Impact and utility. Developmental Medicine and Child Neurology, 46(1), 60-5.]

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6. Manual ability classification system for children with cerebral palsy

Manual Ability Classification System provides a systematic method to classify how children with cerebral palsy use their hands when handling objects in daily activities. MACS is a subjective classification system and is based upon self-initiated manual abilities, with a particular emphasis on handling objects in a individual's personal space. MACS is designed for children with cerebral palsy, aged 4 to 18 years.
[Gajewska, E., Sobieska, M. & Samborski, W. (2006). Manual ability classification system for children with cerebral palsy [Polish]. Chirurgia Narzadow Ruchu i Ortopedia Polska, 71(4), 317-9.]

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7. 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.
[Bax, M., Goldstein, M., Rosenbaum, P., Leviton, A., Paneth, N., Dan, B., Jacobsson, B., Damiano, D. & Executive Committee for the Definition and Cerebral Palsy. (2005). Proposed definition and classification of cerebral palsy, April 2005. Developmental Medicine and Child Neurology, 47(8), 571-6.]

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8. Validity and reliability of the guidelines of the surveillance of cerebral palsy in Europe for the classification of cerebral palsy

The validity and reliability of the guidelines of the Surveillance of Cerebral Palsy in Europe (SCPE) for the classification of cerebral palsy (CP) were tested by administering 10 written case vignettes via an interactive web-based link to 30 SCPE partners. There was a moderately good level of agreement (kappa=0.59) about inclusion as a CP case on the SCPE database. Classification by CP subtype differed in two main areas: assigning spastic versus dyskinetic and judgement of distribution of spastic involvement. Agreement on Gross Motor Function Classification System (GMFCS) level was less good than reported in previous studies. Twenty respondents repeated the test 5 months later and there was good repeatability for case inclusion (kappa= 0.72) but considerable variation in assignment of CP subtype and GMFCS level. There is a need for further collaborative work and training to improve harmonization of the classification of CP, including examination, application of SCPE guidelines, and register coding.
[Gainsborough, M., Surman, G., Maestri, G., Colver, A. & Cans, C. (2008). Validity and reliability of the guidelines of the surveillance of cerebral palsy in Europe for the classification of cerebral palsy. Developmental Medicine and Child Neurology, 50(11), 828-31.]

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9. Reliability and validity of the gross motor function classification system for cerebral palsy

PURPOSE: The purposes of this study were to evaluate interrater reliability using videotapes and criterion-related and construct validity of the Gross Motor Function Classification System (GMFCS), aspects of reliability and validity not previously published. METHODS: Two experienced pediatric physical therapists rated 30 videotapes of children with cerebral palsy (CP) or Down syndrome (DS) to test interrater reliability. Criterion-related validity was evaluated by comparing GMFCS levels with tests of motor and nonmotor development. Construct validity was assessed by comparing GMFCS trends over time in children with CP and DS. RESULTS: Interrater reliability was 0.84. Correlation was higher between GMFCS level and tests of motor development than GMFCS level and tests of nonmotor development. The GMFCS level remained relatively stable in children with CP but tended to improve in children with DS. CONCLUSIONS: This study extends reliability and validity of the GMFCS, supporting its use in clinical practice and research.
[Bodkin, A. W., Robinson, C. & Perales, E. P. (2003). Reliability and validity of the gross motor function classification system for cerebral palsy. Pediatric Physical Therapy, 15(4), 247-52.]

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10. Cerebral palsy

In a prospective study it was found that cerebral palsy constituted 0.29% of total paediatric outdoor cases during the study period of 1988-1990. Seventy-five children suffering from cerebral palsy were studied at SN Children Hospital, Allahabad. Male to female ratio was 1:2. There were 42.8% cases who belonged to the age between one and 2 years. Hypertonic cases were predominant over hypotonic cases (3:1). Hemiplegia was commonest (68%) while triplegia was least common (2.7%). There were 24.5% cases who were mentally retarded. Hypertonic cases improved more frequently than hypotonic cases. An early management by paediatric physiotherapy may play an important role in the treatment of cerebral palsy.
[Chandra, R., Srivastava, A. K., Mishra, P. C., Rahman, H. & Sharan, R. (1993). Cerebral palsy. Journal of the Indian Medical Association, 91(9), 236-7.]

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cerebral palsy origins

Heterogeneous Etiologies