Lynn Dagenais

Comorbidities in cerebral palsy and their relationship to neurologic subtype and GMFCS level

Objective: Utilizing a population-based registry, the burden of comorbidity was ascertained in a sample of children with cerebral palsy and stratified according to both neurologic subtype and functional capability with respect to gross motor skills. Methods: The Quebec Cerebral Palsy Registry was utilized to identify children over a 4-year birth interval (1999–2002 inclusive) with cerebral palsy. Information on neurologic subtype classified according to the qualitative nature and topographic distribution of the motor impairment on neurologic examination, Gross Motor Function Classification System (GMFCS) categorization of motor skills, and the presence of certain comorbidities (cortical blindness, auditory limitations, nonverbal communication skills, gavage feeding status, and coexisting afebrile seizures in the prior 12 months) was obtained. Results: The frequency of individual comorbidities, their proportional distribution, and mean number of occurrences basically falls into a significant dichotomous distribution. Across the spectrum of comorbidities considered, these comorbidities are relatively infrequently encountered in those with spastic hemiplegic or spastic diplegic variants or ambulatory GMFCS status (levels I–III), while these entities occur at a frequent level for those with spastic quadriplegic, dyskinetic, or ataxic-hypotonic variants or nonambulatory GMFCS status (levels IV and V). Conclusion: The enhanced burdens of comorbidity are unevenly distributed in children with cerebral palsy in a manner that can be associated with either a specific neurologic subtype (spastic quadriplegic, dyskinetic, ataxic-hypotonic) or nonambulatory motor status (Gross Motor Function Classification System levels IV and V). This provides enhanced value to the utilization of these classification approaches.

The relationship of cerebral palsy subtype and functional motor impairment: a population‐based study

Aim  Traditionally, cerebral palsy (CP) had been classified according to the distribution and quality of motor impairment. A standardized functional classification of gross motor skills has recently been validated – the Gross Motor Function Classification System (GMFCS). The relationship between the neurological subtype of CP and GMFCS level remains undefined in CP.

Population-based study of neuroimaging findings in children with cerebral palsy

BACKGROUND Neuroimaging is currently recommended as a standard evaluation in children with cerebral palsy (CP). AIMS Utilizing imaging findings from a population-based registry (REPACQ), the frequency and proportion of cerebral radiologic abnormalities in children CP over a four year birth cohort was investigated. METHODS Descriptions of CT and MRI studies were extracted from the Registry dataset and classified into 10 distinct categories. RESULTS Two hundred and thirteen children had imaging available (119 males, 94 females, mean age of 44 months [SD. ± 14 months] at Registry inscription). Eighty seven percent of participants had documented cerebral abnormalities, the most common of which were periventricular white matter injury (PVWMI) (19.2%), diffuse gray matter injury (14.6%), cerebral vascular accident (CVA) (11.7%), and cerebral malformation (11.3%). Also, 18.8% of participants had non-specific radiologic findings and 13.1% of participants had normal imaging results. Severe CP (i.e. GMFCS Level IV-V) and spastic quadriplegic CP were significantly associated with the neuroimaging findings of gray matter injury, while spastic hemiplegic CP was association with CVA, and dyskinetic and spastic diplegic CP were both associated with normal and non-specific neuroimaging findings. CONCLUSIONS Specific patterns of neuroimaging findings in children with CP were found to be associated with neurological subtype, CP severity (i.e. GMFCS Level) and other categorical variables.

Cross-sectional comparison of periventricular leukomalacia in preterm and term children

Objective: To document and contrast the characteristics of preterm and term-born children with cerebral palsy attributed to underlying radiologic periventricular white matter injury (leukomalacia) (PVWMI/PVL). Methods: A comprehensive cerebral palsy population-based registry (REPACQ) for a 4-year inclusive (1999–2002) birth cohort was systematically searched for all children with radiologic evidence for PVWMI/PVL. Clinical features, neurologic subtype, gross motor functional impairment, and comorbidities were compared in those children born preterm (<37 weeks) and those born at term (≥37 weeks). Results: Of 242 children with cerebral palsy in the registry, 213 had available neuroimaging, in which 41 had PVWMI/PVL: 26 preterm born and 15 term born. Neurologic subtype differed significantly between preterm and term-born children with respect to the frequency of spastic hemiplegia (5/26 vs 8/15; p < 0.05) and spastic diplegia (9/26 vs 2/15; p < 0.05). The groups also differed significantly from a functional perspective (Gross Motor Function Classification System for Cerebral Palsy level I–II; 12/26 vs 12/15; p < 0.05). The comorbidity spectrum was similar between the 2 groups except for the occurrence of cortical blindness in the term-born children (3/15 vs 0/26; p < 0.05). Conclusion: Differences between preterm and term-born children with cerebral palsy with periventricular white matter injury (leukomalacia) suggest that despite a common radiologic pattern, these are different clinicopathologic entities with perhaps a different gestational timing of acquired injury.

Spectrum of Visual Disorders in a Population-Based Cerebral Palsy Cohort

BACKGROUND Children with cerebral palsy are known to be at increased risk for visual impairment. METHODS In a population-based sample drawn from a geographically defined registry, the profile of visual impairment in children with cerebral palsy was investigated. RESULTS Close to half (49.8%; 106/213) had a visual impairment. The majority of these individuals had strabismus (55.7%; 59/106) and a slightly lesser fraction had refractive errors (20.7%; 22/106) or severe visual loss (18.9%; 20/106). The vast majority of children with severe visual loss had spastic quadriplegia (83%; 17/20) or were nonambulatory (i.e., Gross Motor Function Classification Scale IV/V, 80%; 16/20). CONCLUSIONS Knowledge of this profile will assist practitioners to heighten their appreciation of potential visual disturbances in certain subsets of children with cerebral palsy.

Predicting Comorbidities With Neuroimaging in Children With Cerebral Palsy

A population-based registry was used to ascertain whether neuroimaging findings of children with cerebral palsy could predict the occurrence of certain comorbidities. Neuroimaging findings and comorbidities data were extracted from the Quebec Cerebral Palsy Registry for children born in a 4-year birth interval (1999-2002) covering half of the provinces population. Neuroimaging studies were classified into 10 mutually exclusive categories (periventricular white matter injury/leukomalacia, cerebral malformation, cerebral vascular accident, deep gray matter injury, superficial gray matter injury, diffuse gray matter injury, intracranial hemorrhage, infection, nonspecific findings, and normal). Comorbidities studied included cortical blindness, severe auditory impairment, inability to communicate verbally, assisted feeding, and the presence of afebrile seizures in the prior 12 months. Neuroimaging results were available for a total of 213 children. Only deep gray matter injury (defined as signal abnormality or volume loss in subcortical gray matter, n = 9) was significantly (P < 0.05) linked with the occurrence of both the inability to communicate verbally (n = 5, 55.6% vs n = 46, 22.5%, P = 0.04) and with a higher mean number of comorbidities (1.67 vs 0.70, P < 0.01), and therefore with increased burden of comorbidities. These findings may improve our ability to prognosticate the outcome of children with cerebral palsy, enabling targeted early direct interventions.

Determinants of Ambulation in Children With Spastic Quadriplegic Cerebral Palsy: A Population-Based Study

The objective of this study was to identify factors that predict ambulation in spastic quadriplegic cerebral palsy. A 4-year registry-based birth cohort was searched for patients with a diagnosis of spastic quadriplegic cerebral palsy. All patients were then divided in 2 groups: (a) Gross Motor Function Classification System level ≤III (ambulant group) and (b) Gross Motor Function Classification System level ≥IV (nonambulant group). Clinical features were then compared between the 2 groups. A total of 85 children with a diagnosis of spastic quadriplegic cerebral palsy were identified. Of these, 65 and 20 were classified in the ‘‘nonambulant’’ and ‘‘ambulant’’ groups, respectively. The presence of seizures in the first 24 or 72 hours of life and the administration of antibiotics during pregnancy/delivery were all associated with an eventual inability to achieve ambulation. A gestational age ≤27 weeks, birth weight <1000 g, Caucasian mother, and the presence of hyperbilirubinemia were significantly linked with independent ambulation.

Congenital non‐central nervous system malformations in cerebral palsy: a distinct subset?

Aim  The aim of this article was to identify and contrast the subset of children with cerebral palsy (CP) and non‐central nervous system (CNS) congenital malformations with children with CP but no coexisting non‐CNS congenital malformations.

Ethical and legal considerations for Canadian registries.

that will impact the creation and operation of neurological disease registries in Canada. This document is not meant to provide legal or ethical advice. In order to ensure that applicable laws and organizational policies are adhered to in an appropriate manner, it is recommended that legal advisors and relevant organizational representatives be consulted. For registries to succeed, it is critical to proactively consider legal and ethical issues such as consent and privacy. Additional ethical and legal considerations include: the involvement of Aboriginal people and their communities, languages and communication; setting up of biobanks; data management; data ownership; and conducting transparent registry operations. The Belmont Report Ethical Principles and Guidelines for the protection of human subjects of research6 and the Government of Canada Tri-Council Policy Statement: Ethical Conduct for Research Involving Humans (TCPS-2)7 should be referred to for the ethical principles that need to be considered during the creation of disease registries. In addition, Registries for Evaluating Patient Outcomes: A User’s Guide5 produced by The Agency for Healthcare Research and Quality provides useful information. However, this document presents perspectives and reviews legislation particularly relevant to the United States which differ in some respects from Canadian law and research policies and practice. In preparation of this guideline, we examined relevant Canadian and international literature as well as Canadian policy and legislation. We also consulted with Canadian privacy officers and specialists in research ethics. Finally, topic themes and issues were discussed with patients and families in project focus groups.

Online neurological registries.

From the 1University of Calgary, Calgary, Alberta; 2Western University – London Health Sciences Centre, London, Ontario; 3University of Calgary Department of Community Health Sciences, Calgary, Alberta; 4Parkinson Society of Canada, Toronto, Ontario; 5McGill University, Montreal, Quebec; 6University of Manitoba, Winnipeg, Manitoba; 7Institute of Public Health, University of Calgary, Calgary, Alberta; 8Hotchkiss Brain Institute, University of Calgary, Calgary, Alberta, Canada. FINAL REVISIONS SUBMITTED JANUARY 28, 2013. Correspondence to: Lawrence Korngut, Clinical Neurosciences, 480060, 4th Floor Administration, South Health Campus, 4448 Front Street SE, Calgary, Alberta, T3M 1M4, Canada. Email: Lawrence.korngut@albertahealthservices.ca. from those where data are entered directly through an online interface into a local or central data repository, and where data are not accessible to local sites to those where aggregate data are available to all sites and to those that facilitate patient networking. A summary of possible features and some case examples of registries that employ these features are below in Table 9.