Helping Children with Cerebral Palsy Thrive

By Alice K. Gong, MD

Cerebral Palsy (CP), the most common motor disability of childhood, is a group of conditions that affects a child’s ability to move and maintain balance and posture. CP can be caused by abnormal brain development or damage to the developing brain of the fetus and newborn. It literally means that the brain has problems connecting with the muscles of the body. The symptoms are variable, ranging from one who walks awkwardly to a person who needs specialized equipment to ambulate to someone who cannot walk and need lifelong care.

William Osler, the father of modern medicine, first introduced the term “cerebral palsies” to describe a group of varied neurological disturbances in children. Not until 2007 did medicine come to a consensus definition, i.e., a group of permanent disorders of the development of movement and posture, causing activity limitation that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain.1

Typically, CP is not diagnosed until about 18 months of age, because the signs and symptoms are not always obvious.3 There are risk factors such as prematurity, especially those whose neonatal course had been compromised by intraventricular hemorrhages (IVH), periventricular leukodystrophy (PVL), or encephalomalacia that accompanies those born exposed to hypoxic-ischemic encephalopathy (HIE). Without biomarkers, physicians must rely upon delays of key milestones which have a broad range of entry in childhood neurodevelopment. Assessments of posture, reflexes and muscle tone can be difficult at times in infants. There was also the need to rule out other causes of findings and a fear of a false positive. Infants have developing voluntary motor possibilities, making it difficult to determine if dysfunction may be permanent. The discussion of the diagnosis with families is not an easy one. Despite all of these, the need for early diagnosis had been recognized for a long time so as to implement early intervention, especially with early brain plasticity.

Early screening for CP for high-risk newborns has gain clinical traction since the publication of the International Clinical Practice Diagnostic Guidelines.2 Maître et al demonstrated a decreased from 19.5 months to 9.5 months for CP diagnosis using Hammersmith Infant Neurologic Examination (HINE), General Movements Assessment (GMA) and standardized assessment of motor function.4

In 2022, American Academy of Pediatrics (AAP) published guidance for a primary care medical home for children with CP.5 Physicians who care for children should be familiar with the definition, manifestations and management of CP. Risk factors for this motor disability include intrauterine and perinatal infections, IVH, HIE, stroke, brain malformations and certain genetic disorders. These causes are permanent and nonprogressive in that the brain disruption does not worsen. The physical manifestations of postural and motion problems do change over time as the child grows and develops. A common system for describing the gross motor abilities is the Gross Motor Functional Classification System (GMFCS). Although CP is a motor disorder, difficulties in other areas of the nervous system and other comorbidities may occur. Common problems include seizures, cognitive, communication disabilities, gastrointestinal and urinary problems as well as pain. The primary care physician’s responsibility is to integrate and coordinate care across multiple sub-specialists while helping families with resources such as early childhood intervention (ECI) for management and therapies, insurance, transition to education systems, and others to help the child thrive.

Preterm birth is a risk factor to about half of the people with CP. In addition to the disparate social factors that contribute to prematurity, population studies reveal that CP is more common among children who are non-Hispanic, black and in families of lower socioeconomic status. As physicians we should be attuned to implicit biases and barriers to screening, identification, treatment, and support for those whose lives are also impacted by social determinates of health.

Over half of children with CP are born at term and do not have identifiable neonatal risk factors making early diagnosis challenging. Rouabhi et al published a prognostic tool for low-risk term newborns using the Canadian Cerebral Palsy Registry with data from the Alberta Pregnancy Outcomes and Nutritional study for controls.6 In their casecontrol study, they were able to develop a prognostic model using 12 clinical variables that identified 2.4-fold more cases with CP than would have if presented with encephalopathy. This tool can be accessed at: https://jamanetwork.com/journals/jamapediatrics/fullarticle/ 2800550?utm_campaign=articlePDF&utm_medium=articlePDFlink&utm_source=articlePDF&utm_content=jamapediatrics. 2022.5177, Supplement 2. This tool is simple, uses information that is routinely collected, and can be quickly calculated in the author’s Excel sheet that is shared. The screening tool found that a threshold greater than 0.3 had sensitivity of 65% and specificity of 71%. These newborns with normal exams should be referred for evaluation with HINE and standardized evaluation of motor function. Monica et al published a new algorithm for early diagnosis of CP.7

Early active movement and intervention helps infants continue with brain development and refinement of cortical connections and dedicated function. Infants’ developing motor behavior help them discover and interact with their surroundings that will control, grow, and develop the neuromuscular system. Early specific interventions have been shown to expand neuroplasticity and decrease deleterious changes to muscle and bone growth and developmental. A medical home for children is CP should be standard care to optimized brain neuroplasticity, prevent complications and enhance parents’ well-being.

References

1. Rosenbaum P, Paneth N, Leviton A, et al. A report: the definition and classification of cerebral palsy April 2006 [published correction appears in Dev Med Child Neurol. 2007;49(6):480]. Dev Med Child Neurol Suppl. 2007;109:8–14.

2. Novak I, Morgan C, Adde L, Blackman J, Boyd RN, BrunstromHernandez J, Cioni G, Damiano D, Darrah J, Eliasson AC, de Vries LS, Einspieler C, Fahey M, Fehlings D, Ferriero DM, Fetters L, Fiori S, Forssberg H, Gordon AM, Greaves S, Guzzetta A, Hadders-Algra M, Harbourne R, Kakooza-Mwesige A, Karlsson P, Krumlinde-Sundholm L, Latal B, Loughran-Fowlds A, Maitre N, McIntyre S, Noritz G, Pennington L, Romeo DM, Shepherd R, Spittle AJ, Thornton M, Valentine J, Walker K, White R, Badawi N. Early, Accurate Diagnosis and Early Intervention in Cerebral Palsy: Advances in Diagnosis and Treatment. JAMA Pediatr. 2017 Sep 1;171(9):897-907. doi: 10.1001/jamapediatrics.2017.1689. Erratum in: JAMA Pediatr. 2017 Sep 1;171(9):919. PMID: 28715518; PMCID: PMC9641643.

3. te Velde A, Morgan C, Novak I, Tantsis E, Badawi N. Early Diagnosis and Classification of Cerebral Palsy: An Historical Perspective and Barriers to an Early Diagnosis. J Clin Med. 2019 Oct 3;8(10):1599. doi: 10.3390/jcm8101599. PMID: 31623303; PMCID: PMC6832653.

4. Maitre NL, Burton VJ, Duncan AF, et al. Network Implementation of Guideline for Early Detection Decreases Age at Cerebral Palsy Diagnosis. Pediatrics. 2020;145(5):e20192126

5. Noritz G, Davidson L, Steingass K, et al; AAP Council on Children With Disabilities, THE AMERICAN ACADEMY FOR CEREBRAL PALSY AND DEVELOPMENTAL MEDICINE. Providing a Primary Care Medical Home for Children and Youth With Cerebral Palsy. Pediatrics. 2022;150(6):e2022060055

6. Rouabhi A, Husein N, Dewey D, et al. Development of a Bedside Tool to Predict the Diagnosis of Cerebral Palsy in Term-Born Neonates. JAMA Pediatr. Published online January 17, 2023. doi:10.1001/jamapediatrics.2022.5177

7. Monica T, Catherine M, Iona N. New Screening Tool for TermBorn Infants Enables Update to the Clinical Practice Guideline for Early Diagnosis of Cerebral Palsy. JAMA Pediatr. Published online January 17, 2023. doi:10.1001/jamapediatrics.2022.5189

Alice K. Gong, MD is Professor of Pediatrics at UT Health, San Antonio. In that role, Dr. Gong also holds the William and Rita Head Distinguished Chair in Developmental and Environmental Neonatology. She is also the Medical Director of the Premiere Program, a follow through clinic for Neonatal Intensive Care Survivors. Dr. Gong is the president-elect for Texas Pediatric Society. She lives in San Antonio with her husband, Richard and is most proud of her three adult children and four grandchildren.