Over my years of practice as a neonatologist and clinical neuroscientist, the most common question I have been asked is, “How bad is it?” quickly followed by “How do I help my child?” Happily, there is now some great data available to help parents sort through conflicting information to find the best possible outcome for their child.
The GMFCS Level and Topography of Cerebral Palsy
Cerebral palsy is the most common motor disability in childhood. The Gross Motor Function Classification Scale (GMFCS) is the commonly used way of classifying the severity of the motor difficulties in sitting and walking.1 In addition to the GMFCS Level, there are 3 major topographies of CP determined by which parts of the body are affected. Based on large population studies, 39% of children with cerebral palsy have hemiplegia affecting one side of the body, 38% have diplegia affecting both legs and 23% have quadriplegia affecting all 4 limbs.2 Each of these topographies can be further sub classified, but first just focusing on which limbs are affected is a good start.
Will my child walk?
Overall, 80% of children with CP are now expected to either walk independently (Level I and II on the GMFCS) or with an aid such as a cane, crutches or walker (Level III).
Adding in the information on the topography makes the information even more useful. It makes a huge difference in the expected outcome. For children with hemiplegia or diplegia, all but 1-2% of children will walk. In contrast, only 23-25% of the children with quadriplegia now walk independently or with an aid.
What about other problems?
There are long lists of co-morbidities or other problems that have been seen in children who also have CP. It is useful information…to a point. As a physician, it is helpful to know about them to make sure that each child is screened properly and treated if needed. I often use the example of hip problems. The incidence a congenital hip problem in newborns is slightly more than 1 in 1,000 infants. Even though it is rare, every newborn is screened for this problem. The goal is early treatment that produces better results.
Later onset hip displacement occurs in roughly 33% (1 in 3) children with cerebral palsy. But the risk for an individual child varies with both the topography and GMFCS severity. The risk is negligible in pure ataxia to near 80% in spastic quadriplegia. It is very rare in children at GMFCS Level 1 and over 60% in children at Level V.
The bottom line for this co-morbidity is simple. Screen all children with Level IV or V spastic quadriplegia every 6 to 12 months and less often for the rest. You should know your child’s risk of this complication and how often a hip x-ray is planned. How often has your child’s hip been screened with an x-ray?
Next week I will discuss children with diplegia at GMFCS Levels I to III. They are the most homogeneous group and the simplest to understand. In 2 weeks, I will do the same for children with hemiplegia at GMFCS Levels I to III. In the following 2 weeks, I will tackle the children at Levels IV and V, first those with spasticity and then those with ataxia and dyskinesia (athetosis and choreoathetosis).
These are the main questions I will discuss.
- What is the brain pathology and what does it mean? What improvements are possible with repair, growth and maturation of the brain?
- What are the risks of other sensory or brain problems and what should be on your checklist to be screened on a regular basis? Most importantly, which professionals are the best sources of help for each problem? The days of the universal expert are over – there is too much information for anyone to know everything.
- What therapies and treatment have the most research back up? These are the evidence based and best practice therapies and interventions where there is sufficient research data to support their use and insurance coverage. This is the basic stuff you really have to know and understand.
What about the rest? Hopefully, if you work through the structure I am presenting in this Cerebral Palsy – GMFCS and Topography series, you will be better able to evaluate other interventions from a more knowledgeable base. Just as there are no more universal experts, best possible outcome means doing the right thing, at the right time and in the right order.
See Also the first blog in this Cerebral Palsy – GMFCS and Topography Series
What Is Your Child’s Label?
- Peter Rosenbaum, et al, “Development of the Gross Motor Function Classification System for cerebral palsy.” Developmental Medicine and Child Neurology, 50 (2008): 249-253.
- Iona Novak, “Evidence-Based Diagnosis, Health Care, and Rehabilitation for Children With Cerebral Palsy”, Journal of Child Neurology, 29 (2014): 1141-1156.