A 5-year-old male came to clinic with increased spasticity for 2 days. He had underlying cerebral palsy and was wheelchair bound and non-verbal. His mother stated that he had not been crying or tearing but had increased spasticity and couldn’t seem to get comfortable. He had some rhinorrhea, but no cough, fever or rash. He had normal urination and had not had any hard stools using her normal bowel regimen. His mother said that when she bathed him she didn’t see any skin lesions or break down. His seizure pattern was also unchanged.
The past medical history showed he was a former 32-week premature infant who had a complicated neonatal intensive care stay. He was followed by multiple specialties including neurology, developmental disabilities, gastroenterology, and orthopaedics and he received comprehensive therapy services at school and in the community. The review of systems was not contributory.
The pertinent physical exam showed him posturing more than normal during the visit but there were times when he relaxed. His vital signs were normal, but a new weight was not obtained. His oxygen saturation was 97% on room air. HEENT showed clear rhinorrhea and a reddened pharynx without exudate but with palatal and peritonsilar vesicles. His ears were normal. Lungs, heart and abdomen were normal. His palms and soles had several 2-3 mm vesicles. There were no other lesions noted. His neurological examination showed increased spasticity in all extremities, but again he could relax at times. He was non-verbal but possibly reacted more during his HEENT examination.
The diagnosis of hand, foot and mouth disease was made. “It looks like he is having hand, foot and mouth infection. For some kids they don’t have many symptoms and for others they can have a lot. Maybe his mouth is bothering him more than we realize or he just plain doesn’t feel good. Why don’t we try to give him some ibuprofen on a schedule and see what happens. He doesn’t seem to be a lot more affected like he’s not having more seizures or doesn’t seem to have a second infection like an ear infection,” the pediatrician discussed. “Okay. He’s had an orthopaedic appointment to watch his hips and a wheel-chair fitting appointment in 2 days, so I could come back then if he’s not getting better,” the mother replied.
The term, cerebral palsy, or CP has gone through many iterations with the first description in 1861 by W.J. Little who described it as “The condition of spastic rigidity of the limbs of newborn children.” The most recent definition is from Rosenbaun et al. in 2007 which states it is “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. The motor disorders of cerebral palsy are often accompanied by disturbances of sensation, perception, cognition, communication, and behaviour, by epilepsy, and by secondary musculoskeletal problems.”
The incidence is about 2-3/1000 live births in high income countries but higher in low income countries. CP can be a severe motor problem for children and adults with up to 40% of patients unable to walk independently.
CP is really a clinical diagnosis with help from neuroimaging. It basically includes using history, some standardized physical examination ratings (i.e. Prechtl General Movement Assessment and Hammersmith Infant Neurological Examination), and cranial ultrasound or MRI which may show findings consistent with the history and physical examination such as intraventricular hemorrhage, cerebral or cerebellar hemorrhage, posthemorrhagic ventricular dilatation, or cystic periventricular leukomalacia. Diagnosis can be made within the first 6 months but this is usually in infants who have more problems. Patients are usually diagnosed after a year when the clinical trajectory of the patient and their individual problems are more consistent. This does not mean that a patient and family should wait for a diagnosis of CP to receive developmental surveillance and treatment if there are risk factors or concerns about the patient.
Any motoric area can be affected and subtypes are named for the area affected and how it is affected. For example, with predominantly the lower limbs (diplegia), the arm and leg on one side (hemiplegia) or all of the body (quadriplegia), and/or spasticity, dystonia (the two most common movement disorders with CP) and or dyskinesia.
The patient’s abnormal movements vary by the patient and the age. Patients with CP have a wide variety of comorbid problems, with the percentages given from high-income countries.
- Chronic pain – 75%
- Intellectual disability – 49%
- Unable to walk independently – 40%
- Epilepsy – 35%
- Hip subluxation – 28%
- Bladder control problems – 24%
- Non-verbal – 23%
- Sleep problems, pathological – 23%
- Sialorrhea – 22%
- Blind, functionally – 11%
- Feeding tube requirement – 6%
- Hearing impairment, severe 4%
Treatment includes managing the overall patient’s health, preventive services, specific services for their problems and early intervention services. Early intervention services are multidisciplinary services that focus on assisting the patient in all developmental areas including potentially physical therapy, occupational therapy, speech therapy, social interactions and cognitive interactions. Data more strongly supports benefits in cognition but less so for motor development. Other needs may include:
- Spasticity and dystonia – physical therapy, occupational therapy, splinting, medications (i.e. Baclofen, Botulinum toxin A, Clonidine, Diazepam, Gabapentin), or neurosurgical interventions (i.e. selective dorsal rhizotomy, deep brain stimulation)
- Hip dysplacement is common and needs ongoing screening particularly if the child is less mobile or immobile
- Feeding and nutrition – difficulty swallowing, aspiration and drooling can be major problems for patients
- Pain – this is common because of constipation, hip dislocation, spasticity and dystonia
- Sleep disturbance – often because of other problems listed above, and may require behavioral interventions and medication
CP’s cause is not entirely known but appears to be multifactorial especially with accumulations of potential insults. Gestational age is a strong factor. Unfortunately for many of the risk factors, there are no easy or obvious treatments or preventive practices for them.
Rick factors for CP include:
- Genetics – higher risk in siblings particularly twins
- Fetal growth restriction
- Maternal obesity
- Multiple births
- Prolonged labor
- Socioeconomic status, lower
- Prematurity – 14.6% in gestation age 22-27 weeks, 6.2% in 28-31 weeks, 0.7% in 32-36 weeks, 0.11% in term infants
- Low birth weight
- Small for gestational age
- Birth defects
- Birth complications – fetal bradycardia, low Apgar score, delayed time to first breath, breech position are some risk factors
- Stroke, perinatal
- Abnormal neuroimaging findings
- Post natal corticosteroid use
- Early surgery
- Mechanical ventilation
- Magnesium sulfate treatment for mother during pregnancy
- Caffeine therapy for the infant
- Brain or body cooling for the infant
Questions for Further Discussion
1. What are indications for referral to a developmental disabilities specialist?
2. What does non-verbal mean? A review can be found here
3. What is the classification for intraventricular hemorrhage? A review can be found here
- Age: School Ager
To Learn More
To view pediatric review articles on this topic from the past year check PubMed.
Information prescriptions for patients can be found at MedlinePlus for this topic: Cerebral Palsy
To view current news articles on this topic check Google News.
To view images related to this topic check Google Images.
To view videos related to this topic check YouTube Videos.
Little W.J. Transaction of the Obstretrical Society of London. Longmans, Green and Co. 1862: 293-344.
Spittle AJ, Morgan C, Olsen JE, Novak I, Cheong JLY. Early Diagnosis and Treatment of Cerebral Palsy in Children with a History of Preterm Birth. Clin Perinatol. 2018;45(3):409-420. doi:10.1016/j.clp.2018.05.011
Graham D, Paget SP, Wimalasundera N. Current thinking in the health care management of children with cerebral palsy. Med J Aust. 2019;210(3):129-135. doi:10.5694/mja2.12106
Korzeniewski SJ, Slaughter J, Lenski M, Haak P, Paneth N. The complex aetiology of cerebral palsy. Nat Rev Neurol. 2018;14(9):528-543. doi:10.1038/s41582-018-0043-6
Lins LAB, Watkins CJ, Shore BJ. Natural History of Spastic Hip Disease. J Pediatr Orthop. 2019;39(Issue 6, Supplement 1 Suppl 1):S33-S37. doi:10.1097/BPO.0000000000001347
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa