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Cerebral palsy

Movement disorders that appear in early childhood

  • 48 min read
  • 10 chapters
Cmdr. John King, a physician embarked aboard the amphibious assault ship USS Kearsarge, assesses the reflexes of a Cerebral Palsy patient at the Arima District Health Facility as part of the humanitar
MC2 Jilleanne Buda · Public domain

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Cerebral palsy is caused by abnormal development or damage to the parts of the brain that control movement, balance, and posture. Most often, the problems occur during pregnancy, but may occur during childbirth or shortly afterwards. Oftentimes the brain will be damaged during prenatal neurodevelopment, and many times the cause is unknown. Risk factors include preterm birth, being a twin, certain infections or exposure to methylmercury during pregnancy, a difficult delivery, and head trauma during the first few years of life. A study published in 2024 suggests that inherited genetic causes play a role in 25% of cases, where formerly it was believed that 2% of cases were genetically determined. In lower- and middle-income countries, cerebral palsy is more prevalent.

Sub-types are classified, based on the specific problems present. For example, those with stiff muscles have spastic cerebral palsy, poor coordination in locomotion have ataxic cerebral palsy, and writhing movements have dyskinetic cerebral palsy. Diagnosis is based on the child's development. Blood tests and medical imaging may be used to rule out other possible causes.

Some causes of CP are preventable through immunization of the mother, and efforts to prevent head injuries in children such as improved safety. There is no known cure for CP, but supportive treatments, medication and surgery may help individuals. This may include physical therapy, occupational therapy and speech therapy. Multiple small studies in China have found improved outcomes with the application of Mouse NGF, and the treatment has been available in China since 2003. Medications such as diazepam, baclofen and botulinum toxin may help relax stiff muscles. Surgery may include lengthening muscles and cutting overly active nerves. Often, external braces and Lycra splints and other assistive technology are helpful with mobility. Some affected children can achieve near normal adult lives with appropriate treatment. While alternative medicines are frequently used, there is no evidence to support their use. Potential treatments are being examined, including stem cell therapy. However, more research is required to determine if it is effective and safe.

Cerebral palsy is the most common movement disorder in children, occurring in about 2.1 per 1,000 live births. It has been documented throughout history, with the first known descriptions occurring in the work of Hippocrates in the 5th century BCE. Extensive study began in the 19th century by William John Little, after whom spastic diplegia was called "Little's disease". William Osler named it "cerebral palsy" from the German zerebrale Kinderlähmung (cerebral child-paralysis). Historical literature and artistic representations referencing symptoms of cerebral palsy indicate that the condition was recognized in antiquity, characterizing it as an "old disease".

Signs and symptoms

Cerebral palsy is defined as "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." While movement problems are the central feature of CP, difficulties with thinking, learning, feeling, communication and behavior often co-occur, with 28% having epilepsy, 58% having difficulties with communication, at least 42% having problems with their vision, and 23–56% having learning disabilities. Muscle contractions in people with cerebral palsy-related high muscle tone are commonly thought to arise from overactivation. Although most people with CP have problems with increased muscle tone, some have low muscle tone instead. High muscle tone can either be due to spasticity or dystonia.

Cerebral palsy is characterized by abnormal muscle tone, reflexes, or motor development and coordination. The neurological lesion is primary and permanent while orthopedic manifestations are secondary to high muscle tone and progressive. In cerebral palsy with high muscle tone, unequal growth between muscle-tendon units and bone eventually leads to bone and joint deformities. At first, deformities are dynamic. Over time, deformities tend to become static, and joint contractures develop. Deformities in general and static deformities in specific (joint contractures) cause increasing gait difficulties in the form of tip-toeing gait, due to tightness of the Achilles tendon, and scissoring gait, due to tightness of the hip adductors. These gait patterns are among the most common gait abnormalities in children with cerebral palsy. However, orthopaedic manifestations of cerebral palsy are diverse. Additionally, crouch gait (also described as knee flexion gait) is prevalent among children who possess the ability to walk. The effects of cerebral palsy fall on a continuum of motor dysfunction, which may range from slight clumsiness at the mild end of the spectrum to impairments so severe that they render coordinated movement virtually impossible at the other end of the spectrum.

Babies born with severe cerebral palsy often have irregular posture; their bodies may be either very floppy or very stiff. Birth defects, such as spinal curvature, a small jawbone, or a small head sometimes occur along with CP. Symptoms may appear or change as a child ages. Babies born with cerebral palsy do not immediately present with symptoms. Classically, CP becomes evident when the baby reaches the developmental stage at 6 to 9 months and is starting to mobilise, where preferential use of limbs, asymmetry, or gross motor developmental delay is seen.

Drooling is common among children with cerebral palsy, which can have a variety of impacts including social rejection, impaired speaking, damage to clothing and books, and mouth infections. It can additionally cause choking.

An average of 55.5% of people with cerebral palsy experience lower urinary tract symptoms, more commonly excessive storage issues than voiding issues. Those with voiding issues and pelvic floor overactivity can deteriorate as adults and experience upper urinary tract dysfunction.

Children with CP may also have sensory processing issues. Adults with cerebral palsy have a higher risk of respiratory failure.

Skeleton

For bones to attain their normal shape and size, they require the stresses from normal musculature. People with cerebral palsy are at risk of low bone mineral density. The shafts of the bones are often thin (gracile), and become thinner during growth. When compared to these thin shafts (diaphyses), the centres (metaphyses) often appear quite enlarged (ballooning). Due to more than normal joint compression caused by muscular imbalances, articular cartilage may atrophy, leading to narrowed joint spaces. Depending on the degree of spasticity, a person with the spastic form of CP may exhibit a variety of angular joint deformities. Because vertebral bodies need vertical gravitational loading forces to develop properly, spasticity and an abnormal gait can hinder proper or full bone and skeletal development. People with CP tend to be shorter in height than the average person because their bones are not allowed to grow to their full potential. Sometimes bones grow to different lengths, so the person may have one leg longer than the other.

Children with CP are prone to low trauma fractures, particularly children with higher Gross Motor Function Classification System (GMFCS) levels who cannot walk. This further affects a child's mobility, strength, and experience of pain, and can lead to missed schooling or child abuse suspicions. These children generally have fractures in the legs, whereas non-affected children mostly fracture their arms in the context of sporting activities.

Hip dislocation and ankle equinus or plantar flexion deformity are the two most common deformities among children with cerebral palsy. Additionally, flexion deformity of the hip and knee can occur. Torsional deformities of long bones such as the femur and tibia are also encountered, among others. Children may develop scoliosis before the age of 10 – estimated prevalence of scoliosis in children with CP is between 21% and 64%. Higher levels of impairment on the GMFCS are associated with scoliosis and hip dislocation. Scoliosis can be corrected with surgery, but CP makes surgical complications more likely, even with improved techniques. Hip migration can be managed by soft tissue procedures such as adductor musculature release. Advanced degrees of hip migration or dislocation can be managed by more extensive procedures such as femoral and pelvic corrective osteotomies. Both soft tissue and bony procedures aim at prevention of hip dislocation in the early phases or aim at hip containment and restoration of anatomy in the late phases of disease. Equinus deformity is managed by conservative methods especially when dynamic. If fixed/static deformity ensues surgery may become mandatory.

Growth spurts during puberty can make walking more difficult for people with CP and high muscle tone.

Eating

Due to sensory and motor impairments, those with CP may have difficulty preparing food, holding utensils, or chewing and swallowing. An infant with CP may not be able to suck, swallow or chew. Gastro-oesophageal reflux is common in children with CP. Children with CP may have too little or too much sensitivity around and in the mouth. Poor balance when sitting, lack of control of the head, mouth, and trunk, not being able to bend the hips enough to allow the arms to stretch forward to reach and grasp food or utensils, and lack of hand-eye coordination can make self-feeding difficult. Feeding difficulties are related to higher GMFCS levels. Dental problems can also contribute to difficulties with eating. Pneumonia is also common where eating difficulties exist, caused by undetected aspiration of food or liquids. Fine finger dexterity, like that needed for picking up a utensil, is more frequently impaired than gross manual dexterity, like that needed for spooning food onto a plate. Grip strength impairments are less common.

Children with severe cerebral palsy, particularly with oropharyngeal issues, are at risk of undernutrition. Triceps skin fold tests have been found to be a very reliable indicator of malnutrition in children with cerebral palsy. Due to challenges in feeding, evidence has shown that children with cerebral palsy are at a greater risk of malnutrition.

Language

Speech and language disorders are common in people with cerebral palsy. The incidence of dysarthria is estimated to range from 31% to 88%, and around a quarter of people with CP are non-verbal. Speech problems are associated with poor respiratory control, laryngeal and velopharyngeal dysfunction, and oral articulation disorders that are due to restricted movement in the oral-facial muscles. There are three major types of dysarthria in cerebral palsy: spastic, dyskinetic (athetotic), and ataxic.

Early use of augmentative and alternative communication systems may assist the child in developing spoken language skills. Overall language delay is associated with problems of cognition, deafness, and learned helplessness. Children with cerebral palsy are at risk of learned helplessness and becoming passive communicators, initiating little communication. Early intervention with this clientele, and their parents, often targets situations in which children communicate with others so that they learn that they can control people and objects in their environment through this communication, including making choices, decisions, and mistakes.

Pain and sleep

Pain is common and may result from the inherent deficits associated with the condition, along with the numerous procedures children typically face. When children with cerebral palsy are in pain, they experience worse muscle spasms. Pain is associated with tight or shortened muscles, abnormal posture, stiff joints, unsuitable orthosis, etc. Hip migration or dislocation is a recognizable source of pain in CP children and especially in the adolescent population. Nevertheless, the adequate scoring and scaling of pain in CP children remains challenging. Pain in CP has a number of different causes, and different pains respond to different treatments.

There is also a high likelihood of chronic sleep disorders secondary to both physical and environmental factors. Children with cerebral palsy have significantly higher rates of sleep disturbance than typically developing children. Babies with cerebral palsy who have stiffness issues might cry more and be harder to put to sleep than non-disabled babies, or "floppy" babies might be lethargic. Chronic pain is under-recognized in children with cerebral palsy, even though three out of four children with cerebral palsy experience pain. Adults with CP also experience more pain than the general population.

Associated disorders

Associated disorders include intellectual disabilities, seizures, muscle contractures, abnormal gait, osteoporosis, communication disorders, malnutrition, sleep disorders, and mental health disorders, such as depression and anxiety. Epilepsy is often found in the child before they are 1 year old, or also before they are four or five. In addition to these, functional gastrointestinal abnormalities contributing to bowel obstruction, vomiting, and constipation may also arise. Adults with cerebral palsy may have ischemic heart disease, cerebrovascular disease, cancer, and trauma more often. Obesity in people with cerebral palsy or a more severe Gross Motor Function Classification System assessment in particular are considered risk factors for multimorbidity. The risk factors for cerebral palsy and the comorbidity rates (co-occurring complications) are much higher in low-resource or low- and middle-income countries compared to high-income countries. This is mostly due to differences in the quality of healthcare services for example in the obstetric and neonatal care, which are generally better in high-income countries. Other medical issues can be mistaken for being symptoms of cerebral palsy, and so may not be treated correctly.

Related conditions can include apraxia, sensory impairments, urinary incontinence, fecal incontinence, or behavioural disorders.

Seizure management is more difficult in people with CP as seizures often last longer. Epilepsy and asthma are common co-occurring diseases in adults with CP. The associated disorders that co-occur with cerebral palsy may be more disabling than the motor function problems.

Managing respiratory illnesses in children with severe CP is considered complex due to the need to manage oropharyngeal dysphagia of both food/drink and saliva, gastroesophageal reflux, motor disorders, upper airway obstruction during sleep, malnutrition, among other factors.

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