JIGMET
RAFTAN
CEREBRAL PALSY
What is Cerebral Palsy
Cerebral Palsy (CP) is a physical disability affecting movement and posture. The name is derived from Latin; cerebral referring to the brain, whereas palsy refers to paralysis. One might think it is a muscle condition, since it affects muscle control and muscle tone, coordination, balance and reflexes, but in fact it is caused by damage to the nervous system or cerebrum. The cerebrum is the part of the brain where the control of muscles takes place. Its outer layer, the cerebral cortex is associated with consciousness, thought, language and memory. That is why people with cerebral palsy may have other impairments. Their sight, hearing, speech, touch, or intellectual capacity might be afflicted. CP it is thus a neurological condition, which hinders a child’s development.
Cerebral palsy has been defined by Rosenbaum et al., (2007) 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. The motor disorders of cerebral palsy are often accompanied by disturbances of sensation, perception, cognition, communication, and behavior, by epilepsy and by secondary musculoskeletal problems.”
Causes of Cerebral Palsy
Cerebral palsy is typically caused during pregnancy or birth. Risk factors are low birth weight, low placental weight, abnormal fetal position and premature separation of the placenta (Torfs et al., 1990). Though, in many cases the exact trigger remains unknown. In the past, doctors assumed that its main cause resulted from asphyxia (deprivation of oxygen) during birth. However, scientists have discovered that this percentage is really small and the relationship between CP and asphyxia at birth is often not causal (Blair and Stanley, 1988). In many cases brain damage rather occurs during pregnancy as the result of maternal bacterial or viral infections. Also, a fetal stroke, or genetic and environmental factors such as mutations of genes disturb fetal brain development. Finally, brain function is also hindered when (cells of) the nervous system are not properly covered by a protective layer called “myelin”. In some cases CP can be diagnosed soon after birth, whereas with some children it only becomes clear when they do not progress as fast as others and developmental milestones (such as rolling over or walking) are delayed.
Types of Cerebral Palsy
Because diverse parts of the brain can be injured, there are different types of CP, affecting people in many ways. Table 1 gives a short overview of the different types of cerebral palsy.
It is possible that a person has a combination of spastic, ataxic or dyskenetic CP.
Also, the gradation may vary: some people are very spastic, whereas others only a little.
A common classification system used, is the one of Palisano et al. (1997). They have described 5 levels of CP according to a Gross Motor Function Classification System (GMFCS). These levels are based on a child’s functional ability, its need for assistive technology such as crutches or a wheelchair, as well as the quality of movement. Children classified as GMFCS level 1 can walk indoors and outdoors, climbing stairs without limitations. They are able to perform gross motor skills but running, jumping, speed, balance and coordination are limited. Going up to level 5, limitations in mobility increase. The last level, level 5, includes physical impairments that restrict voluntary control of movement and children lack the ability to maintain posture of head and trunk. All areas of motor function are limited and thus the child has no means of independent mobility.
For this website I focus on spastic cerebral palsy, which is the most common type of CP. When I first met Jigmet, the pediatrician diagnosed him with spastic hemiplegia, meaning that one side of his body is affected. Below I will give some additional information on spastic CP, relating to Jigmet’s case.
Spastic cerebral palsy and participation limitations
From table 1 it becomes clear that spastic CP is subdivided into 1) hemiplegia, 2) diplegia and 3) quadriplegia. All these types are characterized by muscle stiffness and tightness caused by disturbance of signals from the brain to the muscle. Someone with diplegia commonly has trouble with fine movements of the hands. Hemiplegia on the other hand only affects one side of the body. The affected side of a child with spastic hemiplegia does not develop properly. To give an example: when I first met Jigmet there was a strong asymmetry between the right and left side of his body. His right leg, foot and arm were about 1 cm shorter than the left and lacking muscle strength. He was not able to independently point his toes towards the shinbone. The asymmetry also showed in his pelvis and trunk. As a result, chest expansion when breathing was hindered. Because of the muscle stiffness and underdevelopment he had a strong limping gait and was barely able to use his right hand. However, his speech and intelligence have remained unaffected. Besides his physical disability he is a smart young man!
Many children cope well with their disability if proper care and treatment is provided. However, they will be hindered with activities of daily living (ADL). Jigmet is coping well with his disability. He can go to school, has plenty of friends and plays the sports he loves. Though, he still suffers from his condition on a daily basis. Table 2 gives a simple overview on the effects of spastic cerebral palsy and its consequences regarding activities and participation in society.
Jigmet loves to play sports, but has trouble holding his cricket bat or catching and kicking a ball. He runs, but due to the limping gait he can’t run too fast and has limited endurance as a result of the muscle weakness. Climbing stairs is also hard. He uses his right hand only for support of the left. For example, he has trouble dressing up, as he can barely tie his laces or zip his fly. Use of his right arm and hand for functional activities is very limited.
Management
As stated on the website of Australia’s Cerebral Palsy Alliance: “There is a positive way forward for every individual with cerebral palsy.” When cerebral palsy is managed properly its impact on an individual’s life can be significantly reduced. Intervention should obviously be tailored to each individual case and depends on the type and severity. “Treatments should be goal-oriented and produce positive outcomes” (Koman et al., 2004). Some treatments aim to prevent further disability, whereas others stimulate acquiring new skills. Koman et al., (2004) mention how combined treatment with means such as occupational therapy, physiotherapy, electrical stimulation, orthotics and casting can be used to progress or maintain strength, flexibility and bone structure and improve or normalize motor development. More specifically, programs such as gait training and strength training help reduce limping, while stretching is used to improve flexibility and limit contractures. Functional exercises promote use of the affected limb. Another important aim of intervention is to provide a basis for future health and wellbeing such that participation in everyday life in enhanced (Barber, 2008).
For more information see also the website of Australia’s Cerebral Palsy Alliance:
https://www.cerebralpalsy.org.au/
References:
Barber, C.E. (2008). A guide to physiotherapy in cerebral palsy. Symposium: Special Needs. Elsevier, Paediarics and Child Health, 18(9), 410-413
Blair, E. & Stanley, F.J. (1988). Intrapartum Asphyxia: A rare cause of cerebral Palsy. The Journal of Pediatrics, 112(4), 515-519.
Koman, A.L., Paterson Smith, B., Shilt, J.S. (2004). Cerebral Palsy. Seminar. The Lancet, 363, 1619-1631.
Palisano, R., Rosenbaum, P., Walter, S., Russell, D., Wood, E., & Galuppi, B. (1997). Development and reliability of a system to classify gross motor function in children with cerebral palsy. Developmental Medicine & Child Neurology, 39, 214-223.
Rosenbaum, P., Paneth, N., Leviton, A., Goldstein, M., Bax, M. (2007). A Report: The Definition and Classification of Cerebral Palsy April 2006. Developmental Medicine & Child Neurology, 49(Suppl. 109), 8–14. Erratum in: Dev Med Child Neurol 2007, 49, 480.
Torfs, C.P., van den Berg, B.J., Oechsli, F.W., Cummins, S. (1990). Prenatal and perinatal factors in the etiology of cerebral palsy. The Journal of Pediatrics, 116(4), 615-619