A DETAILED EXPLORATION OF CEREBRAL PALSY – WHAT IS IT? WHERE IS THE BRAIN AFFECTED? HOW DOES IT MANIFEST IN OUR BODY?
Hi there, my name is Mia J Kacen an Accredited Exercise Physiologist in Sydney.
Thank you for taking your time to read our articles on Cerebral Palsy.
At Mia’s Health, we see many extraordinary clients with Cerebral Palsy. Our team specialises in improving a Cerebral Palsy client’s balance, coordination, posture and muscular tone through a customised program which of sports and activities that our client enjoys – whether that is in the pool, gym, park or in the comfort of their own home and/or school. Thanks to Zoom, we now also offer worldwide consultations.
Issue 1 of 4: Tell me – WHAT is Cerebral Palsy?
Cerebral means brain, the cerebrum part of the brain.
Palsy means paralysis.
Cerebral Palsy is usually caused by damage to one or more areas of the brain. Cerebral Palsy (or “CP” as it is commonly known) is a clinical condition, which affects the development of movement, muscle tone and posture causing limitations in activity. This clinical condition is attributed to non-progressive disturbances that occurred in the developing foetal or infant brain. In addition to the motor disorders of CP, disturbances of sensation, perception, cognition, communication, behaviour, epilepsy and secondary musculoskeletal problems are also often present.
But HOW and WHERE is the body affected by Cerebral Palsy?
CP affects movement and posture and the effect is unique for each person that has Cerebral Palsy – it is true that no two experiences are the same!
The Cerebrum is located in the front area of the skull, consisting of two hemispheres, left and right, separated by a fissure. It is responsible for the integration of complex sensory and neural functions and the initiation and coordination of voluntary activity in the body.
Many people with CP may have related conditions (this is known as a co-morbidity which is the presence of a medical condition in addition to the primary condition). Comorbidities include as intellectual disability; epilepsy; problems with vision, hearing, or speech; changes in the spine (such as scoliosis); or joint problems (such as contractures). CP may be associated with pain, bladder problems, hip dysplasia, visual impairment and behavioural issues.
A Cerebral Palsy client may present with:
Neurological signs:
· Muscular weakness.
· Decreased muscular tone.
· Decreased stability and balance.
· Pathological Reflexes.
· Difficulty with swallowing.
Musculoskeletal signs:
· Contractures (that means tight muscles and joints).
Dystonia, a movement disorder that causes uncontrollable tightening of the muscles. This spasm or twist at random times.
· Athetosis, a movement disorder that involves constant twisting and turning of the arms and legs.
So how do exercise physiologists improve mobility, posture and balance?
Mia’s Health specialises in improving a CP client’s balance, coordination, posture and muscular tone in many ways – the most important factor for the team being to work in a way that inspires and motivates our client to move.
We could be working with weights in the gym, balancing on a scooter, riding a bike, shooting basketball hoops, playing netball, learning to swim or focusing on our freestyle stroke correction. You may even see us developing long jump skills, or strengthening our breast stroke kicks and even at an outdoor gym working on our muscle control during a Lat Pull Down.
We have fun working on specific goals together and participating in a variety of activities with modified sports that are individualised for clients to optimise their function. The smiles throughout our sessions from our clients is intrinsically rewarding and further concretes why we do what we do and our approach to achieving goals.
Rain, hail or shine we are working towards a specific goal to improve our client’s quality of life and everyday skills.
Please tell me: who is the team?
When we refer to team we mean Mia’s Health, our client with CP and our client’s parents or carer – a coordinated, consistent and structured group approach is the best formula for achieving goals.
But tell me, what do the parents say?
We kindly asked one of our CP carers to convey their perspective: “Perseverance is what comes to mind when I think of our son who has right hemiplegia. He is five years old and has had approximately 22 000 hours of therapy (give or take a few!) since he was ten weeks old. Therapy has played a vital role in improving his quality of life and ability to function independently. Most recently Mia’s Health Exercise Physiology has been pivotal to identify mobility goals, strengthening exercises and the skills to participate in social sports activities – which ultimately contribute to an overall sense of accomplishment and the ability to confidently participate in activities with other children.
Not to be underestimated is the profound impact that the love, patience and support Mia’s Health (a therapy team) has on person living with a disability and their family, which I believe is the strongest catalyst for progress.”
Now tell me HOW Cerebral Palsy is caused?
Damage can happen to the developing baby brain while the mother is pregnant or during birth. In about 80% of cases, damage occurs during the neonatal period. Although some children have CP without any clear cause, CP is typically caused by:
· Being born early (prematurity); or
· Physical injury to the brain, including bleeding into spaces in the brain (ventricles) that make cerebrospinal fluid; or
· Infections; or
· Lack of oxygen to the brain (anoxic injury).
Please explain WHICH PART OF THE BRAIN CAN BE AFFECTED? And what is the consequence?
There are three areas of the brain which may be affected:
· Cortex: when the Cortex is affected it means that there may be difficulty in moving and muscular stiffness (part of the pyramidal involuntary movements). For example, when the left side of the brain’s motor cortex is affected it can affect the right and lower limb. when the brain is affected bilaterally, the client will have paraplegia or diplegia and have a scissor type gait and stiffness.
· Basal Ganglia: If the Basal Ganglion in the brain is involved there will be dyskinesia with uncontrolled movement.
· Cerebellum: when the Cerebellum in the brain is affected there will be balance and coordination difficulties and gait is once again affected, although in a different way, with a wider gait stance.
Does any of this mean that Cerebral Palsy be cured?
Whilst Cerebral palsy cannot be cured, treatment may help movement, posture and improve muscular tone.
Can Cerebral Palsy worsen over time?
No. CP is non-progressive; however symptoms can change over a person’s lifetime.
Issue 4 of 4: IDENTIFICATION and CLASSIFICATION of Cerebral Palsy
Remind me: WHAT is cerebral palsy?
Cerebral Palsy affects movement and posture and is usually caused by damage to one or more areas of the brain. It is a clinical condition, which affects the development of movement, muscle tone and posture causing limitations in activity (see Issue 1 for a more detailed explanation of the condition).
Please explain: How is Cerebral Palsy identified and classified?
Often classification of CP helps identification of risks of contracture, deformity and outcome interventions. There are several classifications of CP including;
· Topography.
· Movement disorder.
· Function.
Graphic Reference: www.physio-pedia.com
Let’s explore the detail together
Topographical
· Monoplegia: one lower limb is affected – which is uncommon and rare.
· Hemiplegia: one side of the body is affected.
· Diplegia: the lower limbs are affected and potentially fine motor deficiencies will present in the upper limbs.
· Triplegia: usually one arm and both legs are affected; however, it can involve one leg and both arms. Sometimes considered a form of hemiplegia with an overlapping diplegia. This is because the majority of the difficulty is experienced with the legs. If the patient is severely impacted on all three limbs, some medical professionals will categorize the condition as quadriplegia, with less involvement of one arm. Most individuals who suffer with Triplegia exhibit symptoms such as tight muscle tone, poor muscle tone and stiff, jerky movements. They may also have a general lack of balance, involuntary movements and a lack of coordination. As is the case with other types of CP, Triplegia is non-progressive, meaning it will not worsen with time.
· Quadriplegia: all four limbs and trunk are affected. Upper limbs are equally or more affected than lower limbs.
Movement
· Spastic CP: arises from motor cortex damage characterised by resistance to externally imposed movement increases with increasing speed of stretch which varies with direction of joint movement. In this form of CP the muscles appear stiff and movements jerky.
· Dyskinetic forms of CP: are characterised by variable involuntary movements noticeable when a person attempts to move. Some of these involuntary movements include; repetitive movements like twisting (dystonia), slow movements (athetosis), dance like and/or unpredictable movement (chorea).
· Ataxia, “without order”: is the inability to generate normal or expected voluntary movement trajectories, not attributed to weakness or involuntary muscle activity about the affected joints. Characteristically appears as clumsiness or instability that appears jerky, evident in movements such as walking or picking up something.
Now please explain how CP is classified
The Gross Motor Functional Classification System (GMFCS) was developed to create a systematic way to describe the functional abilities and limitations in motor function of children and adolescents with CP.
Another functional classification system is the Cerebral Palsy International Sports and Recreation Association Functional Classification System (CPISRA). Although usage of this system in sports is now limited its functional classification maybe relevant to exercise prescription. This moves from those with most severe spasticity to least and functionally classes 1-4 describe those who are wheelchair users and classes 5-8 as ambulatory.
Upper limb function can be determined using the Manual Ability Classification System (MACS) as it describes how a person with CP uses their hands to handle objects in everyday daily activities.
· Level 1: handles objects easily and successfully.
· Level 2: handles most objects but with somewhat reduced quality and/or speed of achievement.
· Level 3: handles objects with difficulty; needs help to prepare and/or modify activities.
· Level 4: handles a limited selection of easily managed objects in adapted situations. Performs parts of activities with effort and with limited success.
· Level 5: does not handle objects and has severely limited ability to perform even simple actions.
Please tell me: is there a way to classify the effect of CP on communication?
Communication can be assessed using The Communication Function Classification System (CFCS) which classifies patterns of an individual’s communication performance in one of five levels. These levels are:
· Level 1: effective Sender and Receiver with unfamiliar and familiar partners.
· Level 2: effective but slower paced Sender and/or Receiver with unfamiliar and/or familiar partners.
· Level 3: effective Sender and receiver with familiar partners
· Level 4: inconsistent Sender and/or Receiver with familiar partners
· Level 5: seldom Effective Sender and Receiver even with familiar partners
REFERENCE:
1. Patel, D., Neelakantan, M., Pandher, K., & Merrick, J. (2020). Cerebral palsy in children: a clinical overview. Translational Pediatrics, 9(S1), S125-S135. doi: 10.21037/tp.2020.01.01
2. Olney, R. S., Doernberg, N. S., & Yeargin-Allsop, M. (2014). Exclusion of progressive brain disorders of childhood for a cerebral palsy monitoring system: a public health perspective. Journal of registry management, 41(4), 182–189.
3. Johari, R., Maheshwari, S., Thomason, P., & Khot, A. (2016). Musculoskeletal Evaluation of Children with Cerebral Palsy. The Indian Journal Of Pediatrics, 83(11), 1280-1288. doi: 10.1007/s12098-015-1999-5
4. Gorter, J., Verschuren, O., van Riel, L., & Ketelaar, M. (2009). The relationship between spasticity in young children (18 months of age) with cerebral palsy and their gross motor function development. BMC Musculoskeletal Disorders, 10(1). doi: 10.1186/1471-2474-10-108
5. Edwardo Ramos, M., Daniel Moon, D., K. Rao Poduri, D., & Christina Marciniak, M. (2020). Hyperkinetic Movement Disorders – PM&R KnowledgeNow. Retrieved 23 June 2020, from https://now.aapmr.org/hyperkinetic-movement-disorders-including-dystonias-choreas/
6. Alliance, C. (2020). Dyskinetic Cerebral Palsy | Cerebral Palsy Alliance. Retrieved 23 June 2020, from https://cerebralpalsy.org.au/our-research/about-cerebral-palsy/what-is-cerebral-palsy/types-of-cerebral-palsy/dyskinetic-cerebral-palsy/
7. Cerebral Palsy: Hope Through Research | National Institute of Neurological Disorders and Stroke. (2020). Retrieved 23 June 2020, from https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Hope-Through-Research/Cerebral-Palsy-Hope-Through-Research
8. Riebe, D., Ehrman, J., Liguori, G., & Magal, M. (2018). ACSM’s guidelines for exercise testing and prescription. Philadelphia: Wolters Kluwer.
9. Unknown. (2020). Tibial Tubercle Transfer – HSS.edu. Retrieved 23 June 2020, from https://www.hss.edu/conditions_tibial-tubercle-transfer.asp
10. Tibial Tubercle Osteotomy (TTO) Sydney | Knee Patella Alignment Kogarah. (2020). Retrieved 23 June 2020, from http://www.sydneyknee.com.au/tibial-tubercle-osteotomy/?fbclid=IwAR3lMe1yAlpuaddsl-A8XlZQchcHfTzTUo5b40lvRia0i232zfmfmwnfayo
11. Krigger., KW. (2006). Cerebral Palsy: An Overview. American Family Physician. 73(1). 91-100.
O’Shea, TM. (2011). Diagnosis, Treatment and Prevention of Cerebral Palsy in Near-Term/Term Infants. Clinical Obstetrics and Gynecology Journal. 51(4). 816-826.
Royal Children’s Hospital Melbourne
12. Website Accessed September 2020 www.Cdc.com
13. Website Accessed September 2020 www.Armandoh.org
Special thanks to our Mia’s Health team: CP Parent, and Mirren.
Mia J Kacen 2020
Cerebral Palsy
The clinical condition, Cerebral palsy (CP) encompasses a group of disorders affecting the development of movement, muscle tone and posture (Patel, Neelakantan, Pandher & Merrick, 2020) causing activity limitations. This clinical condition is attributed to nonprogressive disturbances that occurred in the developing fetal or infant brain (Olney, Doernbery & Yeargin-Allsop, 2014). In addition to the motor disorders of CP disturbances of sensation, perception, cognition, communication, behaviour, epilepsy and secondary musculoskeletal problems are also often present (Patel, Neelakantan, Pandher & Merrick, 2020).
Often classification of CP helps identification of risks of contracture, deformity and outcome interventions. However, there are several classifications of CP including;
1. Topography
2. Movement disorder
3. Function
(Johari, Maheshwari, Thomason & Khot, 2016).
Classification & Information
1. Topographical
Includes:
Monoplegia (affecting one lower limb – is uncommon),
Hemiplegia (one side of the body),
Diplegia (affects involvement of the lower limbs maybe some fine motor deficiencies will still be present in upper limbs),
Triplegia,
Quadriplegia (all 4 limbs and trunk are affected, upper limbs are equally or more affected than lower limbs)
(Johari, Maheshwari, Thomason & Khot, 2016).
2. Movement
Includes:
Spastic, dyskinetic, ataxic, mixed (Gorter, Verschuren, van Riel & Ketelaar, 2009).
Spastic CP arises from motor cortex damage characterised by resistance to externally imposed movement increases with increasing speed of stretch which varies with direction of joint movement. In this form of CP the muscles appear stiff and movements jerky (Gorter, Verschuren, van Riel & Ketelaar, 2009).
Dyskinetic forms of CP are characterised by variable involuntary movements noticeable when a person attempts to move (Alliance, 2020). Some of these involuntary movements include; repetitive movements like twisting (dystonia), slow movements (athetosis), dance like and/or unpredictable movement (chorea) (Edwardo Ramos, Daniel Moon, K. Rao Poduri & Christina Marciniak, 2020).
Ataxia, “without order”
Is the inability to generate normal or expected voluntary movement trajectories, not attributed to weakness or involuntary muscle activity about the affected joints. Characteristically appears as clumsiness or instability that appears jerky, evident in movements such as walking or picking up something ) (Johari, Maheshwari, Thomason & Khot, 2016).
3. Function
Includes:
Gross motor function, upper limb function, communication.
(Johari, Maheshwari, Thomason & Khot, 2016.
Gross motor functional classification system (GMFCS)
Developed to create a systematic way to describe the functional abilities and limitations in motor function of children and adolescents with CP.
(Johari, Maheshwari, Thomason & Khot, 2016.
Another functional classification system is the Cerebral Palsy International Sports and Recreation Association Functional Classification System (CPISRA).
Although usage of this system in sports is now limited its functional classification maybe relevant to exercise prescription. This moves from those with most severe spasticity to least and functionally classes:
1-4 describe those who are wheelchair users and
Classes 5-8 as ambulatory
(Riebe, Ehrman, Liguori & Magal, 2018).
Upper limb function
This can be described using the manual ability classification system (MACS) as it describes how a person with CP uses their hands to handle objects in everyday daily activities.
Level 1 = handles objects easily and successfully
Level 2 = handles most objects but with somewhat reduced quality and/or speed of achievement.
Level 3 = handles objects with difficulty; needs help to prepare and/or modify activities.
Level 4 = Handles a limited selection of easily managed objects in adapted situations. Performs parts of activities with effort and with limited success.
Level 5 = Does not handle objects and has severely limited ability to perform even simple actions
(Johari, Maheshwari, Thomason & Khot, 2016.)
Communication
Can be assessed using, The Communication Function Classification System (CFCS) which classifies patterns of an individuals communication performance in one of five levels(Johari, Maheshwari, Thomason & Khot, 2016).
Level 1 = Effective Sender and Receiver with unfamiliar and familiar partners.
Level 2 = Effective but slower paced Sender and/or Receiver with unfamiliar and/or familiar partners.
Level 3 = Effective Sender and receiver with familiar partners
Level 4 = Inconsistent Sender and/or Receiver with familiar partners
Level 5 = Seldom Effective Sender and Receiver even with familiar partners
Figure 1 copied from Edwardo Ramos, Daniel Moon, K. Rao Poduri & Christina Marciniak, 2020
Function Scales:
Physical Therapy Treatment in CP
Though there is no known cure for CP, many treatments to improve a person’s capabilities exist including the use of physical therapy.
Physical therapy usually begins in the first few years of a person’s life or soon after diagnosis and includes activities to improve muscle strength, balance and motor skills (“Cerebral Palsy: Hope Through Research | National Institute of Neurological Disorders and Stroke”, 2020)
Passive gentle range of motion exercises and stretches across major joints have been used to prevent or reduce joint contractures.
Low intensity regular progressive resistance exercise involving all major muscle groups to increase muscle strength, improve local muscular endurance, balance, posture control, gait and mobility (Patel, Neelakantan, Pandher & Merrick, 2020).
Functional strength training combined with plyometric exercises and balance training to improve function as plyometric exercises improve muscle power which includes strength and speed. Specifically a 12 week adaptive bungee trampoline program involving bouncing, hopping, heel jumping, jumping with eyes closed, sequence jumps and games such as dodgeballs, has been used to improve lower limb muscle strength (Patel, Neelakantan, Pandher & Merrick, 2020). 3-4 sessions per week over 3-4 months of treadmill training were documented to have led to improved gait velocity, stepping movements and independence of walking.
ACSM exercise prescription recommendations and considerations:
Generally the FITT principle recommendations for general population should be applied to individuals with CP however it is important to note that individuals with CP have decreased physical fitness levels compared with their peers without disability as well as decreased mobility with age and associated high pain and fatigue. Special considerations should be applied when adopting the general population prescription (Riebe, Ehrman, Liguori & Magal, 2018, p 317)
Aerobic exercise programs should start with frequent but short bouts at moderate intensity (40-50% oxygen uptake reserve or RPE 12-13 on our 6-20 scale) with recovery periods of intensity is exceeded. Progressively increase exercise bouts to reach 50-80% VO2R for 20 minutes.
If balance deficits are present- leg ergometry or recumbent stationary cycling for lower extremities or hand cycling for upper extremities can be used as they allow for wider ranges of power output and movements occur in a closed chain and is a minimal risk to injury from loss of balance control.
Recumbent stepping is feasible and safe in individuals with significant motor impairment and can often be performed without significant post exercise pain.
Fatigue
Fatigue is common due to poor economy of movement (because of altered movement control, energy expenditure is high even at low power output levels). Fatigue can deteriorate the voluntary movement patterns of hypertonic muscles. Exercise programs can be more effective when several shorter sessions are conducted, relaxation and stretching routines are included throughout the session and new skills are introduced early in the session.
Reduced muscular strength and endurance
Resistance training increases strength in individuals with CP without adverse effects on muscle tone. Emphasize the role of flexibility training with any resistance training program. Targeting weak muscles groups that oppose hypertonic muscle groups improve the strength of the weak muscle groups and normalise the tone in the opposing hypertonic muscle group through reciprocal inhibition.
Before open kinetic chain strengthening exercises (dumbbells, barbells, free weights) always check impact of primitive reflexes on performance (position of head, trunk, proximal joints of extremities) and if the individual has adequate neuromotor control to exercise with these.
Hypertonic muscles
Should be stretched slowly to their limits throughout the workout program to maintain length. Stretching for 30 improves muscle activation of antagonist group whereas sustained stretching for 30mins effectively, temporarily, reduces spasticity in the muscle being stretched. Ballistic stretches should be avoided.
Susceptible to overuse injuries because of higher incidence of inactivity
One of our incredible clients had the following procedure:
Tibial tubercle transfusion (osteotomy)
Tibial tubercle transfer (bony realignment, osteotomy), is a surgical procedure that involves moving a small portion of bone from the patella tendon and repositioning or transferring it to a location on the tibia. This procedure is designed to correct the underlying problem, instability, arthritis, cartilage defects affecting patellofemoral joint.
Exercises to address referral
VMO strengthening – Thanks to Physi Track, here are some great exercises to help strengthen muscles.
Stretches:
References:
Patel, D., Neelakantan, M., Pandher, K., & Merrick, J. (2020). Cerebral palsy in children: a clinical overview. Translational Pediatrics, 9(S1), S125-S135. doi: 10.21037/tp.2020.01.01
Olney, R. S., Doernberg, N. S., & Yeargin-Allsop, M. (2014). Exclusion of progressive brain disorders of childhood for a cerebral palsy monitoring system: a public health perspective. Journal of registry management, 41(4), 182–189.
Johari, R., Maheshwari, S., Thomason, P., & Khot, A. (2016). Musculoskeletal Evaluation of Children with Cerebral Palsy. The Indian Journal Of Pediatrics, 83(11), 1280-1288. doi: 10.1007/s12098-015-1999-5
Gorter, J., Verschuren, O., van Riel, L., & Ketelaar, M. (2009). The relationship between spasticity in young children (18 months of age) with cerebral palsy and their gross motor function development. BMC Musculoskeletal Disorders, 10(1). doi: 10.1186/1471-2474-10-108
Edwardo Ramos, M., Daniel Moon, D., K. Rao Poduri, D., & Christina Marciniak, M. (2020). Hyperkinetic Movement Disorders – PM&R KnowledgeNow. Retrieved 23 June 2020, from https://now.aapmr.org/hyperkinetic-movement-disorders-including-dystonias-choreas/
Alliance, C. (2020). Dyskinetic Cerebral Palsy | Cerebral Palsy Alliance. Retrieved 23 June 2020, from https://cerebralpalsy.org.au/our-research/about-cerebral-palsy/what-is-cerebral-palsy/types-of-cerebral-palsy/dyskinetic-cerebral-palsy/
Cerebral Palsy: Hope Through Research | National Institute of Neurological Disorders and Stroke. (2020). Retrieved 23 June 2020, from https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Hope-Through-Research/Cerebral-Palsy-Hope-Through-Research
Riebe, D., Ehrman, J., Liguori, G., & Magal, M. (2018). ACSM’s guidelines for exercise testing and prescription. Philadelphia: Wolters Kluwer.
Unknown. (2020). Tibial Tubercle Transfer – HSS.edu. Retrieved 23 June 2020, from https://www.hss.edu/conditions_tibial-tubercle-transfer.asp
Tibial Tubercle Osteotomy (TTO) Sydney | Knee Patella Alignment Kogarah. (2020). Retrieved 23 June 2020, from http://www.sydneyknee.com.au/tibial-tubercle-osteotomy/?fbclid=IwAR3lMe1yAlpuaddsl-A8XlZQchcHfTzTUo5b40lvRia0i232zfmfmwnfayo
