Cerebral Palsy Makrina Morozowski Introduce Personal Experience Learnt lots Questions throughout at and at the end

Outline Definition Causation Associated Problems Diagnostic Criteria Multidisciplinary Team Treatments Baclofen Botox Diagnostic Criteria includes Gross motor functional classification system Functional Assessment Tools Gross Motor Functional Classification Tools Ashworth Spasticity measures Gillette Functional Assessment Questionnaire Gait Analysis Common Treatments include Baclofen and Botox

What Is Cerebral Palsy (CP)? Perinatal Males Not genetic Life long Is not communicable 2/1000 Cerebral palsy, which I will not relate to as CP throughout this lecture Cerebral concerns the brain, and PALSY means paralysis or inability to move As a whole Cerebral Palsy undertakes a big role, and as we will see is an umbrella term and encompasses range symptoms and disorder types. The damage that causes CP cannot be cured, but it will not spread throughout the body. I will describe how spasticity can change throughout daily life. The hall mark limitation is mobility Most often develop in the fetal period, but can develop within the first few years of life. PERINATAL More males than females CP is not Genetic All children grow and develop into bigger kids. The growth may make function more difficult due to the extra weight and length of limbs growing. Muscles of children with CP are tighter than the average kids the same age, therefore as the bones grow, the muscles are stretched further and get tighter. LIFE LONG CP is not a disorder you can catch from having a cold. The prevalence rates in countries like Canada and The United States of America range 2 in 1000 births. In poorer countries were health care is lower, it increases to 1 in every 200 live births.

Causation Asphyxia, lack of oxygen Low birth weight Prematurity Trauma or injury Stroke Low birth weight Prematurity Maternal infection during pregnancy Blood incompatibility It has been thought for a great period of time that loss of oxygen during the birthing process was the primary cause of Cerebral Palsy. Many people thing or have thought that CP is a direct result of no oxygen in the brain. Asphyxia, has now been found to only cause 10-20% of CP Birthing trauma, injury, or stroke before, during, or right after the birth process can lead to CP Ulitmately those causes are antedenents to a lack of oxygen Born before 37 weeks or less than 5.5 pounds puts baby at higher risk for breathing complication Prematurity is being norm before 28-32 weeks, or less than 1500grams Maternal infections such as German measles, shingles, and flu can potentially cause CP Blood types include A, B, and O, in addition to a Rhesus factor, either positive or negative. Incompatibility occurs when moms blood and baby’s blood is not the same. During pregnancy, moms blood can cross placenta, and if different, moms blood develops antibodies that can attack the babies blood which can lead to problems and cause Jaundice.

Co-morbid Disorders/ Associated Problems Intellectual capacity or mental impairment Speech difficulties Epilepsy and seizures Growth problems There are a number of co morbid disorders and associated problems that can associate with the development of Cerebral Palsy Intellectual ability can range from having no impact to needing some assistance throughout their childhood, to having no processing capabilities Can be a learning disability or a form of mental retardation. Learning disabilities is the need some assistance, and mental retardation were a person functions at lower than normal intelligence. One half of children with CP will have an intellectual impairment. It is important to consider that muscles in the face area can be affected so it may take longer for the child to respond, it is not that they’re slow, its that their muscles make it harder to speak. 40% of people with CP can have some type of mental impairment There can be hearing difficulties, hard to hear, or difficulty in locating sounds. Some children with CP can have dysarthria, its hard for to control and coordinate their mouths. 30% of people have problems associated with hearing, speech, and language. Epilepsy, also known as seizures, is abnormal electric impulses in the brain that cause involuntary muscle contraction that can range from slight to severe spasms in which a child loses consciousness. Epilepsy can affect up to half of the children with cerebral palsy. ‘Failure to thrive’ can occur when children fall behind in development, and can occur if premature and low weight Can be due to feeding and growth difficulties. They can be secondary to a variety of problems such as: Swallowing mechanism,, hyperactive gage reflex, weak suck, hyptonia, poor coordination. Lead to poor nutrition Many of these impairments range from being present, in different ranges, or not at all. The environment in which cerebral develops in can impact other factors of development

Diagnostic Criteria Reaching milestones Primitive reflexes Physiological classification Geographical classification There are normal developmental milestones such as reaching (3-4 months), sitting (6-7months), and walking (10-14 months) Either slower to develop or do not develop. All infants are born with primitive reflexes that are present in early life and thought to be controlled by the primitive regions of the nervous system. Examples include suckling and hand grasping. These reflexes normally disappear around 1 year, and for some children with CP persist beyond infancy. These reflexes can precede postural reactions. There are key things that the doctor may look at when suspecting a diagnosis of CP. - Physiological, what type of movements is it (ABSENT OR PRESENT) -Geographic, where is the disorder occurring,

Physiological Classification Spastic Athetoid /Dyskinetic Ataxic Hypotonic Mixed There are 4 main types of CP that occur Tight/stiff muscles are common in cerebral palsy. The muscles can impair how a child walks, sits, lies down. Occurs in 70-80% of people with CP The motor areas and pathways of the cortex are damaged. Spastic diplegia affects either both arms or both legs, most common in legs Spastic Hemiplegia, is one half of the body Spastic quadriplegia, affects all 4 limbs Athetiod is unwanted or uncontrolled movement. Muscles can either be stiff or floppy. There is involuntary and controlled movement Children often have a hard time holding themselves up Affects 10-20% of people with CP The basal ganglia is affected Ataxic Cerebral Palsy is rare Shakiness, or tremor like movement is found in ataxic like CP. As a result, muscles may come stiff to overcome this. Difficulty with balance, coordination, and depth perception Affects 5-10%. Commonly has trouble with precise movements, such as writing The cerebellum is affected Hypotonic is which a child has low tone. Can also be from cerebellum. Mixed cases, can include features of two or more.

Geographical Classification Cerebral Palsy can affect different parts of the body. This all depends on what was damaged. Monoplegia, is very rare, and it is when one extremity is involved, usually a lower limb Diplegia, occurs in 50% of cases, and the lower extremities are more involved than upper extremities. Motor and sensory function is fine in the upper body Motor Cortex, the leg portion of it is affected Hemiplegia, occurs in 30%, this is when the arm and the leg on the same side are affected, typically arm is more affected than leg The part of the brain that is affected, it is the opposite side that is affected (Right then left) Quadriplegia, smaller percent, all extremities are involved equally, normal head and neck control Motor Cortex, the arms and legs of the humunculi are affected Mix words. Spastic diplegic CP It is important to note that CP does not get worse or spread to different areas. Tightness and spasticity can get worse if a person fails to stretch and maintain flexibility

Multi-Disciplinary Team Pediatrician Orthopedic Doctor Neurologist Physiotherapist Occupational therapist Speech therapist Orthotist Social worker Psychologist The physician can be the leader of the treatment a team and works to pull together professional assistance Orthopedic doctors specialize in treating bones, muscles, tendons, and other skeletal problems. Neurologist doctors specialize in the brain and the nervous system Physio-therapists work with children with CP to maintain posture and movement Exercise and Stretches and strengthening Occupational therapists work on children's function, visual perception, and fine motor control Daily Living Skills Speech therapists work on eating, drinking, and communication Orthotists make braces suitable for each individual to aid in maintaining neutral foot stance, keeping the ankle stretched and at a good position. Social worker, helps patients and their families find financial, educational, and medical assistance in their community Advocacy for the child and their families Ex. Driving Lisense Psychologist typically helps patients and their families cope with anxieties, pressures, and demands that are placed on the individuals and families. Boundaries between specialties are blurred and often overlap

Baclofen Botox TREATMENTS

What Is Baclofen? Muscle relaxant Action potential GABA B agonist Oral baclofen Intrathecal baclofen Side Effects Baclofen is a type of treatment used to help children with cerebal palsy. It is a muscle relaxant that is primarily used to reduce spasticity. Baclofen is a GABA B agonist, its role in action potentials is to keep the cell negative so they cannot happen. If the cell cannot fire, no action potentials occur. If the cells cannot communicate with the muscles, they stay relaxed So a normal action potential looks like this. As the cell depolarizes, sodium gates open and come into the cell, that makes the cell more positive. Enough sodium causes the cell to spike, and reach action potential Following AP, the cell repolarizes, and allows potassium to flows out. Potassium gates take longer to close, so typically to more potassium gets out, making the cell hyperpolarized (more negative). Often it is first tested by oral medication, but it does affect all parts of the body including hands and brain. Intrathecal baclofen can be surgically inputted so the medicine goes in the spine and directly affects muscles below the spinal cord Pump is placed in spine around Thoracic levels5-7. The pump is programmable, so a person can find the right amount of dosage that works for the spastic problems that are occuring. Bolus or Continuous The reservoir sits in the lower stomach and can be refilled when needed. 2-6 months

Baclofen In Action Brochard, Remy-Neris, Fillipetti, and Bussel (2008). Less supportive Hoving et al., (2009). Control and pump Spasticity Brochard, Remy-Neris, Fillipetti, and Bussel (2008). Evaluated intrathecal baclofen in children that could walk. 12 had spastic dip CP and 8 had spastic quad 7 children changes to a less supportive walking device Hoving et al., (2009). Ashworth is a scale which measures tightness and spasticity. Ranges 0-4. 0 there is no increase in muscle. 4 completely rigid in flexion or extension The participants were randomized in the continuous infusion of intrathecal baclofen (CITB) group- 9 and the control group-8 Spasticity reduced in the Baclofen group

What Is Botulinum Toxin Type A (Botox)? Neurotoxin Acetylcholine (ACH) Administered Common Effects Another common treatment for CP is Botox. Botox is a sterile, purified neurotoxin and is used to treat disorders involving rigidity and spasticity It works by blocking the nerve-muscle junction by inhibiting the release of acetylcholine Results in weakening of the muscle as it causes local paralysis by chemical denervetating the muscle Can be inserted, by needle into tight muscles, commonly gastrocnemius, soleus, hamstrings and adductors but also into salivary glands to reduce drooling Common side Effects include Pain, tenderness, bruising at injection site. Other effects that can be found is weakness, chest pain, and skin rash. Positive effects such as muscle relaxation, greater range of motion and flexibility

How Botox Works Cell communication Botox SNARE complex Nerve impulses SNAP-25 Nerve impulses Does everyone remember how cells communicate through presynaptic and post synaptic communication? Neurons send information to each other. Presynaptic terminals send pieces of information to the post synaptic cleft. This communicates to the muscle to contract to move. In CP, there are more signals that get sent to the muscle. This leads to more spasticity and spasms. The goal of injected botox is to reduce spasticity. Botox remains at the injection site and targets nerve endings. One part of the botox chain breaks part of the SNARE complex off, SNAP 25. This causes no ACH to bind, and no activation of the muscle. Therefore, no muscle contractions. In essence, the brain still sends messages to the muscle but the muscle does not respond or react to the impulse

Summary Defined CP Causation Co-morbid Disorders Diagnostic Criteria Team of Professionals Assessment Tools Treatments We defined CP as paralysis or inability move due to injury of the brain. We discussed events that occur perinattaly that can lead to CP such as asphyxia and prematurity I pointed out disorders that commonly can occur with CP such as seizure and mental impairment The Diagnostic Criteria was reviewed, I explained where the disorder originates from, what causes it. A team of professionals work together to create a better QOL and improve ability in each child. An orthopedic doctor/surgeon and physiotherapist are the most common We discussed assessment tools such as the gross motor functional classification scale and gait analysis I then explained common treatments such as Baclofen and botox. Diagnostic Criteria can include Milestones Reflexes Geographical and Physiological Classification GMFCS and we discussed the Ashworth Scale We reviewed what Gait Analysis is Disorders associated with CP such as epilepsy and mental impairment Risk factors such as low birth weight and low oxygen Professionals involved can be numerous but typicall involve an orthopedic physician as well as a physiotherapist We then discussed treatments to help increase the QOL and focus on the ability of children and discussed Baclofen and Botox.

Questions Please feel free to ask my any questions regarding CP

References Agarwal, A., & Verma, I. (2012). Cerebral palsy in children: An overview. Journal of Clinical Orthopaedics and Trauma, 3, 77-81. doi: 10.1016/j.jcot.2012.09.001 Bandholm, T., Jensen, B, R., Neilsen, L, M., Rasmussen, H., Bencke, J., Curtic, D., … Sonne-Holm, S. (2012). Neurorehabilitation with versus without resistance training after botulinum toxin treatment in children with cerebral palsy: a randomized pilot study. NeuroRehabilitation, 30, 277-286. doi: 10.3233/NRE-2012- 0756 Brochard, S., Remy-Neris, O., Filipetti, F., & Bussel, B. (2008). Intrathecal baclofen infusion for ambulant children with cerebral palsy. Pediatric Neurology, 40(4), 265-270. doi: 10.1016/j.peidatrneurol.2008.10.020 Haslaas, Y. (n.d.). Xeomin injections- Are you a candidate? [On-line]. Retrieved from http://www.facialplasticsurgery.net/xeomin-injections.htm Hinchcliffe, A. (2007). Children with cerebral palsy. A manual for therapists, parents and community workers. (2nd.ed.). What is cerebral Palsy and how does it affect children? (pp.13-24). Thousans Oaks, California: Sage Publications Inc. Hoon, A, H., & Tolley, F. (2013). Cerebral Palsy. In Batchaw, M, L., Roizen, N, J., & Lotrecchiano, G, R. (Eds.), Children with Disabilities (7 ed.). (pp. 423- 450). Baltimore, Maryland: Paul H. Brookes Publishing Co. Hoving, M, A., Van Raak, E, P, M., Spincermaille, G, H, J, J., Palmans, L, J., Becher, J, G., & Vles, J, S, H. (2009). Efficacy of intrathecal baclofen therapy in children with intractable spastic cerebral palsy: a randomized controlled trial. European Journal of Peadiatric Neurology, 13, 240-246. doi: 10.1016/j.ejpn.2008.04.013

References http://www.byhealth.com/cerebral-palsy-cp http://www.bcchildrens.ca/Services/SurgeryAndSurgSuites/OrthopaedicSurgery/Orthopaed ic+Cerebral+Palsy+Clinic/cerebral+palsy.htm http://suppversity.blogspot.ca/2011/08/suppversity-emg-series-gluteaus-maximus.html http://www.rnoh.nhs.uk/clinical-services/rehabilitation-and-therapy/gait-analysis-laboratory http://www.skincarebydesign.com/Reference/BotoxComparePage.shtmlhttp://multiple- sclerosis-research.blogspot.ca/2013/02/research-spasticity-neuros-need-to.html http://www.fortmacleodgazette.com/2012/students-share-their-story-of-overcoming- challenges/ http://www.mushymamma.com/2011/11/remembering-my-twin-boys-during-prematurity- awareness-month/ http://www.cmpa- acpm.ca/cmpapd04/docs/ela/goodpracticesguide/pages/communication/Team_Communicati on/tools_and_techniques-e.html http://www.interactivemetronome.com/IMW/IMProvider/eNews/Provider_E- News_FebMar_10.htm

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