CLINICAL Cerebral Palsy: an Overview REVIEW Indexing Metadata/Description › Title/condition: Cerebral Palsy: an Overview › Synonyms: Palsy, cerebral: an overview › Anatomical location/body part affected: Central nervous and musculoskeletal systems › Area(s) of specialty: Neurological rehabilitation, orthopedic rehabilitation, pediatric rehabilitation › Description • Cerebral palsy (CP) is an umbrella term that refers to various neurological disorders caused by a static (i.e., nonprogressive) lesion in the developing brain, all of which result in abnormal postural tone and atypical movement patterns(1) • The incidence of CP is 2-2.5 per 1,000 live births(2) • Anatomical classification of CP –Diplegia – lower extremities are more involved than upper extremities - For additional information on diplegia, see Clinical Review…Cerebral Palsy: Spastic Diplegia;Item Number: 708898 –Hemiplegia – one side of the body is affected by CP; injury to brain located principally in one hemisphere(2) - For additional information on hemiplegia, see Clinical Review…Cerebral Palsy: Spastic Hemiplegia;Item Number: 708899 –Quadriplegia/tetraplegia – all extremities are affected similarly(3) –Double hemiplegia – all extremities are involved, but significant asymmetry is present in the level of involvement between the two sides(4) –Paraplegia – only the lower extremities are involved; very rare(4) –Monoplegia – one extremity is involved; very rare(4) –Triplegia – the lower extremities and one upper extremity are affected; very rare(5) Authors › ICD-9 codes Amy Lombara, PT, DPT • 343.0 congenital diplegia Andrea Callanen, MPT • 343.1 congenital hemiplegia Cinahl Information Systems, Glendale, CA • 343.2 congenital quadriplegia Reviewers • 343.3 congenital monoplegia Diane Matlick, PT • 343.4 infantile hemiplegia Cinahl Information Systems, Glendale, CA • 343.8 other specified infantile cerebral palsy Debra Seal, PT, DPT, PCS • 343.9 infantile cerebral palsy, unspecified Cinahl Information Systems, Glendale, CA Rehabilitation Operations Council › ICD-10 codes Glendale Adventist Medical Center, • G80 cerebral palsy Glendale, CA –G80.0 spastic quadriplegic cerebral palsy –G80.1 spastic diplegic cerebral palsy Editor –G80.2 spastic hemiplegic cerebral palsy Sharon Richman, MSPT Cinahl Information Systems, Glendale, CA –G80.3 dyskinetic cerebral palsy –G80.4 ataxic cerebral palsy –G80.8 other cerebral palsy August 28, 2015 –G80.9 cerebral palsy, unspecified

Published by Cinahl Information Systems, a division of EBSCO Information Services. Copyright©2015, Cinahl Information Systems. All rights reserved. No part of this may be reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, or by any information storage and retrieval system, without permission in writing from the publisher. Cinahl Information Systems accepts no liability for advice or information given herein or errors/omissions in the text. It is merely intended as a general informational overview of the subject for the healthcare professional. Cinahl Information Systems, 1509 Wilson Terrace, Glendale, CA 91206 › (ICD codes are provided for the reader’s reference, not for billing purposes) • G-Codes –Mobility G-code set - G8978, Mobility: walking & moving around functional limitation, current status, at therapy episode outset and at reporting intervals - G8979, Mobility: walking & moving around functional limitation; projected goal status, at therapy episode outset, at reporting intervals, and at discharge or to end reporting - G8980, Mobility: walking & moving around functional limitation, discharge status, at discharge from therapy or to end reporting –Changing & Maintaining Body Position G-codeset - G8981, Changing & maintaining body position functional limitation, current status, at therapy episode outset and at reporting intervals - G8982, Changing & maintaining body position functional limitation, projected goal status, at therapy episode outset, at reporting intervals, and at discharge or to end reporting - G8983, Changing & maintaining body position functional limitation, discharge status, at discharge from therapy or to end reporting –Carrying, Moving & Handling Objects G-codeset - G8984, Carrying, moving & handling objects functional limitation, current status, at therapy episode outset and at reporting intervals - G8985, Carrying, moving & handling objects functional limitation, projected goal status, at therapy episode outset, at reporting intervals, and at discharge or to end reporting - G8986, Carrying, moving & handling objects functional limitation, discharge status, at discharge from therapy or to end reporting –Self Care G-code set - G8987, Self care functional limitation, current status, at therapy episode outset and at reporting intervals - G8988, Self care functional limitation, projected goal status, at therapy episode outset, at reporting intervals, and at discharge or to end reporting - G8989, Self care functional limitation, discharge status, at discharge from therapy or to end reporting –Other PT/OT Primary G-code set - G8990, Other physical or occupational primary functional limitation, current status, at therapy episode outset and at reporting intervals - G8991, Other physical or occupational primary functional limitation, projected goal status, at therapy episode outset, at reporting intervals, and at discharge or to end reporting - G8992, Other physical or occupational primary functional limitation, discharge status, at discharge from therapy or to end reporting –Other PT/OT Subsequent G-code set - G8993, Other physical or occupational subsequent functional limitation, current status, at therapy episode outset and at reporting intervals - G8994, Other physical or occupational subsequent functional limitation, projected goal status, at therapy episode outset, at reporting intervals, and at discharge or to end reporting - G8995, Other physical or occupational subsequent functional limitation, discharge status, at discharge from therapy or to end reporting › . G-code Modifier Impairment Limitation Restriction CH 0 percent impaired, limited or restricted CI At least 1 percent but less than 20 percent impaired, limited or restricted CJ At least 20 percent but less than 40 percent impaired, limited or restricted CK At least 40 percent but less than 60 percent impaired, limited or restricted CL At least 60 percent but less than 80 percent impaired, limited or restricted CM At least 80 percent but less than 100 percent impaired, limited or restricted Source: http://www.cms.gov G-code Modifier Impairment Limitation Restriction CN 100 percent impaired, limited or restricted Source: http://www.cms.gov

. › Reimbursement: No specific issues or information regarding reimbursement have been identified › Presentation/signs and symptoms: Classification for physiological types of CP vary; the types listed below are from United Cerebral Palsy (UCP). Signs and symptoms as well as certain secondary impairments are listed from various references. The clinician should recognize that although the lesion is static, the secondary conditions (e.g., muscle weakness) can worsen over time(6) • General signs and symptoms of CP –Persistent neonatal reflexes (e.g., asymmetric tonic neck, Moro reflex)(7) –Poorly developed protective and equilibrium reactions(7) –Atypical muscle tone(1) –Atypical deep tendon reflexes(1) –Pain may be present as a result of arthritis, overuse syndromes, or musculoskeletal deformities(1) –Secondary musculoskeletal complications, including scoliosis, joint contractures, cervical instability, osteophytes, herniated discs, and cervical spinal stenosis, may be observed as the patient ages(1) –Due to muscle weakness and limited range of motion (ROM), radiculopathies, myelopathies, and nerve entrapment may occur(1) –Difficulty with motor control, coordination, and isolating muscle groups for movement –Two thirds of individuals with CP have cognitive impairments ranging from mild to severe(8) –Dyspraxia and other speech and language disorders(9) –Seizures(9) –Hearing loss(9) –Feeding difficulties(9) –Incontinence(9) –Visual impairments(10) • Spastic CP –~ 70-80% of CP cases(11) –Initially may present with hypotonia in infancy(11) –Hypertonicity and increased cocontraction of muscle groups(4) –Hyperreflexia –Muscle weakness(4) –Difficulty with motor control, coordination, and isolating muscle groups for movement –Delayed developmental motor milestones –Decreased ROM –Impaired balance, postural tone, coordination, and protective reactions(4) –Severe musculoskeletal deformities may develop as a result of prolonged spasticity(7) - Thoracolumbar scoliosis with pelvic obliquity is most prevalent; marked lordosis is typical in these patients; the scoliosis is often a C-shaped curve (no compensatory curve)(7) - Femoral anteversion, acetabular dysplasia, coxa valga, and pelvic obliquity(7) - Hip subluxation and dislocation may occur(7) - Muscle and joint contractures may develop(7) - Calcaneal varus or valgus, usually with a plantar flexion bias(7) - Dynamic equinus is a frequent finding in patients with lower extremity spasticity(12) - Patella alta due to history of prolonged flexed gait(1) • Ataxic CP –Rare form; ~ 5-10% of CP cases(11) –Damage to the cerebellum and/or its pathways results in absent co-contraction of agonists/antagonists, making gravity-resisted movement patterns extremely challenging(4) –Ataxia in its pure form is extremely uncommon(4) –Affects primarily balance, coordination, depth perception, gait, and rapid alternating movements(11) –Gait is characterized by a wide base of support and unsteadiness –Tremor on intention may be observed(11) –Hypotonia; low postural tone(4) –Developmental delay in motor milestones(4) –Visual impairments may be present, such as difficulty with visual tracking, difficulty isolating cervical movement from ocular movement, and eye-hand coordination challenges(4) • Dyskinetic CP –~ 10-20% of CP cases(11) –Dyskinesia is an umbrella term for various movement disorders, including chorea, athetosis, and dystonia - Chorea: abrupt, jerky distal movements - Athetosis: slow, writhing, persistent movements in the proximal extremities and trunk - Dystonia: writhing movements leading to sustained atypical postures –Abnormal involuntary movement patterns –Postural tone fluctuates and is typically hypotonic(4) –Postural instability(4) –Impaired co-contraction of muscle groups(4) and isolated motor control –Inability to grade desired movements(4) –Hypermobility present in most joints –Patterns of respiration are often altered(4) • Mixed CP –Presence of symptoms of two types of CP; often spastic CP with athetoid movements(4) • For more information regarding CP, see Clinical Review…Cerebral Palsy: Spastic Hemiplegia; Item Number: T708899; Clinical Review…Cerebral Palsy: Spastic Diplegia; Item Number: T708898;Clinical Review…Cerebral Palsy: Botulinum Toxin Type A Injections and Nonstandard Treatment Interventions; Item Number:;T708990;Clinical Review…Cerebral Palsy: Speech Therapy; Item Number:T708777; and Clinical Review…Dysphagia: Cerebral Palsy (Children); Item Number: T708945

Causes, Pathogenesis, & Risk Factors › Causes • Congenital CP – occurs as a result of injury to the child’s brain prior to or during birth(11) • Acquired CP – occurs as a result of injury to the brain after birth, or during the first few years of life (typically by age 2 years). It can be the result of an infection, traumatic brain injury (TBI), anoxia, or ischemia(5,11) –~ 10% of cases of CP are acquired(11) • ~ 20% of cases of CP are associated with known risk factors • Current evidence does not support that perinatal asphyxia is a major cause of CP(122) › Pathogenesis • An injury occurs in a region or multiple regions in the brain, resulting in permanent damage to brain tissue during the period of infant brain development(11) • A review of the literature on brain imaging patterns in children with CP published during the period 1995-2012 indicates that brain abnormalities were observed in 86% of neuroimaging scans. White matter injury was the most common imaging pattern, but with high heterogeneity. Grey matter injury, malformations, focal vascular insults, and miscellaneous findings (in descending order) were also observed(111) › Risk factors (prenatal) • Gestational age (< 32 weeks’(8) or ≥ 42 weeks’ gestation)(13) • Low birth weight (i.e., < 5 pounds, 8 ounces)(8) • Multiple birth • Prolonged labor – second stage of labor lasting > 4 hours(14) • Menkes syndrome– copper cannot be absorbed by the GI tract(3) • TORCH (toxoplasmosis, other infections [e.g., syphilis], rubella, cytomegalovirus, herpes) syndrome • Intraventricular hemorrhage(5) • Maternal infection(11) • Chorioamnionitis(15) – the membranes that cover the fetus become inflamed(3) • In vitro fertilization (IVF)(16) • Mother and infant blood type incompatibility(11) • Function of placenta breaks down(11) • Intrauterine growth retardation(8) • Intracranial hemorrhage(8) • Periventricular leukomalacia (PVL)(17) • Antepartum vaginal bleeding(14) › Risk factors (postnatal) • Severe jaundice(11) • Bacterial meningitis(8) • Viral encephalitis(8) • Falls(8) • Child abuse(8)

Overall Contraindications/Precautions › New clinicians may require training on how to address the emotional needs of the parents of a child with CP › Patients with CP have an increased risk for fractures(1) • Usually in the lower extremities and are usually caused by pathology, not trauma (falls) • 41% of fractures occur after surgery and often during physical therapy • Immobility often leads to osteoporosis, predisposing the patient to fractures • Patients with CP who are ambulatory have an increased risk of traumatic and stress fractures › Seizures are common in children with CP.(9,96) During a seizure:(18) • Stay with the patient • Eliminate dangerous objects from area • Release restrictive clothing • Do not hold down extremities • Use aspiration precautions: rotate head to side, assess airway, wait for tonic-clonic activity to pass before administering artificial respiration if indicated › Clinicians should be aware of any feeding tubes and take appropriate precautions during examination and treatment › A patient with oral motor dysfunction may not be able to safely manage liquids/foods and may be at risk for aspiration. Consult with family, nurse, and/or speech-language pathologist (SLP) for restrictions › Consult with family/health care providers for restrictions/health care plan related to a diagnosis of gastroesophageal reflux disease (GERD). Try to schedule therapy sessions either before or several hours after a meal › In patients with shunts, monitor for signs/symptoms of shunt malfunction (e.g., seizures, irritability, headaches, vomiting)(19) › See specific Contraindications/precautions under Assessment/Plan of Care Examination › History • History of present illness/injury –Etiology of illness - What type of CP does the patient have? - If known, where is the lesion located in the brain? - What was the birth history? –Course of treatment - Medical management - Orthopedic surgery may be indicated for:(20) - Significant contractures - Joint subluxation/dislocation - Curvature of the spine affecting (or potential to affect) overall function and/or respiration - Difficulty with hygiene and/pain due to musculoskeletal deviations and/or spasticity - The patient’s medical team needs to carefully decide how (and if) to treat spasticity in patients with CP. Generally a reduction in spasticity is useful to the patient; however, in some instances spasticity can facilitate function in otherwise weak muscles. To avoid inadvertent loss of function, the entire clinical picture should be evaluated prior to implementing any treatment strategies to reduce spasticity(21) - Children may require gastrostomy due to significant feeding/swallowing impairments. Based on a 2014 systematic review, evidence for the effectiveness of surgical interventions for feeding difficulties is low. Studies of gastrostomy typically demonstrate significant improvements in weight gain(119) - Spasticity management may consist of the following (Note: The level of investigation into the efficacy various treatment methods varies):(21) - Physical therapy and/or orthotic management - Oral medications (e.g., diazepam) - Chemodenervation – botulinum toxin, phenol, or alcohol injections - Dorsal rhizotomy - Intrathecal baclofen - Orthopedic surgery - Document other aspects of medical management,includingrespiratory functionand growth and nutrition - Medications for current illness/injury - Determine what medications clinician has prescribed, if any; are they being taken? Do they effectively control symptoms? - Botulinum toxin, intrathecal baclofen, and diazepam may be used for spasticity;pamidronate to increase bone mineral density; and glycopyrrolate to reduce drooling - Antiseizure medications may be prescribed as indicated(22) - Dopamine receptor blockers may be prescribed to decrease involuntary movements - Patients may also be treated with medications for pain management, bowel and bladder dysfunction, and behavioral problems (e.g., anxiety, attention deficit hyperactivity disorder [ADHD]) - Diagnostic tests completed: Testing may consist of: - MRI to assess location and size of brain injury(2) - Cranial ultrasound(23) - X-ray to assess for orthopedic concerns such as scoliosis, femoral anteversion, hip dysplasia, etc. - Electroencephalogram (EEG) assessment may be completed(22) - Home remedies/alternative therapies: Document any use of home remedies (e.g., ice or heating pack) or alternative therapies (e.g., acupuncture, Brain Gym, hyperbaric chamber treatment) and whether or not they help - In a study investigating the effects of hippotherapy (therapeutic horseback riding) conducted in South Korea, researchers found that an 8-week program with two 30-minute sessions per week resulted in significant improvements in both gross motor function and balance in 33 children with CP(97) - In a follow-up randomized controlled study conducted by the same South Korean researchers, hippotherapy positively affected gross motor function and balance in 91 children with CP presenting at various GMFM levels (I-IV). The GMFM-88, GMFM-66, and Pediatric Balance Scale (PBS) were used as outcome measures(123) - In a stratified single-blind randomized controlled trial conducted in Spain with 19 subjects with CP in an experimental hippotherapy simulator group and 19 subjects in a control, “no-movement” simulator group, researchers found significant improvement in balance in the children with higher levels of disability but no changes in overall function in the children in the experimental group(81) - Please see Clinical Review…Hippotherapy;. Item Number: T901881 for more information on this topic - Previous therapy: Document whether patient has had occupational and/or physical therapy for this or other conditions and what specific treatments were helpful or not helpful. How long did the patient receive rehabilitation services? –Aggravating/easing factors (and length of time each item is performed before the symptoms come on or are eased) –Body chart: Use body chart to document location and nature of symptoms –Nature of symptoms: Document nature of symptoms (constant vs. intermittent, sharp, dull, aching, burning, numbness, tingling). Pain may be caused by medical and/or surgical procedures; physical, occupational and/or speech therapy treatments; and/or gastrointestinal, orthopedic and/or neuromuscular disorders.(24) The specific cause of pain may be difficult to isolate –Rating of symptoms - Use a visual analog scale (VAS) or 0-10 scale to assess symptoms at their best, at their worst, and at the moment (specifically address if pain is present now and how much) - Pain in patients with CP is highly prevalent(104) - Musculoskeletal pain experienced by children with CP can have a significant negative impact on health-related quality of life(82) - The Oucher scale, a self-report pain VAS, may be used with children aged 3.3 years and older.(25) FLACC scale for younger or nonverbal patients –Pattern of symptoms: Document changes in symptoms throughout the day and night, if any (A.M., mid-day, P.M., night); also document changes in symptoms due to weather or other external variables –Sleep disturbance: Document number of wakings/night, if any - Children with CP may have difficulty staying asleep for various reasons, including pain, seizures, gastrointestinal problems, and/or respiratory problems such as sleep hypoventilation, central sleep apnea, and obstructive sleep apnea –Other symptoms: Document other symptoms patient may be experiencing that could exacerbate the condition and/ or symptoms that could be indicative of a need to refer to physician (e.g., dizziness, bowel/bladder dysfunction, saddle anesthesia). In children with shunts, the clinician should monitor for signs and symptoms of shunt malfunction –Respiratory status: Document if the patient has received or is receiving respiratory therapy, or oxygen. Obtain results of pulmonary function tests (PFTs) where available –Barriers to learning - Are there any barriers to learning? Yes__ No__ - If Yes, describe ______• Medical history –Past medical history - General inquiry - What is the present reason for evaluation? - What is patient’s age and adjusted age for prematurity? (When performing standardized tests, typically adjust for prematurity until age 24 months, but this varies regionally and based on testing purpose) - At what age was the patient diagnosed with CP? - Any hospitalizations? If so, how long was the patient hospitalized? - Who comprises the patient’s medical team? - Any recent x-rays? - Does the child require a feeding tube? If so, for what percentage of total nutrition? - Has the patient undergone any surgical procedures, specifically orthopedic surgeries? - Any spasticity management interventions, botulinum toxin, phenol, baclofen pump, etc.? - If relevant (e.g., depending on patient’s age), inquire about pregnancy and birth history. Note pertinent findings such as Apgar scores, gestational age, adjusted age, birth weight, need for ventilation, manner of delivery, need for forceps or vacuum, nuchal cord complications, NICU stay, fetal exposure to alcohol or drugs, etc. - Comorbid diagnoses: Ask patient/caregiver about other problems, including diabetes, obesity, epilepsy, sensory integration disorder,(9) GERD, cancer, autism spectrum disorder (ASD),heart disease, psychiatric disorders, orthopedic disorders, etc. - Data from the 2009-2010 U.S. National Survey of Children with Special Health Care Needs indicate that adolescents with CP did not have higher rates of attention deficit hyperactivity disorder (ADHD), depression, anxiety, oppositional or conduct disorders, or ASD compared to a control group(120) - Medications previously prescribed: Obtain a comprehensive list of medications prescribed and/or being taken (including over-the-counter drugs and herbal supplements) - Other symptoms: Ask patient/caregiver about other symptoms the patient may be experiencing that are inhibiting his or her motor development • Social/occupational history –Patient’s goals: Document what the patient and caregiver hope to accomplish with therapy and in general - Does the family feel they have necessary support in place? –Vocation/avocation and associated repetitive behaviors, if any - What is child’s grade level if school-aged? - Does the child travel to school? - Is the child in an integrated classroom? - Can the child access the classroom in a timely manner? - What therapeutic services does the child receive through the school district? - Is the child involved in any additional activities, hobbies, and/or sports? - How much daily physical activity does the patient typically get? - Physical Activity Scale for Individuals with Physical Disabilities can be used to determine self-reported level of activity - Children and young adults with CP participate in significantly lower levels of habitual physical activity than their typically developing peers(98) - Is the older patient living independently, working, and driving? - How is the patient’s endurance throughout the day? –Functional limitations/assistance with ADLs/adaptive equipment - What is the patient’s primary means of mobility (indoor/outdoor)? - Does the patient utilize any assistive or adaptive devices for ADLs? - How does the child manage bowel and bladder function? –Living environment - Inquire about stairs, number of floors in the home, with whom patient lives, caregivers, etc. Identify if there are barriers to independence in the home - Is the patient able to safely negotiate all environments (home, school, work setting)? - What is the child’s primary mode of mobility within the home/school? - Are any modifications necessary to access the home or within the home? › Relevant tests and measures: (While tests and measures are listed in alphabetical order, sequencing should be appropriate to patient medical condition, functional status, and setting.) May be age and setting specific; recommendations are a guide and should be implemented as appropriate for each unique patient • Anthropometric characteristics –Is there a functional/true leg length discrepancy present? –Note height and weight and calculate body mass index (BMI). Compare to age- and sex-matched growth curves • Arousal, attention, cognition (including memory, problem solving) –Assess orientation to name, place, date, and time (as age appropriate) –Assess ability to follow simple commands. How many steps? –The Neonatal Behavioral Assessment Scale may be used for newborns –Note any adaptive communication devices used by child/family • Assistive and adaptive devices –What devices does the patient have? Assess safety and fit of current devices, as well as need for additional devices –The Family Impact of Assistive Technology Scale is designed to rate the effect assistive technology (e.g., postural control devices) has on a child with disability and his/her family. The test is geared toward children who cannot sit independently. Further testing needs to be completed to fully assess validity and reliability of this new instrument(26) • Balance –Assess static and dynamic balance reactions in developmentally appropriate postures –The PBS can be used on patients older than age 5 years –The Timed Up and Go (TUG) test can be used as an objective measure of balance and mobility –The Bruininks-Oseretsky Test of Motor Proficiency, Second Edition (BOT-2) assesses higher level balance skills in patients aged 4-21 years –Five-stand-test (FTSST) has been found to be a reliable measure for assessment of functional balance ability and lower limb strength in children with mild to moderate CP(88) –Based on a 2014 systematic review, the Pediatric Reach Test (PRT), Sitting Assessment for Children with Neuromotor Dysfunction (SACND), Segmental Assessment of Trunk Control (SATCo), and Trunk Control Measurement Scale (TCMS) are recommended for use in clinical practice to assess sitting balance in children and adolescents with CP(113) • Cardiorespiratory function and endurance –Document vital signs at rest and during mobility –Document distance patient can safely propel wheelchair - The 6-minute push test (6MPT) and the one-stroke push test (1SPT) are reproducible functional tests for children and adolescents with CP who self-propel a wheelchair(106) –Six-minute walk for distance test (6MWT) or TUG test may be used in ambulatory population –An accelerometer can be used to measure habitual physical activity in ambulant children with CP(124) - The ActiGraphGT3X+ accelerometer has been found to be reliable under controlled walking and stepping conditions, as well as in a community environment, to measure physical activity in independently ambulating children and adolescents with CP(124) –Note respiratory rate, use of diaphragm and accessory muscles, atypical breathing patterns, and color changes in lips, skin, and fingers –In a study in the Netherlands that involved 70 children with CP and 31 typically developing children, researchers found that children with CP had decreased aerobic and anaerobic exercise responses compared with typically developing children(95) –Results of a study conducted in South Korea indicate that children with spastic diplegic CP and hemiplegic CP have lower respiratory function in terms of maximum inspiratory and expiratory pressures (MIP and MEP), forced vital capacity (FVC), and forced expiratory volume in 1 second (FEV1) compared to children with normal development.(125) No significant differences were found between children with spastic diplegic CP and hemiplegic CP(125) • Circulation –Assess pedal pulses –Note capillary refill time –Note temperature and color of skin • Ergonomics/body mechanics –Body mechanics are often impaired; the clinician should document compensatory strategies • Functional mobility (including transfers, etc.) –WeeFIM for overall functional assessment –Quick functional assessment tests that can be used for adolescents with CP - Gross Motor Function Measure (GMFM)(121) - Timed Up and Down Stairs (TUDS) test - TUG test - Sit-to-Stand (STS) test - Lateral Step-Up (LSU) test - 1-minute walk test - 10-meter walk test • Gait/locomotion –Assess child’s gait and document any gait abnormalities and/or asymmetries –Common atypical gait patterns include decreased and/or absent heel strike, decreased step and/or stride lengths, increased stance phase, decreased speed, increased hip/knee flexion in all phases of gait, increased hip internal rotation with adduction, decreased trunk/pelvic disassociation, decreased reciprocal arm swing, and wide base of support(27) –Formal gait analysis, ideally at a computerized motion analysis lab, is beneficial prior to surgical intervention in order to properly identify abnormal gait biomechanics due to spastic muscle patterns - Three-Dimensional Gait Analysis (3-DGA) is used to evaluate gait in an objective manner and is reported to have high intrarater reliability. Limitations of this method of assessment include the cost and high skill level required to interpret data(28) - Researchers also suggest gait analysis 2-3 years after surgery since functional gait improvements observed immediately following surgery may not last(29) –In a controlled study conducted in the United States involving 30 children with CP and 27 typically developing children found that the children with CP expended more energy walking than their typically developing peers. Energy cost was measured by the gas dilution method(30) - There was a significant correlation between the Gross Motor Function Classification System (GMFCS) level and the energy cost of walking: the higher the level of disability, the greater the energy expenditure(30) –In a study with 209 children and adolescents with CP conducted in the United States, researchers found that in order to reliably measure the stepping activity of children and adolescents with CP, it is necessary to monitor walking activity over a period of: - At least 8, 6, and 2 days for individuals aged 2 to 5 years who are at GMFCS levels of I (walking without restrictions), II (walking without assistive devices but some assistance and limitations for stairs/community walking), and III (walking only with assistive devices),respectively(80) - At least 6, 5, and 4 days for individuals aged 6 to 14 years who are at GMFCS levels of I, II, and III, respectively(80) • Joint integrity and mobility –High risk for developing joint contractures in children with spasticity since full ROM is not achieved; document any joint contractures –Assess upper and lower extremities for joint crepitus –Document joint hypermobility using Beighton Hypermobility Scale –Assess hip joint integrity via Galeazzi sign and/or Ortolani maneuver • Motor function (motor control/tone/learning) –Assess upper and lower extremities and trunk for coordination, hypertonicity, hypotonicity, and dystonia –Assess spasticity using Modified Ashworth Scale or Tardieu Scale - The Tardieu Scale was found to have good to excellent intrarater and interrater reliability when assessing ankle plantar flexors or elbow flexors in a study comprised of 6 examiners; reliability was significantly increased following 1 day of training(31) –Assess for ankle clonus and indicate number of beats, if present –Assess movement pattern during age-appropriate functional and/or play activities (e.g., ball handling, jumping, kicking, running)(32) - Observe quality of movement patterns and the ability to isolate muscle groups (such as hip abductors and hip extensors when rolling) - Indicate any abnormal patterns, reflexes, or tone observed with specific activity –Refer for assessment for adaptive play and leisure equipment when appropriate; adaptive bicycles and other adaptive equipment can allow children with CP to experience and participate in play and leisure activities with their able-bodied peers(85) • Muscle strength –Use manual muscle testing (MMT) and/or handheld dynamometry as indicated. MMT is not valid in the presence of abnormal tone. Appropriate for children aged 4-5 years and above who can follow directions for testing. Evaluate lower extremities completely and screen upper extremities. Assess trunk strength for flexion and extension and isolate upper and lower trunk rotation.(17)Document the patient’s ability to isolate muscle groups against gravity (i.e., hip abductors in side-lying, hip extensors in prone) –Handheld dynamometry has been found to have acceptable reliabilityin children with CP for testing hip flexors, hip extensors (in supine), knee flexors and extensors, and ankle dorsiflexors (with stabilization). To analyze changes in muscle strength in children in relation to an intervention or maturation, strength should be reported relative to body weight or as a torque measurement (relative to lever length). Measurement error varies across more impaired and less impaired limbs(126) –Use the Selective Control Assessment of the Lower Extremity (SCALE) to determine extent of selective (isolated) motor control available(107) –Assess strength through function in the younger population and in those who are unable to follow verbal commands due to cognitive deficits –Ten children in Australia with spastic diplegic CP who were ambulatory had their lower extremity strength measured via a handheld dynamometer. The test-retest reliability was analyzed with the following findings:(41) - In groups, mean lower extremity strength needs to increase at least 7 kg (30%) to be viewed as a true change in strength - In individuals, strength needs to increase more than 16.8 kg (70%) for knee extension and increase more than 4.3 kg (25%) for plantar flexion to be viewed as a true change in strength - Hip extension measurements were not shown to be reliable - The authors concluded that evaluating strength via handheld dynamometers is most reliable in assessing changes in strength among groups of patients with CP • Neuromotor development: Use one of the following functional tests to assess developmental skills –Test of Infant Motor Performance (TIMP) - Clinical tool designed to assess motor performance in infants 34 weeks postconceptional age to 4 months postterm(33) - Can be used to document progress in postural and motor control(34) - Reported to have excellent test-retest and rater reliability (in clinicians with proper instruction on use of TIMP)(33) - Sensitivity is 92% and specificity is 76% for predicting motor performance at 1 year(33) - For additional information on the TIMP, see Clinical Review…Test of Infant Motor Performance (TIMP); Item Number: T902532 –The WeeFIM is a clinically useful tool for assessing self-care, sphincter control, transfers and locomotion, and communication and social cognition(35,36) - The WeeFIM is most useful in children aged 2-5years(36) - The WeeFIM has been studied in various countries (including in a recent study in Turkey involving 86 children, approximately half of whom had CP) and found to be valid in gross domain assessment(36) –The Functional Walking Test (FWT) measures a child’s ability to kneel, stand from kneeling position, stand, walk, and climb stairs(37) –Researchers in Taiwan found that the Peabody Developmental Motor Scales, Second Edition (PDMS-2) had good test-retestreliability over the course of 1 week and appropriate responsiveness and sensitivity to change over a 3-monthperiod in a non-controlled study of 32 children with CP(38) –The GMFM and the Pediatric Evaluation of Disability Inventory (PEDI) are appropriate outcome measures(39) - The GMFM is an outcome measure that classifies children with CP based on their level of independence with gross motor skills(23) - Can be used to track progress (or regression) in gross motor skills, as it is sensitive to motor change - The original GMFM had 88 items (GMFM-88); the latest version has 66 items (GMFM-66). Based on a 2014 systematic review, both versions of the GMFM are effective in measuring change in gross motor function in children with CP(109) - A Chinese version of the GMFM is available. It has been reported to be a valid measure of functional ability in Chinese school children with CP and reliable for use by Chinese healthcare providers(118) - PEDI(23) - Developed for use with infants and children (aged 6 months to 7.5 years) - Can be used to track progress (or regression) in domains of self-care, mobility, and social function - In a study conducted in the Netherlands evaluating 55 children with CP for the responsiveness of the GMFM and the PEDI, researchers found that both tests were responsive to alterations in motor performance and were most sensitive in children under 4 years of age(40) - Results from a 2014 systematic review indicate the PEDI has the strongest psychometric properties in school-aged children(110) –The Manual Ability Classification System (MACS) is a classification system designed to categorize children 4 to 18 years of age with CP based on the use of their hands for functional activities(108) - Level 1: Children are able to handle most objects easily and successfully on their own - Level II: Children are able to handle most objects on their own but with minimal decreased ability and/or speed - Level III: Children are able to handle objects but with difficulty and require help in preparing and/or modifying activities - Level IV: Children are able to handle a limited selection of easily managed objects in adapted situations - Level V: Children cannot handle objects and have severely limited ability to perform simple actions - Results of a 2014 Swedish study that included 1,267 children with CP suggest MACS levels are stable over time and the classification system has excellent predictive value(108) • Observation/inspection/palpation (including skin assessment) –Assess skin integrity and monitor tolerance to orthotics, if in use; document wearing schedule –Note redness, ability to blanch, any breakdown in skin; measure and stage any wounds and notify MD • Oral mechanism exam and related tests –Screen patient for oral motor dysfunction/feeding problems that may compromise nutritional/health status. Examine for signs of weak suck, delayed/absent tongue lateralization, tongue thrust, weak lip closure, uncoordinated swallowing mechanism, tonic bite, hyperactive gag reflex, and poor seating/posture during feeding secondary to unstable trunk(17) • Posture –Assess posture in sitting and standing, with and without braces –Assess posture in wheelchairas indicated –Observe for signs of scoliosis (rib hump, shoulder elevation, pelvic asymmetry)(14) - 25% of individuals with CP develop scoliosis - 60-75% of severely involved individuals with quadriplegia develop scoliosis - The course of scoliosis in CP varies from idiopathic curves. In CP the curve can progress after skeletal maturity - For additional information on scoliosis, see Clinical Review…Scoliosis; Item Number: T708447 • Range of motion –Assess passive and active ROM for upper and lower extremities; use goniometry –In the younger population, ROM can be assessed though functional movement if patient is resistive to hands-onassessment –Flexibility assessments should include the following tests:(17) - Hip abduction with knees flexed (adductor magnus, brevis, and longus muscles) - Hip extension in the Thomas test position with the knee extended (iliopsoas muscle) and flexed (rectus femoris muscle) - Knee extension with hip flexion (popliteal angle –hamstring muscles) - Ankle dorsiflexion with knee flexed (soleus muscle) and knee extended (gastrocnemius muscle) • Reflex testing/protective reactions –Assess for anterior, posterior and lateral protective reactions in sitting, prone, and standing –Assess for primitive reflexes (e.g., Moro, asymmetrical tonic neck, parachute), which may be abnormally absent or persistent –Assess deep tendon reflexes bilaterally for C5, C6, C7, L4, and S1; hyperreflexia often elicited • Self-care/activities of daily living (objective testing) –Evaluate or inquire about ADLs; note any assistive devices used –The Canadian Occupational Performance Measure (COPM) may be used to assess function in the domains of self-care,leisure, and productivity(42) –The Assessment of Life Habits (LIFE-H) tool may be used to evaluate ADLs in children aged 5-13 years(43) –Results of a 2014 systematic review suggest the Assessment of Motor and Process Skills (AMPS) is the most effective measure to evaluate ADL performance or capacity and is suitable for children and adolescents, but further researcher should examine the reliability of the AMPS over time in children and adolescents with CP(110) • Sensory testing –Stereognosis, joint position sense, and two-pointdiscrimination are often impaired in children with CP(44) - Assess tactile sensation using Semmes Weinstein monofilaments (SWMs)(45) - Assess two-point discrimination using paper clips(45) - Use the Sensory Integration and Praxis Test (SIPT) to assess stereognosis and graphanesthesia(45) –In a prospective cross-sectional study conducted in the United States involving 62 children with CP and 65 typically developing children, researchers found the following:(44) - Sensory testing comprised of light touch, position sense, vibration sense, direction of scratch, and pain sensation can begin as early as age 5 years in children with CP - Among the children with CP, the greatest inaccuracies during testing were observed during proprioception of the great toe position, vibration sense, and direction of scratch compared to their typically developing peers

Assessment/Plan of Care › Contraindications/precautions • Patients with this diagnosis are at risk for falls;(101) follow facility protocols for fall prevention and post fall prevention instructions at bedside, if inpatient. Ensure that patient and family/caregivers are aware of the potential for falls and educated about fall prevention strategies. Discharge criteria should include independence with fall prevention strategies –Adults with CP often experience mobility decline in early to middle adulthood with reduced balance and increased fall risk(93) • Only those contraindications/precautions applicable to this diagnosis are mentioned below, including with regard to modalities. Rehabilitation professionals should always use their professional judgment and knowledge of best practice evidence • Clinicians should include the family/caregivers in the treatment sessions, explain and demonstrate therapeutic treatment interventions, and provide oral and written instructions for a home program as indicated. Whenever possible, interventions for patients with CP should be family-centered and function-focused(102) • Clinicians should follow the guidelines of their clinic/hospital and what is ordered by the patient’s physician. The summary listed below is meant to serve as a guide, not to replace orders from a physician or a clinic’s specific protocols • Cryotherapy contraindications(46) –Raynaud’s syndrome –Cold urticaria –Paroxysmal cold hemoglobinuria –Impaired circulation • Cryotherapy precautions(46) –Hypertension – cold can lead to an increase in blood pressure –Hypersensitivity to cold –Avoid aggressive treatment with cold modalities over an acute wound –Avoid placement over superficial nerves for extended periods (> 1 hour) –Cold may be counterproductive if being used to facilitate muscle relaxation and reduce in pain in patients who do not tolerate the modality • Thermotherapy contraindications(46) –Decreased circulation –Decreased sensation –Acute/subacute traumatic and inflammatory conditions –Skin infections –Impaired cognition or language barrier –Malignant tumors –Tendency for hemorrhage or edema –Heat rubs › Diagnosis/need for treatment: Most children with CP will require periodic courses of physical therapy throughout their lifespan with the degree/intensity of physical therapy varying depending on the child’s needs. Intervention should be tailored to meet the needs of the child/family at each particular stage of development as well as recovery from postsurgical procedures. The goals of physical therapy are numerous but generally include prevention of musculoskeletal deformity, maximization of mobility and age-appropriate functional skills, and patient/family education • It is important to note that although early diagnosis is key to initiating rehabilitative services, up to 50% of children with CP diagnosed before the age of 2 years have spontaneous resolution of symptoms and, therefore, can be inaccurately diagnosed(6) › Rule out • Progressive neurologic disorder • Idiopathic toe-walking • Pelizaeus-Merzbacher disease: X-linked genetic disorder, slowly progressive, begins in infancy › Prognosis • In children with CP who are not walking by the age of 2 years, independent predictors of the ability to walk a minimum of 20 feet independently by age 6 years include rolling, sitting and/or pulling to stand at 2 years of age, type of CP other than spastic quadriparesis, and intact visual function(47) –Based on a retrospective study conducted in the United States involving 2,295 children with CP who were not walking at age 2-3.5 years and were evaluated at age 6 years –At the age of 6 years, 12.8% walked independently (at least 20 feet) and 18.4% walked with support • In a cross-sectional study conducted in Sweden comprised of children with CP, the inability to walk independently at age 5 years was associated with type of CP other than unilateral spastic, IQ <50, presence of active epilepsy, and severe visual and/or hearing impairment(48) –Based on a record review of 9,012 children with CP born in Europe between 1976 and 1996 –At age 5 years, 54% walked unaided, 16% walked with aids, and 30% were unable to walk –Study included detailed distribution of walking ability based on CP type, degree of intellectual impairment, epilepsy activity, and degree of visual and hearing impairment • Gross motor progress can be predicted in children with CP(49) –Based on prospective cohort study conducted in Canada –657 children aged 1-13 years with CP followed for up to 4 years –Authors provide curves predicting gross motor function scores by age for 5 levels of gross motor function • Canadian researchers foundthatof 258 children and adolescents with CP, 53.2% of children and 57.5% of adolescents attended regular schools; however, 41% of these children and adolescents required special education resources(116) –The remainder of the children and adolescents in the study attended special schools and 84.6%of all children and adolescents were receiving at least one rehabilitative service, with physical therapy and occupational therapy being the most common services provided –Services were primarily one time a week and conducted in the school setting for both children and adolescents, but for the adolescents, services were more frequently consultations rather than direct weekly therapies –Children with lower IQ, greater motor impairments, and greater activity limitations were significantly more likely to receive occupational therapy, physical therapy, speech-language therapy, or special education • The TUG test may be best predictor of walking ability in adults with spastic CP(50) –Based on a cross-sectional study conducted in Norway –126 adults with unilateral or bilateral spastic CP were evaluated –Sex, type of CP, popliteal angle, pain, and TUG test values were reported to be significant predictors of walking ability • Factors associated with lower physical activity in children aged 8-17years with CP include decreased participation in the home and community, female sex, older age (adolescents), and decreased performance on the 6MWT(127) –Older age and reduced community participation were associated with high inactivity –GMFM-Section E (walking, running, jumping) score is also an important predictor of daily physical activity(128) • Survival(1) –~ 90% of individuals with CP live to age 20 –~ 75% of individuals with quadriplegic CP live to age 30 –~ 95% of individuals with diplegic CP survive to age 30 • A cross-sectional study conducted in the Netherlands of 54 adults with CP aged 25-36 years assessed health concerns in this population(51) –59% of group sample reported pain –15% had a joint deformity in their most affected upper extremity –57% had a joint deformity in their most affected lower extremity –~ 20% had swallowing and speech impairments –22% of the group sampled had vision concerns › Referral to other disciplines • Surgeon (including neurosurgeon and orthopedic surgeon): The typical goal of surgery in this population is to improve ROM and manipulate musculoskeletal deformities with the aim of improving overall function(29) –In a small randomized controlled trial conducted in Canada of 24 children with CP, researchers evaluated the impact of a selective dorsal rhizotomy (SDR) for the management of spasticity and found SDR in combination with physical therapy/ occupational therapy had a significant positive impact on functional motor skills 1 year postop when assessed against physical therapy/occupational therapy alone(52) • Counselors, therapists, social workers, and neuropsychologists: Many anecdotal reports of psychosocial issues were reported in the literature as parents and children try to cope with the effects of CP. A referral to a specialist may be warranted, and each case should be evaluated on an individual basis. The clinician needs to avoid generalizations based on level of impairment and assess the needs of each family separately • SLP: If feeding or speech concerns are present, a speech therapy evaluation is indicated –Researchers in the United Kingdom reported that in a population of adolescents with spastic diplegic CP (N=346), 63% had impaired speech of varying severity; 32% of the 346, reported being provided with 1 or more types of augmentative and alternative communication (AAC), but 75% of these adolescents reported that AAC was only for use at school and for communication assistance(115) • Occupational therapy as indicated for feeding and fine motor deficits › Other considerations • Musculoskeletal pain experienced by children with CP can have a significant negative impact on health-related quality of life.(82) Parents of children with CP do not always understand the impact of this pain on the child’s well-being and mental health; hence, a self-reported measure of quality of life should be administered whenever possible in order to best meet the needs of the patient(82) • There are numerous assessment tools available to assist in monitoring various domains related to patient/family quality of life; examples include:(43) –Pediatric Quality of Life Inventory –Lifestyle Assessment Questionnaire –Family Support Scale –Health Utilities Index –Strength and Difficulties Questionnaire • School setting –In a prospective cohort study conducted in Israel, 148 children with CP in an elementary school were divided into two groups, fully-included (FI) and self-contained (SC). The children were assessed using the School Function Assessment (SFA) after the first school semester. The following findings were reported:(53) - The average participation (within the school) rating in the FI group was significantly higher in most of the 6 school environments evaluated - Adaptations and assistance was significantly greater in the SC group than in the FI group - Physical task supports granted to both the FI and SC groups were significantly higher than cognitive/behavioral supports provided - The authors concluded that more cognitive/behavioral adaptations are required in place of physical assistance to bolster participation level among CP students in the school setting › Treatment summary • Strength training –Guidelines of the Cincinnati Children’s Hospital Medical Center recommend including a progressive strength training exercise program as part of comprehensive physical therapy management for individuals with CP aged 4 to 20 years(105) –Muscle strengthening may not yield clinically relevant improvements in strength, motor function, or walking speed in ambulatory children and adolescents with CP(54) - Based on a systematic review with meta-analysis - Review included 6 trials (randomized or quasi-randomized controlled trials); 5 were included in the meta-analysis - Some concerns have been raised about the findings of this review, including:(55) - The combination of interventions in the meta-analysis (e.g., electrical stimulation and progressive resistance exercise) - The treatments within the individual studies may not have been of ample intensity - The method of calculation of effect sizes –Results from a systematic review of 7 articles investigating the effects of progressive resistive exercise in individuals with CP suggest an increase in muscle performance following progressive resistive exercise, but a clear correlation between the increase in strength and resulting improvement in functional outcomes could not be made(56) –Functional progressive resistance strength training may lead to gains in muscle strength in children with CP; however, mobility and spasticity may not be significantly affected(58) - Based on a small randomized trial conducted in Australia - 51 children with spastic CP (mean age, 10.4 years) were randomized to 1 of 2 groups: - Intervention group - Exercise circuit including leg press and lower extremity functional strength exercises - 3x/week, 12 weeks, 45-60 minutes per session - Control group – 1-3 typical physical therapy sessions per week for 12 weeks - Participants were followed for 18 weeks total - Results - No significant differences between groups in mobility or spasticity were detected post intervention - The intervention group experienced a significantly greater increase in: - Knee extensor strength - Hip abductor strength - Total isometric muscle strength - 6 repetition maximum strength - 6 weeks after strength training ended, gains in strength diminished –In a randomized controlled trial conducted in Germany comprised of 39 children with CP, researchers investigated the effects of a postoperative home-based strength training program on walking ability. The study found no significant difference between the treatment group and the control group measured 1 year after the surgery(29) - The control group participated in a physical therapy program that focused on restoring ROM following surgery - The treatment group participated in typical physical therapy treatment with the addition of a strength training program. After discharge from the hospital, the strengthening exercises were performed at home with parents assisting - The strength training (treatment group) was a 9-monthprogram comprised of 7 lower extremity exercises, 2 sets of 5 reps; Theraband was used once gravity could be overcome - There were no significant differences between the treatment and control groups - Authors speculated that the lack of significant change may have been due to variable postop outcomes or decreasing motivation due to length of study and that more positive results may have been observed if treatment time was shorter/ more intense –In a pilot study conducted in the United States, researchers demonstrated that increased ankle strength may lead to increased gait speed and improved function in children with CP(59) - 12 participants were randomly assigned to one of 4 groups: strengthening of plantar flexors, dorsiflexors, plantar and dorsiflexors, or a control group. Most participants demonstrated strength gains in the specific muscles that underwent training - Kinematics of gait either significantly improved or demonstrated trends toward improvement in all treated patients. Gait speed increased in some but not all training groups. Quality of life as measured by the PedsQL improved significantly for the entire training group –Progressive resisted exercises performed using circuit training to increase trunk and lower extremity strength may improve overall strength, balance, and function(117) - Based on a randomized controlled trial with 60 children with spastic diplegic CP conducted in Saudi Arabia - Children in the experimental group participated in a 6-week supervised circuit strengthening program in conjunction with traditional physical therapy; the control group participated solely in traditional physical therapy. Strength training sessions were 3 times a week. Six to 10 repetitions per exercise were completed and repetitions progressed to a higher weight when the subject could perform 3 sets of 12 repetitions - Balance, strength, and functional ability improved significantly compared to the control group as measured by the PBS, handheld dynamometer, and the GMFM –Results of a feasibility study conducted in Australia suggest that a community-based, low-dose group exercise program can improve balance and strength in children with CP(129) - Ten children with CP, aged 8-15 years, participated in an 8-week program in a community gym using simple equipment - Strength of elbow flexors, hip abductors, ankle dorsiflexors, and ankle plantar flexors improved. Functional strength as measured by seated throw, distance jump, and lateral step-up and balance as measured by the Movement Assessment Battery for Children (MABC), the lateral and forward reach tests, and the BOTalso showed improvement - The researchers noted that this study demonstrates that results of strength and balance training studies conducted in clinical research settings with specialized equipment translates into clinical practice in a low-cost, low-dose community program –Strength training combined with botulinum toxin type A treatments might lead to greater reductions of spasticity in children with CP(89) - Based on a study with 15 children with spastic diplegic CP conducted in Australia(89) - Children were receiving bilateral lower-limbbotulinum toxin type A treatments from their physicians(89) - The strength-training was done 3 times per week for 10 weeks and was coordinated and progressed by a physical therapist every 2 weeks - Lower limbs were targeted for the strength training exercises and each program was individualized to the specific study participant’s needs - Researchers found that over the course of the study period the injections appeared to reduce spasticity and the strength training did not increase it; strength and functional gains were found in all muscles that were both trained and treated with injections • Functional training –A systematic review of studies investigating the effectiveness of static weight-bearing (SWB) exercises in children with CP included 10 studies after 638 did not meet the inclusion criteria. The authors’ conclusions from the compiled results of these studies are listed below(6) - The use of SWB exercises in the lower extremities may improve bone mineral density in the spine or femur - Spasticity may be reduced in the lower extremities in the short term through SWB exercises (for the lower extremities) via a prolonged stretch - Limited research exists to support SWB exercises in the upper extremities in an effort to decrease spasticity and improve hand function - No evidence other than anecdotal reports exists for the use of SWB activities in preventing hip dysplasia, improving self-esteem, improving urinary and bowel function, or improving children’s communication abilities –Physical therapy with a functional emphasis may result in greater motor improvements than physical therapy with an emphasis on normalizing quality of movement(60) - Based on a randomized blocked design study conducted in the Netherlands comprised of 55 children with CP - GMFM results post treatment were not significantly different between groups; however, when measured by the PEDI (self-care and mobility domains) greater improvements were observed in the functional treatment group(60) –Goal-directed functional therapy (GDT) was reported to be more effective than activity-focused therapy (AT) for children with CP, leading to improved abilities in self-careand mobility(79) - Based on a nonrandomized, comparative study conducted in Sweden - 44 children with CP participated in the study - The children were divided into 2 groups and received 1 of 2 interventions - Group 1 – GDT; group session 1x/week; daily practice; 12 weeks duration - Group 2 – AT; individual session 1x/week; daily practice; 12 weeks duration - GDT is different from AT in that GDT:(81) - Identifies individually tailored treatment goals - Implements regular group meetings for therapy - Incorporates specific parental education at the beginning of therapy - Results - Preintervention – The PEDI caregiver assistance scale in self-care was significantly higher in group 2 - Postintervention – The between group comparison revealed group 1 experienced significantly greater improvements in mobility and self-care • Gait training –Gait trainer walking exercises might be more effective than traditional physical therapy to improve ambulation index and stance time, but not gait speed or step length, in children with hemiparetic CP(61) - Based on a randomized controlled trial of 30 children with hemiparetic CP conducted in Egypt - The children in the gait trainer group participated in traditional physical therapy exercises and gait training, in addition to walking exercises on the Biodex Gait Trainer 2 - The gait trainer provided visual and auditory feedback and automatically adjusted the speed of the treadmill belt depending on the child’s performance –Lower body positive pressure supported treadmill training might improve balance, walking speed, and lower extremity strength in children with CP(62) - Based on a research study of 9 children with CP conducted in the United States - The children participated in treadmill training on a specialized treadmill that used an inflatable bag to provide partial body weight support 2 days per week for 6 weeks. The air pressure was uniformly distributed over the lower body, which reduced the uncomfortable pressure points that typically occur in more traditional body weight support systems –Body-weight supported treadmill walking (BWSTW) can have a positive impact on nonambulatory patients with CP(83) - Based on a case study of an 18-year-old male patient with CP who was nonambulatory(83) - Training sessions took place twice a week for 6 weeks with a harness system supporting approximately 40% of his body weight(83) - Outcome measures: The over-ground 3-minute walk test distance and associated physiological cost index (PCI) did not change. BWSTW exercise time increased by 67%, and the BWSTW speed increased by 43% while the patient’s working heart rate decreased by 8% on the treadmill. The BWSTW PCI decreased by 26%, and there were improvements in the PEDI in the areas of self-care and mobility(83) –Treadmill gait training might be more effective for improving functional balance in children with CP as compared to over-ground gait training(100) - Based on a randomized controlled trial conducted in Brazil with 15 children with CP (7 randomized to experimental/ treadmill training group and 8 to the control/over-ground gait training) - Treadmill training protocol consisted of two 30-minute sessions per week for 7 weeks; the child was instructed to walk at 60% of maximal speed for the first and final 5 minutes of each session and 80% of maximal for the 20 minutes in between - Although both the treadmill and over-ground gait training protocols led to within-group improvements in functional balance, the mean increase on the Berg Balance Scale score in the treadmill group was 14.7 points whereas the mean increase for the control group was 3.2 points –Researchers in Germany found a high degree of variability in responses to robot-enhanced repetitive treadmill training in patients with early-developed movement disorders that included CP(130) - 83 patients aged 4-18 years participated in treadmill walking for a mean total of 7.2 hours - Determinants of inter-individual variability in treatment responses included gross motor abilities at baseline and age • Postural control techniques –Physical therapy treatment based on dynamics of postural control might be more effective than traditional physical therapy to improve balance in children with CP(63) - Based on a randomized controlled trial conducted in India of 38 children with CP who participated in traditional physical therapy or physical therapy treatment based on dynamics of postural control –Balance training using the Biodex balance system is more effective than traditional physical therapy in improving balance in children with CP(112) - Based on a randomized controlled trial in Egypt - in which 30 children with spastic diplegic CP (GMFM-66 and GMFM-88) were randomly selected to participate in traditional physical only or traditional physical therapy in conjunction with Biodex balance training for 30 minutes a day, 3 days a week, for 3 months - Both groups had significant improvement in balance parameters at the end of 3 months; however, the Biodex training group had greater improvement in balance and stability compared to the control group - Outcome measures were the PBS, fall risk balance testing on the Biodex, static and dynamic balance stability on the Biodex, and directional control on the Biodex • Aerobic training –Research is limited on the effects of aerobic training in the CP population –A series of 3 case studies reported that aerobic training in college-aged students with CP leads to increased combined mean lower extremity strength, at least a partial improvement in the energy expenditure index, increased gross motor function, and improvement in participant self-perception(64) • Neurodevelopmental treatment (NDT) –NDT may not be more effective than other therapeutic strategies in the treatment of individuals with CP(65) - Based on the report by the American Academy for Cerebral Palsy and Developmental Medicine - A more intense NDT program may not yield better outcomes than standard frequency of treatment - Task-oriented approaches, strengthening, endurance training, and use of assistive technologies may yield better results, but more research is needed to support this theory –In a study conducted in Greece, 34 children (mean age, 7 years) with spastic CP (hemiplegia, diplegia, tetraplegia) were randomly allocated to 2 treatment groups, both of which received NDT at varying intensities(66) - Both treatment groups had significant improvements in gross motor function after the 16-week treatment regimen according to the GMFM - The group that underwent intensive (5 days/week) therapy had greater improvements in function than the group who received NDT 2 days/week. Sessions were 50 minutes long in each group • Electrotherapeutic modalities –There is currently insufficient evidence to support the routine use of electrical stimulation to improve gait in children with CP(67,68,69,70,71,72,90) –Neuromuscular electrical stimulation (NMES) combined with dynamic bracing of the upper extremity temporarily improves grip strength, upper extremity coordination and function, and posture in children with CP(73) - Based on a randomized trial comprised of 24 children with spastic hemiplegic CP in the United States • Prescription, application of devices and equipment –Ankle-foot orthosis (AFO) - There is substantial evidence that AFOs that control the foot and ankle in stance and swing phases can improve gait efficiency in ambulant children with CP (GMFCS levels I-III). Use of AFOs may reduce the energy cost of gait(131) - AFOs are typically used to optimize gait dynamics through application of a mechanical constraint to the ankle that controls motion - AFOs might improve plantarflexor tone in the lower extremities of patients with CP(74) - Based on a controlled trial of 30 children with CP conducted in India - All the children received traditional physical therapy. Half of the children wore AFOs during the treatment period - The children who wore AFOs had a greater improvement in plantar flexor muscle tone –Seating/positioning interventions - Optimal seating/positioning interventions have yet to be established; more research with better methodology is warranted to clarify which interventions are superior to others as well as demonstrate improved function as a result of these positioning strategies(75) - The early implementation and use of postural equipment in nonambulatory children with CP may reduce the risk of hip subluxation and the need for surgical intervention at 5 years of age(76) - Based on a prospective cohort study of 39 nonambulatory children with CP diplegia conducted in the United Kingdom - Children were ≤ 18 months at the start of the study and were followed up to the of age 5 years - The children received various methods of postural support, in various positions, in an effort to prevent hip subluxation –Environmental modifications (e.g., hand rails, car wheelchair hoists, portable ramps) improve mobility, social function, and self-care in children with CP, according to their parents(77) - Based on a research study conducted in Norway involving 95 children with CP and their parents - ~ 50% of parents stated the environmental modifications had moderate to very large effect on their child’s independence during mobility; 25% stated it improved independence during self-care;and ~ 20% stated it improved independence during social function - ~ 65% stated that the modifications decreased the level of caregiving during mobility - ~ 75% stated that the modifications decreased the level of caregiving during self-care - ~ 25% stated that the modifications decreased the level of caregiving during social function –A review of the literature, investigating the effect wheelchair positioning had on the upper extremity function in children with CP, compiled the following recommendations:(78) - The child’s pelvis should be held in neutral - A functional sitting position (FSO) is recommended in facilitating the use of the child’s upper extremities - FSO – hip belt, abduction orthosis, a cutout tray, footrests, and orientation in space of 0-15º. Also included was a forward sloped seat of 0-15º - The child’s head, shoulders, and trunk should be slightly anterior to the ischial tuberosities –Early use of powered mobility can have a positive impact on the psychosocial and play skills of children with CP or orthopedic disabilities(86) - Based on a study conducted in the United States of 23 children with CP or another type of disability aged 18 months to 6 years(86) - Significant increases in both parental perception of positive social skills and the number of mobility activities during free play (although no increase in the interaction with toys and objects was seen)(86) • Passive stretching –Results from a systematic review investigating the efficacy of passive stretching in children with CP indicate that supportive evidence for this intervention was minimal and its use as a method of increasing ROM, improving gait efficiency, and diminishing spasticity has not been established. The authors concluded further research is required(57) • Constraint-induced therapy –Constraint-induced therapy can improve grasping ability, daily function, and temporal/spatiotemporal control of reaching in children with unilateral CP(84) - Based on a randomized controlled trial conducted in Taiwan with 24 children with CP receiving constraint-induced therapy and 23 children with CP receiving traditional rehabilitation; children were 6 to 12 years of age and had unilateral spastic CP(84) - Both groups received individualized therapist-basedinterventions at home for 3.5-4 hours per day, 2 days per week for 4 weeks(84) - In the experimental group, the participants wore an elastic bandage and restraint mitt that limited both finger and wrist movement in the less affected arm; therapy sessions involved function tasks (reaching, grasping, manipulating household items) with the more affected arm(84) - At the conclusion of the 4 weeks of the study, the children in the experimental group demonstrated significantly greater improvement on outcome measures(84) - Improved grasping ability measured by the PDMS-2 - Improved functional use of affected arm measured by the Pediatric Motor Activity Log - Improved temporal and spatiotemporal efficiency of reaching as measured by reaction time, movement time, and peak velocity –Modified constraint-induced movement therapy can improve hand dexterity and grip strength in children with CP(91) - Based on a small randomized controlled trial conducted in South Korea(91) - 20 subjects with CP total; 10 received traditional rehabilitation and 10 participated in a modified constraint-induced movement therapy program(91) - All participants participated in 30-minutetraditional sessions that took place semi-weekly;the experimental group additionally received 60-minute constraint-induced movement therapy sessions with the nonaffected arm restrained by a string and splint during which participants were guided through 13 functional activities with the un-constrained hand(91) - At the conclusion of the study, the children in constraint-induced movement therapy group showed significantly greater improvement in the following measures: block and box test (hand dexterity test), grip strength, and WeeFIM(91) –A combination of constraint-induced movement therapy and bimanual training might produce the best outcomes for children with CP(94) - Based on a systematic review that included 7 studies; authors of the review found that both constraint-induced movement therapy and bimanual therapy produced similar improvements in the abilities of the affected arm as well as overall functional performance. Because the constraint-induced movement therapy appears to yield more improvements in unimanual tasks with the impaired arm and bimanual training is more effective for improving performance of bimanual and functional tasks, authors recommended considering an approach to therapy that includes both types of training(94) –Results of a randomized controlled trial conducted in Israel suggest that both modified constraint-induced movement therapy and bimanual therapy provided in a school-basedsetting can lead to improvements in bimanual skill and quality of movement patterns(132) - 12 children with hemiplegic CP were randomized to receive modified constraint-induced movement therapy or hand-arm bimanual intensive therapy for 2 hours per day, 6 days per week in a special education preschool/kindergarten - After 8 weeks of intervention, both groups demonstrated comparable improvement on the Assisting Hand Assessment and the Quality of Upper Extremity Skills Test –In a pilot study conducted in Taiwan, researchers found that group constraint-induced movement therapy could be a feasible and effective alternative to individual, one-on-oneconstraint-induced movement therapy in clinical practice(99) • Vibration treatment –In a randomized controlled trial conducted in Croatia with 89 children with CP, researchers found that the addition of sound-based vibration treatment significantly reduced spasticity and increased motor function as compared to traditional physical therapy interventions alone(87) - Intervention period was 12 weeks, during which time all subjects received three 40-minute sessions per week; children in the intervention group received the addition of sound-based vibration treatment twice per week(87) - Vibration treatment was delivered using a vibroacoustic bedpad; children were placed on the bedpad in supine position for 20 minutes(87) - At the conclusion of the study, the children in the experimental group showed a significantly greater reduction in spasticity (as measured by the Modified Ashworth Scale) and a significantly greater increase in gross motor function (as measured by the GMFM) than the group with traditional physical therapy interventions only(87) - Researchers emphasized that vibration treatment should be used as a complement to traditional physical therapy interventions and should not replace active therapist-basedintervention(87) –A trunk-targeted intervention combined with vibration can improve posture and gait in children with CP(92) - Based on a study conducted in South Africa with 27 children with CP aged 6 to 13 years(92) - Intervention consisted of selective trunk-targetedexercises to activate and strengthen abdominal muscles; all exercises were performed on a vibrating platform; therapy program was 4 weeks in length with 2 sessions in the first week, 3 sessions in the second, and 3 to 4 sessions in the third and fourth weeks(92) - Significant improvements were seen in gait speed, sitting posture, standing posture, and abdominal strength(92) –Whole-body vibration may improve strength and balance in children with CP(114) - Based on a randomized controlled trial conducted in Egypt with 30 children with spastic diplegic CP. All children received traditional physical therapy 1 hour a day, 5 days a week for 3 months. In addition to traditional therapy, the experimental group received whole-body vibration training for 9 minutes a day, 5 days per week for the 3 months - Using the Biodex balance system, the children in the whole-body vibration group showed a significant improvement in knee extensor peak torque at 60°/sec and 90°/sec and overall stability index compared to the control group –Whole-body vibration training can improve mobility in children with CP(103) - Based on a randomized controlled trial conducted in South Korea with 30 children with CP. All children received traditional physical therapy three times per week for 8 weeks and 15 of the children also underwent whole-body vibration treatments in addition to physical therapy(103) - Whole-body vibration took place on a vibrating platform bed on which participants progressed more deeply to a squat position from 30 to 100° of knee flexion - Improvements in gait speed, stride length, cycle time, and ankle angle were significantly greater in the experimental (vibration) group –For additional information on whole-body vibration training, see Clinical Review…Whole-Body Vibration Training: an Overview; Item Number: T709253

. Problem Goal Intervention Expected Progression Home Program Gross motor delay Maximize age- NDT, functional Progress each patient Implement a home appropriate gross motor training, GDT as indicated and program that function _ appropriate incorporates activities Referral to adaptive that foster carryover of physical education newly acquired skills programs as indicated _ _ _ _ Ensure patient and (See Treatment parent/caregiver receive summary) proper education on home program Gait deviations Improve gaitwith Gait training, ankle Progress each patient Implement a home with decreased increased strengthening, GDT, as indicated and program that independence independence, balance training appropriate incorporates activities improved energy _ that foster carryover of efficiency, and (See Treatment newly acquired skills appropriate use of summary) adaptive equipment as _ indicated _ Prescription, application of devices and equipment _ Assess need for assistive devices and adaptive equipment and implement as indicated _ _ (See Treatment summary) Decreased functional Increase independence Functional training, Progress each patient Implement a home capacity/impaired with ADLs and leisure strength training as indicated and program that ability to perform activities, including _ appropriate incorporates activities ADLs and leisure mobility on/off Referral to adaptive that foster carryover of activities playground equipment physical education newly acquired skills programs _ _ Prescription, application of devices and equipment _ Assess need for assistive devices and adaptive equipment, and implement as indicated _ Constraint-induced therapy _ _ (See Treatment summary) Contractures of muscle Prevent secondary Functional training Progress each patient Implement a and joint tissue complications such as _ as indicated and home program _ contractures, decreased ROM, stretching, and appropriate that incorporates _ bone mineral density, positioning programs as maintenance of Decreased bone mineral pain, and orthopedic indicated functional ROM density (osteopenia/ deformities as able _ osteoporosis)(8) _ Assess need for orthotics _ _ (See Treatment summary) Musculoskeletal Independent with Prescription, Progress each patient Home program should deformities such orthotic management application of devices as indicated and incorporate orthotic as scoliosis and and equipment appropriate management hip dysplasia. Hip _ dysplasia can lead Assess need for to hip subluxation/ assistive devices and dislocation adaptive equipment, and implement as indicated _ _ Referral to an orthotist as indicated _ _ (See Treatment summary) Decreased strength, Improve strength Functional training Progress patient as Provide patient and muscle imbalances, and _ _ appropriate family/ caregivers with atrophy _ Therapeutic exercise written instructions Improve muscle can be implemented to regarding functional balance strengthen weakened activities that can be _ muscles(21) performed at home as _ _ indicated Reduce/prevent atrophy _ For the younger child strengthening can be accomplished through play and functional/ developmental activities _ _ (See Treatment summary) Decreased endurance Improve endurance Functional training, Progress as indicated Implement a home _ aerobic training, gait program that Increase daily physical training incorporates endurance activity _ activities (See Treatment summary) Muscle tone Normalize muscle tone Interventions to N/A Implement a home abnormalities as able normalize muscle tone program that _ _ _ incorporates tone _ _ Stretching may serve to management techniques Abnormal postural tone Improve postural decrease hypertonicity _ alignment for brief periods (e.g., _ hours)(21) Persistent neonatal _ reflexes _ Therapeutic exercise should be implemented to strengthen weakened muscles(21) _ _ Teach caregiver/patient tone management techniques _ _ (See Treatment summary) Decreased coordination Improve coordination Functional training Progress as indicated Balance activities _ _ _ may be added to the _ _ Incorporate strategies patient’s home exercise Decreased balance Improve balance to improve the patient’s program as indicated. reactions reactions balance Ensure the caregivers _ _ _ can safely carry out the _ _ _ prescribed interventions Decreased protective Improve protective (See Treatment reactions reactions summary) Skin breakdown due to Prevent skin breakdown Integumentary N/A Home program should impaired sensation _ repair and protection include the care/ _ techniques prevention of wounds Increase awareness of _ as indicated sensory limitations/ Instruct the patient pressure-relieving and family how to strategies perform pressure- relieving techniques in the wheelchair or in bed _ _ Any wounds should be staged and monitored closely Poor alignment in Improve alignment in Prescription, N/A Home program should wheelchair wheelchair application of devices incorporate positioning and equipment strategies _ Complete a wheelchair assessment as appropriate and indicated _ _ Inquire about primary use of wheelchair (e.g., home, community, school, all settings) _ _ Clinicians must recognize the diverse needs of children with CP and the importance of a custom-built wheelchair. Ensure there is room for growth as funding may be limited _ _ (See Treatment summary) Nutritional deficits Reduce nutritional Therapeutic N/A N/A due to feeding deficits intervention difficulties/oral motor _ dysfunction(8) Refer to appropriate specialists as indicated

.

Desired Outcomes/Outcome Measures › Desired outcomes • Attainment of gross motor milestones • Improved gait pattern with increased independence • Appropriate use of adaptive equipment • Increased independence with ADLs and leisure activities • Restored or maintained ROM • Prevention of secondary complications such as contractures, decreased bone mineral density, pain, skin breakdown, and orthopedic deformities as able • Independence with orthotic management • Improved strength, reduced/prevention of atrophy • Improved endurance • Normalized muscle tone as able • Improved postural alignment, improved alignment in wheelchair • Improved coordination • Improved balance and protective reactions • Increased awareness of sensory limitations/pressure-relievingstrategies • Increased daily physical activity › Outcome measures • Goniometric measurement of ROM • MMT, handheld dynamometry • Skin integrity • Measures of endurance (6MWT) • Independence during functional activities • Use of assistive/adaptive devices • GMFM, TIMP, WeeFIM, PDMS-2, PEDI, GAS, BOT-2, PBS, COPM, MACS, AMPS • Accelerometry, self-reported physical activity

Maintenance or Prevention › Children and adolescents with CP are less likely to participate in sports, clubs, and organized activities compared to peers without CP or developmental disabilities(120) › Adults with CP have been shown to participate in lower levels of physical activity and spend increased time in sedentary behavior(133) › Results of a randomized controlled trial conducted in the Netherlands indicate that a lifestyle intervention program geared toward more favorable physical behavior in adolescents and young adults with spastic CP was not effective in increasing physical activity(134) • 57 patients with spastic CP aged 16 to 25 years completed the study • A 6-month lifestyle intervention, consisting of fitness training and counseling on physical behavior and sports participation, was implemented • Physical activity was measured objectively using activity monitor and subjectively using self-reported Physical Activity Scale for Individuals with Disabilities • The intervention was ineffective at eliciting the desired behavioral change › Healthcare services to manage the needs of adults with CP are often inadequate.(123)Children and adolescents with CP would benefit from more proactive planning for the transition to adult health care to continue to receive optimal intervention(120) › Home exercise program should be implemented with a focus on: • Preventing ROM loss • Preventing pressure sores • Orthotic wearing schedule • Exercises/activities to maintain strength and function

Patient Education › National Institute of Neurological Disorders and Stroke, http://www.ninds.nih.gov/disorders/cerebral_palsy/ cerebral_palsy.htm › United Cerebral Palsy, http://www.ucp.org/

Coding Matrix References are rated using the following codes, listed in order of strength:

M Published meta-analysis RV Published review of the literature PP Policies, procedures, protocols SR Published systematic or integrative literature review RU Published research utilization report X Practice exemplars, stories, opinions RCT Published research (randomized controlled trial) QI Published quality improvement report GI General or background information/texts/reports R Published research (not randomized controlled trial) L Legislation U Unpublished research, reviews, poster presentations or C Case histories, case studies PGR Published government report other such materials G Published guidelines PFR Published funded report CP Conference proceedings, abstracts, presentation

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