Cerebral Palsy General Assessment

Introduction

Cerebral palsy (CP) is a non-progressive neuromotor disorder. The primarily impairments secondary to CP include movement, muscle tone and posture along with a range of secondary conditions over time which may affect functional abilities. Injury to the developing brain in the prenatal through neonatal period is the underlying pathophysiology for CP.

General Diagnosis

In the peadiatric practice setting, it is difficult to make a certain diagnosis of CP during the first 1-2 years of life. During this time period, delays in development could be part of a normal variation and may resolve. Therefore, a more reliable diagnosis is made after 2 years of age based on clinical findings typically:

• failure to attain certain key milestones at an expected age
• persistence of primitive reflexes or
• primary motor patterns beyond the expected age

Despite a definitive diagnosis being made after the age of 2, a diagnosis can be made for "high risk of CP" prior to this age. This risk category requires motor dysfunction and either an clinical history indicating risk of CP and/or MRI scan abnormality. Novak et al., (2017)proposed ways to predict CP in low birth weight infants:

• infants ≤5 months corrected age: term-age magnetic resonance imaging (MRI) (86–89% sensitivity), the Prechtl Qualitative Assessment of General Movements (98% sensitivity), and the Hammersmith Infant Neurological Examination (90% sensitivity)
• infants ≥6 months corrected age: MRI (86–89% sensitivity), the Hammersmith Infant Neurological Examination (90% sensitivity), and the Developmental Assessment of Young Children (83% C index)
  

Specific Tests

The General Movements Assessment

The General Movements Assessment has a high sensitivity and specificity in the prediction of cerebral palsy and can be used in children from birth to 20 weeks of age (corrected for prematurity).14 The best age to perform the assessment is 12–14 weeks after the baby’s due date, and is therefore best suited to high risk neonatal follow-up. The assessment is made from a 3–5-minute video of the child’s movements taken in a standardised method

The Hammersmith Infant Neurological Examination

The Hammersmith Infant Neurological Examination is not a new tool nor is it specific for cerebral palsy, but it has recently been promoted for use in high risk infant follow-up programs.16 It can be performed from age 2 months to 2 years and is fast to perform and score, but some training in its use is recommended. Using this standardised neurological assessment provides a framework for monitoring and allows early identification of deviation from normal development, facilitating faster referral for diagnostic assessment and treatment.16 It has been shown to have high sensitivity and specificity for predicting cerebral palsy, with defined cut-off scores corresponding to severity of potential cerebral palsy. This assessment helps identify children at risk of cerebral palsy who do not have immediate infant risk factors.erebral palsy is a developmental disorder of movement and posture which is often associated with comorbidities. • While there is currently a limited range of evidence-based treatments that change the underlying pathology of cerebral palsy, there are many areas in which health care professionals can change the natural history of cerebral palsy and improve participation and quality of life for children with this condition. • Early identification has become of paramount importance in the management of cerebral palsy, and it is hoped that it will allow earlier access to cerebral palsy interventions that may improve the natural history of the condition. • Common challenges in the management of cerebral palsy include spasticity and dystonia, management of pain, hip surveillance, sleep and feeding, swallowing and nutrition. • The six Fs framework (function, family, fitness, fun, friends and future) provides a guide to developing shared goals with families in the management of cerebral palsy. 130 Narrative reviews MJA 210 (3) ▪ 18 February 2019 high sensitivity and specificity for predicting cerebral palsy, with defined cut-off scores corresponding to severity of potential cerebral palsy. This assessment helps identify children at risk of cerebral palsy who do not have immediate infant risk factors.

The Gross Motor Function Classification System (GMFCS) is used to describe gross motor function, especially the ability to walk, for children from 2 to 18 years of age (19,20). GMFCS is used to describe self-initiated movements as well as movements assisted by devices such as walkers, crutches, canes or wheelchairs (19,20). The Manual Ability Classification System (MACS) is used to describe the typical use of both hands and upper extremities for children from 4 to 18 years of age (21). The Communication Function Classification System (CFCS) is used to describe the ability of persons with CP for daily routine communication (sending or receiving a message) (22). The CFCS considers all methods of communication including vocalizations, manual signs, eye gaze, pictures, communication boards or speech generating devices (22). The Eating and Drinking Ability Classification System (EDACS) is used to describe the eating and drinking function for children 3 years and older (23). The EDACS assesses the eating and drinking safety (risk for aspiration or chocking), and eating and drinking efficiency (the amount food lost and the time taken to eat) (23).and TABLE 3

For this purpose, four systems are used for functional classification of CP which include GMFCS, MACS, CFCS, and EDACS

Treatment

Cerebral palsy management is aimed at improving functional ability and independency and managing secondary complications. Physical and occupational therapies, mechanical aids, orthopedic surgery to address patients’ motor problems, and optimal medical and surgical treatment of medical comorbidities are the main management strategies [35]. An increase in neonatal care and decreased prevalence showed a promising impact on early diagnosis [4]Addressing functions like physical issues, cognition, communication, eating and drinking, vision, and sleep helps in improving the overall health of the child, and cooperation of the family and environment modification are the major factors for improvement Management of CP children requires a team approach which includes a list of multidisciplinary team members such as an audiologist, medical social worker, nurse, nutritionist, occupational therapist, pediatric gastroenterologist, pediatric neurologist, pediatric orthopedic, surgeon, pediatric pulmonologist, pediatric surgeon, pediatrician, physiatrist, physiotherapist, psychologist, speech-language therapist, and special educator [10]. Many recent advances in the management of CP have come up including intrathecal baclofen, selective dorsal rhizotomy, and sensory integratio

A multidisciplinary team (Table 4) approach provides the best model for medical care of children and adults with CP across their lifespan to manage various associated and secondary conditions as well as address support system and psychosocial need- TABLE 4

The following CP interventions were shown to be effective (“green light go interventions”): 1) botulinum toxin, diazepam, selective dorsal rhizotomy for reducing muscle spasticity, 2) casting for improving and maintaining ankle range of motion, 3) hip surveillance for maintaining hip joint integrity, 4) constraint-induced movement therapy, bimanual training, context-focused therapy following botulinum toxin, home programmes for improving motor activity performance and/or self-care, 5) fitness training for improving fitness, 6) bisphosphonates for improving bone density, 7) pressure care for reducing the risk of pressure ulcers, 8) anticonvulsants for managing seizures. The following therapies were shown to be ineffective for improved motor activities and self-care in children with CP (“red light stop interventions”): craniosacral therapy, hip bracing, hyperbaric oxygen, neurodevelopmental therapy, sensory integration. The authors suggested that these interventions should be discontinued from CP care.91

Spasticity

reatment of spasticity involves systematic rehabilitation, if necessary, assisted by pharmacotherapy, physiotherapy or surgical interventions. Selection of a pharmacological treatment method depends on the intensity and location of the disease. In the case of generalized spasticity, the most frequently applied is general treatment. The main drugs used in this kind of treatment include baclofen – an analogue of gamma-aminobutyric acid (GABA), acting on the spinal cord, and benzodiazepine derivatives, such as diazepam, clonazepam or tetrazepam (these act on the central nervous system by releasing endogenic GABA). In the case of focal spasticity, one of the basic therapies is intramuscular administration of botulinum toxin type A (botulin). Botulinum toxin hinders the secretion of acetylcholine in neuromuscular junctions, resulting in a decrease of tension in the group of muscles subjected to treatment. Multi-level administration of botulinum toxin can be effective in the treatment of generalized spasticity. The improvement lasts approximately 3–8 months. At that time, systematic motor rehabilitation is necessary so as to make full use of the botulinum toxin effect. By reducing spasticity, botulinum toxin increases the range of passive and active motion, facilitates posture correction, and reduces discomfort and pain related to enhanced muscle tension, which is also felt during rehabilitation. Treatment with botulinum toxin results in a better quality of life for children with CP, both in the case of focal spasticity being confined to single muscle groups, and generalized spasticity. Botulinum toxin can also reduce pain after orthopaedic operations in children with spastic types of CP.81–86

The most common movement disorders seen in cerebral palsy are spastic muscles and dystonia with difficulties in coordination, strength, and selective motor control. Spasticity is the major challenge in the management of CP children. It causes spasticity-induced bone and joint deformity, pain, and functional loss [10]. Commonly used medicines found in the literature to relieve spasticity are baclofen, diazepam, clonazepam, dantrolene, and tizanidine. Baclofen and diazepam help in relaxing the muscles but have many side effects [27]. First-line treatment for spasticity is physiotherapy, occupational therapy and botulinum toxin injections, selective dorsal rhizotomy, and intrathecal baclofen [8]. A selective dorsal rhizotomy is a surgical procedure that is effective in CP children that improve their walking ability and range of movement. It rectifies the spasticity that impairs gait by improving the ankle joint junctions [41]. Administration of intrathecal baclofen is done via an implantable pump. It is reserved for GMFCS levels IV and VI as it is used in extreme spasticity cases. However, it is expensive, and relief is of short duration. Intramuscular onabotulinum toxin type (Botox) weakens the skeletal muscles by impairing the release of neurotransmitters at the NMJ. This slows down the contraction of skeletal muscle. The injection is first given between 18 and 24 months of age [8, 10]. Surgical management including lengthening of the soft tissues such as adductors and hamstrings, multilevel surgery of the ankle and foot, nerve blocking, tendon transfers, and joint stabilization are some of the surgical techniques used in CP age appropriately [27].

Management of Balance and Movement Disorder

he degree or severity of spasticity in CP varies depending upon the stage of arousal of the child at the time and the duration since the inciting event that lead to spasticity (11,16,28-32). Muscle spasticity in a child with CP may interfere with certain functions as well as may serve to facilitate certain functions. Therefore, reduction of spasticity should be considered within the context of its functional impact and multiple factors (Table 7) need careful consideration Different treatment interventions (Table 8) have been used to treat spasticity in children with CP (11,16,28-32). The decision to use any particular treatment intervention is guided by the goal of the treatment. In some cases, the goal may be to reduce focal spasticity, whereas in others it may be to reduce generalized spasticity. Also, the risks and benefits of any particular intervention should be carefully considered. Physiotherapy and occupational therapy by age 4–5 years of age have been shown to be relatively more effective than if started at a later age (33,34). Botulinum toxin injection is used to treat focal spasticity with optimal effectiveness between 1 and 6 years of age for the treatment of lower extremity spasticity and between 5 and 15 years of age for spastic hemiplegia (11,16,28-32). Spasticity management is best guided by a physician with expertise and experience with the use of different treatment interventions.TABLE 7,8

Management of Balance and Movement Disorder

Balance and movement disorders are crucial management issues in CP children as they are necessary for maximum activities of daily living. The traditional management approach in CP includes physiotherapy, occupational therapy, hyperbaric oxygen therapy, sensory integration, NDT, hippotherapy, CIMT, BWSTT, acupuncture, and the Vojta method [8, 42]. Two treatment techniques called whole-body vibration along with core stability exercises are found to be effective in managing balance issues, the former being more effective [43]. Another therapy having prospects of improving balance is virtual reality. Giving it for 20 minutes, twice a week for 6 weeks gives very good results in balance improvement [44]. Nintendo Wii therapy is another balance-improving treatment that can be considered an effective treatment for improving functional and dynamic balance. It can be combined with physiotherapy techniques for 30 minutes for a minimum of 3 weeks for effective results [44]. Management of movement disorders however for CP patients includes trihexyphenidyl, tetrabenazine, baclofen, levodopa, benzodiazepines, and deep brain stimulation

Management of Hand Dysfunction

Injury in the brain may cause disturbances in hand functioning which may be unilateral or bilateral. In the former case, motor control and function on one side of the body are affected. In this type of CP, children experience difficulties using their hands on the affected side. Constraint-induced movement therapy (CIMT) is a technique that is being used to improve the function of the affected hand. It is based on the principles that not using the good hand and intensive use of the affected hand improves hand function by neuroplasticity of the brain. A study reveals that the therapeutic effect of CIMT is independent of age. No differences were found between boys and girls for this therapy. CIMT is found to be effective in the literature to improve hand function; however, its effect on muscle tone and protective extension is yet to be investigated. Hand-arm intensive bimanual therapy is a similar technique that improves hand function; however, both hands need to be used in this technique. In a study of children with hemiplegic cerebral palsy, it is found that both of these strategies are promising techniques to improve hand function; however, the latter is more tolerable in children than CIMT. CIMT has also shown improvement in somatosensory functioning and neural processing in such children [46–49].

Management of Hip and Ankle Deformities

36% of CP children suffer from hip disorders which lead to problems such as dislocation, subluxation, and other related problems which can be managed surgically. Hip surveillance programs are recommended to screen cases of hip deformities [40]. In younger children who cannot walk due to hip disorders, reconstructive procedures are useful as they provide long-term results; in cases of degeneration of the hip, reconstruction surgeries such as osteotomy or arthroplasty are done [41]. In ankle equines, the deformity is seen in cerebral palsy children. Orthotic devices can help improve the ankle range which is beneficial in improving the gait of the child. Specific types of AFOs improve joint function and gait parameters. AFOs reduce energy expenditure in children with spastic CP. The HKAFO is very helpful in improving gait parameters and is evident in energy conservation in hemiplegic CP children. Further studies are required for better evidence regarding this [50–52].

Orthopedic surgical procedures

Children with CP develop multiple secondary, often progressive, musculoskeletal conditions (Table 9) that may require orthopedic surgical interventions that are best managed by orthopedic surgeons with experience and expertise in these surgical procedures (6,10,13,16,33,34). The type and severity of these conditions vary depending upon the type and severity of CP. A number of factors are considered in planning any of these surgical interventions that include the age of the child, severity and progressive or nonprogressive nature of the condition, support system for post-operative and long term follow-up and care, potential for functional improvement, and potential for amelioration or prevention of complications. A common practice consideration is to perform most procedures as a single event multiple level surgery (SEMLS) to avoid multiple exposure to anesthesia risk and other operative risks (16,33). Also, this approach allows for a planned course of post-operative rehabilitation.- TABLE 9

PT

Physiotherapy has been shown to improve muscle strength, local muscular endurance and joint range of movement in children with CP (35,36). Physiotherapy exercises are used to prevent or reduce joint contractures; this is achieved by passive gentle range of motion exercises and stretches across major joints. Increased muscle strength is achieved by performing regularly scheduled progressively increasing resistive exercises involving all major muscle groups. Low resistance, high repetition exercises of major muscle groups improve local muscular endurance. Specific physiotherapy exercises are designed to improve balance, postural control, gait, and assist with mobility and transfers (for example from bed to wheelchair). Functional strength training combined with plyometric exercises and balance training have been used to improve function in individuals with CP (31,35-38). Plyometric exercise improves muscle power, which includes strength and speed (35,36). In regards to functional strength training, studies have shown that targeting specific muscles is most effective in muscle activation (35,36,38). A study has shown that 12 weeks of an adaptive bungee trampoline program improved lower limb muscle strength (39). This bungee trampoline program included bouncing, hopping, heel jumps, jumping with eyes closed, practicing a sequence of jumps, and games such as dodgeball. The use of constraint-induced therapy centers on the idea of selective upper extremity strengthening in children with CP (8,12-14,40,41). The intervention focuses on having the child use the affected limb, while simultaneously restraining the use of normally functioning limb. Prolonged restraint and disuse of the normally functioning upper limb may result in disuse weakness. The application of conductive education based on the concept that children with or without motor deficits learn the same way (14,30,31,35,42). The conductive education specialist attempts to unify developmental areas including emotional, cognitive, motor and communicative domains in order to improve integration and global functionality in participants (14,30,31,35,42). The effectiveness of conductive education (CE) in improving functional capabilities of children with CP has not been clearly established (14,30,31,35,42).

Physiotherapy

Physiotherapy has provided great achievement in the field of cerebral palsy. It helps in improving the muscle structure and function and joint range of motion and reduces contractures; some techniques used to achieve this are muscle stretching, joint range of motion exercises, low resistance repetitive exercises, progressive resistance training, functional strength training, balance training, plyometrics, and selective muscle activation by techniques such as constraint-induced movement therapy. A study was done on the effects of neurodevelopmental therapy in CP children which revealed improved function in various activities of children after the application of the intervention technique. NDT also reduced spasticity and improved overall function in CP children; however, there was not much improvement in walking, running, and jumping [8, 10, 17, 21, 64]. Another emergent therapy called hippotherapy has improved neck control and posture control in sitting along with the upper extremity and trunk. There is an overall posture improvement due to stimulation of balance reactions which has a positive effect on balance and spasticity. 30–45 min sessions, twice weekly for 8–12 weeks, produce a positive effect on gross motor function in children with CP [18]. Deep brain stimulation in the case of dyskinetic CP and electrical stimulation via tens and NMES in spastic CP are two techniques to improve the strength and function of muscles [8]. Serial casting is a technique used to stretch tight muscles to improve the range of motion by application of a cast to the affected part [10]. The robot-assisted gait training regimen is effective in improving gross motor function in children whose both sides are affected. There was a positive effect on all the measures of gross motor function after this intervention. It also improved the locomotor ability in ambulatory children [19]. Functional gait training or practice walking on a treadmill with limited body weight support helps in standing erect with a decreased load on the lower extremity joint. This facilitates gait training with good posture and control and is most effective in GMFCS grades IV and V. It can be done with or without a treadmill. Virtual reality and biofeedback can be incorporated; it produces a positive effect [19, 20]. Biofeedback is a common strategy used in rehabilitation that can be used to represent any biological parameters and their changes. The changes can be detected in a variety of ways such as visual, audio, and haptic responses. It is effective in improving motor function by identifying effective motor performance and motor learning [65].

Treadmill training

Treadmill training as an intervention for children with CP aims at improving balance as well as lower extremity symmetry (10,30,31,35,43-46). It provides important measures in developing an understanding of how to walk independently. Specific methods of treadmill training vary, with protocols demonstrating differences in training speeds (varied based on age at intervention), use of or lack of body weight support techniques (support under the arms or with utilization of a harness on the patient while on the treadmill), and frequency or duration of the training (10,30,31,35,43-46). Studies have demonstrated that 3–4 sessions per week over a period of 3–4 months of treadmill training in children under 6 years of age with ambulatory capability have led to improvement in gait velocity and enhancement of stepping movements, as well as independence with walking (10,30,31,35,43-46).

Treadmill training is time and labor intensive. Robotic gait training has been shown to reduce the time and labor burden associated with traditional treadmill training (47). Robotic assisted device can harness the child appropriately and can be programmed to simulate normal gait. Studies have shown increased walking speed and endurance with the use of robotic gait training. Randomized controlled trials have shown effectiveness of robot assisted gait training in improving gait velocity, spatiotemporal station, and endurance in children with CP (47).

Occupational therapy

Occupational therapy is an integral component in the interdisciplinary treatment of individuals with CP, with various studies demonstrating its long-term effects on promoting improvement in fine motor functionality (35). A major focus of occupational therapy is to improve fine motor function of upper extremities to assist the child in performing activities of daily living more efficiently. Occupational therapist also works in organization of child’s play areas, providing adaptive equipment for self-care and learning and to modify child’s learning environment to facilitate attention and information processing (35).

Orthotics, adaptive equipment, and assistive technology

In the long-term management of children with CP, it is important to determine how much assistance is required on a daily basis for optimal functioning. Orthoses, adaptive equipment and assistive technology devices (Table 6) are used to improve child’s functional abilities and facilitate activities of daily living (13,14,32-34,48-50). Assistive technology plays an important role in the management of persons who have CP and other developmental disorders. According to the United States Individuals with Disabilities Education Act (IDEA), the term “assistive technology device” means any item, piece of equipment, or product system, whether acquired commercially off the shelf, modified, or customized, that is used to increase, maintain, or improve functional capabilities of a child with a disability (48). The term does not include a medical device that is surgically implanted, or the replacement of such device. The term “assistive technology service” means any service that directly assists a child with a disability in the selection, acquisition, or use of an assistive technology device. A significant amount of variability exists within adaptive technology for children with CP, as these devices are ideally tailored to the individual’s existing muscle constraints.

Others

Many other specific interventions or intervention approaches have been used in the treatment of CP; however, the evidence for effectiveness and recommendation for routine use of such interventions is equivocal and limited. Some of these interventions or approaches include acupuncture, neurodevelopmental training, sensory integration, electrical stimulation, suit therapy, hippotherapy, music therapy, video game therapy, and stem cell therapy

BTX-A produces a dose-related temporary tone reduction of the muscle injected by inhibiting the release of acetylcholine from the motor endplates [5]. BTX-A treatment is most often indicated for spasticity problems in a limited number of muscles, both in children with unilateral and bilateral spasticity. SDR is a neurosurgical procedure that involves partial sensory deafferentation at the lumbar and first sacral nerve rootlets. This procedure results in permanent reduction of muscle tone in the lower limbs [6]. SDR is most often used for people with walking ability and generally high spasticity level in both lower extremities. ITB is a continuous administration of baclofen into the intrathecal space from an implanted pump and through a catheter entering the spinal canal. Baclofen reduces the increased muscle tone from spasticity and/or dystonia, by binding to GABA-receptors and blocking excitatory neurotransmitters [7]. The baclofen dosage can be adjusted by telemetry. ITB is most often used for people with severe gross motor impairment with a generally high spasticity level.