Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ONGOING

spastic hemiplegia

1st line – occupational therapy (OT), physiotherapy (PT), and speech therapy

Back
ONGOING
|
spastic hemiplegia
1st line – 

occupational therapy (OT), physiotherapy (PT), and speech therapy

Therapy is recommended early (0-3 years), when the clinical picture is evolving and parents are learning about their child's needs and abilities. OT and PT are directed towards strengthening of weak muscles, stretching of shortened muscles, and coordination activities, with an emphasis on bimanual upper-extremity activities and encouraging symmetry of gait and posture.[132]

Therapies are tailored to the individual patient. Evidence supports the use of training-based interventions, such as bimanual training, constraint-induced movement therapy (CIMT), goal-directed training, mobility training, and treadmill training.[63] Bottom-up, generic, and/or passive motor interventions (e.g., neurodevelopmental treatment) are less effective or ineffective.[63] The emphasis is on practising real-life tasks and activities, using active movements, targeted at achieving an identified goal.

There is some evidence that exercise interventions and fitness programmes have positive effects on function, social participation, self-perception, and quality of life.[133][134][135][136] Even in children who are non-ambulatory, as little as 6 weeks of exercise intervention may improve gross motor function.[137]

CIMT is a treatment approach for children with hemiplegic CP that involves restraining the use of the less affected limb (e.g., using a splint, mitt, or sling) and intensive therapeutic practice of the more affected limb. One Cochrane review concluded that CIMT was more effective at improving bimanual performance and unimanual capacity in children with hemiplagic CP than standard care or other low-intensity interventions (e.g., OT), and was as effective as intensive or dose-matched comparators (e.g., individualised OT, bimanual therapy).[138]

Therapy combining soft constraint, bimanual play, reach training with a sticky mitten, graduated motor-sensory training, and parent education was effective in children aged under 2 years with asymmetric CP. These therapeutic approaches avoid sensory deprivation that may occur in other forms of constraint therapy.[139][140] 

Mechanically assisted walking training aims to help children with CP to walk further by using equipment such as a treadmill, a gait trainer (a wheeled walking aid), or a robotic training device. One Cochrane review concluded that mechanically assisted walking training probably improves walking speed (with or without body weight support) and may improve motor function (with body weight support). There was some evidence of a greater beneficial effect of such training without body weight support compared with overground walking for improving speed and function. However, the clinical significance of the results is unclear.[141]

The importance of considering the environment or context of interventions has been highlighted.[63] Context-focused therapy emphasises modification of the task or environment, including the use of compensatory movements, to achieve a functional task. Compensatory strategies and environmental adaptations ought to be considered for every child in attempting to improve their overall function.[63][108]

Interactive adaptive gaming offers the opportunity for participation and social interaction.[155] There is some evidence that virtual reality training may be as effective as conventional interventions for improving motor function in children with CP.[156][157][158]

Speech therapy is directed towards educating and assisting the family in helping the child achieve developmental milestones and overcome communication difficulties, which may be underestimated because of the severity of motor disability.

Consider – orthoses

Back
ONGOING
|
spastic hemiplegia
Consider – 

orthoses

Additional treatment recommended for SOME patients in selected patient group

May be used to improve function and maintain range of motion during walking or upper-extremity activities.[148][149][150][151]

Casting is used to correct deformity, while splinting is used to maintain correction and to prevent recurrence. Bracing and casting are also used to enhance function.

Spasticity that is not well managed may develop into a fixed contracture, although the severity of contracture and deformity tends to be less in patients with hemiplegia than in those with more global involvement. Contractures are best prevented by a home exercise programme of stretching combined with appropriate positioning to maintain adequate range of motion. Fixed contractures are treated by serial casting and/or intensive physiotherapy. Once achieved, range of motion is maintained by bracing or splinting.

Spasticity that interferes with walking may be managed by an orthosis to correct the gait deviation. Care must be taken to determine the specific impairment causing the gait deviation to ensure the most appropriate form of orthosis is used. Excessive plantar flexion may require an ankle-foot orthosis to assist with dorsiflexion during swing or to prevent excessive plantar flexion during stance.

Consider – adaptive equipment

Back
ONGOING
|
spastic hemiplegia
Consider – 

adaptive equipment

Additional treatment recommended for SOME patients in selected patient group

Therapy is directed at providing patients with necessary equipment aids depending on their physical abilities.

Aids for walking include braces, crutches, canes, and walkers. Patients with poor trunk control may use a gait trainer, which also provides trunk and pelvic stability. Children with difficulty advancing the limbs during swing may use a reciprocating gait orthosis. Gait trainers and reciprocating orthoses are typically used with assistance for therapeutic purposes and are not considered independent mobility aids.

For non-ambulatory patients and those with walking limitations, mobility aids such as specially adapted scooters, tricycles, and automobiles, as well as positioning equipment for electric and non-electrically driven wheelchairs, may be used. Power mobility should be considered from an early age to promote independence.

Adaptive technology both to enhance communication and to enable access to computers should be provided. Appropriate augmentative and alternative communication systems should be made available to children at an early age if required. Provision should be re-assessed regularly to ensure that aids are still appropriate as the patient’s needs and abilities change.[56][153][154]

Consider – injectable pharmacological therapy

Back
ONGOING
|
spastic hemiplegia
Consider – 

injectable pharmacological therapy

Additional treatment recommended for SOME patients in selected patient group

Injectable therapy for spasticity includes botulinum toxin type A.[63]​[109][110][111]​​[112][113] Phenol or ethyl alcohol may be used for severe spasticity if botulinum toxin is not available, but they are less effective and are associated with more adverse effects than botulinum toxin.[63][114]

Botulinum toxin type A acts locally in the injected muscle. There is a delayed onset of action of 1-2 days; muscle consistency is altered by day 10. It temporarily weakens muscle but is followed by re-innervation, accounting for the short duration of therapeutic effects (typically 3-4 months).

Botulinum toxin type A products have shown efficacy in improving upper limb spasticity (as an adjunct to OT), lower limb spasticity, gait, joint range of motion, satisfaction, and pain in children with CP.​[112][113][159]​ 

Botulinum toxin type A products are approved for use in adults with upper limb spasticity and cervical dystonia. OnabotulinumtoxinA (botulinum toxin type A) and abobotulinumtoxinA (botulinum toxin type A) are approved for upper limb and lower limb spasticity caused by CP in children aged 2 years and over. IncobotulinumtoxinA (botulinum toxin type A) is approved for the treatment of chronic sialorrhoea in adults and children aged 2 years and older. OnabotulinumtoxinA is also used for drooling in adults and children; however, this is an off-label use and there are no approved dosing schedules. Botulinum toxin type B is approved in some countries for treating cervical dystonia and chronic sialorrhoea in adults, but is not widely used in CP.

Mild transient side effects of botulinum toxin type A product lasting 1 to 2 days (local rash, flu-like symptoms, constipation, weakness) may occur. Rare adverse reactions, possibly dose-related, include respiratory distress and dysphagia, although a direct causal relation to botulinum toxin has not been established.[112][113] Use is contraindicated in children with respiratory compromise, severe dysphagia, or presence of neuromuscular junction disease (e.g., Eaton-Lambert syndrome, myasthenia gravis). It must not be given with aminoglycoside antibiotics or tubocurarine-type muscle relaxants. AbobotulinumtoxinA is contraindicated in patients with hypersensitivity to any form of botulinum toxin or to cow’s milk protein. IncobotulinumtoxinA is contraindicated in patients with hypersensitivity to sucrose or albumin.

There is some evidence of muscle damage and incomplete recovery after botulinum toxin type A treatment, particularly in shorter-term animal models.[161][162] Following recommended dosing schedules, including repeating injections no more than twice a year, along with accurate placement of the medication using ultrasound and/or electrical stimulation, are ways to minimise damage. Studies suggest that humans recover up to 90% of pre-injection muscle strength and volume at 1 year and that injections should be accompanied by an organised exercise programme for muscle strengthening.[162] 

It is important for practitioners to note that doses differ for each botulinum toxin type A and they are not interchangeable.

Phenol and ethyl alcohol may be used for severe spasticity when botulinum toxin type A is not available. Phenol is used mainly for motor nerves or during open surgical procedures where branches of nerves may be electrically stimulated to ensure they are purely motor, or for persistent elbow flexion. Phenol may be injected using the closed motor point or the open surgical method. Use a slightly higher concentration for closed injection into the motor point. Phenol is contraindicated for mixed or sensory nerves due to potential dysaesthesias. Ethyl alcohol, in lower concentrations, acts as a local anaesthetic; at higher concentrations it denatures proteins indiscriminately.

Primary options

botulinum toxin type A: children and adults: consult specialist for guidance on dose

Secondary options

phenol: children and adults: consult specialist for guidance on dose

OR

ethyl alcohol: children and adults: consult specialist for guidance on dose

Consider – orthopaedic surgery

Back
ONGOING
|
spastic hemiplegia
Consider – 

orthopaedic surgery

Additional treatment recommended for SOME patients in selected patient group

For resistant contractures or bony deformity, orthopaedic surgery may be necessary. The goals of surgery are to prevent progressive deformity and remove mechanical or anatomic barriers in order to maximise mobility and function. Single-event multilevel surgery (i.e., combining several surgeries into a single surgical event) is recommended in order to minimise total recovery time, rather than 'birthday surgery' (a procedure nearly every year).[63][181]

Orthopaedic surgery is ideally offered in specialty CP clinics where all the manifestations of a patient’s condition can be identified and prioritised.

Tendon transfers to improve wrist and finger/thumb extension, along with first web-space release and flexion contracture release, can improve hand function. Wrist fusion to address residual wrist-flexion deformity is often sought prior to a patient beginning university or participating in the workplace. It may improve function as a 'helping hand'. For hygiene issues, tendon lengthening and long-term bracing usually suffice. Release of contractures is also considered to facilitate placement of hand in space and prevent hygiene problems. If deformity is is long-standing, more extensive releases, including release of the joint capsules, may be needed. Elbow flexor and forearm pronation contracture release may help an adolescent fit in socially.

Hip dysplasia and deformity is rare in patients with hemiplegia, but should not be ignored.[85] If possible, surgery to correct knee flexion and ankle equinus contractures is best deferred until adolescence to avoid weakness, but recalcitrant cases will require earlier treatment. Femoral derotational osteotomy can improve gait mechanics and appearance.

spastic diplegia

1st line – occupational therapy (OT), physiotherapy (PT), and speech therapy

Back
ONGOING
|
spastic diplegia
1st line – 

occupational therapy (OT), physiotherapy (PT), and speech therapy

Treatment for spastic diplegia is dependent on the patient's age and gross motor function classification system (GMFCS) level. Therapy is recommended early (0-3 years), when the clinical picture is evolving and parents are learning about their child's needs and abilities. OT and PT are directed towards strengthening of weak muscles, stretching of shortened muscles, and coordination activities, with an emphasis on bimanual upper-extremity activities and encouraging symmetry of gait and posture.[132] 

Therapies are tailored to the individual patient. Evidence supports the use of training-based interventions, such as goal-directed training, mobility training, and treadmill training.[63] Bottom-up, generic, and/or passive motor interventions (e.g., neurodevelopmental treatment) are less effective or ineffective.[63] The emphasis is on practising real-life tasks and activities, using active movements, targeted at achieving an identified goal.

Physical activity participation in the real world is notoriously difficult to measure in this group, complicating attempts to demonstrate the usefulness of exercise.[204][205] There is some evidence that fitness programmes have positive effects on social participation, self-perception, and quality of life.[133][134][206] Even in children who are non-ambulatory, as little as 6 weeks of exercise intervention may improve gross motor function.[137]

Mechanically assisted walking training aims to help children with CP to walk further by using equipment such as a treadmill, a gait trainer (a wheeled walking aid), or a robotic training device. One Cochrane review concluded that mechanically assisted walking training probably improves walking speed (with or without body weight support) and may improve motor function (with body weight support). There was some evidence of a greater beneficial effect of such training without body weight support compared with overground walking for improving speed and function. However, the clinical significance of the results is unclear.[141]

The importance of considering the environment or context of interventions has been highlighted.[63] Context-focused therapy emphasises modification of the task or environment, including the use of compensatory movements, to achieve a functional task. Compensatory strategies and environmental adaptations ought to be considered for every child in attempting to improve their overall function.[63]​[108]​​ 

Interactive adaptive gaming offers the opportunity for participation and social interaction.[155] There is some evidence that virtual reality training may be as effective as conventional interventions for improving motor function in children with CP.[156][157][158]

Speech therapy is directed towards educating and assisting the family in helping the child achieve developmental milestones and overcome communication difficulties, which may be underestimated because of the severity of motor disability.

Consider – orthoses

Back
ONGOING
|
spastic diplegia
Consider – 

orthoses

Additional treatment recommended for SOME patients in selected patient group

May be used to improve function and maintain range of motion during walking or upper-extremity activities.[148][149][150][151]

Casting is used to correct deformity, while splinting is used to maintain correction and to prevent recurrence. Bracing and casting are also used to enhance function.

Spasticity that is not well managed may develop into a fixed contracture. Contractures are best prevented by a home exercise programme of stretching combined with appropriate positioning to maintain adequate range of motion. Fixed contractures are treated by serial casting and/or intensive physiotherapy. Once achieved, range of motion is maintained by bracing or splinting.

Spasticity that interferes with walking may be managed by an orthosis to correct gait deviation. Care must be taken to determine the specific impairment causing the gait deviation to ensure the most appropriate form of orthosis is used. Orthoses that limit dorsiflexion may improve stability when plantar flexor weakness is the primary cause; however, remaining contributors, such as hip or knee flexion contractures and tight hamstrings creating a crouched gait, must also be addressed.

Consider – adaptive equipment

Back
ONGOING
|
spastic diplegia
Consider – 

adaptive equipment

Additional treatment recommended for SOME patients in selected patient group

Therapy is directed at providing patients with necessary equipment and aids depending on their physical abilities.

Aids for walking include braces, crutches, canes, and walkers. Patients with poor trunk control or persistent scissoring may use a gait trainer, which also provides trunk and pelvic stability. Children with difficulty advancing the limbs during swing may use a reciprocating gait orthosis. Gait trainers and reciprocating orthoses are typically used with assistance for therapeutic purposes and are not considered independent mobility aids.

For non-ambulatory patients and those with walking limitations, mobility aids such as specially adapted scooters, tricycles, and automobiles, as well as positioning equipment for electric and non-electrically driven wheelchairs, may be used. Power mobility should be considered from an early age to promote independence.

Adaptive technology both to enhance communication and to enable access to computers should be provided. Appropriate augmentative and alternative communication systems should be made available to children at an early age if required. Provision should be re-assessed regularly to ensure that aids are still appropriate as the patient’s needs and abilities change.[56][153][154]

Consider – oral pharmacological therapy

Back
ONGOING
|
spastic diplegia
Consider – 

oral pharmacological therapy

Additional treatment recommended for SOME patients in selected patient group

Oral pharmacological therapy may have a role from mid-childhood onwards for significant spasticity interfering with function, and is the first line of treatment.[109] It is not used for the management of mild spastic CP (e.g., gross motor function classification system [GMFCS] level I, II, or possibly III). Oral pharmacological therapy is effective in some patients, but adverse effects such as lethargy may limit tolerability. 

Many of the medications used are crossovers from clinical experience with epilepsy and other conditions.

Diazepam: good for transient use for acute spasms, post-surgery; however, the patient may develop dependence and it should be discontinued slowly.

Tizanidine: a centrally acting alpha-2-adrenergic agonist. Liver function must be monitored due to risk of hepatotoxicity.

Dantrolene: acts peripherally on muscle fibres. Liver function must be monitored in view of reversible hepatotoxicity and, rarely, of fatal hepatitis.

Baclofen: may potentiate seizures.[207] The dosage must be titrated in all age groups until effective or presence of adverse effects.[208] Abrupt withdrawal may also cause seizures and/or psychosis. Lack of an effect following oral treatment is not a contraindication to try intrathecal baclofen.

Primary options

diazepam: children: consult specialist for guidance on dose; adults: 2-10 mg orally two to four times daily

OR

tizanidine: children: consult specialist for guidance on dose; adults: 2-4 mg orally three times daily initially, increase gradually according to response, maximum 36 mg/day

OR

dantrolene: children ≥5 years of age: 0.5 mg/kg orally once daily for 7 days, followed by 0.5 mg/kg three times daily for 7 days, then 1 mg/kg three times daily for 7 days, then 2 mg/kg three times daily thereafter, maximum 400 mg/day; adults: 25 mg orally once daily for 7 days, followed by 25 mg three times daily for 7 days, then 50 mg three times daily for 7 days, then 100 mg three times daily thereafter, maximum 400 mg/day

OR

baclofen: children 2-7 years of age: 5 mg orally three times daily initially, increase gradually according to response, maximum 40 mg/day; children ≥8 years of age: 5 mg orally three times daily initially, increase gradually according to response, maximum 60 mg/day; adults: 5 mg orally three times daily initially, increase gradually according to response, maximum 80 mg/day

Consider – injectable pharmacological therapy

Back
ONGOING
|
spastic diplegia
Consider – 

injectable pharmacological therapy

Additional treatment recommended for SOME patients in selected patient group

Injectable therapy for spasticity includes botulinum toxin type A.[63]​[109][110][111]​​[112][113] Phenol or ethyl alcohol may be used for severe spasticity if botulinum toxin is not available, but they are less effective and are associated with more adverse effects than botulinum toxin.[63][114]

Botulinum toxin type A acts locally in the injected muscle. There is a delayed onset of action of 1-2 days; muscle consistency is altered by day 10. It temporarily weakens muscle but is followed by re-innervation, accounting for the short duration of therapeutic effects (typically 3-4 months).

Botulinum toxin type A products have shown efficacy in improving upper limb spasticity (as an adjunct to OT), lower limb spasticity, gait, joint range of motion, satisfaction, and pain in children with CP.​[112][113] [159]​

Botulinum toxin type A products are approved for use in adults with upper limb spasticity and cervical dystonia. OnabotulinumtoxinA (botulinum toxin type A) and abobotulinumtoxinA (botulinum toxin type A) are approved for upper limb and lower limb spasticity caused by CP in children aged 2 years and over. IncobotulinumtoxinA (botulinum toxin type A) is approved for the treatment of chronic sialorrhoea in adults and children aged 2 years and older. OnabotulinumtoxinA is also used for drooling in adults and children; however, this is an off-label use and there are no approved dosing schedules. Botulinum toxin type B is approved in some countries for treating cervical dystonia and chronic sialorrhoea in adults, but is not widely used in CP.

Mild transient side effects of botulinum toxin type A product lasting 1 to 2 days (local rash, flu-like symptoms, constipation, weakness) may occur. Rare adverse reactions, possibly dose-related, include respiratory distress and dysphagia, although a direct causal relation to botulinum toxin has not been established.[112][113] Use is contraindicated in children with respiratory compromise, severe dysphagia, or presence of neuromuscular junction disease (e.g., Eaton-Lambert syndrome, myasthenia gravis). It must not be given with aminoglycoside antibiotics or tubocurarine-type muscle relaxants. AbobotulinumtoxinA is contraindicated in patients with hypersensitivity to any form of botulinum toxin or to cow’s milk protein. IncobotulinumtoxinA is contraindicated in patients with hypersensitivity to sucrose or albumin.

There is some evidence of muscle damage and incomplete recovery after botulinum toxin type A treatment, particularly in shorter-term animal models.[161][162] Following recommended dosing schedules, including repeating injections no more than twice a year, along with accurate placement of the medication using ultrasound and/or electrical stimulation, are ways to minimise damage. Studies suggest that humans recover up to 90% of pre-injection muscle strength and volume at 1 year and that injections should be accompanied by an organised exercise programme for muscle strengthening.[162] 

It is important for practitioners to note that doses differ for each botulinum toxin type A and they are not interchangeable.

Phenol and ethyl alcohol may be used for severe spasticity when botulinum toxin type A is not available. Phenol is used mainly for motor nerves or during open surgical procedures where branches of nerves may be electrically stimulated to ensure they are purely motor, or for persistent elbow flexion. Phenol may be injected using the closed motor point or the open surgical method. Use a slightly higher concentration for closed injection into the motor point. Phenol is contraindicated for mixed or sensory nerves due to potential dysaesthesias. Ethyl alcohol, in lower concentrations, acts as a local anaesthetic; at higher concentrations it denatures proteins indiscriminately.

Primary options

botulinum toxin type A: children and adults: consult specialist for guidance on dose

Secondary options

phenol: children and adults: consult specialist for guidance on dose

OR

ethyl alcohol: children and adults: consult specialist for guidance on dose

Consider – neurosurgical techniques

Back
ONGOING
|
spastic diplegia
Consider – 

neurosurgical techniques

Additional treatment recommended for SOME patients in selected patient group

Intrathecal baclofen (ITB) decreases spasticity, improves endurance, comfort, and ease of care, and reduces fatigue and pain.[118][166][167]

Before implantation of the ITB pump, a trial dose is administered via lumbar puncture and is considered effective if the modified Ashworth scale scores decrease 1 or more points in most affected muscles 2-4 hours following injection. Pump doses are started at the bolus dose and may be titrated 10% to 15% at each follow-up. Typically, if the first dose is unsuccessful, a second dose 25 micrograms higher may be administered 24 hours later.[168] 

Baclofen withdrawal can be caused by failure of the pump or battery; failure to refill drug before scheduled alarm date; or a leak, a disconnection, or breakage in the catheter. Withdrawal can result in exaggerated rebound spasticity, rhabdomyolysis, and multiple organ failure. The condition may resemble autonomic dysreflexia, sepsis, malignant hyperthermia, and neuroleptic-malignant syndrome.[172][173] Although not always leading to withdrawal, the reported incidence of catheter-related complications ranges from 7% to 9%.[174][175] Catheter micro-cracks can occasionally lead to under-dosage or over-dosage.[176]

Selective dorsal rhizotomy (SDR; also called selective posterior rhizotomy) is a one-time irreversible treatment to ameliorate spasticity.[180] It does not address factors other than spasticity that may impede walking. The best candidates for SDR are patients with preserved voluntary selective motor control, strong trunk and pelvic muscles, and good dynamic balance. Post-operative weakness is common and patients require long-term, aggressive physiotherapy. Ankle-foot orthoses are recommended post-operatively for at least 6 months to protect weak plantar flexor muscles from overstretch.

Primary options

baclofen intrathecal: children and adults: consult specialist for guidance on dose

Consider – orthopaedic surgery

Back
ONGOING
|
spastic diplegia
Consider – 

orthopaedic surgery

Additional treatment recommended for SOME patients in selected patient group

For resistant contractures or bony deformity, orthopaedic surgery may be necessary. The goals of surgery are to prevent progressive deformity and remove mechanical or anatomic barriers in order to maximise mobility and function. Single-event multilevel surgery (i.e., combining several surgeries into a single surgical event) is recommended in order to minimise total recovery time, rather than 'birthday surgery' (a procedure nearly every year).[63][181]

Orthopaedic surgery is ideally offered in specialty CP clinics where all the manifestations of a patient’s condition can be identified and prioritised.

The hips should be screened from age 3 years to detect progressive subluxation and, if necessary, reduced with early (before age 5 years) tendon releases.[184] Correction of rotational deformities with osteotomy should be carried out between ages 3 and 7 years. Late surgeries are less effective in preventing or reducing sequelae such as pain and progressive dislocation. Late-presenting dislocation (age 9+ years) requires either observation or surgical reduction, depending on the shape of the acetabulum and how recently the hip was last known to be located. Adult presentation depends on symptoms: hip replacement, osteotomy, or resection for significant pain; or extreme osteotomy to position the femoral head away from the acetabulum.

Knee flexion contracture or ankle equinus is acceptable in non-ambulatory patients up to the point at which care or positioning is impaired. In ambulatory patients (gross motor function classification system [GMFCS] I, II, or III), tendon releases or transfers at the knee or ankle should be preceded with a gait study including electromyogram and kinematic data. The more mobile the patient, the more likely tendons will be transferred and lengthened rather than released. Aggressive lengthening of the triceps surae in young ambulatory children should be avoided, as it may contribute to a crouched gait as the child ages.[189] When possible in diplegia, the soleus is left intact, and only the gastrocnemius fascia is lengthened.[189] Excessive dorsiflexion may also be due to hypermobility in the subtalar joint that requires stabilisation. Significant knee flexion contractures should be addressed before adolescence in order to prevent the development of a severely crouched gait.[191][192][193] This frequently involves distal femoral shortening extension osteotomies with advancement of the patellar tendons.[191][194] Lever disease or the malpositioning of the functional plane of motion of the knee or ankle can be corrected with femoral or tibial rotational osteotomies, or both.

Foot deformities may be treated with a bony operation because of the unpredictability of the deforming forces.

The upper extremities are usually not significantly involved in diplegia so are rarely a major problem, although occasionally a contracture release or tendon transfer is indicated.

Spine abnormalities are less common and surgery should be avoided until at least the age of 7 or 8 years. If the spinal curve is significant or progressive, surgical fusion may be indicated. Posterior fixation devices are usually sufficient, although anterior release may also be required for severe deformities.

spastic quadriplegia

1st line – occupational therapy/physiotherapy/speech therapy

Back
ONGOING
|
spastic quadriplegia
1st line – 

occupational therapy/physiotherapy/speech therapy

Realistic goals are established by assessing the patient's physical and intellectual abilities and energy demands. In most cases of spastic quadriplegia, the focus is on improving care and comfort rather than on improving function.

Therapy is recommended early (0-3 years) when the clinical picture is evolving and parents are learning about their child's needs and abilities. It is directed towards strengthening of weak muscles, stretching of shortened muscles, and coordination activities with an emphasis on bimanual upper-extremity activities and encouraging symmetry of posture.[132] Upright activities, either seated or standing with support, are encouraged. A primary goal of therapy is the achievement of independent mobility. This maybe achieved through floor mobility or with walkers, wheelchairs, or other assistive devices.

Therapies are tailored to the individual patient. Evidence supports the use of training-based interventions.[63] Bottom-up, generic, and/or passive motor interventions (e.g., neurodevelopmental treatment) are less effective or ineffective.[63] The emphasis is on practising real-life tasks and activities, using active movements, targeted at achieving an identified goal.

There is some evidence that exercise interventions and fitness programmes have positive effects on function, social participation, self-perception, and quality of life.[133][134][135][136] Even in children who are non-ambulatory, as little as 6 weeks of exercise intervention may improve gross motor function.[137]

The importance of considering the environment or context of interventions has been highlighted.[63] Context-focused therapy emphasises modification of the task or environment, including the use of compensatory movements, to achieve a functional task. Compensatory strategies and environmental adaptations ought to be considered for every child in attempting to improve their overall function.[63]​[108]​​ 

Interactive adaptive gaming offers the opportunity for participation and social interaction.[155] There is some evidence that virtual reality training may be as effective as conventional interventions for improving motor function in children with CP.[156][157][158]

Speech therapy is directed towards educating and assisting the family in helping the child achieve developmental milestones and overcome communication difficulties, which may be underestimated because of the severity of motor disability.

Consider – orthoses

Back
ONGOING
|
spastic quadriplegia
Consider – 

orthoses

Additional treatment recommended for SOME patients in selected patient group

Casting is used to correct deformity, while splinting is used to maintain correction and to prevent recurrence. Bracing and casting are also used to enhance function.

Spasticity that is not well managed may develop into a fixed contracture. Contractures are best prevented by a home exercise programme of stretching combined with appropriate positioning to maintain adequate range of motion. Fixed contractures are treated by serial casting and/or intensive physiotherapy. Once achieved, range of motion is maintained by splinting or bracing.

In the ambulatory patient, care must be taken to determine the specific impairment causing the gait deviation to ensure the most appropriate form of orthosis is used. Ankle-foot orthoses may provide the additional stability to allow walking for short distances (gross motor function classification system [GMFCS] IV), to assist with dorsiflexion during swing or to prevent excessive plantar flexion during stance. Orthoses that limit dorsiflexion may improve stability when plantar flexor weakness is the primary cause.

Consider – adaptive equipment

Back
ONGOING
|
spastic quadriplegia
Consider – 

adaptive equipment

Additional treatment recommended for SOME patients in selected patient group

Therapy is directed at providing patients with the necessary equipment and aids depending on their physical abilities.

For a subset of patients who are capable, the goal is independent, powered mobility with an adapted seating system and a means to communicate basic needs. Some patients (typically gross motor function classification system [GMFCS] level IV) may be able to walk for short distances using assistive devices including a walker and orthoses; however, walking is rarely the primary means of mobility in this population. For patients with more involvement, a manual wheelchair with a seating system for postural support is needed, with emphasis on care and comfort.

Adaptive technology both to enhance communication and to enable access to computers should be provided. Appropriate augmentative and alternative communication systems should be made available to children at an early age if required. Provision should be re-assessed regularly to ensure that aids are still appropriate as the patient’s needs and abilities change.[56][153][154]

Consider – oral pharmacological therapy

Back
ONGOING
|
spastic quadriplegia
Consider – 

oral pharmacological therapy

Additional treatment recommended for SOME patients in selected patient group

Oral pharmacological therapy may have a role from mid-childhood onwards for significant spasticity interfering with function, and is the first line of treatment.[109] It is not used for the management of mild spastic CP (e.g., motor function classification system [GMFCS] level I, II, or possibly III). Oral pharmacological therapy is effective in some patients, but adverse effects such as lethargy may limit tolerability.

Many of the medications used are crossovers from clinical experience with epilepsy and other conditions.

Diazepam: good for transient use for acute spasms, post-surgery; however, the patient may develop dependence and it should be discontinued slowly.

Tizanidine: a centrally acting alpha-2-adrenergic agonist. Liver function must be monitored due to risk of hepatotoxicity.

Dantrolene: acts peripherally on muscle fibres. Liver function must be monitored in view of reversible hepatotoxicity and, rarely, of fatal hepatitis.

Baclofen: may potentiate seizures.[207] The dosage must be titrated in all age groups until effective or presence of adverse effects.[208] Abrupt withdrawal may also cause seizures, psychosis. Lack of an effect following oral treatment is not a contraindication to try intrathecal baclofen.

Primary options

diazepam: children: consult specialist for guidance on dose; adults: 2-10 mg orally two to four times daily

OR

tizanidine: children: consult specialist for guidance on dose; adults: 2-4 mg orally three times daily initially, increase gradually according to response, maximum 36 mg/day

OR

dantrolene: children ≥5 years of age: 0.5 mg/kg orally once daily for 7 days, followed by 0.5 mg/kg three times daily for 7 days, then 1 mg/kg three times daily for 7 days, then 2 mg/kg three times daily thereafter, maximum 400 mg/day; adults: 25 mg orally once daily for 7 days, followed by 25 mg three times daily for 7 days, then 50 mg three times daily for 7 days, then 100 mg three times daily thereafter, maximum 400 mg/day

OR

baclofen: children 2-7 years of age: 5 mg orally three times daily initially, increase gradually according to response, maximum 40 mg/day; children ≥8 years of age: 5 mg orally three times daily initially, increase gradually according to response, maximum 60 mg/day; adults: 5 mg orally three times daily initially, increase gradually according to response, maximum 80 mg/day

Consider – injectable pharmacological therapy

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spastic quadriplegia
Consider – 

injectable pharmacological therapy

Additional treatment recommended for SOME patients in selected patient group

Injectable therapy for spasticity includes botulinum toxin type A.[63]​[109][110][111]​​[112][113] Phenol or ethyl alcohol may be used for severe spasticity if botulinum toxin is not available, but they are less effective and are associated with more adverse effects than botulinum toxin.[63][114]

Botulinum toxin type A acts locally in the injected muscle. There is a delayed onset of action of 1-2 days; muscle consistency is altered by day 10. It temporarily weakens muscle but is followed by re-innervation, accounting for the short duration of therapeutic effects (typically 3-4 months).

Botulinum toxin type A products have shown efficacy in improving upper limb spasticity (as an adjunct to OT), lower limb spasticity, gait, joint range of motion, satisfaction, and pain in children with CP.​[112][113][159]​ 

Botulinum toxin type A products are approved for use in adults with upper limb spasticity and cervical dystonia. OnabotulinumtoxinA (botulinum toxin type A) and abobotulinumtoxinA (botulinum toxin type A) are approved for upper limb and lower limb spasticity caused by CP in children aged 2 years and over. IncobotulinumtoxinA (botulinum toxin type A) is approved for the treatment of chronic sialorrhoea in adults and children aged 2 years and older. OnabotulinumtoxinA is also used for drooling in adults and children; however, this is an off-label use and there are no approved dosing schedules. Botulinum toxin type B is approved in some countries for treating cervical dystonia and chronic sialorrhoea in adults, but is not widely used in CP.

Mild transient side effects of botulinum toxin type A product lasting 1 to 2 days (local rash, flu-like symptoms, constipation, weakness) may occur. Rare adverse reactions, possibly dose-related, include respiratory distress and dysphagia, although a direct causal relation to botulinum toxin has not been established.[112][113] Use is contraindicated in children with respiratory compromise, severe dysphagia, or presence of neuromuscular junction disease (e.g., Eaton-Lambert syndrome, myasthenia gravis). It must not be given with aminoglycoside antibiotics or tubocurarine-type muscle relaxants. AbobotulinumtoxinA is contraindicated in patients with hypersensitivity to any form of botulinum toxin or to cow’s milk protein. IncobotulinumtoxinA is contraindicated in patients with hypersensitivity to sucrose or albumin.

There is some evidence of muscle damage and incomplete recovery after botulinum toxin type A treatment, particularly in shorter-term animal models.[161][162] Following recommended dosing schedules, including repeating injections no more than twice a year, along with accurate placement of the medication using ultrasound and/or electrical stimulation, are ways to minimise damage. Studies suggest that humans recover up to 90% of pre-injection muscle strength and volume at 1 year and that injections should be accompanied by an organised exercise programme for muscle strengthening.[162] 

It is important for practitioners to note that doses differ for each botulinum toxin type A and they are not interchangeable.

Phenol and ethyl alcohol may be used for severe spasticity when botulinum toxin type A is not available. Phenol is used mainly for motor nerves or during open surgical procedures where branches of nerves may be electrically stimulated to ensure they are purely motor, or for persistent elbow flexion. Phenol may be injected using the closed motor point or the open surgical method. Use a slightly higher concentration for closed injection into the motor point. Phenol is contraindicated for mixed or sensory nerves due to potential dysaesthesias. Ethyl alcohol, in lower concentrations, acts as a local anaesthetic; at higher concentrations it denatures proteins indiscriminately.

Primary options

botulinum toxin type A: children and adults: consult specialist for guidance on dose

Secondary options

phenol: children and adults: consult specialist for guidance on dose

OR

ethyl alcohol: children and adults: consult specialist for guidance on dose

Consider – neurosurgical techniques

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spastic quadriplegia
Consider – 

neurosurgical techniques

Additional treatment recommended for SOME patients in selected patient group

Intrathecal baclofen decreases spasticity, improves endurance, comfort, and ease of care, and reduces fatigue and pain.[118][166][167]

Before implantation, a trial dose is administered via lumbar puncture and is considered effective if the modified Ashworth scale scores decrease 1 or more points in most affected muscles 2 to 4 hours following injection. Pump doses are started at the bolus dose and may be titrated 10% to 15% at each follow-up.

Baclofen withdrawal can be caused by failure of the pump or battery; failure to refill drug before scheduled alarm date; or a leak, a disconnection, or breakage in the catheter. Withdrawal can result in exaggerated rebound spasticity, rhabdomyolysis, and multiple organ failure. The condition may resemble autonomic dysreflexia, sepsis, malignant hyperthermia, and neuroleptic-malignant syndrome.[172][173] Although not always leading to withdrawal, the reported incidence of catheter-related complications ranges from 7% to 9%.[174][175] Catheter micro-cracks can occasionally lead to under-dosage or over-dosage.[176]

Selective dorsal rhizotomy (SDR; also called selective posterior rhizotomy) is a one-time irreversible treatment to ameliorate spasticity.[180] It does not address factors other than spasticity that may impede walking. The best candidates for SDR are patients with preserved voluntary selective motor control, strong trunk and pelvic muscles, and good dynamic balance.

Post-operative weakness is common and patients require long-term, aggressive physiotherapy. Ankle-foot orthoses are recommended post-operatively for at least 6 months to protect weak plantar flexor muscles from overstretch.

Primary options

baclofen intrathecal: children and adults: consult specialist for guidance on dose

Consider – pressure care

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spastic quadriplegia
Consider – 

pressure care

Additional treatment recommended for SOME patients in selected patient group

Non-ambulatory patients are at risk of pressure ulcers and appropriate preventive measures should be taken to avoid pressure ulcer development.

Consider – multidisciplinary orthopaedic management

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spastic quadriplegia
Consider – 

multidisciplinary orthopaedic management

Additional treatment recommended for SOME patients in selected patient group

The goal of surgery for treating non-ambulatory patients with spastic quadriplegia is the facilitation of care and comfort; for example, comfortable sitting in a mobile chair, either manual or powered, with a balanced spine. This should be monitored throughout childhood. Surgery is performed to prevent progressive deformity and remove mechanical or anatomical barriers in order to maximise mobility and function. Single-event multilevel surgery (i.e., combining several surgeries into a single surgical event) is recommended in order to minimise total recovery time, rather than 'birthday surgery' (a procedure nearly every year).[63][181]

Orthopaedic surgery is ideally offered in specialty CP clinics where all the manifestations of a patient’s condition can be identified and prioritised.

The hips should be screened from age 3 years to detect progressive subluxation and, if necessary, reduced with early (before age 5 years) tendon releases.[184] Correction of rotational deformities with osteotomy should be carried out between ages 3 and 7 years. Late surgeries are less effective in preventing or reducing sequelae such as pain and progressive dislocation. Late-presenting dislocation (age 9+ years) requires either observation or surgical reduction, depending on the shape of the acetabulum and how recently the hip was last known to be located. Adult presentation depends on symptoms: hip replacement, osteotomy, or resection for significant pain; or extreme osteotomy to position the femoral head away from the acetabulum.

Knee flexion contracture or ankle equinus is acceptable in non-ambulatory patients up to the point at which care or positioning is impaired.

Foot deformities may be treated with a bony operation because of the unpredictability of the deforming forces. The ability to wear shoes comfortably is the goal.

Tendon transfers to improve wrist and finger/thumb extension, along with first web-space release and flexion contracture release, can improve hand function. Wrist fusion to address residual wrist-flexion deformity is often sought prior to a patient beginning university or participating in the workplace. It may improve function as a 'helping hand'. For hygiene issues, tendon lengthening and long-term bracing usually suffice. Release of contractures is also considered to facilitate placement of hand in space and prevent hygiene problems. If deformity is long-standing, more extensive releases, including release of the joint capsules, may be needed. Elbow flexor and forearm pronation contracture release may help an adolescent fit in socially.

dyskinetic

1st line – occupational therapy (OT), physiotherapy (PT), and speech therapy

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ONGOING
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dyskinetic
1st line – 

occupational therapy (OT), physiotherapy (PT), and speech therapy

Therapy is recommended early (0-3 years), when the clinical picture is evolving and parents are learning about their child's needs and abilities. It should be directed towards facilitating the patient's strengths to optimise independence and participation in school, work, and the community. The aim is to teach the child alternative strategies to optimise independence.

Therapies are tailored to the individual patient. Evidence supports the use of training-based interventions.[63] Bottom-up, generic, and/or passive motor interventions (e.g., neurodevelopmental treatment) are less effective or ineffective.[63] The emphasis is on practising real-life tasks and activities, using active movements, targeted at achieving an identified goal.

There is some evidence that fitness programmes have positive effects on social participation, self-perception, and quality of life.[133][134][136]​​[206]​​ Even in children who are non-ambulatory, as little as 6 weeks of exercise intervention appears to improve gross motor function.[137]

Mechanically assisted walking training aims to help children with CP to walk further by using equipment such as a treadmill, a gait trainer (a wheeled walking aid), or a robotic training device. One Cochrane review concluded that mechanically assisted walking training probably improves walking speed (with or without body weight support) and may improve motor function (with body weight support). There was some evidence of a greater beneficial effect of such training without body weight support compared with overground walking for improving speed and function. However, the clinical significance of the results is unclear.[141]

The importance of considering the environment or context of interventions has been highlighted.[63] Context-focused therapy emphasises modification of the task or environment, including the use of compensatory movements, to achieve a functional task. Compensatory strategies and environmental adaptations ought to be considered for every child in attempting to improve their overall function.[63]​[108]​​  

Interactive adaptive gaming offers the opportunity for participation and social interaction.[155] There is some evidence that virtual reality training may be as effective as conventional interventions for improving motor function in children with CP.[156][157][158]

Speech therapy is directed towards educating and assisting the family in helping the child achieve developmental milestones and overcome communication difficulties, which may be underestimated because of the severity of motor disability.

Consider – orthoses

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dyskinetic
Consider – 

orthoses

Additional treatment recommended for SOME patients in selected patient group

May be used, although patients with dystonia rarely tolerate bracing due to the fluctuating tone and movement abnormality.

Consider – adaptive equipment

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ONGOING
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dyskinetic
Consider – 

adaptive equipment

Additional treatment recommended for SOME patients in selected patient group

Therapy is directed at providing patients with the necessary equipment and aids depending on their physical abilities.

Aids for walking include braces, crutches, canes, and walkers. Patients with functional upper extremities who require assistance for balance and stability may use a standard walker with wheels.

For non-ambulatory patients and those with walking limitations, mobility aids such as specially adapted scooters, tricycles, and automobiles, as well as positioning equipment for electric and non-electrically driven wheelchairs, may be used. Power mobility should be considered from an early age to promote independence.

Adaptive technology both to enhance communication and to enable access to computers should be provided. Appropriate augmentative and alternative communication systems should be made available to children at an early age if required. Provision should be re-assessed regularly to ensure that aids are still appropriate as the patient’s needs and abilities change.[56][153][154]

Consider – carbidopa/levodopa

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ONGOING
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dyskinetic
|
with dystonia
Consider – 

carbidopa/levodopa

Additional treatment recommended for SOME patients in selected patient group

A therapeutic trial of carbidopa/levodopa may be considered to assess for dystonia due to dopamine-responsive dystonia (also known as Segawa's disease), in which patients show a dramatic response. In many cases, full physical functionality including walking, running, speaking, and writing is restored or preserved.

Diagnosis of dystonia is not made by one definitive test, but by a series of clinical observations and specific biochemical assessments. Defining the exact cause may not be possible. Dystonia Medical Research Foundation: dopa-responsive dystonia Opens in new window High-protein foods slow the absorption of this drug.

Primary options

carbidopa/levodopa: children ≥3 months of age: 250 micrograms/kg orally two to three times daily, increase gradually according to response, maximum 1 mg/kg three times daily; adults: 25/100 mg orally (immediate-release) two to three times daily initially, increase gradually according to response, maximum 200/2000 mg per day

More

Consider – oral pharmacological therapy

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ONGOING
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dyskinetic
|
with dystonia
Consider – 

oral pharmacological therapy

Additional treatment recommended for SOME patients in selected patient group

If there is a modest response to carbidopa/levodopa, it can be continued and combined with oral medications such as diazepam or trihexyphenidyl.

Non-responsiveness to carbidopa/levodopa does not predict response to the other medications, and an attempt to treat the dystonia on a chronic basis with alternative oral medications should be considered. The drugs may diminish the dyskinesia in selected patients. It is possible to combine drugs to enhance the therapeutic response, while reducing or minimising adverse effects.

One systematic review concluded that trihexyphenidyl is possibly ineffective in reducing dystonia, improving motor function, and easing care-giving in patients with dystonia in CP, and that there is no evidence on the effectiveness of benzodiazepines.[121] One Cochrane review concluded that there is insufficient evidence regarding the effectiveness of trihexyphenidyl for patients with dystonic CP.[122]

Trihexyphenidyl is an anti-parkinsonian agent. It is better tolerated in children than with adults. Use with caution in older patients. Adverse effects limit its use at high doses.

Primary options

diazepam: children: consult specialist for guidance on dose; adults: 2-10 mg orally two to four times daily

OR

trihexyphenidyl: children: consult specialist for guidance on dose; adults: 1 mg orally once daily initially, increase gradually according to response, maximum 10 mg/day given in 3-4 divided doses

Consider – injectable pharmacological therapy

Back
ONGOING
|
dyskinetic
|
with dystonia
Consider – 

injectable pharmacological therapy

Additional treatment recommended for SOME patients in selected patient group

Injectable therapy includes botulinum toxin type A.

Botulinum toxin targeted to individual muscles lessens the force and frequency of dystonic contractions, and may be trialled to improve function (e.g., accessing a switch for wheelchair mobility or computer communications). It also facilitates care-giving. There is a delayed onset of action of 1-2 days; muscle consistency is altered by day 10. It temporarily weakens muscle but is followed by re-innervation, accounting for the short duration of therapeutic effects (typically 3-4 months).

Botulinum toxin type A products are approved for use in adults with upper limb spasticity and cervical dystonia. OnabotulinumtoxinA (botulinum toxin type A) and abobotulinumtoxinA (botulinum toxin type A) are approved for upper limb and lower limb spasticity caused by CP in children aged 2 years and over. IncobotulinumtoxinA (botulinum toxin type A) is approved for the treatment of chronic sialorrhoea in adults and children aged 2 years and older. OnabotulinumtoxinA is also used for drooling in adults and children; however, this is an off-label use and there are no approved dosing schedules. Botulinum toxin type B is approved in some countries for treating cervical dystonia and chronic sialorrhoea in adults, but is not widely used in CP.

One systematic review concluded that there is limited evidence on the effectiveness of botulinum toxin for dystonia in CP, but some trials show promising results for the treatment of the upper extremity and cervical muscles for patients with dystonic CP.[121][123][124]

Mild transient side effects of botulinum toxin type A product lasting 1 to 2 days (local rash, flu-like symptoms, constipation, weakness) may occur. Rare adverse reactions, possibly dose-related, include respiratory distress and dysphagia, although a direct causal relation to botulinum toxin has not been established.[112][113] Use is contraindicated in children with respiratory compromise, severe dysphagia, or presence of neuromuscular junction disease (e.g., Eaton-Lambert syndrome, myasthenia gravis). It must not be given with aminoglycoside antibiotics or tubocurarine-type muscle relaxants. AbobotulinumtoxinA is contraindicated in patients with hypersensitivity to any form of botulinum toxin or to cow’s milk protein. IncobotulinumtoxinA is contraindicated in patients with hypersensitivity to sucrose or albumin.

There is some evidence of muscle damage and incomplete recovery after botulinum toxin type A treatment, particularly in shorter-term animal models.[161][162] Following recommended dosing schedules, including repeating injections no more than twice a year, along with accurate placement of the medication using ultrasound and/or electrical stimulation, are ways to minimise damage. Studies suggest that humans recover up to 90% of pre-injection muscle strength and volume at 1 year and that injections should be accompanied by an organised exercise programme for muscle strengthening.[162] 

It is important for practitioners to note that doses differ for each botulinum toxin type A and they are not interchangeable.

Primary options

botulinum toxin type A: children and adults: consult specialist for guidance on dose

Consider – neurosurgical techniques

Back
ONGOING
|
dyskinetic
|
with dystonia
Consider – 

neurosurgical techniques

Additional treatment recommended for SOME patients in selected patient group

Neurosurgical treatment is indicated for selected patients after less-invasive options have been exhausted. Options include an intrathecal baclofen (ITB) pump and deep brain stimulation (DBS).[121][125][128][129]

Studies on the effect of intrathecal baclofen in children and young adults with dyskinetic CP (gross motor function classification system [GMFCS] levels IV and V) found improvements in sitting, communication, fine motor skills, and goal attainment scaling of individual treatment goals.[126][127]

Before implantation of the ITB pump, a trial dose is administered via lumbar puncture. Pump doses are started at the bolus dose and may be titrated 10% to 15% at each follow-up. Typically, if the first dose is unsuccessful, a second dose 25 micrograms higher may be administered 24 hours later.[168] 

Baclofen withdrawal can be caused by failure of the pump or battery; failure to refill drug before scheduled alarm date; or a leak, a disconnection, or breakage in the catheter. Withdrawal can result in exaggerated rebound spasticity, rhabdomyolysis, and multiple organ failure. The condition may resemble autonomic dysreflexia, sepsis, malignant hyperthermia, and neuroleptic-malignant syndrome.[172][173] Although not always leading to withdrawal, the reported incidence of catheter-related complications ranges from 7% to 9%.[174][175] Catheter micro-cracks can occasionally lead to under-dosage or over-dosage.[176]

DBS is the use of electrical stimulation by electrodes placed in the posteroventral lateral globus pallidus internus to decrease extra-pyramidal movement disorders. This treatment has been successful in patients with primary (or genetic) dystonia, and some evidence suggests it may be successful in patients with secondary dystonia.[121][128][129]

Primary options

baclofen intrathecal: children and adults: consult specialist for guidance on dose

Consider – neuroleptic therapy

Back
ONGOING
|
dyskinetic
|
with athetosis
Consider – 

neuroleptic therapy

Additional treatment recommended for SOME patients in selected patient group

Oral medications may be tried, although evidence for efficacy is limited.[130]

Primary options

tetrabenazine: children and adults: consult specialist for guidance on dose

OR

carbidopa/levodopa: children and adults: consult specialist for guidance on dose

OR

diazepam: children and adults: consult specialist for guidance on dose

OR

dantrolene: children and adults: consult specialist for guidance on dose

ataxic

1st line – occupational therapy (OT), physiotherapy (PT), and speech therapy

Back
ONGOING
|
ataxic
1st line – 

occupational therapy (OT), physiotherapy (PT), and speech therapy

Therapy is recommended early (0-3 years) when the clinical picture is evolving and parents are learning about their child's needs and abilities. Therapy should be directed towards strengthening of weak muscles(particularly, proximal musculature of the pelvis, scapula, and trunk), and coordination and balance activities.

Therapies are tailored to the individual patient. Evidence supports the use of training-based interventions.[63] Bottom-up, generic, and/or passive motor interventions (e.g., neurodevelopmental treatment) are less effective or ineffective.[63] The emphasis is on practising real-life tasks and activities, using active movements, targeted at achieving an identified goal.

There is some evidence that exercise interventions and fitness programmes have positive effects on function, social participation, self-perception, and quality of life.[133][134][135][136] Even in children who are non-ambulatory, as little as 6 weeks of exercise intervention may improve gross motor function.[137]

Mechanically assisted walking training aims to help children with CP to walk further by using equipment such as a treadmill, a gait trainer (a wheeled walking aid), or a robotic training device. One Cochrane review concluded that mechanically assisted walking training probably improves walking speed (with or without body weight support) and may improve motor function (with body weight support). There was some evidence of a greater beneficial effect of such training without body weight support compared with overground walking for improving speed and function. However, the clinical significance of the results is unclear.[141]

The importance of considering the environment or context of interventions has been highlighted.[63] Context-focused therapy emphasises modification of the task or environment, including the use of compensatory movements, to achieve a functional task. Compensatory strategies and environmental adaptations ought to be considered for every child in attempting to improve their overall function.[63]​[108]​​ 

Interactive adaptive gaming offers the opportunity for participation and social interaction.[155] There is some evidence that virtual reality training may be as effective as conventional interventions for improving motor function in children with CP.[156][157][158]

Speech therapy is directed towards educating and assisting the family in helping the child achieve developmental milestones and overcome communication difficulties, which may be underestimated because of the severity of motor disability.

Consider – orthoses

Back
ONGOING
|
ataxic
Consider – 

orthoses

Additional treatment recommended for SOME patients in selected patient group

Lower-extremity orthoses have not been shown to be effective or well tolerated in patients with ataxic CP, and in some cases their use may limit the patient's ability to compensate distally for proximal impairment.

Consider – adaptive equipment

Back
ONGOING
|
ataxic
Consider – 

adaptive equipment

Additional treatment recommended for SOME patients in selected patient group

Therapy is directed at providing patients with necessary equipment and aids depending on their physical abilities.

Aids for walking include braces, crutches, canes, and walkers. Patients with functional upper extremities who require assistance for balance and stability may use a standard walker with wheels. For non-ambulatory patients, mobility aids such as specially adapted scooters, tricycles, and automobiles, as well as positioning equipment for electric and non-electrically driven wheelchairs, may be used. Power mobility should be considered from an early age to promote independence.

Adaptive technology both to enhance communication and to enable access to computers should be provided. Appropriate augmentative and alternative communication systems should be made available to children at an early age if required. Provision should be re-assessed regularly to ensure that aids are still appropriate as the patient’s needs and abilities change.[56][153][154]

Adaptive equipment such as weighted devices, modified utensils, and walking aids may compensate for the poor proximal control that often contributes to instability in patients with ataxic CP. Protective gear, such as helmets, may be used to protect those prone to frequent falling. Equipment such as weighted vests or visual cues may provide additional sensory feedback to improve stability and safety.

Consider – pharmacological therapy for symptom management

Back
ONGOING
|
ataxic
Consider – 

pharmacological therapy for symptom management

Additional treatment recommended for SOME patients in selected patient group

Pharmacotherapy may sometimes be used to treat accompanying symptoms such as tremor. Emotional support and medication to treat any associated depression should also be considered.

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