Course:KIN366/ConceptLibrary/Spasticity

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Movement Experiences for Children
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KIN 366
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Instructor: Dr. Shannon S.D. Bredin
Email: shannon.bredin@ubc.ca
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Spasticity is associated with increased muscle tone or stiffness that results in uncontrolled and awkward movements (Children’s Hospital of Pittsburgh, 2002). Spasticity is a common symptom seen in many neurological conditions, including single insult events as well as chronic neurological conditions (National Institute for Health and Clinical Excellence, 2012). Many of these conditions implicate children, and consequently have lasting effects on child movement experiences (World Health Organization, 2001).

Background

The term “spasticity” is derived from the Greek words spasticos and spaon, meaning “to draw out, to stretch” (Thilmann, 1993, p. 1). In clinical terms, it is often observed as a “spastic catch”, “clonus”, or “clasp-knife phenomenon” (Sagner et al., 2003, p.89). Spasticity is most commonly defined as a motor disorder characterized by “a velocity-dependent increase in tonic stretch reflexes (muscle tone) with exaggerated tendon jerks, resulting from hyper-excitability of the stretch reflex” (Sagner, Delgado, Gaebler-Spira, Hallett, & Mink, 2003, p.89). The variation in terminology used to describe spasticity highlights an issue in contemporary society, in which there are discrepancies in the literature regarding the definition of spasticity (Adams & Hicks, 2005). Whereas symptoms such as clonus (involuntary, rhythmic muscle contractions in response to sustained stretch), hyperactive tendon reflexes, and spasms are occasionally used within the umbrella term “spasticity”, other authors discuss these symptoms as related yet separate from spasticity (Biering-Sorensen, Nielsen, & Klinge, 2006, p.44). This inconsistency is problematic, because a unified definition is critically importance for research, clinical evaluation, drug evaluation, and choice of treatment. Despite the ambiguous distinctions between the characteristics that define spasticity, as compared to those that are merely associated with spasticity, The National Institutes of Health Task Force on Childhood Motor Disorders defined spasticity as ‘a hypertonia in which one or both of the following signs must be present: (1) muscle resistance to externally imposed movement increases with increasing speed of stretch and varies with the direction of joint movement and/or (2) muscle resistance to externally imposed movement rises rapidly above a threshold speed or joint angle’ (Tilton et al, 2010). Spasticity seldom exists in isolation and is usually accompanied by one or more components of an upper motor neuron syndrome. Upper motor neurons (UMN) are defined as neurons that originate in the motor region of the cerebral cortex or the brainstem and carry motor information down to the lower motor neurons (Barnes, 2001). Upper motor neuron syndrome is associated with enhanced stretch reflexes, released flexor reflexes in the lower limbs, loss of dexterity, and weakness. (Tilton et al, 2010) In children, the impact of spasticity on co-existing motor disorders and their early musculoskeletal development varies; common problems include impaired motor function, pain from muscle spasms, motor developmental delay, and difficulties with daily care (Barnes, 2001). Some examples of disorders associated with spasticity include cerebral palsy, multiple sclerosis, stroke, traumatic brain injury, central nervous system tumor or infarct, metabolic disorders, and hydrocephalus. (Tilton et al, 2010)

Pathophysiology

While the mechanism for spasticity is not entirely known, altered firing rate of gamma motor neurons and their regulating interneurons has been postulated to be involved, as well as an increase in alpha motor neuron activity, which causes an exaggerated stretch reflex to fast stretch of the muscle (Knierim, 2007). In some cases the resistance may become so great that the inverse stretch reflex is initiated, relaxing the muscles such that they suddenly give away, a reflex known as the “clasp-knife phenomenon” (Bax et. al, 2005). Spasticity refers to an increased muscle tone, or tension (Dimitrijević et al, 2014). Normally muscles possess a degree of tone in order to maintain posture, perform movements against, provide flexibility and control the speed of movements (Dimitrijević et al, 2014) Spasticity is usually caused by damage to descending motor pathways at cortical, brainstem, or spinal cord levels that control voluntary movement. (Ghai et al, 2013) This damage causes an imbalance in the command signals between the nervous system and the muscles, which leads to increased activity in the associated muscles (Kheder & Nair, 2012). The command signal to increase muscle tone, travels to the spinal cord from the muscle on sensory nerve fibres (which tell the spinal cord how much tone the muscle has). The command signal to reduce muscle tone (flexibility) comes to the spinal cord from the brain. It is these two commands that must be well coordinated in the spinal cord for muscles to work smoothly while maintaining strength (Dimitrijević et al, 2014). Due to the imbalance between inhibitory and excitatory inputs to spinal motor neurons (with the favor placed on excitation), motor neurons respond to stretch at a lower threshold than normal, with long discharges (American Association of Neurological Surgeons, 2006). In children, spasticity can be detrimental to muscle development. Many disorders involving spasticity in children result from injury to developing motor pathways in the cortex. Other brain structures commonly injured include the basal ganglia, thalamus, cerebellum, brainstem, central white matter or spinal cord (Sagner et. al, 2003). After an injury has occurred, muscle tone is flaccid with hyporeflexia before spasticity is seen. The gap between injury and spasticity varies from days to months according to the level of the lesion. (Ghai et al, 2013)

Associated Disorders

Spasticity is found in several neurological disorders such a cerebral palsy, stroke, multiple sclerosis, spinal cord injury and traumatic brain injury (National Institute for Health and Clinical Excellence, 2012). As well as certain conditions such as central nervous system tumor or infarct, metabolic disorders, and hydrocephalus. (Tilton et al, 2010)

Cerebral Palsy

The most common cause of spasticity in children is cerebral palsy (CP) (Tilton et al 2010). Understanding the underlying causes and effective treatment methods is crucial as CP is a devastating neurodevelopmental condition that affects about 1 in every 500 individuals living in British Columbia (The Cerebral Palsy Association of BC, 2013). Cerebral palsy (CP) describes a group of disorders that affect body movement and muscle coordination due to damage in the brain from early pregnancy until about three years of age (Bax et. al, 2005). Cerebral palsy (CP) is a motor impairment that results from a lesion that occurs in the developing brain. CP varies in the timing and the location of the lesion in the brain, the clinical presentation and the severity of the impairments (Dimitrijević et al, 2014). For some unknown reason the lesion in the brain usually occurs in the area that controls muscle tone and movement of limbs. Consequently the brain is unable to control the amount of muscle flexibility. (Dimitrijević et al, 2014) According to the U.S. Centres for Disease Control and Prevention about 80% of people with CP have varying degrees of spasticity. Spasticity can vary from mild muscle stiffness to severe, painful, and uncontrollable muscle spasms (Dimitrijević et al, 2014). For unknown reasons, the damage tends to be in the area of the cerebral motor cortex that controls muscle tone and movement of the arms and legs, thus people with CP are unable to influence the amount of flexibility muscles should have (Bax et. al, 2005). In addition to tight and stiff muscles associated with spasticity, children with CP often have speech impairments, lack of coordination, different walking patterns, difficulty with gross and fine motor skills, and abnormal perception and sensation (BC Children’s Hospital, 2013)

Stroke

Stroke is characterized by a sudden loss of brain function that can be caused by an interruption of blood flow to the brain (ischemic stroke) or by the rupture of blood vessels in the brain (hemorrhagic stroke). As a result of a stroke, neurons in the affected areas of the brain begin to die, which can impact several areas of function depending on the location of the stroke (Heart and Stroke Foundation, 2012). When a stroke occurs in the regions of the brain involved with movement, post-stroke spasticity is common (Thilbaut et. al, 2013). Muscle weakness and loss of dexterity are most commonly seen acutely after the injury (Trompetto et al 2014). More time after the stroke other symptoms can appear characterised by muscle overactivity such as: ‘spasticity, increased deep tendon reflexes (also called tendon jerks), clonus, extensor spasms, flexor spasms, Babinski sign, positive support reaction, cocontraction, spastic dystonia, and associated reactions.’ (Trompetto et al 2014). Post-stroke spasticity does not usually set in until weeks, months, or sometimes years after stroke (Thilbaut et. al, 2013). According to the National Stroke Association, spasticity affects up to 58% of stroke survivors and significantly impacts stroke survivor quality of life and caregiver burden. Although the mechanism for the onset of spasticity remains unclear, the inability to relax the muscle (i.e., spastic dystonia) is likely to be connected to prolonged firing of α-motoneurons (Trompetto et al 2014). Furr-Stimming et al (2014) reported that post stroke spasticity is thought to happen due to a breakdown of control of the spinal stretch reflex mechanisms which cause muscle overactivity, which limits the range of movement of joints and limbs. This is what limits the recovery of motor function and dexterity in patients poststroke (Furr-Stimming et al 2014). Strokes can occur at any point along the lifespan, each year 200 to 300 Canadian children will experience a perinatal stroke (Heart and Stroke Foundation, 2012).


Multiple Sclerosis

Multiple Sclerosis (MS) is a disabling disease of the central nervous system (CNS), in which the protective myelin sheaths surrounding the nerves of the CNS are attacked (National Multiple Sclerosis Society, 2013). MS is the most common disabling neurological disorder affecting young adults (Morley et al, 2013). The causes of MS are currently unknown; however the effects include loss of balance, impaired speech, extreme fatigue, double vision, paralysis and spasticity. According to the MS Society of Canada, about 80% of people living with MS have varying degrees of spasticity, which is particularly significant because an estimated 100,000 Canadians are living with MS, ranking Canada as having one of the highest rates of MS in the world. Although MS is often diagnosed in adult’s ages 15-40 years old, it has also been known to affect children at ages as young as two years old, with no cure currently known to combat MS (National Multiple Sclerosis Society, 2013). In people dealing with MS, spasticity-related problems include: ‘involuntary muscle contractions, loss of dexterity, loss of balance, incontinence and pain’ (Morley et al, 2013) which were seen to lead to distress and embarrassment within the individual. Spasticity-related problems such as ‘falls, difficulty with toileting, dressing, transfers, stairs, mobility, driving, shopping, sleeping, writing, typing and eating’ (Morley et al 2013) had detrimental effects on the individuals emotional and social relationships through change of their self image, employment, fear of the future and loss of their role in their family. Understanding the pathology of MS is improving, however treatments are limited to disease modification and symptom management.

Spinal Cord Injury

Approximately 70% of individuals with spinal cord injury (SCI) have been shown to be spastic one year after injury (American Association of Neurological Surgeons, 2006). Spinal cord injurys (SCI) in children are rare, but it does represent approximately <4% of the overall incidence of SCI annually (National Spinal Cord Injury Statistical Center, 2004). The incidence increases with age, with >30% of injuries occurring between the ages of 17 and 23, and 53% occurring between the ages of 16 and 30 (Parent et al, 2011). The rate of recovery following SCI in young children is also thought to be faster. The American Spinal Injury Association has suggested that the classification of SCI severity (incomplete/complete) and the level of injury in the spinal cord may predict the likelihood of developing spasticity. Specifically, it has been thought that the more severe and higher up the injury, the greater the risk of developing spasticity, although further research needs to be done to confirm this claim. Spasticity develops months or years after the acute spinal cord injury and can lead to an increased loss of function (Rekand et al 2012). In a particular spinal cord injury study, it was discovered that of those patients reporting spasticity, 40% reported their spasticity to be problematic, such that activities of daily life were restricted and/or that the spasticity caused pain (Adams & Hicks, 2005). In addition to pain, functional consequences associated with SCI include impaired ability to walk, difficulties with self-care, fatigue, disturbed sleep, compromised safety, development of contractures, pressure ulcers, infections (Adams & Hicks, 2005), problems transferring (from bed to chair, chair to car etc), it can affect the placing of legs in their wheelchair, and cause problems with catheterisation. (Rekand et al 2012). However Rekand et al (2012) reported that ‘Light to moderate spasticity may have a positive effect on function’. Spasticity may allow for people with a SCI (with lower limb paralysis) to stand, and witness their paralysed legs move. Light to moderate spasticity helps improve circulation in the legs which decreases the likelihood of edema and reduces the risk of deep vein thrombosis. (Rekand et al 2012). These outcomes are one of the few positive roles of spasticity in those dealing with certain neurological conditions. However progressing from light-moderate spasticity, pronounced spasticity may contribute to incorrect posture, ulcers and pain. Treatment should start as soon as possible (Rekand et al 2012).

Traumatic Brain Injury

Spasticity often occurs in traumatic brain injury (TBI) patients who have damage to the brainstem, cerebellum or midbrain, which are all regions of the brain involved with movement (American Association of Neurological Surgeons, 2006). A TBI has the potential to affect reflex centres of the brain by interrupting message flow along different nerve pathways, which can result in changes to muscle tone, movement, and reflexes (Ross, Elie, Lombard, & Lisa, 2004). Since reflex centres in the brain are more complex than those found in the spinal cord (which by contrast are monosynaptic), treatment of spasticity in TBI patients is often more difficult than in SCI patients. Shortly after brain injury, many patients experience a period of hypertonicity in which their body posture becomes very rigid, elbows are commonly held stiffly at the sides, wrists and fingers bent, and fists are clenched. The legs are usually extended at the hips and knees, with ankles and toes flexed (American Association of Neurological Surgeons, 2006). As at TBI patient undergoes recovery, nerve signals that control motor functions main change, such that some signals may not reach the nerve centres of the brain, or the brain may send excessive signals, causing the muscles not to respond properly. The resulting spasticity can be endured permanently if not treated appropriately (Ross, et. al, 2004). While CP, stroke, MS, SCI and TBI are commonly cited in the literature to involve spasticity, other disorders associated with spasticity include: encephalitis, meningitis, neurodegenerative illnesses, amyotrophic lateral sclerosis (ALS), phenylketonuria (PKU), adrenoleukodystrophy, and brain damage caused by hypoxia (a lack of oxygen). (Ross, et. al, 2004)

Characteristics

Velocity Dependence and Exaggerated Stretch Reflex

Spasticity is a velocity-dependent resistance of a muscle to stretch. This means that an increase in speed of stretch results in an increase in muscle resistance until a certain point (Biering-Sorensen et. al, 2006). The velocity dependency distinguishes spasticity from other syndromes where changed resistance to passive movement of a joint may be present, for example in muscle rigidity seen in Parkinson’s patients and in contractures, which is a permanent tightening of non-bony tissues (Biering-Sorensen et. al, 2006). The increased resistance is usually not directly proportional to the speed of stretch in spastic individuals (and often only shows a modest dependence); however resistance must be different for high versus low speeds of passive movement and for flexion versus extension about the joint (Kheder & Nair, 2012). Spasticity may also manifest as an increased sensitivity in the stretch reflex response, which distinguishes spasticity from dystonia, which is a disorder characterized by involuntary muscle contractions that cause slow repetitive movements or abnormal postures (Kheder & Nair, 2012). Muscle activity is thus not seen in spastic patients as rest, but only manifests when the stretch reflex is activated voluntarily or involuntarily (Adams & Hicks, 2005).

Functional Impact

The functional impact of spasticity on a child’s movement experiences can range widely, from minor discomfort to complete immobility with pressure sores and contractures (Thompson, Jarrett, Lockley, Marsden, & Steveson, 2005). If poorly managed, spasticity can be responsible for muscle shortening and the development of tendon and soft tissue contractures; once present the secondary characteristics become extremely difficult to modify, with long lasting functional implications (Kheder & Nair, 2012). With children in particular, spasticity can cause the failure of normal muscle growth, torsion of long bones, join instability and degeneration. Walking can become slower or more difficult, children can experience frequent falls, and even the ability to self-propel a wheelchair can be compromised (National Institute for Health and Clinical Excellence, 2012). Daily living activities such as washing, dressing, toileting, sexual activity, and general self-care can also be implicated. Because of these detrimental affects spasticity can have consequences on emotional, mental and social health of the individual. Interestingly, although the effects of spasticity are primarily detrimental, there are cases in which spasticity can have positive effects, for instance in situations where muscle weakness in the absence of spasticity would otherwise inhibit individuals from standing or walking. It may also permit transfers or bed mobility through utilizing spasms (Kheder & Nair, 2012). For someone dealing with a SCI, where they normally cannot move their legs, spasms occurring within their legs can bring hope and joy knowing they can still move their legs. Spasticity can also vary depending on a child’s state of alertness, activity, or posture and can increase with anxiety, emotional state, surface contact, or other non-harmful sensory input (Thompson, et. al, 2005). It may worsen with movement of the involved muscles against gravity; however spasticity is not specific to particular tasks (Thompson et. al, 2005).

Associated Symptoms

Additional symptoms commonly associated with spasticity include:  Involuntary movements  Spasms  Clonus  Muscle fatigue  Contractures  Bone and joint deformities  Involuntary crossing of the legs  Abnormal posture  Delayed motor development  Inhibition of longitudinal muscle growth  Inhibition of protein synthesis in muscle cells  Speech impairments (American Association of Neurological Surgeons, 2006)

Assessment

Diagnosis and assessment of spasticity is generally separated into two different domains: (1) patient history and (2) the clinical examination.

Patient History

Obtaining patient history is a critical component of the physician’s diagnosis and assessment of spasticity. When children are impacted, patient history is often gathered in the presence of the child, caregivers, and physician such that the treatment goals important to both the patient and family can be discussed. It is recommended for physicians to ask open-ended questions to reveal the specific areas of concern and also to understand the child and family’s expectations (Rekand, 2010). Open-ended questions have been shown to be a useful way to set the scene and allow systematic assessments to flow naturally, while ensuring that treatment options remain focused on the priorities of the child and family (Kheder & Nair, 2012). The patient history portion of the assessment is also a critical opportunity for the physician to explore any specific symptoms the child may be experiencing and any treatments that have already been tried, for example medication use and dosages, use of recent therapy programs, and/or use of orthotics or splints (Rekand, 2010). It is also important to consider the impact of the child’s mood, self-image and motivation for therapy as this can influence potential treatment outcomes. For disorders with a genetic basis, it is especially critical to obtain any history of neurological or muscular disorders found in the family (Adams & Hicks, 2005).

The Examination

The clinical examination portion of the assessment is used to confirm the patient history, differentiate spasticity from other causes of hypertonicity, obtain quantitative measurements of spasticity, assess the impact on function, and to identify potential triggers (Rekand, 2010). The examination phase can be broken down into three stages. The first stage is purely observational, in which the physician documents posture, alignment and presence/absence of spontaneous spasms. The second stage involves an assessment of active involvement, in which the physician establishes whether spasticity or weakness is the main factor that is limiting function. The third and final stage involves an assessment of resistance to passive movement, which identifies the contributions of neural and non-neural components (Rekand, 2010).

Modified Ashworth Scale

In 1964 the Ashworth Scale was developed, however it was inadequate in measuring spasticity (Ghai et al 2013).This led to the creation of the Modified Ashworth Scale (MAS) which was developed in 1987 in response to concerns that the Ashworth Scale was indiscrete. Ghai et al (2013) reported that the MAS has an inter-rater reliability of 86.7% in assessment of elbow flexor muscle spasticity. The MAS the most frequently used clinical method to measure resistance to limb movement (Khedar & Nair, 2012). For this test, the patient is placed in the supine position (i.e. dorsal side is down, ventral side is up), as this will garner the most accurate and lowest score since tension anywhere in the body will increase spasticity. If the physician is testing a muscle that primarily flexes a joint, the joint is placed in a maximally flexed position and is moved to a position of maximal extension over one second (it is common to count “one thousand one”). Conversely, if the physician is testing a muscle that primarily extends a joint, then the joint is placed in a maximally extended position and is moved to a position of maximal flexion over one second (Adams & Hicks, 2005). The speed of joint movement is fast, due to the fact that spasticity is velocity dependent. The movement is then scored on a scale of 0 (no increase in muscle tone) to 4 (affected parts rigid in flexion or extension) to quantify the spasticity (Rekand, 2010). It is recommended that the test be done on both sides of the body to obtain a comparison point, and for the test to be done a maximum of three times at each joint to avoid the short-term effect of a stretch from impacting subsequent scores. Additionally, due to the influence of anxiety on muscle tone, the patient should be relaxed as much as possible during the examination (Adams & Hicks, 2005). The components of the Modified Ashworth Scale (MAS) reported by Ghai et al (2013) are as follows: 0 = No increase in muscle tone 1 = Slight increase in muscle tone, manifested by a catch and release or by minimal resistance at the end of the range of motion (ROM) when the part is moved in flexion or extension/abduction or adduction, etc. 1+ = Slight increase in muscle tone, manifested by a catch, followed by minimal resistance throughout the remainder (less than half) of the ROM 2 = More marked increase in muscle tone through most of the ROM, but the affected part is easily moved 3 = Considerable increase in muscle tone, passive movement is difficult 4 = Affected part is rigid in flexion or extension (abduction or adduction, etc.).

Severity of pain is assessed on a visual analog scale (VAS) where zero represents no pain and 10 represents the worst possible pain.

Triple flexion of the lower limb, consecutive to the flexor muscle spasm, is assessed using a 4-point scale: 0 = Hip and/or knee flexion <30[degrees], with or without mild gait disability 1 = Hip and/or knee flexion between 30[degrees] and 45[degrees], with moderate gait disability 2 = Hip and/or knee flexion between 45[degrees] and 60[degrees], with severe gait disability 3 = Hip and/or knee irreducible flexion with gait inability.

Range of Motion of the hip abductors is assessed passively by the abduction of the hip joint and graded as 0-3. 0 = Ability to abduct the thigh easily to 45[degrees] 1 = Ability to abduct the thigh to 45[degrees] with mild effort 2 = Ability to abduct the thigh to 45[degrees] with major effort 3 = Inability to abduct the thigh to 45[degrees].

Number of spasms experienced is recorded on spasm frequency scale. 0 = No spasm 1 = One spasm or fewer per day 2 = Between one and five spasms per day 3 = Between five and nine spasms per day 4 = Ten or more spasms per day.

Perineal hygiene is assessed using a 4-point scale, considering the ability of the patient to perform perineal hygiene care, related to the degree of adductor muscles spasticity. Hygiene score 0 = Hygiene performance with relative ease 1 = Hygiene performance with mild difficulty 2 = Hygiene performance with moderate difficulty 3 = Hygiene performance with severe difficulty.

Gait is assessed, when possible, using a 4-point score, representing the effect of obturator neurolysis on spasm and leg crossing: 0 = Patient able to walk with mild difficulty 1 = Patient able to walk with moderate difficulty 2 = Patient able to walk with severe difficulty 3 = Patient unable to walk.

While the Modified Ashworth Scale (MAS) provides a quick and easy method to measure spasticity, there are limitations to this method of assessment (see “Common Problems”). Other tests used to evaluate the severity and impact of spasticity include the 10-meter timed walk, goniometry tests, and the Medical Research Council grading scale of muscle strength (Adams & Hicks, 2005).

Treatments & Practical Applications

Since spasticity is only one component of upper motor neuron syndromes, current recommendations are that treatments for spasticity should be function focused, rather than simply targeted at reducing spasticity alone (Thompson et. al, 2005). Tilton et al (2010) suggests that a careful pre-treatment assessment of all the different components of the motor disorder should be performed in order to obtain accurate and objective information for the patient which they can use to predict their response to treatment. When spasticity leads to functional or postural loss, management of spasticity must be dealt with a progressive approach, from the most conservative treatment to the most invasive therapy (Ghai et al 2010). Spasticity in children is a difficult condition to treat and takes special consideration from Pediatric neurologists, developmental pediatricians, orthopedists, neurosurgeons, and physiatrists (Tilton et al 2010). Reduction in spasticity may not always be the desirable outcome; this is because some people may experience a decline in function when spasticity is decreased. It takes determining if spasticity has detrimental effects on patient function, quality of life and comfort. Spasticity doesn’t always need treatment, especially if there is no room for functional gain (Graham 2013).Specific and measurable goals of treatment should be established with both the children and parents ( Tilton et al 2010). Treatment options include pharmacological interventions, occupational therapy and physical therapy programs, functional electrical stimulation, muscle relaxant injections, and in severe cases, surgery.

Pharmacological Treatment

Currently, there is no agreed evidence-based model available to guide the choice of agent or dosing schedule; rather medications are administered based on a logical and pragmatic approach (Yelnik et. al, 2009). The drugs chosen and time of administration are based on the specific treatment goals outlined in the patient history portion of the physician’s assessment, and thus vary from patient to patient. Nonetheless, there is consensus in the literature that medications should be used alongside education and an effective physical therapy program rather than being administered in isolation (Thibaut et. al, 2013). The most common oral agents used to manage and/or reduce spasticity are baclofen, tizanidine, dantrolene, benzodiazepines and gabapentin, however the evidence base for all agents is fairly limited with few placebo-controlled trials (Yelnik et. al, 2009).

Diazepam

One medication often used for the treatment of spasticity is a low dose of diazepam. In children it is generally introduced at night to avoid sedative side effects during the day (Tickner et al 2012). A daytime dose can be added if the desired response is not achieved, however sedation should be monitored by the parents and clinician. The British National Formulary for Children recommends the total daily dose should not exceed 40 mg in children aged 12–18 years. The American Academy of Neurology (AAN) concluded that ‘diazepam should be considered as a short-term antispasticity treatment in children with CP, as there is insufficient evidence to support or refute the use of diazepam to improve motor function in this population’ (Tickner et al 2012). Common side effects include: drowsiness, sedation (which can be useful to aid sleep), weakness, ataxia and hypersalivation (Tickner et al 2012). Tolerance and dependence are issues when considering the long-term use of diazepam. Common withdrawal symptoms include: ‘agitation, twitching, nausea, seizures, insomnia and hyperpyrexia’ (Tickner et al 2012). Diazepam can still be used, however, when needed for long periods of time, a clinician who is experienced in withdrawal should monitor discontinuation in patients.

Baclofen

Baclofen is the most common oral agent used for patients experiencing spasticity (Thompson et. al, 2005). Clinical trials have involved patients with cerebral palsy, multiple sclerosis, spinal cord injury and stroke, with most studies showing positive effects in reducing hypertonia and spasms, however little attention has been paid to the functional benefits (Thompson et. al, 2005). Baclofen works by binding to the GABAB receptors which triggers ‘hyperpolarization of neurons, prevention of calcium influx, facilitation of potassium conductance, and decrease in excitatory neurotransmitter release’ (Furr-Stimming et al 2014). These events lead to presynaptic inhibition at the level of the spinal cord. Oral baclofen is absorbed in the gastrointestinal tract, but has a limited ability to cross the blood–brain barrier (Furr-Stimming et al 2014). Maximum doses of baclofen in children and adolescents vary from 40 to 80 mg daily which are divided in 3 or 4 doses (Tilton et al 2010). Caution must also be taken when administering baclofen to children who have epilepsy, as it has been found to potentially reduce the seizure threshold (Thompson et. al, 2005). Although oral baclofen is effective in reducing mild-to-moderate spastic tone, there are side effects which include: sedation, confusion, headache, and lethargy. These have been reported to affect up to 45% of baclofen users (Yelnik et. al, 2009). Intrathecal baclofen is a treatment in which a programmable pump is implanted into the abdomen, from which a catheter conveys the baclofen into the intrathecal space. Intrathecal baclofen (ITB) allows for a higher concentration of the drug to reach sensorimotor pathways of the spinal cord with fewer side effects (Furr-Stimming et al 2014). Doses by this method are often lower than oral doses, treatment effects have been shown to be greater, and side effects have been shown to be fewer (Albright, 1996). Specifically, this treatment is associated with improved gait and upper extremity function, and in long-term follow up studies the benefits have been shown to be sustainable over a longer period of time. However there are risks of complications such as inadvertently overdosing from baclofen which can result in death in extreme cases (Yelnik et. al, 2009). In a study on one patient with MS by Furr-Stimming et al (2014), they found that ITB therapy alongside physical therapy on a regular basis allowed the patient to walk without a walker, sometimes using a walking stick and no longer has spasms. The patient reported an improved quality of life. In patients who have suffered from a stroke, ITB therapy led to a 63% increase in both arm and leg strength (Furr-Stimming et al 2014). Use of the spastic right arm/hand improved to around 75% of their prestroke usage. The patient reported increase quality of life and mobility (Furr-Stimming et al 2014). Motor recovery is usually the greatest within the first 3 to 6 months poststroke, and is influenced by changes in muscle tone caused by spasticity, however studies have shown that an increase in function can occur up to 6 years poststroke (Furr-Stimming et al 2014). In children suffering from cerebral palsy, research evidence is limited regarding oral baclofen's effect for young children. Tilton et al (2010) conducted as study which revealed reduced spasticity and improved range of motion in children with CP on baclofen versus placebo. However another study did not see improvement in spasticity or scores on the Pediatric Evaluation of Disability Inventory. This leads to the conclusion that there is insufficient evidence to support or refute the use of baclofen for spasticity or functional impairment in childhood CP. Comparatively ITB has been well evaluated for children with CP (Tilton et al, 2010). However ITB is expensive, so careful analysis of the benefits of spasticity reduction and functional gain must be considered. Similarly with oral baclofen there insufficient to support or refute the use of ITB for spasticity in children with CP (Tilton et al, 2010) Walter et al (2014) concluded that ITB is an effective therapy option for children with congenital brain injuries suffering from severe spasticity. However the patients, parents/caregivers and clinicians have to be aware of the high incidence of complications and the need for considerable follow-up.

BTA Injections

Muscle relaxant injections such as botulinum toxin-A (BTA) injections are the most widely used treatment for focal spasticity (Moore et. al, 2008). BTA injections in children have been shown to improve arm and leg function and decrease pain, by inhibiting the release of acetylcholine at the neuromuscular junction. BTA induces a temporary weakness in the focal area that it is treating. Early treatment of spasticity with BTA injections can prevent contractures and deformities, which can help avoid surgical treatment (Dimitrijević et al 2014). BTA injections last for around three months, the muscle will recover and form new neuromuscular junctions as well as regenerate the original neuromuscular junctions. Only two BTA’s are available: BoNT-A (botox and disport) and BoNT-B. Only BoNT-A is licensed for use in children (Tickner et al 2012). BoNT-B has been used for children in a small number of studies but the evidence is limited. Both botox and dysport have a similar onset and duration(apparent at 12-72 hrs after injection, peak at 3-4 weeks) (Tickner et al 2012). Improvements are expected after 2 weeks, and the dosage repeated after 12 weeks. Administering BTA injections in children may require the need for a topical anaesthetic. (Tickner et al 2012).

Cannabis

Recently there has been an increased interest in the use of cannabis and cannabinoids to treat both pain and spasticity in the MS population (Yelnik et. al, 2009). THC is the active ingredient in cannabis which is produced as a pharmaceutical product; recent studies have shown improvements in spasticity as measured by the Ashworth Scale for THC-treated individuals, however there is still a need for further trials to assess the efficacy of cannabis (Thibaut et. al, 2013). Recent trials have also supported symptomatic benefits for using whole-plant extracts, although further trials are also needed to confirm preliminary findings. Possible long-term side effects of using cannabis treatment for spasticity include psychomotor slowing, increased risk of psychosis, and cognitive changes, which are particularly concerning for child cognitive development (Thibaut et. al, 2013).

Occupational Therapy and Physical Therapy Programs

Occupational therapy (OT) and physical therapy (PT) programs are often focused on muscle stretching and range of motion exercises in spasticity patients (Thompson et. al, 2005). The aims of physiotherapy treatment should be to improve function whether its mobility or dexterity, ease pain, reduce the risk of unnecessary complication, decrease spasms and assist with the daily tasks of hygiene, dressing, and transferring to decrease caregiver burden (Ghai et al 2013). Braces are occasionally used to prevent tendon shortening; however the use of orthotics requires careful assessment where frequent spasms and significant levels of spasticity are present. In these cases, splinting should be used with caution and soft splints (such as foam or sheepskin) are often recommended (Yelnik et. al, 2009). Mobilization of the affected limbs as well as the prevention of a prolonged shortened muscle position of the affected limb are probably the most important things to do in order to prevent and treat muscle spasticity (Trompetto et al, 2014). Effective OT and PT programs have been shown to improve postural control and help to avoid muscle retractions, however they have not been shown to attenuate spasticity in the long-term (Thibaut et. al, 2013). Combining muscle stretching and exercise programs with drug treatment has been shown to be an effective treatment method (Anttila, Autti-Ramo, Suoranta, Makela, & Malmivaara, 2008). Home-based PT programs are often recommended particularly with spastic children, as the majority of a child’s spasticity management realistically occurs in the home with the assistance of family members. Attention is primarily placed on posture and positioning of the trunk, head and limb posture, with the main goals of minimizing changes in visco-elastic properties of muscles, joints, and connective tissue and to maintain the child’s range of movement while preventing the development of contractures. A key component of home-based PT programs is changing the patterns of spasticity or spasms to prevent them from becoming self-perpetuating, this is often achieved through daily stretching programs which become incorporated into the child and family’s daily routine (Anttila et. al, 2008). The main modality of neurophysiotherapists is to restore ‘good’ normal movement patterns versus quick movements using abnormal muscle patterns which can increase tone and can limit long-term rehabilitation (Graham 2013) Performing an active or passive movement program into the patient’s daily life is crucial to maintaining adequate range of joint movement and preventing contracture.

Standing

Research studies in patients with SCI, acquired brain injury, CP, stroke and MS have demonstrated beneficial effects of standing, with changes found in the passive range of movement and in spasticity (Thompson et. al, 2005). The beneficial effects of standing have been postulated to be secondary to the promotion of anti-gravity muscle activity in the trunk and lower limbs, maintenance in soft tissue and joint flexibility, modulation of neural component of spasticity through prolonged stretch and altered sensory input, reduction of lower limb spasms and positive psychological effect (Thibaut et. al, 2013). Standing using a standing frame or weight-bearing on a tilt table allows a stretch at the ankle joint and helps to combat contracture development (Graham, 2013). However, there is no clear guidance from the literature regarding the optimum time and frequency for standing; advice is often individualized to the child and their lifestyle (Thompson et. al, 2005).

Active Movement

Active movement is defined as movement produced by the individual’s own muscles (Thibaut et. al, 2013). Although it was initially feared that active movement would increase spasticity, studies in patients with CP, stroke, and MS has shown to increase levels of function and decrease fatigue (Thiabut et. al, 2013). Active movement has also been shown to increase strength, re-educate movement patterns, and improve cardiovascular fitness in patients. In fact, the effects of active movement in spasticity have been shown to be greater than those seen in passive movement alone (Thompson et. al, 2005). Passive Movement Passive movement is defined as movement of the body or of the extremities of a patient performed by another person or mechanical aid, without voluntary motion on the part of the patient (Thibaut et. al, 2013). It may be useful when active movement is not possible or in cases where active movement induces severe spasticity or spasms. The premise behind passive movement is the idea that by moving all affected body parts through their available range of motion on a daily basis, secondary non-neural changes can be prevented and comfort improved Splinting and plaster casting are both used to aid passive stretching to maintain range of joint movement (Graham 2013). However, there is little evidence to support this premise, and little evidence to suggest that changes are seen immediately after movement is sustained (Anttila et. al, 2008).

Positioning

Positioning is a critical component of home-based PT programs to avoid the formation of pressure ulcers in patients with severe spasticity (Anttila et. al, 2008). It is also important that once passive and/or active movements are completed, benefits are maintained by ensuring the child is positioned or seated appropriated afterwards to avoid the typical positions which are commonly adopted as a result of spasticity. Correct posture may be maintained while in bed using a range of positioning tools such as a ‘T-Bar’ or trunk wedge to help trunk stability and reduce adductor spasticity (Graham, 2013). Using these aids allows for an increase in trunk stability and can help alleviate limb spasticity and maximising motor control. Adequate posture while in a wheelchair can be accomplished tilting the back rest of the wheelchair and using a suitable head rest or cervical collar (Graham 2013). A correct seating system should try to stabilize the pelvis without lateral tilt or rotation, but with a slight anterior tilt, so that the spine adopts its normal curves (Ghai et al 2013). Incorrect positioning in bed, especially in the early stages poststroke or brain injury, is a major contributor of unnecessary spasticity. An orthosis or splint is ‘a device designed to apply, distribute, or remove forces to or from the body in a controlled manner to control body motions and/or alter the shape of body tissues, e.g., ankle foot orthosis, insoles, ankle supports, wrist/hand/elbow splints, knee splints, spinal brace, hip brace, neck collar’ (Ghai et al 2013). Using splints and casts can prevent contractures in the spastic limb and serial casting can improve the range of motion in a joint that is contracted, using a new cast every few days also improves the range of motion (Ghai et al, 2013).


Experimental Techniques

Cooling of muscles

Cooling the muscles inhibits monosynaptic stretch reflex and lowers receptor's sensitivity, which could inhibit spastic muscles, however this effect is short term (Ghai et al 2013). Different techniques like quick icing and evaporating spray like ethyl chloride are occasionally used to help with spasticity (Ghai et al 2013).

Heat

Heat may increase the elastic property of muscles, which may cause relaxation of the spastic muscles. Techniques using heat include ultrasound, fluidotherapy, paraffin, and superficial heat. Using these techniques should be combined with active and passive stretching and exercise. (Ghai et al 2013)

Electrical therapy

Functional electrical stimulation can benefit selected individuals with spasticity affected by upper motor neuron pathologies. This can be accomplished using a Functional Electrical Stimulation (FES) bike, where electrodes are placed on major muscle groups: Quadriceps, Hamstrings, Gluts. These electrodes send impulses which contract the muscles and allow them to perform the movement of pedalling the stationary bike by themselves. Due to the loss of sensation from stroke, brain injury or SCI, more stimulation can be tolerated causing a greater contraction of the muscle. Transcutaneous electrical nerve stimulation (TENS) has been found to reduce spasticity through its nociceptive action and reduction of pain (Ghai et el, 2013). This modality is especially helpful in dealing with spinal cord injuries, where electrical stimulation can be tolerated well and can actually contract muscles, reducing spasms, increasing metabolism and improving circulation of the affected limb to reduce the likelihood of developing pressure sores.

Vibration

Chen et al (2015) conducted a study which evaluated the effect of whole body vibration (WBV) on lower limb spasticity and ambulatory function in children with cerebral palsy (CP). The Whole-body vibration was applied to children with CP while they were standing supported on a vibrating platform. The methodology behind this technique is that the ‘vibrations are believed to initiate muscle contractions by stimulating muscle spindles and alpha motor neurons’ (Chen et al 2015). The results revealed that spasticity (measured with the Wartenburg Pendulum test and Modified Ashworth Scale) was decreased. The ambulatory function of the affected limbs improved significantly after the WBV. The assisted range of motion for both knee and ankle joints also increased. The results of this study showed that the WBV is an effective modality for controlling spasticity and improving ambulatory function. These effects might be able to promote children’s active participation in exercise training protocols and therapeutic interventions. WBV provides an chance for patients with neuromuscular disorders (SCI, Stroke, brain injury, CP) who lack motivation to engage in muscle strengthening exercises.WBV is also thought to contribute to an increased oxygen consumption, muscle temperature, skin blood flow and muscle power (Cochrane et al., 2008 and Lohman Iii et al., 2007).When applied repetitively, there has been positive long-term effects on muscle strength, balance, and bone density (Rauch, 2009). This study and results support the clinical use of WBV in rehabilitation for people/children with CP. (Chen et al 2015)

Surgery

In cases of severe spasticity where other less-invasive treatment methods have proven to be ineffective, surgery may be done to cut a nerve-muscle pathway (selective dorsal rhizotomy), or done to surgically implant intrathecal baclofen (Albright, 1996). However surgery in children is risky and can come with complications. Treating spasticity with other modalities should be the first step before surgery is considered.

Common Problems

Inconsistencies in Definition

As discussed earlier, the lack of a consistent definition of the term “spasticity” poses problems both clinically and in the field of research. Childhood hypertonia, dystonia and rigidity are terms which are often used interchangeably with spasticity although they all manifest as different clinical features (Thompson et. al, 2005). The lack of general agreement over terminology in clinical situations leads to inconsistencies in labeling of pediatric signs and symptoms by clinicians, as well as researchers (Adams & Hicks, 2005). There is currently a need for a consistent set of well-established definitions in order to allow accurate communication between clinicians and to enable accurate selection of children for medical therapy and clinical research trials.

Inconsistencies in Severity Classification

Inconsistencies also exist in the methods used to classify the severity of spasticity (Adams & Hicks, 2005). Although it is often easy to recognize spasticity, it is not as easy to quantify it, as few quantitative measures for establishing the degree of severity exist (Antilla et. al, 2008). Studies of appropriate rehabilitative interventions in chronic movement disorders in children have been hampered by the difficulty of establishing homogenous cohorts for study, due to the inconsistencies in the classification system for spasticity severity (Thibaut et. al, 2013). It is important to have quantitative measures in order to be able to evaluate the potential effect of treatment interventions, which is a current weakness in the literature. Although manual tests such as the Ashworth Scale are often used in the clinical setting, biomechanical and electrophysiological methods used for research are often more objective and quantifiable, and should therefore be investigated further (Adams & Hicks, 2005).

Weakness of the Modified Ashworth Scale

A major concern established with the MAS is the high inter-rater variability (Haas, Bergstrom, Jamous, & Bennie, 1996). Circumstances under which the child was tested such as the time of day, hours and type of activity before the test, ambient temperature, emotional status, general health, drug use, clothing and especially the testing position may also contribute to the variability in scores (Thibaut et. al, 2013). To minimize these confounding variables, it is recommended that strict guidelines for assessment procedures are established and followed with regular training for clinicians. For instance, it is important that clinicians manipulate the limb at speeds below the threshold of stretch reflex activity and compare such resistance to resistance generated at higher speeds of movement. In addition to various speeds of movement, various joint directional movements should also be tested, with the clinician noting any presence of increased resistance and/or velocity dependence (Haas et. al, 1996). It has also been recommended that the clinician include information of passive range of movement, the resting limb posture before stretch and possible pain elicited during the stretch. If the range of movement is decreased, for example, due to contracture, one has to be aware that the results obtained from clinical spasticity assessment may as well be limited (Haas et. al, 1996). Likewise, if pain is present during the procedure, the value of the measurements may be questionable (Adams & Hicks, 2005). The modified Ashworth Scale also says little about the loss of function related to spasticity and is not suitable for assessing the effect of treatment on spasticity (Rekand et al, 2012)

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