What is spasticity

Spasticity is stiff or rigid muscles or more commonly referred to as “tightness” or “stiffness” ¹. Spasticity is a condition in which there is an abnormal increase in muscle tone, unusual tightness or stiffness of muscle, which might interfere with movement, speech, or be associated with discomfort or pain. Reflexes (for example, a knee-jerk reflex) are stronger or exaggerated. Spasticity can interfere with walking, movement, or speech. Spasticity can present variably in a clinical setting, sometimes with a subtle neurological manifestation and, other times, with severely increased muscle tone leading to immobility of joints.

Spasticity is often caused by damage to the part of the brain that is involved in movements under your control. Spasticity may also occur from damage to the nerves that go from the brain to the spinal cord that control muscle movement.

Spasticity may occur in association with spinal cord injury, multiple sclerosis (MS), cerebral palsy, stroke, brain or head trauma, amyotrophic lateral sclerosis (ALS), hereditary spastic paraplegias, and metabolic diseases such as adrenoleukodystrophy, phenylketonuria (PKU), and Krabbe disease.

Spasticity symptoms may include hypertonicity (increased muscle tone), clonus (a series of rapid muscle contractions), exaggerated deep tendon reflexes, muscle spasms, scissoring (involuntary crossing of the legs), and fixed joints (contractures). The degree of spasticity varies from mild muscle stiffness to severe, painful, and uncontrollable muscle spasms. Spasticity can interfere with rehabilitation in patients with certain disorders, and often interferes with daily activities.

Spasticity may also affect speech. Severe, long-term spasticity may lead to contracture of muscles. This can reduce range of motion or leave the joints bent.

Exercise, including muscle stretching, can help make symptoms less severe. Physical therapy is also helpful.

See your health care provider if:

• The spasticity gets worse
• You notice deformity of the affected areas

Treatment may include such medications as baclofen, diazepam, tizanidine or clonazepam. Physical therapy regimens may include muscle stretching and range of motion exercises to help prevent shrinkage or shortening of muscles and to reduce the severity of symptoms. Targeted injection of botulinum toxin into muscles with the most tome can help to selectively weaken these muscles to improve range of motion and function. Surgery may be recommended for tendon release or to sever the nerve-muscle pathway.

Spasticity vs Spasm

Spasms are contractions of the muscles of the hands, thumbs, feet, or toes. Spasms are usually brief, but they can be severe and painful.

Spasms symptoms depend on the cause. They may include:

• Cramping e.g., Charley horse, leg cramps
• Fatigue
• Muscle weakness
• Numbness, tingling, or a “pins and needles” feeling
• Twitching
• Uncontrolled, purposeless, rapid motions

Nighttime leg cramps are common in older people.

Spasms in the muscles often have no clear cause.

Possible causes of hand or foot spasms include:

• Abnormal levels of electrolytes, or minerals, in the body
• Brain disorders, such as Parkinson disease, multiple sclerosis, dystonia, and Huntington disease
• Chronic kidney disease and dialysis
• Damage to a single nerve or nerve group (mononeuropathy) or multiple nerves (polyneuropathy) that are connected to muscles
• Dehydration (not having enough fluids in your body)
• Hyperventilation, which is rapid or deep breathing that can occur with anxiety or panic
• Muscle cramps, usually caused by overuse during sports or work activity
• Pregnancy, more often during the third trimester
• Thyroid disorders
• Too little vitamin D
• Use of certain medicines

Home care for spasms

If vitamin D deficiency is the cause, vitamin D supplements may be suggested by the health care provider. Calcium supplements may also help.

Being active helps keep muscles loose. Aerobic exercise, especially swimming, and strength building exercises are helpful. But care must be taken not to overdo activity, which may worsen the spasms.

Drinking plenty of fluids during exercise is also important.

Spasticity vs Tone

Muscle tone is regulated by signals that travel from the brain to the nerves and tell the muscles to contract. Normally, even when relaxed, muscles have a very small amount of contraction that gives them a springy feel and provides some resistance to passive movement. A characteristic of a muscle brought about by the constant flow of nerve stimuli to that muscle, which describes its resistance to stretching. Abnormal muscle tone can be defined as: hypertonus or hypertonia (increased muscle tone, as in spasticity); hypotonus or hypotonia (reduced muscle tone); flaccid (paralysis); atony (loss of muscle tone).

Spasticity causes

Spasticity is considered to be a positive sign of the upper motor neuron syndrome, which refers to motor behaviors resulting from lesions proximal to the alpha motor neuron, therefore within the spinal cord or brain. Other positive features of upper motor neuron syndrome include exaggerated muscle stretch reflexes and up-going plantar reflex. Negative features include motor weakness, slowed movement, loss of dexterity, or selective motor control. An upper motor neuron injury leads to loss of inhibition downstream and hypersensitivity of the reflex arc within the spinal cord. Many clinical scenarios can lead to spasticity, such as stroke, cerebral palsy (CP), anoxia, traumatic brain injury (TBI), spinal cord injury, multiple sclerosis (MS), and other central nervous system (CNS) neurodegenerative diseases.

Spasticity may be caused by any of the following:

• Adrenoleukodystrophy (disorder that disrupt the breakdown of certain fats)
• Brain damage caused by lack of oxygen, as can occur in near drowning or near suffocation
• Cerebral palsy (group of disorders that can involve brain and nervous system functions)
• Traumatic brain injury
• Multiple sclerosis (MS)
• Neurodegenerative illness (illnesses that damage the brain and nervous system over time)
• Phenylketonuria (disorder in which the body can’t break down the amino acid phenylalanine)
• Spinal cord injury
• Stroke

This list does not include all conditions that can cause spasticity. Of the diseases mentioned, spasticity affects approximately 35% of those with stroke, more than 90% with cerebral palsy, about 50% of traumatic brain injury patients, 40% of spinal cord injury patients, and between 37% and 78% of multiple sclerosis patients ¹.

Spasticity signs and symptoms

Symptoms of spasticity include:

• Abnormal posture
• Carrying the shoulder, arm, wrist, and finger at an abnormal angle because of muscle tightness
• Exaggerated deep tendon reflexes (the knee-jerk or other reflexes)
• Repetitive jerky motions (clonus), especially when you are touched or moved
• Scissoring (crossing of the legs as the tips of scissors would close)

Spasticity may cause pain, fatigue and other problems. Also, spasticity can become a barrier to your daily activities, walking, sitting, positioning and sleep.

Typically, the most common sign on exam is resistance to a passive change in a joint angle. It is most commonly noted in the flexor muscles of the upper extremities, the proximal extensor muscles of the lower extremities, and the distal flexor muscles of the lower extremities. As such, depending on the insult, specific patterns may arise that can aide in treatment.

Cerebral palsy

Children with cerebral palsy tend to exhibit one of the following spasticity patterns:

• Diplegic pattern: Scissoring, crouching, and toe walking
• Quadriplegic pattern: Diplegic patterning in addition to flexion of the elbow, flexion of the wrist and fingers, adduction of the thumb, and internal rotation, pronation, or adduction of the arms
• Hemiplegic pattern: Plantar flexion of the ankle, flexion of the knee, adduction of the hip, flexion of the wrist and finger, adduction of the thumb, and flexion, internal rotation, pronation, or adduction of the arms

Equinovarus positioning of the foot is a common posture in the lower extremity, and it can be a major limitation to functional transfers or gait as a child grows older.

Spasticity of the upper extremities

The following patterns present in patients with cerebral palsy, stroke, or traumatic brain injury (TBI) ²:

• Adduction and internal rotation of the shoulder
• Flexion of the elbow and wrist
• Pronation of the forearm
• Flexion of the fingers and adduction of the thumb

The following flexor patterns can occur in patients with cerebral palsy, multiple sclerosis (MS) , or traumatic brain injury or who have suffered a stroke ²:

• Hip adduction and flexion
• Knee flexion
• Ankle plantar flexion or equinovarus positioning

The following extensor patterns may be seen in patients following traumatic brain injury:

• Knee extension or flexion
• Equinus and/or valgus ankle
• Great toe dorsiflexion or excessive toe flexion

Spasticity complications

Complications of spasticity can vary. In a severe state, spasticity can interfere with daily function. It can cause extreme discomfort or pain for the patient and interfere with hygiene and the caregiver’s ability to provide care. This, in turn, can increase the risk of developing pressure ulcers, which can lead to infection and sepsis. It also can lead to bone fractures, subluxation, dislocation, and increased the risk of heterotopic ossification.

Spasticity diagnosis

Clinicians may be presented with a patient who has (1) new-onset spasticity as an initial symptom of an underlying neurological illness or (2) as existing spasticity that has worsened as a result of the progression of the known chronic neurologic condition or an aggravating factor. When evaluating a patient with new spasticity, the clinician must obtain a history and progression of the symptoms, including any motor weakness, altered sensation, pain, bladder and/or bowel dysfunction, and sexual dysfunction. Additionally, a complete history should include family history, travel history, diet, and any compromised immunity. The physical exam should include a neurological evaluation of muscle tone, motor power, reflexes, and sensation.

A patient may present with new-onset spasticity after suffering a stroke, spinal cord injury, or traumatic brain injury. On the other hand, they may have been diagnosed with MS years ago or have had cerebral palsy since infancy and present with new or worsening of pre-existing spasticity. On physical exam, hallmark findings include high muscle tone in muscle groups such as the shoulder adductors; elbow, wrist, and finger flexors; and forearm pronators. A characteristic finding in the hand includes a “thumb in palm” deformity, where excessive finger flexion and adduction of the thumb results in a clenched fist, with fingers wrapping around the thumb. In the lower extremities, the increased tone is especially prominent in the hip adductors, knee flexors and extensors, and plantar flexors and invertors of the ankle. Patients may report difficulty with footwear if their spasticity involves constant, high tone of the extensor hallucis longus or long toe flexors. On physical exam, the clinician will notice that spasticity varies with speed of movement; meaning the faster the muscle is moved or stretched, the greater the resistance to stretch or passive elongation is felt. Additional physical exam findings include clonus, spastic co-contractions, and spastic dystonia. Clonus is defined as an alternating muscle contraction and relaxation of the agonist and antagonist muscles. Spastic co-contractions are abnormal antagonist contractions that present during voluntary agonist effort. Spastic dystonia is a muscle contraction that is present at rest, leading to a constant clinical posture that is highly sensitive to stretch.

In patients with a previous neurologic insult, a thorough history and physical examination is necessary to rule out any factors that can exacerbate spasticity (e.g., medication changes, noxious stimuli, increased intracranial pressure).

Your doctor will perform a physical exam and ask about your symptoms, including:

• When was it first noticed?
• How long has it lasted?
• Is it always present?
• How severe is it?
• Which muscles are affected?
• What makes it better?
• What makes it worse?
• What other symptoms are present?

For a patient with worsening of chronic spasticity, which is often a more common reason for consultation than new onset spasticity, a clinician must evaluate for any triggers, disease progression, and the possibility of a new disease. Triggers may include skin, visceral, drug-related, or device-related issues. Skin issues may present as ulcers, ingrown toenails, boils, and infections. Visceral issues include constipation, urinary tract infections or calculi. Rapid withdrawal of antispasmodic agents can lead to worsening spasticity. Lastly, poor seating, an ill-fitting orthotic, or failure of an intrathecal baclofen pump can all be device-related triggers. Spasticity also can be worsened by other noxious stimuli such as infections, injuries, deep vein thromboses (DVT), or stress.

Laboratory studies (e.g., complete blood count [CBC] and culturing of urine, blood, cerebrospinal fluid) may help to rule out infection.

Spasticity is difficult to quantify ³, but clinically useful scales include the following:

• Ashworth Scale/Modified Ashworth: From 0-4 (normal to rigid tone)
• Tardieu Scale
• Penn Spasm Frequency Scale

Spasticity is frequently graded using the modified Ashworth scale, which is graded 0 to 4. Other commonly used scales include the Tardieu scale and Penn spasm frequency scale.

Ashworth Scale

• Zero is defined as no increase in tone.
• 1 is a “catch and release,” or minimal resistance towards end-of-range of motion (ROM).
• 1+ is similar to a grade of 1, but with a catch that is followed by resistance through less than half of range of motion.
• 2 is increased muscle tone through the majority of range of motion while still able to move the affected part.
• 3 is difficult passive movement throughout the majority of range of motion.
• 4 presents with the affected part in rigid flexion or extension.

Tardieu Scale

• Velocity to stretch is graded from V1 (as slow as possible) to V3 (as fast as possible).
• Muscle reaction is graded from 0 (no resistance through passive movement) to 5 (joint being immobile).
• Spasticity angle is graded as R1 (angle of catch at velocity V2 or 3) or R2 (full range of motion when the muscle is a rest and tested at V1 velocity).

Penn Spasm Frequency Scale

• 0 is no spasms.
• 1 is no spontaneous spasms; only elicited through vigorous sensory and motor stimulation.
• 2 is occasional spontaneous spasms and easily induced spasms, occurring less than once per hour.
• 3 is spasms occurring between 1 and 10 times per hour.
• 4 is more than 10 spasms per hour.

Spasticity treatment

When considering treatment for spasticity, the clinician must take into account the cause of the spasticity, the timing of onset, medical comorbidities, the patient’s support system, and the overall goals of management. As with other conditions, treatment options follow a stepwise approach, starting with more conservative routes and extending to more invasive surgical procedures. One of the initial treatment approaches involves identifying and avoiding noxious stimuli, such as infection, pain, DVT (deep vein thrombosis), heterotopic ossification, pressure ulcers, urinary retention or stones, and ingrown toenails. Next, it is crucial to implement physical modalities and therapeutics, such as stretching, splinting, serial casting, heat and cold modalities, direct tendon pressure, functional electrical stimulation, vibration, and biofeedback. Pharmacotherapy offers several options currently approved by the Food and Drug Administration (FDA). These include baclofen, tizanidine, dantrolene, and diazepam. These systemic medications may provide relief in cases of mild to moderate spasticity, and have their best effects in spasticity secondary to spinal cord injury or MS. Though these agents may reduce tone and decrease pain, they have not been shown to improve function significantly.

After determining the cause of your spasticity, the doctor may refer you to a physical therapist. Physical therapy involves different exercises, including muscle stretching and strengthening exercises. Physical therapy exercises can be taught to parents who can then help their child do them at home.

Treatment for spasticity usually involves a combination of the following options:

• Exercises. Physical and occupational therapists can teach you stretching, positioning and exercise activities that may help maintain range of motion and prevent shortening or tightening of the muscles (contracture).
• Oral medications. Certain prescribed medications given by mouth (orally) may help reduce muscle spasticity. These need to be taken as instructed.
• Intrathecal therapy. In rare cases, a pump used to directly deliver medicine into the spinal fluid and nervous system. Sometimes, spasticity may be treated with medications administered 24 hours a day directly into the fluid surrounding your spinal cord. The medication is delivered via an implantable pump and catheter system.
• Injections.
  • Botulinum toxin (Botox) injections into affected muscles may decrease the muscle signals that cause spasticity. The injections provide temporary relief, allowing you to move and strengthen your muscles. You may have injections every three months.
  • Phenol or alcohol injections into your peripheral nerve near the spastic muscles may reduce your muscle spasms.
• Neurosurgery and orthopedic surgery procedures. Surgical procedures to destroy (ablate) motor nerves of sensory spinal roots may stop the spasticity.

Bracing

Bracing is a first line option for children with gait abnormalities resulting from spasticity. Spastic muscles are inherently weak, so flexible deformities can be managed with braces in order to augment function.

Spasticity exercises

Physical therapists use exercise and therapeutic play activities to address movement, coordination, balance, strength and endurance issues in children and young adults.

Spasticity medication

Your doctor may prescribe medicines for you to take to help with muscle spasticity. Some common ones are:

• Baclofen (Lioresal)
• Dantrolene (Dantrium)
• Diazepam (Valium)
• Tizanidine (Zanaflex)

These drugs have side effects. Call your doctor if you have any of the following side effects:

• Being tired during the day
• Confusion
• Feeling “hung over” in the morning
• Nausea
• Problems passing urine

DO NOT just stop taking these medicines, especially Zanaflex. It can be dangerous if you stop abruptly.

Baclofen

This functions as a gamma-aminobutyric acid (GABA) agonist at GABA receptors, thereby increasing the overall inhibitory effects within the reflex pathway. By activating presynaptic GABA receptors, the influx of calcium is reduced, suppressing the release of excitatory neurotransmitters from the presynaptic axon. Activation of postsynaptic GABA receptors increases potassium egress and maintains membrane polarization, making it more difficult to depolarize the postsynaptic cell and decreasing the effect of any excitatory neurotransmitters released from presynaptic axon. As a result, this decrease in neuron excitability also decreases input to muscle fibers as well as muscle spindle sensitivity. Side effects of baclofen include sedation and drowsiness, to which the patient may develop tolerance with time. Baclofen may also lower the seizure threshold and cause muscle weakness, gastrointestinal symptoms, tremors, insomnia, and confusion. Baclofen is the drug of choice for spinal forms of spasticity and MS and also is used for traumatic brain injury-induced spasticity. Sudden withdrawal of baclofen can lead to seizures, hallucinations, rebound spasticity with associated fever, renal failure, and death. Thus, discontinuation of baclofen requires a slow taper. Baclofen is renally metabolized, so in patients with the underlying renal disease, it must be renally dosed. If changing to intrathecal baclofen from oral, there may be an association with withdrawal, as the cerebral concentration is relatively low compared to oral use. Baclofen also poses a theoretical interference with recovery after traumatic brain injury. Dosing typically starts at 5 mg two or three times a day, and may be increased by 5 mg every 3 to 5 days, up to 80 mg/day, the FDA-recommended maximum dose. However, higher doses have been reported to be tolerated just as well.

Tizanidine

This drug is an alpha-2 adrenergic agonist, chemically related to clonidine. Its mechanism of action is to enhance presynaptic inhibition of the spinal reflex. Its side effects include sedation and drowsiness in up to 50% of patients, liver damage, hypotension, dry mouth, bradycardia, and dizziness. Clinical trials have shown tizanidine to be as effective as oral baclofen or diazepam but with a better overall tolerability. Precautions to take with tizanidine include frequent monitoring of liver function tests (LFTs), as it is metabolized by the liver. Additionally, tizanidine requires frequent dosing due to its short half-life and is contraindicated if simultaneously using intravenous ciprofloxacin due to cytochrome P450 inhibition. Dosing for tizanidine begins at 2 to 4 mg/day, typically at bedtime, and may gradually be increased in dose and frequency to 36 mg/day divided 3 to 4 times a day, depending on patient tolerance.

Dantrolene Sodium

This is unique to spasticity treatment when compared to other agents because it acts peripherally at the level of the muscle by blocking the release of calcium from the sarcoplasmic reticulum. As a result, a reduction of extrafusal muscle fiber contraction strength and muscle spindle sensitivity is achieved. Dantrolene minimally affects smooth or cardiac muscle. An important adverse effect is that 1% of patients suffer from liver toxicity. The highest risk is in female patients over the age of 30 who have been taking higher doses for more than 2 months. This liver toxicity carries with it the risk of hepatonecrosis, so liver function tests should be monitored closely. Additional adverse effects include drowsiness, sedation, weakness, fatigue, paresthesias, diarrhea, nausea, and vomiting. Dantrolene is the preferred agent for spasticities of cerebral origin, such as cerebral palsy or head injury. Its use is often limited in spinal cord injury and MS due to its associated adverse effect of weakness. It is also used as a treatment for malignant hyperthermia, neuroleptic malignant syndrome, and hyperthermia from baclofen withdrawal. Dosing begins at 25 mg twice daily to be increased to 400 mg daily, divided into doses two or three times a day.

Diazepam

This functions by facilitating GABA’s effects on GABA receptors, leading overall to membrane hyperpolarization and decreased firing of neurons. Its net effect is increased presynaptic inhibition and reduced reflexes. Of all the aforementioned antispasmodic agents discussed, diazepam is the most sedating. It also can lead to memory impairment and decreased REM sleep. However, it has shown to be beneficial for spasticity due to MS and spinal cord injury. It is often unsuitable in traumatic brain injury patients due to its side effect of memory impairment. Diazepam undergoes hepatic metabolism, so its clearance can be affected by concurrent use of other hepatically metabolized agents, and it can have a very long half-due to its active metabolites. If used together with alcohol, it can lead to the significant central nervous system (CNS) depression. Diazepam overdose can be treated with flumazenil, and like other agents, may lead to withdrawal symptoms if not tapered gradually. The starting dose is typically 4 mg at bedtime or 2 mg twice a day and can be increased to 60mg per day.

In addition to oral agents, spasticity can be managed with local interventions, such as diagnostic nerve blocks, chemical neurolysis, chemodenervation with botulinum toxin, and motor point blocks. These procedures are the typical choice for treatment of focal spasticity or when the systemic effects of the oral agents mentioned above are prohibitive at required treatment doses.

Diagnostic Nerve Blocks

A local anesthetic is injected perineurally using electrostimulation as guidance to block nerve conduction for a few hours. This temporary measure allows the clinician to plan for more permanent interventions such as chemoneurolysis, botulinum toxin, or possible surgery because seeing the temporary reduction in spasticity allows for assessment of potential benefit to be obtained from longer lasting procedures. These local anesthetics function by blocking the voltage-gated sodium channels on the axon, thereby preventing the depolarization of the axon membrane and interrupting travel of the signal along the axon. Local anesthetics should not be injected through infected skin or skin that cannot be properly cleaned. Common agents used include lidocaine and bupivacaine.

Chemoneurolysis

Chemoneurolysis is achieved by the injection of medication into select muscles. Both Botulinum Toxin A (sold under the commercial name Botox) and phenol are used for chemoneurolysis procedures. These drugs can temporarily reduce spasticity (with the effect typically lasting four to six months), and may delay the need for surgery.Chemical neurolytic agents function for anywhere between months and years. These agents cause demyelination and axonal destruction via protein denaturation and axonal necrosis. These agents also are injected using electrostimulation or electromyography (EMG) guidance. Agents used for these procedures include phenol and ethyl alcohol. Phenol typically is used in concentrations ranging from 2% to 7%. Lower concentrations achieve demyelination, leading to a transient anesthetic effect, but achieve minimal axonolysis. Effects of higher concentrations are neurolytic as they destroy axons, lasting greater than 6 months. Ethyl alcohol should be used at a concentration of 45% to 100% to achieve neurolytic effects. It is less commonly used than phenol and is less toxic. A common adverse effect of chemoneurolysis is dysesthesias, which means pain in the sensory distribution of the blocked nerve. Reported incidence ranges from 10% to 30%, lasting weeks to months. Additional adverse effects are muscle weakness (which may be permanent), transient swelling or nodule formation within the muscle itself, DVT, sprains, and skin sloughing (more common when injecting phenol). Phenol, if injected intravascularly, can lead to serious systemic reactions such as convulsions, CNS depression, and cardiovascular failure. Therefore, the usual dose of phenol is well below its lethal dose (8.5 grams), limited to 20 to 30 mL of 5% concentration. Ethyl alcohol has minimal systemic side effects if injected intravascularly.

Botulinum toxin injections

Botulinum toxin or botulinum neurotoxin is a purified form of the same toxin made by the bacteria Clostridium botulinum that causes some forms of food poisoning. Botulinum neurotoxin is prepared for several uses and is used safely by neurologists who inject small and safe doses of botulinum neurotoxin into muscles to block nerve signals that cause muscle spasms or pain.

The FDA has approved three type A Botulinum toxins, onabotulinumtoxin A, incobotulinumtoxin A, and abobotulinumtoxin A, and one type B Botulinum toxin, rimabotuinumtoxin B, for clinical use ¹. Currently, onabotulinumtoxin A is approved for use in both upper and lower limb spasticity in five specific muscles, and incobotulinumtoxin A has been approved for upper limb spasticity. All serotypes function at the neuromuscular junction, where they block the presynaptic release of acetylcholine.

One use of botulinum toxin is injections (shots) for treatment of localized spasticity. Botulinum toxin works by blocking the chemical messages between the nerve and the muscle. The doctor will decide which muscle groups to treat and the number of injections you will need. Onabotulinumtoxin A is typically injected in units ranging from 25 to 200 per muscle, depending on muscle size, function, patient weight, and amount of spasticity.

Botulinum toxin will not work right after the injection. It takes several days (24 to 72 hours) to work, with the greatest effect seen in 3 to 6 weeks. The effects of botulinum toxin last an average of 3 months, at which point the patient may need re-injection. Doctors refer to the “rule of 3s”: 3 days for initial effect, 3 weeks for peak effect, 3 months duration. Adverse effects are typically benign and include unwanted weakness in adjacent muscles, hematoma, local bruising or swelling, flu-like symptoms, dysphagia from the cervical injection (transient), and injection site pain.

Will botulinum neurotoxin help improve spasticity in adults?

Neurologic injury may cause overly stiff muscles, which interfere with moving and walking. These problems, called spasticity, are common in cerebral palsy, traumatic brain injury, stroke, multiple sclerosis, and spinal cord injury. If you have had a stroke, you may have tightness in your arm, wrist, and hand muscles on your affected side. There is strong evidence that in adults, botulinum neurotoxin safely and effectively treats spasticity in the limbs. If you have multiple sclerosis and severe tightening or stiffness of your thigh muscles, botulinum neurotoxin may provide you comfort when in bed or a wheelchair.

How does botulinum neurotoxin control spasticity in children with cerebral palsy?

There is strong evidence that botulinum neurotoxin injections help control spasticity when injected into the calf muscles of children with cerebral palsy with tightness of the foot muscles, causing them to walk on their toes. There is good evidence that botulinum neurotoxin may be appropriate to treat thigh adductor spasticity (which squeezes the thighs together) and for pain control for children undergoing adductor-lengthening surgery. Good evidence also supports botulinum neurotoxin as a treatment option for children experiencing arm spasticity. Clinical studies have shown small improvements in walking when botulinum neurotoxin is injected into the hamstrings, which are muscles on the back of the thigh.

How is botulinum toxin injection given?

Botulinum toxin injections are given into the affected muscle. If the muscle is large, several shots will be given into the same muscle to allow the medicine to be evenly distributed.

The injection is somewhat painful for a while because it is given into the muscle. To make the needle pokes less painful, the doctor will apply an anesthetic cream to the skin areas ½ hr before the injections. Your child will still feel some discomfort, such as pressure or a stinging feeling during the injections.

A sedative medicine is usually given to children to help them relax for the procedure. The medicine causes children to become drowsy and will help them to forget the discomfort. The doctor will discuss the use of sedation medicine with you before the procedure.

What are the side effects of botulinum toxin injections?

Botulinum neurotoxin was introduced 28 years ago. When used appropriately, its risks are low and adverse side effects are rare. The most common side effect is mild muscle weakness. Other side effects include pain where botulinum neurotoxin was injected, dry mouth, flu-like symptoms, risk of falling, inability to maintain control of bladder and bowel movements, and difficulty swallowing. All side effects generally go away quickly.

Less than 5 percent of children who receive botulinum toxin injection may have a fever between 101° F and 103° F (38.3 °C and 39.4 °C). Fever is not an indication of whether or not botulinum toxin will work.

Some children may have less energy for a few days after the procedure. Some children may have a short-term decrease in function if they were using their spasticity to support themselves.

Rarely, a child could have gagging, choking, or trouble swallowing. If this happens, call the doctor right away.

What happens after the injections are done?

If sedation was used, your child will need to be observed for a short time after the procedure. The nurse will give you instructions for caring for your child.

Your child should not do any strong stretching of the muscle for 72 hours after receiving the injections. The doctor will give you instructions for activity restrictions.

For a few days, your child may feel discomfort in the affected muscles. If so, check with your doctor about giving a pain-relieving medicine to increase comfort.

When should I call for help?

Call your doctor if:

• trouble swallowing
• choking or gagging
• trouble breathing – call your local emergency services number

Intrathecal Baclofen Pump

Another management option is an intrathecal baclofen pump. This device allows for direct delivery of baclofen into the cerebrospinal fluid (CSF) in the intrathecal space. This allows a patient to receive a high concentration of the medication directly to the spine, while decreasing the CNS risks associated with high oral doses of baclofen, with a ratio of 100:1 for the baclofen concentration at the spinal cord level when administered intrathecally versus orally. The components include a pump and reservoir implanted subcutaneously within the abdominal wall and a catheter placed into the intrathecal space. A programmable battery-powered pump stores and delivers the baclofen via an electronic schedule. The pump is refilled on an intermittent basis via transcutaneous injection. The frequency of refill depends on infusion rate and the size of the pump reservoir. The dose can be adjusted via the electronic programmer at any time. The pump also has a programmable alarm system to alert the patient, caregiver, and clinician when the reservoir is running out of medication or when there has been a pump malfunction.

Symptoms of baclofen overdose include hypotonia or severe weakness, somnolence, nausea/vomiting, hypotension, respiratory depression, and seizures. Symptoms of baclofen withdrawal include fever, nausea, hyperthermia, dizziness, insomnia, pruritis, hallucinations, altered mental status, and exaggerated rebound spasticity.

Intrathecal baclofen pump pump is indicated for patients with generalized spasticity who either cannot tolerate or lack response to more conservative agents (oral, nerve blocks, etc.). Once again, the clinician must be careful to evaluate the utility of a patient’s spasticity to their daily function. Before implanting an intrathecal baclofen pump, the patient must undergo trials through a single intrathecal bolus or continuous infusion through a percutaneous catheter. Multiple trials of increasing doses may be performed to establish clinical benefit. If the patient demonstrates a significant decrease in tone or spasms, he or she may be a good candidate for pump placement. The initial pump infusion dosing is typically calculated by doubling the initially clinically effective dose and using this for the initial 24-hour infusion dose. An intrathecal baclofen pump pump requires strict compliance, as it needs regular monitoring and refilling, and withdrawal can be lethal.

Surgery

The most invasive management options are surgical. These include orthopedic surgeries such as tendon lengthening, tendon transfer procedures (such as the split anterior tibial tendon transfer known as SPLATT) as well as neurosurgical procedures such as a sectioning at the level of a peripheral nerve (neurectomy), central electrical stimulators, rhizotomy, and neuroablative procedures.

Orthopaedic surgery

Spastic muscles do not grow normally, and over time, permanent muscle contracture (tightening) and deformity can develop.

In children with diplegic cerebral palsy, contractures worsen around the ages of 4 to 5, and the child’s ability to walk either does not improve or deteriorates. These contractures can lead to hip displacement or dislocation, gait abnormalities, or neuromuscular scoliosis.

When contractures occur, orthopaedic surgery is often the best intervention to address them. Common types of orthopaedic surgery are as follows:

• Muscle lengthening: Muscle lengthening procedures may be performed surgically to improve joint motion and gait (walking), and to prevent deformities. Lengthening procedures can also be used to decrease the need for bony surgery in younger children, and their effects last longer than those of botulinum toxin.
• Tendon transfers: Tendon transfers allow muscles to partially be transferred physically to a different location, which can balance the forces across a joint in a more advantageous fashion.
• Bony reconstruction: Bony reconstruction allows for direct restoration of anatomic position of joints (in the case of neuromuscular hip dislocation/dysplasia), or relief of rotational abnormalities which result in brace intolerance.
• Joint fusion: In cases where the deformity is too severe to be managed with simple realignment, fusion can provide a durable option to provide long term support of a patient’s skeleton.

Neurosurgery

Neurosurgery is another option to treat spasticity in children with cerebral palsy. Selective dorsal rhizotomy (SDR) is a surgery that reduces the tone and spasticity of the legs to a greater degree than other treatments. In selective dorsal rhizotomy, neurosurgeons aim to reduce the spasticity of your child’s legs by cutting a portion of the dorsal roots of spinal nerves as they leave the spinal column.

Self-care

These things may make your spasticity worse:

• Being too hot or too cold
• The time of day
• Stress
• Tight clothing
• Bladder infections and spasms
• Your menstrual cycle (for women)
• Certain body positions
• New skin wounds or ulcers
• Hemorrhoids
• Being very tired or not getting enough sleep

Your physical therapist can teach you and your caregiver stretching exercises you can do. These stretches will help keep your muscles from getting shorter or tighter.

Being active also helps keep your muscles loose. Aerobic exercise, such as swimming, and strength-building exercises are helpful as are playing sports and doing daily tasks. Talk with your health care provider or physical therapist first before starting any exercise program.

Your provider may place splints or casts on some of your joints to keep them from becoming so tight that you cannot move them easily. Make sure to wear these as your provider tells you to.

Be careful about getting pressure sores from exercise or being in the same position in a bed or wheelchair for too long.

Muscle spasticity can increase your chances of falling and hurting yourself. Be sure to take precautions so you do not fall.

Spasticity prognosis

The prognosis for those with spasticity depends on the severity of the spasticity and the associated disorder(s). If a patient’s spasticity responds well to treatment, whether physical modalities, therapy, or pharmacological intervention, the patient may carry a favorable prognosis in terms of managing the spasticity symptomatically. Additionally, as previously mentioned, spasticity may carry with it certain benefits for the patient, such as helping the patient with ambulation thereby preventing deep vein thrombosis, maintaining muscle bulk, weight-bearing, and in turn preventing osteoporosis.

MS spasticity

Multiple sclerosis (MS) is an inflammatory and neurodegenerative autoimmune demyelinating disease of the central nervous system with a varied and unpredictable course ⁴:942–55.)). Disability in MS include mobility, spasticity, pain, manual dexterity, sensory, tremor/coordination, bowel/bladder function, fatigue, and cognitive function ⁵:146–58.)). The severity of impairment increases as the disease progresses, although the patterns of disabling symptoms can vary widely among individuals ⁵:146–58.)). The most frequent symptoms associated with spasticity in patients with MS, beyond mobility worsening, are spasms, pain, poor sleep quality (linked to pain and nocturnal spasms) and urinary dysfunction ⁶.

A number of large studies have shown that spasticity produces some of the most common and disabling symptoms associated with MS, occurring in up to 80% of affected MS persons ⁷. Between 30 and 50% of MS patients rate their spasticity as moderate to severe ⁸, and some of the most commonly reported associated symptoms included rigidity, spasms, pain, worsening of movement difficulties and/or urinary dysfunction and sleep disturbances.

Spasticity is one of the most frequent symptoms associated with MS and yet it is often overlooked and poorly managed. In the majority of patients with MS spasticity and its associated symptoms contribute to disability, interfere with performance of everyday activities, and impair quality of life ⁹. Even under treatment with oral antispasticity drugs, about a third of MS patients continue to experience spasticity of moderate to severe intensity, underscoring the need for additional treatment options ⁹. The efficacy of tetrahydocannabinol and cannabidiol (THC:CBD) oromucosal spray as add-on therapy in patients with refractory MS spasticity has been demonstrated in clinical trials and observational studies ⁹.

Patients with MS spasticity have greater overall disability compared to patients without spasticity, with restricted mobility being commonly reported ¹⁰. As severity worsens, MS spasticity (spasms, muscle rigidity) and its associated symptoms place an increasingly greater demand on healthcare resources ¹⁰ and have an increasingly greater negative impact on patients’ quality of life ¹¹:154–62.)).

First-line treatment options for MS spasticity include oral antispasticity drugs such as baclofen and tizanidine, often associated with drugs such as gabapentin, pregabalin, and antidepressants ¹²:1386–96.)). Despite pharmacological treatment, around a third of patients with MS spasticity continue to experience symptoms of moderate to severe intensity, highlighting a considerable unmet need ¹³. Tetrahydocannabinol and cannabidiol (THC:CBD) oromucosal spray (Sativex®: nabiximols) is indicated as add-on therapy in patients who show nonresponsiveness or develop tolerance to first-line antispasticity agents ¹⁴. The efficacy of tetrahydocannabinol and cannabidiol (THC:CBD) oromucosal spray for symptomatic relief of MS spasticity has been demonstrated in large phase 3 clinical trials ¹⁵, as add-on therapy, in subjects with refractory spasticity caused by multiple sclerosis. Eur J Neurol. 2011 Sep;18¹⁶:1122–31.)) and in routine clinical practice ¹⁷. Tetrahydocannabinol and cannabidiol (THC:CBD) oromucosal spray has also shown a trend towards improving objective measures of spasticity such as the timed 10-meter walk test ¹⁸.

MS spasticity triggers

Triggers may include skin, visceral, drug-related, or device-related issues. Skin issues may present as ulcers, ingrown toenails, boils, and infections. Visceral issues include constipation, urinary tract infections or calculi. Rapid withdrawal of antispasmodic agents can lead to worsening spasticity. Lastly, poor seating, an ill-fitting orthotic, or failure of an intrathecal baclofen pump can all be device-related triggers. Spasticity also can be worsened by other noxious stimuli such as infections, injuries, deep vein thromboses (DVT), or stress.

Symptoms that may influence MS spasticity which include, among others ¹⁹:

• Musculoskeletal symptoms (e.g. tendinitis);
• Orthopedic symptoms (e.g. osteoarthritis);
• Urinary and bowel disorders;
• Balance disorders (e.g. visual, oculomotricity, sensory or vestibular disturbances);
• Fatigue/deconditioning (very common);
• Uhtoff syndrome (spasticity worsens when temperature is raised); and
• Arm weakness.

What does MS spasticity feel like

In addition to muscle stiffness and increasing mobility restrictions, symptoms commonly associated with multiple sclerosis (MS) spasticity are spasms, sleep disturbances, worsening of pain, fatigue,movement difficulties, sleep disturbances and bladder dysfunction ²⁰. The most impairing symptoms of MS spasticity were muscle stiffness (77% of patients) and mobility restrictions (69%) ¹⁹. MS spasticity restricted daily activities at least several times a week in about two-thirds of patients (as assessed by physicians and self-reported by patients using the EQ-5D) and, for the majority of patients, caused ‘some’ or ‘moderate’ problems in the subdomains of mobility, usual activities and pain/discomfort. Overall, 48% of physicians and 34% of patients were at least partly dissatisfied with the effectiveness of available pharmacotherapy options for MS spasticity.

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