1. SPASTICITY

2. OBJECTIVES
1. Understand the definition of spasticity as part of the upper motor neuron syndrome
2. Understand and describe how spasticity can interfere with hygiene, function, and comfort
3. Correctly measure spasticity using the Modified Ashworth Scale
4. Choose appropriate therapeutic options to manage spasticity
5. Describe when it is appropriate to not manage spasticity

3. DEFINITION
Disordered sensorimotor control resulting from an upper motor neuron (UMN) lesion, presenting as intermittent or sustained involuntary activation of muscles.

4. Spasticity Clinical Features
In the upper extremities, the tone is usually high in adductors of the shoulders; flexors of elbows, wrist, and fingers; and pronators of forearm.
Excessive flexion of the fingers and adduction of the thumb results in the characteristic clenched fist with “thumb in palm” deformity.

5. CLINICAL FEATURES continued
In the lower extremities, the high tone due to spasticity is particularly prominent in adductors of the hips, flexors and extensors of the knees, and plantar flexors and inverters of the ankle.
Hyperextension of the big toe as a result of persistent high tone of extensor hallucis longus is another feature of spasticity
High muscle tone in lower extremity spasticity characteristically affects the antigravity muscle groups.

6. Video 1
File sent as attachment in

7. CLINICAL FEATURES continued
The high muscle tone associated with spasticity varies with the speed of the movement, that is, the faster the stretch, the greater the resistance.
In other words, spasticity is VELOCITY-DEPENDENT increase in tone, as part of the UPPER MOTOR NEURON SYNDROME

8. CLINICAL FEATURES continued
Other clinical features of spasticity are clonus, spasms, spastic dystonia, and spastic co-contractions
Clonus: Involuntary rhythmic contractions triggered by stretch; these can interfere with walking, transfers, sitting, and care.
Spasms: Sudden involuntary movements that often involve multiple muscle groups and joints; can be in response to somatic or visceral stimuli

9. CLINICAL FEATURES continued
Spastic dystonia: Tonic muscle overactivity without any triggers owing to the inability of motor units to cease firing after a voluntary or reflex activity; results in characteristic limb postures and contractures.
Spastic co-contraction: Inappropriate activation of antagonistic muscles during voluntary activity, due to lack of reciprocal inhibition, causing a loss of dexterity and slowness in movements.

10. Quiz question Which of the following is FALSE regarding spasticity?
Spasticity involves increased muscle tone that is velocity-dependent
Typical manifestations are increased tone in elbow flexors, hip adductors, and ankle dorsiflexors
Clonus is an involuntary rhythmic contraction triggered by stretch
Spastic co-contraction involves antagonist muscles activating simultaneously

11. Answer
(B) Elbow flexors and hip adductors are true, but ankle dorsiflexors are not involved, ankle plantarflexors are.

12. DIFFERENTIAL DIAGNOSIS
Includes: contractures, rigidity, and catatonia
Contractures:
Shortening and reduction in elasticity of muscles, tendons, ligaments, joint capsules, and skin, which is accompanied by an increase in the resistance to passive stretch.
After immobilization, sarcomeres shorten and muscle and elastic tissue are replaced with connective tissue and fat. Left unchecked, this process can result in permanent loss of motion in joints.
Unlike spasticity, contractures do not show dynamic changes such as clasp knife phenomenon, catch, and variation of tone with speed of movement and position of limb.

13. DIFFERENTIAL DIAGNOSIS continued
Rigidity:
Associated with disorders of the basal ganglia.
Unlike spasticity, the high tone in rigidity is non-selective and affects all muscles acting on a joint equally. The resistance to passive movements due to rigidity is felt throughout the range of movement and does not change with speed of movement.
In Parkinson’s disease, rigidity associated tremors give a “cog wheel” feel on passive movement.
Rigidity does not respond well to spasticity interventions.

14. DIFFERENTIAL DIAGNOSIS continued
Catatonia:
A neuropsychiatric syndrome characterized by abnormal posturing, gegenhalten, and “waxy flexibility.”
Gegenhalten or counterhold is an increase in muscle tone proportional to the force applied.
In waxy flexibility, patients maintain their limbs in positions placed by others for a long time. When the limb is moved passively, the muscles stiffen in proportion to the force applied, as if the patient is actively opposing movement.
Catatonia is caused by several psychiatric, neurological, and medical conditions. Presence of signs such as excitement, impulsivity, perseveration, combativeness, automatic obedience, stupor, mutism, staring, grimacing, stereotypical movements, echolalia, echopraxia, and withdrawal should raise a suspicion of catatonia.

15. MEASUREMENT OF SPASTICITY
Modified Ashworth Scale
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 when the affected part(s) is moved in flexion or extension
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 affected part(s) easily moved
3 = Considerable increase in muscle tone, passive movement difficult
4 = Affected part(s) rigid in flexion or extension

16. MEASUREMENT OF SPASTICITY continued
Spasm Frequency Scale 0 = None 1 = No spontaneous spasms; but vigorous sensory or motor stimulation results in spasms 2 = Occasional spontaneous spasms or easily induced spasms 3 = Greater than one but less than ten spontaneous spasms per hour 4 = Greater than ten spontaneous spasms per hour

17. MEASUREMENT OF SPASTICITY continued
Tardieu scale (can insert PDF file)

18. APPROACH TO WORSENING SPASTICITY
In general practice, a long-standing neurological condition may be a more common reason for consultations involving spasticity compared with new- onset spasticity.
Patients with worsening spasticity may experience a variety of symptoms, such as pain, stiffness, involuntary movements, deterioration of mobility, increase in care needs, and sexual dysfunction.
Can be due to: triggers, disease progression or new disease.

19. APPROACH TO WORSENING SPASTICITY continued
Triggers
Visceral or somatic stimuli below the level of injury. Examples:
• Skin—ulcers, ingrown toenails, boils, and skin infections
• Visceral—constipation, urinary tract infections, urinary tract calculi, kinked indwelling urinary catheter, pain during menstruation
• Devices—improper seating, ill-fitting orthoses, failure of intrathecal baclofen pump
• Drugs—rapid withdrawal of antispasticity drugs
• Others—infections, injuries, deep vein thrombosis, stress

20. APPROACH TO WORSENING SPASTICITY continued
Disease progression
Worsening of spasticity could be a sign of the progression of the primary neurological disease, as in progressive forms of multiple sclerosis.
In people with spinal cord injury, worsening spasticity may indicate development of syringomyelia.
New disease
Worsening of spasticity could indicate a coincidental new disease.

21. APPROACH TO WORSENING SPASTICITY continued
First step in the assessment of patients with worsening spasticity is to look for triggers
Education of patients and caregivers in recognizing these triggers is an important part of management
If no underlying triggers are identified or if the spasticity continues to worsen, coincidental new disease should be excluded

22. Quiz question Which of the following can worsen spasticity?
Urinary tract infection
Progression of multiple sclerosis
Ingrown toenail
All of the above

23. Answer
(D) All of the above: a new neurologic disease, or progression of neurologic disease, as well any noxious stimulus, can be the cause of worsened spasticity, and must be considered before changing management

24. Impact of spasticity Effects in three broad categories
Pain: painful spasms, uncomfortable sensation of tightness, pain with stretching
Hygiene- difficulty accessing folds such as axilla, antecubital fossa, palm, groin, popliteal fossa
Function- dressing, reaching, grasp, fine-motor ADLs, walking, transfers, standing, sitting, sexual

25. Aims of management of spasticity
Relief of pain and discomfort.
Improvement of hygiene in areas such as palm, axilla, and groin
Facilitation of sitting, standing, and walking
Improvement of posture
Reduction in burden of care
Improvement in body image and self esteem
Prevention of complications such as pressure ulcers

26. When NOT to treat spasticity
If it’s helpful: e.g., elbow flexion to hold objects, finger flexion to grasp during transfers, knee extension during gait
If risk of intervention outweighs benefit: e.g., bleeding risk of injection when on anticoagulation, infection risk with injection in grossly edematous limb, risks of distal spread of medication in patients susceptible to adverse effects
If the spasticity isn’t causing any problems

27. Management of Spasticity
Non-drug interventions
Stretching
Splinting
Postural management
Strengthening exercises
Physical modalities

28. Management of Spasticity continued
Drug interventions
Oral Medications
baclofen, benzodiazepines, tizanidine, dantrolene
Injections
botulinum toxin, chemical neurolysis (ethyl alcohol or phenol)

29. Management of spasticity continued Non-drug interventions
Stretching
Often part of a home management program, and considered to be cornerstone of the management of spasticity.
The effect, however, is short-lived, so must be taught to the patient and/or caregiver in order to be repeated multiple times per day. 

30. Management of spasticity continued Non drug interventions
Splinting
Provides a stretch over a more prolonged period compared with manual stretching
The use of Lycra-based spring-controlled garments (dynamic fabricated orthoses) is another way of applying a stretch over longer periods while not rigidly fixing a joint
Wrist extension dynamic splint

31. Management of spasticity continued Non-drug interventions
Postural management
Involves managing postural alignment to prevent or reduce contracture and spasticity in people with severe symptoms.
Includes systems to maintain alignment when sitting, standing, and in bed.
Standing frames allow people to remain upright for prolonged periods by providing the required support at the ankle, knee, or hip.

32. Management of spasticity continued Non-drug interventions
Strengthening Exercises
Strengthening of spastic muscles does not increase spasticity, and can improve function since a spastic muscle is also often weak during voluntary contraction
Needs to be task-specific, based on mutually agreed-upon goals
Strengthening of the antagonist muscle can help overcome the spastic muscle 

33. Management of spasticity continued Non-drug interventions
Physical modalities
Extracorporeal shock wave therapy, transcutaneous electrical stimulation, repetitive transcranial magnetic stimulation, transcranial direct current stimulation, and electromagnetic therapy.
Superficial heat and cold cause temporary reduction in spasticity, so can be used prior to stretching and exercises

34. Quiz question
True or False? Stretching provides several days of relief from spasticity

35. Answer
False: the effect of stretching lasts only minutes to hours, so ideally it should be repeated a few times a day

36. Management of spasticity Drug interventions
Medication
Mechanism of action
Type of injury
Dosing
Considerations 
Baclofen
GABA-B agonist
Spinal cord>stroke
5mg TID to 80mg/d divided TID
Sedation common
Withdrawal: itchiness, worsened spasticity, change in mood.
Lowers seizure threshold. Renally metabolized/excreted (and not dialyzed)
Can raise liver enzymes
Benzodiazepines
(diazepam/Valium)
GABA-A agonist
supraspinal
5mg qhs to 60mg/d divided TID
Tizanidine (Zanaflex)
Alpha-2 agonist (↓glutamate, aspartate)
supraspinal, spinal
2mg qhs to 36mg/d divided TID
Similar to clonidine- can ↓BP
Dantrolene
Prevents calcium release in sarcoplasmic reticulum
Supraspinal
25mg to 100mg 4x/d
Risk of fulminant liver failure, especially if F/30/300/60 (female over 30 taking 300mg/d for over 60d)
Also effects cardiac muscle

37. Quiz question
True or false? Baclofen can be given in low doses to patients on hemodialysis

38. Answer
False: The effect of baclofen in patients with end stage renal disease on hemodialysis is unpredictable and can result in overdose with even small doses. Baclofen is not removed with hemodialysis. So, oral baclofen should not be used.

39. Botulinum toxin
Causes selective weakness of the target muscle by blocking the release of acetylcholine at the neuromuscular junction.
Results in a focal reduction in spasticity in the injected muscles without side effects of global weakness or sedation.
Botulinum toxin is recommended for the treatment of patients with focal spasticity.
The schematic shows the sites where botulinum toxin types C and D act to prevent fusion of the ACh vesicle with the presynaptic membrane, so ACh can’t be released into the neuromuscular junction
The schematic shows the sites where botulinum toxin types C and D act to prevent fusion of the ACh vesicle with the presynaptic membrane, so ACh can’t be released into the neuromuscular junction

40. Botulinum toxin
In the United States, is approved by the Food and Drug Administration (FDA) only for limited uses (blepharospasm, cervical dystonia, chronic migraine, overactive bladder, axillary hyperhidrosis, strabismus, and upper limb spasticity in select muscles), but botulinum toxin is very commonly used throughout the body to treat spasticity.
The botulinum toxins licensed for this purpose are onabotulinum toxin A (Botox; Allergan), abobotulinum toxin A (Dysport; Ipsen, Biopharm), incobotulinum toxin A (Xeomin; Merz Pharma) and RimabotulinumtoxinB (Myobloc).

41. Botulinum toxin Toxin type Site of action Vial sizes FDA-approved uses
Onabotulinum A (Botox)
Neuromuscular junction: SNAP-25
100 Units, 200 U
Cervical dystonia, strabismus, blepharospasm, severe primary axillary hyperhidrosis, glabellar lines, some elbow/wrist/finger muscles in adults with upper limb spasticity 
AbobotulinumtoxinA (Dysport)
NMJ: SNAP-25
500 U
Cervical dystonia, glabellar lines
IncobotulinumtoxinA (Xeomin)
50 U, 100 U
Cervical dystonia, blepharospasm, glabellar lines
RimabotulinumtoxinB (Myobloc)
VAMP/synapto-brevin
2500, 5000, U
Cervical dystonia

42. Botulinum toxin
Therapeutic effects are seen in 7-10 days, peak in 3 weeks, and wane by 12 weeks.
Patients should be reassessed about four weeks after the initial injections to determine their efficacy and whether treatment goals have been attained.
If required, further injections should be planned once every 12 weeks.
Ultrasound guided injection of botulinum toxin in hamstring muscles.

43. Botulinum toxin Adverse events of botulinum toxin:
Dry mouth, injection site pain, respiratory tract infections, muscle weakness, urinary incontinence, falls, fever, and pain.
Rarely, due to spread of the toxin distal from the site of injection, transient dysphagia requiring temporary nasogastric/percutaneous gastric feeding or difficulty breathing requiring temporary ventilator support, can occur.
Patients should be counseled about these adverse events and advised to seek medical help if they develop signs and symptoms of toxin spread.

46. Quiz question The average duration of effect of botulinum toxin is:
7-10 days
3 weeks
3 months
6 months

47. Answer
(C) 3 months, which is the interval at which the injections are typically repeated

48. INTRATHECAL BACLOFEN
A pump in the subcutaneous lower abdomen stores and delivers programmable doses of baclofen through a catheter into the spinal subarachnoid space.
A relatively small dose of baclofen administered intrathecally achieves a high concentration of the drug within the spinal cord resulting in good muscle relaxation without systemic side effects.
The drug is effective in the treatment of spasticity secondary to spinal cord disorders, stroke, traumatic brain injury, and multiple sclerosis.
Intrathecal baclofen pump

49. INTRATHECAL BACLOFEN Procedure-related complications
Infection, skin erosions, cerebrospinal fluid leak and seroma formation around the pump.
Abrupt withdrawal of intrathecal baclofen due to catheter block or breakage, battery failure, pump failure.
Patient with baclofen pump seen in right lower quadrant
X-ray of abdomen, shows baclofen pump on the right side.

50. INTRATHECAL BACLOFEN Acute baclofen withdrawal:
Signs: High fever, confusion, rebound spasticity, muscle rigidity, seizures.
Baclofen withdrawal can be life-threatening!
Noncompliance may not be a patient’s fault (lack of insurance coverage, having to travel long distances to reach clinic for appts, poor social support limiting patient’s ability to make it to appts), but regardless of cause it is reason to not have a baclofen pump implanted at all, since withdrawal is serious and can be deadly
Patients need to comply with treatment and attend appointments regularly for monitoring and pump refills. Patient non-compliance can cause a clinical emergency.
The treatment of acute intrathecal baclofen withdrawal is intrathecal baclofen through a temporary external catheter. Pending this, patients should be started on oral baclofen mg/day divided q2hours (to maximize GI absorption), diazepam 2-5mg PO/IV/IM q3hours.
Usually oral baclofen is given in 8 hour intervals, but in the case of withdrawal maximal absorption is needed to combat symptoms. Only a very small portion of the small intestine absorbs baclofen, so giving smaller doses at more frequent intervals will cause more of the drug to be absorbed

51. Images showing intrathecal baclofen pump re-fill equipment

52. Chemical neurolysis
Involves the injection of an agent directly onto a nerve, in order to damage a portion of it, impeding conduction
Motor nerves are targeted as muscle as possible, to denervate their muscles
Injection can be performed using alcohol (50-98% ethanol or 5-12% phenol) for this purpose.
Ultimately, these interventions interfere with the muscle stretch reflex arc, and thus can be used to treat spasticity.

53. Chemical neurolysis Local anesthetic block
Done for diagnostic purposes, using temporary local anesthetic instead of destructive concentrated alcohol
Can be used to help determine the extent of contribution of spasticity versus contracture affecting limited range of motion in a limb, especially in an old injury
Can be used to determine which muscle is contributing to a pathologic posturing
Can be used in situations where it is uncertain if a nerve block will cause more harm than good, particularly when significant function is at stake, or when blocks may affect more than one aspect of a function, such as ambulation.

54. Chemical neurolysis
The most common technique for injection employs the use of a nerve stimulator device which delivers a pulsed current for accurate localization to the target nerve. Musculoskeletal ultrasound has also been implemented by some clinicians to allow direct visualization of the target nerve.

55. Chemical neurolysis
Nerve blocks are usually performed using a 24-gauge needle that is insulated except at the bevel tip, and connected to the electrical stimulator, so that a pulsed electrical stimulus can be transmitted to the nerve only when it is in the proximity of the bevel itself. A ground electrode is placed to complete the circuit.
The needle is inserted, and pulsed stimulation delivered. When a rhythmic response is observed the physician checks to be sure that the appropriate muscle or group of muscles is contracting.

56. Video of ultrasound-guided electrical stimulation of thoracodorsal nerve (lattisimus dorsi)

57. Alcohol vs. Botulinum toxin
Location/ mechanism of action
Cost
Duration of effect
Dose range
Selectivity of response
Alcohol
Nerve/muscle destruction
pennies
~9 months
“no limit” in adults, can repeat at desired intervals
Unpredictable magnitude
Botulinum toxin
Prevents release of ACh into
neuromuscular junction
$550/100 Units
(Botox)
~3 months
Up to Units per session (Botox) in adults, all at one session, q3mos
Dose-dependent, muscle-specific

58. Surgical Management Selective dorsal rhizotomy Fractional lengthening
Tendon transfer

59. Surgical management Selective dorsal rhizotomy
Surgical transection of selected dorsal nerve roots of the lumbosacral spinal cord
Reduces the sensory input into spinal motor neuron pools, reducing their excitability

60. Surgical management Selective dorsal rhizotomy
Potential side effects include sensory loss, urinary incontinence, low-back pain, spinal deformity, loss of ambulation
Recently, less invasive percutaneous radiofrequency ablation of dorsal root ganglion showed promising results