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Spasticity: Introduction and Overview R. Samuel Mayer, MD Assistant Professor Vice Chair of Education Department of Physical Medicine and Rehabilitation Johns Hopkins University School of Medicine Deputy Director, Quality Improvement, Department of Physical Medicine and Rehabilitation The Johns Hopkins Hospital Objectives • Assess spasticity by history and physical examination and testing. • Distinguish between generalized and localized impairments. • Discuss treatment options for specific upper motor neuron syndromes. Consequences of Spasticity • May interfere with mobility, exercise, and joint range of motion • May interfere with activities of daily living • May cause pain and sleep disturbance • Can make patient care more difficult Possible Advantages of Spasticity • Maintains muscle tone • Helps support circulatory function • May prevent formation of deep vein thrombosis • May assist in activities of daily living • May assist in maintaining erect posture • May assist in gait Pathophysiology Central Nervous System (CNS) Tracts --Spinothalamic --Corticospinal --Corticothalamic --Corticocerebellar Mechanical vs Spastic Contracture • Fixed • Tendon and/or ligament • Charcot joint • Heterotopic ossification • Dynamic • Slow stretch • Palpable antagonistic muscle action • Increased reflexes Most often, both mechanical and spastic factors Assessing Muscle Tone • History • Physical examination • Measurement tools Key Questions in History • Location? • Time of day, aggravating/alleviating factors? (diary) • Painful spasms? • Functional limitations? • Need for extensor tone in legs for standing? Physical Examination • Standard musculoskeletal examination including range of motion (measured with a goniometer) • Standard neurologic examination • Clonus • Ashworth scale Modified Ashworth Scale (MAS) • • • • • 0 = Normal tone 1 = Slight “catch” 1+ = Significant “catch” 2 = Mild, limb moves easily 3 = Moderate, passive range of movement difficult • 4 = Severe, rigid limb Quantitative Evaluation • Gait lab with surface electromyography (mostly research use) • Fugl-Meyer test for upper limb dexterity Goal Attainment Scales (GAS) • Select 2 SMART (specific, measurable, achievable, realistic, time-frame) goals with patient • • • • • -2 did not achieve by a lot -1 did not achieve by a little 0 achieved +1 exceeded by a little +2 exceeded by a lot Spectrum of Care for Management of Spasticity Prevent Nociception Intrathecal Baclofen (ITB™) Therapy Rehabilitation Therapy Oral Drugs Patient Orthopedic Treatments Injection Therapy Neurosurgeries Treatment Paradigms “Old School” “New School” • • • • • • • Generalized vs localized • Brain vs spinal (spinal cord injury [SCI]) • Acute vs chronic • Patient-specific analysis of side effects Stepwise approach Modalities Medications Injections Tendon surgery Nerve ablation Generalized Spasticity Remove Nociceptive Factors Stretch/Splint Positioning Oral Medication Intrathecal Medication Myelotomy Dorsal Rhizotomy Modalities Oral Medications (1) • Baclofen • Binds GABA-b receptors • SCI > brain • Side effects—weakness, lethargy, rebound seizures—if stopped abruptly GABA = gamma-aminobutyric acid. Oral Medications (2) • Dantrolene • Blocks Ca++ channels in muscle • No CNS effects, but causes significant weakness • Hepatic dysfunction rare CNS = central nervous system. Oral Medications (3) • Tizanidine • Alpha-2 agonist • Brain = SCI • Side effects: lethargy, orthostasis, dry mouth Oral Medications (4) • Diazepam • Enhances GABA through benzodiazepine receptors in brain • Brain > SCI • Rapid development of tolerance • Side effects: drowsiness, delirium, abuse potential, withdrawal seizures Children with CP Guidelines • Level B evidence for diazepam • Level C evidence for tizanidine • Level U evidence for baclofen, dantrolene CP = cerebral palsy. Delgado MR, et al. Neurology. 2010;74:337-343. Intrathecal Baclofen • Generalized spasticity • Not controlled or side effects from oral medications • Delivers @ 1/50 dose baclofen direct to cerebrospinal fluid • Infinitely programmable • Refill every 3 to 12 months • Mechanical complications Efficacy of Intrathecal Baclofen • CP: statistically significant gains in Gillette Functional Assessment Questionnaire1 • Stroke: improved Sickness Impact Profile at 3 and 12 months2 • Cost/quality-adjusted life-year: $10,550 to $19,5703 1. Brochard S, et al. Pediatr Neurol. 2009;40:265-270; 2. Ivanhoe CB, et al. Arch Phys Med Rehabil. 2006;87:1509-1515; 3. Sampson FC, et al. J Neurosurg. 2002;96:1052-1057. Intrathecal Baclofen in Cortical vs Spinal Spasticity • No difference in outcomes, dosing, or adverse events among patients with SCI, TBI, stroke, or CP • Important note: MS patients required far smaller dosing TBI = traumatic brain injury. Saval A, Chiodo AE. J Spinal Cord Med. 2010;33:16-21. Case #1 • 25-year-old man with C7 ASIA A tetraplegia from transverse myelitis for 5 years; now with worsening spasticity in all 4 limbs and trunk, which is painful and limiting transfers ASIA: American Spinal Injury Association. Question 1 In managing this patient, would you: A. Inject his biceps, quadriceps, and hamstrings bilaterally with botulinum toxin B. Look for nociceptive causes for his increased tone such as urinary tract infection (UTI) or pressure sore C. Instruct him in stretching 10 minutes daily D. Refer him immediately for an intrathecal baclofen trial Answer B. Look for nociceptive causes for his increased tone such as UTI or pressure sore Localized Spasticity Prolonged Stretch Bracing/Splinting Ultrasound Smaller Muscles Shorter Acting Botulinum Toxin A Botox, Dysport Botulinum Toxin B Myobloc Larger Muscles Semipermanent Phenol Alcohol Tendon Surgery OnabotulinumtoxinA (Botox®), AbobotulinumtoxinA (Dysport®), RimabotulinumtoxinB (Myobloc®). Phenol and Alcohol • Causes degeneration of both motor sensory fibers • Can do nerve blocks in pure motor nerves, but avoid mixed or sensory nerves • Motor point blocks • Lasts 4 to 9 months Phenol vs Alcohol • 100% effective in gastrocnemius injections for 6 months • 90% of patients injected with alcohol had relief at 9 months, 70% of those with phenol Kocabas H et al. Eur J Phys Rehabil Med. 2010;46:5-10, Botulinum Toxins • Can be injected using anatomic landmarks or as motor point block • 4 formulations in United States: onabotulinumtoxinA (Botox®) + abobotulinumtoxinA (Dysport®), incobotulinumtoxinA (Xeomin®), and rimabotulinumtoxinB (Myobloc®) • Lasts 2 to 4 months • NOT FDA approved for spasticity, except onabotulinumtoxinA for upper limb only. FDA = US Food and Drug Administration. Botulinum Toxin Efficacy • Stroke: improvement at 6 months in MAS and GAS, but not Assessment of Quality of Life1 • CP: meta-analysis shows 4 of 6 Class I trials in lower limb, 4 of 6 Class II trials in upper limb showed significant functional improvement2 • Better than tizanidine for upper limb spasticity3 1. McCrory P, et al. J Rehabil Med. 2009;41:536-544; 2. Lukban MB, et al. J Neuro Transm. 2009;116:319-331; 3. Simpson DM, et al. J Neurol Neurosurg Psychiatry. 2009;80:380-385. Botulinum Toxin Dosing • Toxin dosing among formulations are NOT equivalent • Dosing mostly an art, not science, based on size of the muscle (and patient) and degree of spasticity • Dosing table available in Sheean et al1 • Dosing peak effects for various muscle in Yablon et al2 1. Sheean G, et al. Eur J Neurol. 2010; 17:74-93; 2. Yablon SA, et al. Mov Disord. 2010;26:209-215. Upper Motor Neuron Syndromes • • • • • • • • • Shoulder flexion/internal rotation Elbow flexion/pronation Wrist flexion Finger flexion Hip flexion Knee flexion Knee hyperextension Equinovarus Toe flexion/extension Shoulder • Internal rotation/adduction Case #2 • 70-year-old woman with spastic hemiparesis with painful right shoulder, adductor, and internal rotation contractures • How would you treat? Question 2 The MOST appropriate treatment would be: A. Tizanidine 4 mg by mouth 3 times daily B. Intrathecal baclofen trial C. Botulinum toxin injection into subscapularis and pectoralis major D. Botulinum toxin injection into deltoids and infraspinatus Answer C. The subscapularis and pectorals are the major adductors and internal rotators of the shoulder. The spasticity is well localized, and hence treated best by local injection. Elbow • • • • • • Flexion/pronation Muscles? Biceps (2-joint rule) Brachialis Brachioradialis Pronator teres Hand • Flexed wrist, MCPs, PIPs, DIPs • Muscles? • FCR, FDS, FDP, lumbricals DIP = distal interphalangeal; FCR = flexor carpi radialis; FDP = flexor digitorum profundus; FDS = flexor digitorum superficialis; MCP = metacarpophalangeal; PIP = proximal interphalangeal. Hand • Thumb in palm • Muscle? • Flexor pollicis longus Lower Limb • Hip flexion/adduction • Muscles? • Iliopsoas, rectus femoris/add mag • Knee flexion • Muscles? • Hamstrings • Equinovarus • Muscles? • Gastrocnemius/soleus Case #3 • 40-year-old man with spastic diplegic CP has a scissoring gait with bilateral equinovarus, hamstring, and hip flexion/adduction spasticity with minimal mechanical contracture. He has not responded to oral medications. Question 3 How would you treat this patient? A. Intrathecal baclofen trial B. Phenol injections into bilateral adductors, rectus femoris, medial hamstrings, and gastrocnemius C. Botulinum toxin injections into bilateral adductors, rectus femoris, medial hamstrings, and gastrocnemius D. Options A and B are both reasonable. Answer D. Options A and B are reasonable depending on patient preference and social and financial issues. Aggressive splinting or serial casting is probably needed as well. Ankle and Foot • • • • Inversion Muscle? TP Great toe, small toe flexion • Muscles? • FHL, FDL FD = flexor digitorum longus; FHL = flexor hallucis longus; TP = terminal phalanx.