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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 Injection Therapy Neurosurgeries Orthopedic Treatments Rehabilitation Therapy Prevent Nociception Intrathecal Baclofen (ITB) Therapy Oral Drugs Patient Treatment Paradigms “Old School” Stepwise approach Modalities Medications Injections Tendon surgery Nerve ablation “New School” Generalized vs localized Brain vs spinal (spinal cord injury SCI) Acute vs chronic Patient-specific analysis of side effects Generalized Spasticity Remove Nociceptive Factors PositioningModalitiesStretch/Splint Oral Medication Intrathecal Medication Myelotomy Dorsal Rhizotomy Oral Medications (1) Baclofen Binds GABA-b receptors SCI brain Side effectsweakness, lethargy, rebound seizuresif 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 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 spasticity 3 3 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

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