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Spasticity Management in Neurological Conditions


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Spasticity Management in Neurological Conditions

  1. 1. Spasticity Management in Neurological Conditions A Quick-Start Guide George F. Wittenberg MD PhD 28 January 2010
  2. 2. Outline <ul><li>The Upper Motor Neuron Syndrome </li></ul><ul><li>Spasticity Management through Oral Medications </li></ul><ul><li>Spasticity Management through Parenteral Medications </li></ul><ul><ul><li>Botulinum Toxins </li></ul></ul><ul><ul><li>Intrathecal Baclofen </li></ul></ul><ul><li>Emergency Management </li></ul>
  3. 3. Upper Motor Neuron Syndrome <ul><li>Positive Signs (Excessive normal resting state) </li></ul><ul><ul><li>Spasticity </li></ul></ul><ul><ul><li>Rigidity </li></ul></ul><ul><ul><li>Hyperreflexia </li></ul></ul><ul><ul><li>Primitive reflexes </li></ul></ul><ul><ul><li>Clonus </li></ul></ul><ul><li>Negative Signs (Less than normal resting state) </li></ul><ul><ul><li>Lack of strength </li></ul></ul><ul><ul><li>Lack of motor control </li></ul></ul><ul><ul><li>Lack of coordination </li></ul></ul>Young RR, Emre M, Nance PW, et al. Current issues in spasticity management. Neurologist . 1997; 3:261-275. Young RR. Treatment of spastic paresis. N Engl J Med. June 1989;320(23):1553-1555.
  4. 4. Examples of UMN Lesions <ul><ul><li>Stroke </li></ul></ul><ul><ul><li>Multiple Sclerosis </li></ul></ul><ul><ul><li>Corticobasal Ganglionic Degeneration </li></ul></ul><ul><ul><li>Traumatic Brain Injury </li></ul></ul><ul><ul><li>Acquired Brain Injury </li></ul></ul><ul><ul><li>Spinal Cord Injury </li></ul></ul><ul><ul><li>Cerebral Palsy </li></ul></ul>
  5. 5. Pathophysiology of Spasticity
  6. 6. Spasticity: Consequences <ul><li>Pain and discomfort </li></ul><ul><li>Contractures </li></ul><ul><li>Increased energy cost of movement </li></ul><ul><li>Skin breakdown–shear </li></ul><ul><li>Interferes with breathing </li></ul><ul><li>Hampers gait and transfers </li></ul><ul><li>Interferes with hygiene </li></ul><ul><li>More work for caregiver </li></ul><ul><li>Poor safety </li></ul><ul><li>Sexual difficulties </li></ul><ul><li>Insomnia </li></ul><ul><li>Poor posture </li></ul>
  7. 7. Spasticity: Assessment <ul><li>Patient report </li></ul><ul><li>Deep tendon reflexes </li></ul><ul><li>Passive range of motion </li></ul><ul><li>Test for clonus </li></ul><ul><li>Functional observation </li></ul><ul><ul><li>Determine nature of hypertonicity </li></ul></ul><ul><ul><li>Assess interference with function (gait) and effect of stress and/or fatigue </li></ul></ul><ul><ul><li>Assess adaptive shortening of muscles vs. irreversible contracture </li></ul></ul><ul><li>Consider aggravating factors (e.g., UTI, infection, excessive activity, strengthening exercises) </li></ul>
  8. 8. Modified Ashworth Scale <ul><li>0. No increase in muscle tone </li></ul><ul><li>(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. </li></ul><ul><li>(1+) Slight increase in muscle tone, manifested by a catch, followed by minimal resistance throughout the reminder (less than half) of the ROM (range of movement). </li></ul><ul><li>(2) More marked increase in muscle tone through most of the ROM, but affected part(s) easily moved. </li></ul><ul><li>(3) Considerable increase in muscle tone passive, movement difficult. </li></ul><ul><li>(4) Affected part(s) rigid in flexion or extension. </li></ul><ul><li>(Original Ashworth score in parentheses; Modified after Bohannon and Smith Phys Ther. 1987 Feb;67(2):206-7. </li></ul>
  9. 9. Considerations in Reducing Spasticity <ul><li>Possible Advantages of Spasticity </li></ul><ul><ul><li>Maintains muscle tone/bulk </li></ul></ul><ul><ul><li>Helps support circulatory function </li></ul></ul><ul><ul><li>May prevent formation of deep vein blood thrombosis </li></ul></ul><ul><ul><li>May assist in activities of daily living </li></ul></ul><ul><ul><li>May assist with postural control </li></ul></ul>
  10. 10. Spasticity: Rehabilitation Intervention <ul><ul><li>Static stretch </li></ul></ul><ul><ul><li>Gait training (emphasis on swing and heel strike) </li></ul></ul><ul><ul><li>Positioning/posture </li></ul></ul><ul><ul><li>Cooling </li></ul></ul><ul><ul><li>Patient education </li></ul></ul><ul><ul><li>Relaxation </li></ul></ul><ul><ul><li>Bio-feedback </li></ul></ul><ul><ul><li>Reflex-inhibiting movement patterns </li></ul></ul><ul><ul><li>Avoiding noxious stimuli </li></ul></ul><ul><ul><li>Medications </li></ul></ul><ul><ul><li>ITB Therapy </li></ul></ul><ul><ul><li>Botulinum Toxin </li></ul></ul>
  11. 11. Spasticity: Medication <ul><li>Baclofen (Lioresal) </li></ul><ul><ul><li>GABA agonist </li></ul></ul><ul><ul><li>Max. dose 180 mg daily, divided </li></ul></ul><ul><ul><li>Side effects : sedation, incontinence, weakness, withdrawal seizures </li></ul></ul><ul><li>Tizanidine (Zanaflex) </li></ul><ul><ul><li>Alpha 2 adrenergic agonist </li></ul></ul><ul><ul><li>Max. dose 24-32 mg daily </li></ul></ul><ul><ul><li>Side effects : sedation, hypotension, weakness, hepatotoxicity </li></ul></ul><ul><li>Dantrolene - reserve for specialists </li></ul><ul><li>Diazepam et al. (Valium) </li></ul><ul><li>Gabapentin (Neurontin) </li></ul>
  12. 12. Considerations in Starting Oral Medications <ul><li>Titrate slowly: increase dose every five days, starting at: </li></ul><ul><ul><li>5 mg baclofen qHS </li></ul></ul><ul><ul><li>2 mg tizanidine qHS </li></ul></ul><ul><li>Inform patients to back off to previous dose if they encounter problems with sedation, other expected effects. </li></ul><ul><li>Effectiveness variable, often marginal </li></ul>
  13. 13. Spasticity: Invasive ℞ <ul><li>Botulinum Toxins A & B </li></ul><ul><li>Baclofen pump </li></ul><ul><ul><li>Intrathecal delivery via implanted pump (ITB) </li></ul></ul><ul><li>Surgical interventions </li></ul><ul><ul><li>Tenotomy (Tendon Lengthening) </li></ul></ul><ul><ul><li>Selective Rhizotomy </li></ul></ul>
  14. 14. Botulinum Toxins <ul><li>Derived from Clostridium botulinum bacteria </li></ul><ul><li>Multiple forms, but only A & B clinically approved </li></ul><ul><li>Produce reduction in muscle overactivity from 2-200 days after injection </li></ul><ul><ul><li>Peak effect begins 7-10 days after </li></ul></ul><ul><ul><li>Should not be reinjected before 90 days </li></ul></ul><ul><ul><li>May eventually become ineffective due to antibodies </li></ul></ul>
  15. 15. Injection Procedure <ul><li>Monitor with EMG (optional) </li></ul><ul><ul><li>Allows identification of muscles </li></ul></ul><ul><ul><li>Can help identify best targets </li></ul></ul><ul><li>Injected near motor points of muscles </li></ul><ul><li>Multiple injections per muscle </li></ul>
  16. 16. Common Spasticity syndromes treatable with Botulinum Toxin <ul><li>Thumb in palm – and clenched hand/flexed wrist in general </li></ul><ul><li>Elbow flexion </li></ul><ul><li>Striatal/ Clenched toes </li></ul><ul><li>Equinovarus deformity </li></ul><ul><li>Scissoring Gait </li></ul>
  17. 17. Side Effects of Botulinum Toxins <ul><li>Systemic Botulism </li></ul><ul><li>Respiratory compromise </li></ul><ul><li>Dysphagia </li></ul><ul><li>Dry mouth </li></ul><ul><li>Muscle Pain </li></ul><ul><li>Weakness </li></ul>
  18. 18. Response Problems <ul><li>Poor response may be primary (with first injection) or secondary </li></ul><ul><li>Reasons for poor response </li></ul><ul><ul><li>Poor muscle injection technique </li></ul></ul><ul><ul><li>Incorrenct muscle selection </li></ul></ul><ul><ul><li>Dosing inadequate </li></ul></ul><ul><ul><li>Muscle involvement has changed </li></ul></ul><ul><ul><li>Soft tissue contracture </li></ul></ul><ul><li>Neutralizing antibodies may be present (but rare in spasticity) </li></ul><ul><ul><li>Tests for nonresponse: frontalis test, </li></ul></ul><ul><ul><li>antibody assays (limited sensitivity, specificity) </li></ul></ul>
  19. 19. Dosing of Botulinum Toxin <ul><li>Botox (onabotulinumtoxinA) available in 100 unit vials of lyophilized powder </li></ul><ul><ul><li>Max dose not established, but FDA warns against dosing more than 400 units </li></ul></ul><ul><li>MyoBloc (rimabotulinumtoxinB) units are about 50x less potent than Botox </li></ul><ul><li>So 100 units Botox are approximately equivalent to 5000 units MyoBloc </li></ul>
  20. 20. ITB Therapy <ul><li>Intrathecal Baclofen Therapy delivers liquid baclofen directly into the intrathecal space around the spinal cord </li></ul><ul><li>Drug delivered via an implanted and programmable pump connected to a catheter </li></ul><ul><li>Approved by FDA in 1992 for spinal origin spasticity and 1996 for cerebral origin spasticity. </li></ul><ul><ul><li>Components are completely implanted (except the programmer) </li></ul></ul><ul><ul><li>Programmer is used to adjust the pump. </li></ul></ul><ul><ul><li>Implanting the pump is the beginning of therapy, takes 30-90 days to adjust dosage </li></ul></ul>
  21. 21. SynchroMed II Pump
  22. 22. Pump Compatibility <ul><li>The following are unlikely to affect pump operation or damage the pump: </li></ul><ul><ul><li>Electrocautery </li></ul></ul><ul><ul><li>Diagnostic Ultrasound </li></ul></ul><ul><ul><li>Low-Power Therapeutic Ultrasound – i.e. the type used in P.T. </li></ul></ul><ul><ul><li>Pacemakers/ICD’s </li></ul></ul><ul><ul><li>Diagnostic X-rays </li></ul></ul><ul><ul><li>TENS </li></ul></ul><ul><ul><li>Laser Procedures </li></ul></ul><ul><ul><li>Pressurized Aircraft </li></ul></ul><ul><ul><li>Theft detectors/Security Devices </li></ul></ul><ul><ul><li>Home Appliances </li></ul></ul><ul><ul><li>Tanning Bed </li></ul></ul>
  23. 23. Advantages of ITB ℞ <ul><li>Non-destructive and reversible </li></ul><ul><li>Potential for fewer side effects as compared to oral baclofen </li></ul><ul><li>Dose can be adjusted to optimal effect </li></ul><ul><li>May decrease spasticity-related pain </li></ul>
  24. 24. Potential Risks of ITB ℞ <ul><li>Most common side effects: weakness, drowsiness, nausea/vomiting, headache, and dizziness </li></ul><ul><li>Overdose, although rare, could lead to respiratory depression, loss of consciousness, coma, and in extreme cases, may be life-threatening </li></ul><ul><li>Infection </li></ul>
  25. 25. Potential Risks of ITB ℞ <ul><li>Abrupt discontinuation can result in high fever, altered mental status, worsened spasticity, and muscle rigidity </li></ul><ul><ul><li>needs to be treated as life threatening </li></ul></ul><ul><li>Causes: </li></ul><ul><ul><li>Empty pump reservoir </li></ul></ul><ul><ul><li>Catheter failure: disconnecting from pump, kinking, migrating or breaking </li></ul></ul><ul><ul><li>Electromechanical failure, e.g. battery failure </li></ul></ul>
  26. 26. Management Issues: Spasticity has increased <ul><ul><li>Patient not getting enough drug </li></ul></ul><ul><ul><ul><li>Low reservoir </li></ul></ul></ul><ul><ul><ul><li>Programming/refill error </li></ul></ul></ul><ul><ul><ul><li>Pump or catheter problem </li></ul></ul></ul><ul><ul><li>Factor causing increase in spasticity </li></ul></ul><ul><ul><ul><li>Disease progression </li></ul></ul></ul><ul><ul><ul><li>Physiologic response </li></ul></ul></ul>
  27. 27. Management Issues: Spasticity has decreased – hypotonia <ul><ul><li>Patient getting too much drug </li></ul></ul><ul><ul><ul><li>Programming error </li></ul></ul></ul><ul><ul><ul><li>Refill error </li></ul></ul></ul><ul><ul><ul><li>Subdural catheter </li></ul></ul></ul><ul><ul><ul><li>Pump pocket injection </li></ul></ul></ul><ul><ul><ul><li>Oral medication </li></ul></ul></ul>
  28. 28. Management Issues: Altered Mental Status <ul><li>Altered mental status </li></ul><ul><ul><li>Could be sign of overdose or withdrawal </li></ul></ul><ul><ul><ul><li>Has spasticity increased or decreased? </li></ul></ul></ul><ul><ul><ul><li>Other associated symptoms </li></ul></ul></ul>
  29. 29. Circumstances That May Require Added Attention <ul><li>Higher ITB Therapy doses </li></ul><ul><li>Non-verbal patient </li></ul><ul><li>Inability to identify problem/localize pain </li></ul><ul><li>ITB Therapy naïve medical system </li></ul><ul><ul><li>ER/ICU doesn’t contact “pump doctor” until after cascade effect </li></ul></ul><ul><li>After pump replacement </li></ul><ul><li>After pump removal for infection </li></ul>
  30. 30. Lioresal Intrathecal <ul><li>Lioresal Intrathecal is an analog to the naturally occurring inhibitory neurotransmitter gamma-aminobutyric acid (GABA) </li></ul><ul><ul><li>Effects similar to benzodiazepines </li></ul></ul><ul><li>SynchroMed ® II pump reservoir holds 20 or 40 ml of drug </li></ul><ul><ul><li>6-month supply </li></ul></ul><ul><ul><ul><li>500 mcg/ml </li></ul></ul></ul><ul><ul><ul><li>2000 mcg/ml </li></ul></ul></ul>
  31. 31. Overdose <ul><li>Symptoms Are Dose-Dependent </li></ul><ul><ul><li>Mild </li></ul></ul><ul><ul><ul><li>Hypotonia </li></ul></ul></ul><ul><ul><ul><li>Difficulty concentrating </li></ul></ul></ul><ul><ul><ul><li>Progressive decrease in tone to flaccid </li></ul></ul></ul><ul><ul><li>Moderate </li></ul></ul><ul><ul><ul><li>Somnolence </li></ul></ul></ul><ul><ul><ul><li>Obtundation </li></ul></ul></ul><ul><ul><ul><li>Bradycardia </li></ul></ul></ul><ul><ul><li>Severe </li></ul></ul><ul><ul><ul><li>Stupor </li></ul></ul></ul><ul><ul><ul><li>Hypoventilation to apnea </li></ul></ul></ul><ul><ul><ul><li>Coma </li></ul></ul></ul>
  32. 32. Overdose <ul><li>Iatrogenic </li></ul><ul><ul><li>Likely due to human error </li></ul></ul><ul><ul><li>Programming error </li></ul></ul><ul><ul><li>Unanticipated effect of dosage or concentration </li></ul></ul><ul><li>SynchroMed pump vs. catheter malfunction </li></ul><ul><li>Difficult to ascertain cause </li></ul>
  33. 33. Overdose – Mechanical Causes <ul><li>Subdural catheter </li></ul><ul><li>Filling of pump pocket </li></ul><ul><ul><li>18 ml of Lioresal Intrathecal 500 mcg/ml=9 mg </li></ul></ul><ul><ul><li>18 ml of Lioresal Intrathecal 2000 mcg/ml=36 mg </li></ul></ul><ul><ul><li>40 ml of Lioresal Intrathecal 500 mcg/ml=20 mg </li></ul></ul><ul><ul><li>40 ml of Lioresal Intrathecal 2000 mcg/ml=80 mg </li></ul></ul>
  34. 34. Overdose - Treatment <ul><li>Suggested Treatments </li></ul><ul><li>Mild </li></ul><ul><ul><li>Decrease dose </li></ul></ul><ul><li>Moderate </li></ul><ul><ul><li>Stop pump for several hours (2-4 hours) </li></ul></ul><ul><ul><ul><li>Program minimal infusion bolus, then restart at lower dose </li></ul></ul></ul><ul><ul><li>Decrease dose </li></ul></ul><ul><li>Monitor patient carefully for at least 24 hours </li></ul>
  35. 35. Severe Overdose <ul><li>Suggested Treatment </li></ul><ul><li>Maintain airway/breathing/circulation </li></ul><ul><li>Empty pump reservoir to stop drug flow </li></ul><ul><ul><li>Aspirate reservoir if no programmer </li></ul></ul><ul><ul><li>Remember to schedule restart </li></ul></ul><ul><li>Administer physostigmine/pressors </li></ul><ul><ul><li>+/- physostigmine(0.5-1.0mg increments IV) </li></ul></ul><ul><li>LP to withdraw 30 – 40 mL CSF </li></ul><ul><li>Notify patient's ITB Therapy physician </li></ul><ul><li>Continue to monitor closely for symptom recurrence </li></ul>
  36. 36. Suggested Treatment <ul><li>Remove CSF </li></ul><ul><ul><li>An effective treatment </li></ul></ul><ul><ul><ul><li>Effective in first 4 hours </li></ul></ul></ul><ul><ul><ul><li>Still may be effective up to 8 hours </li></ul></ul></ul><ul><ul><li>Catheter access port (CAP) </li></ul></ul><ul><ul><li>30 – 40 mL </li></ul></ul><ul><ul><li>Lumbar puncture if cannot aspirate CAP </li></ul></ul>
  37. 37. Sequelae of Severe Overdose <ul><li>Patient </li></ul><ul><ul><li>Less frequent if airway is protected and hypotension treated rapidly </li></ul></ul><ul><li>Family </li></ul><ul><ul><li>Fear of patient’s death </li></ul></ul><ul><ul><li>Fear of brain damage </li></ul></ul><ul><ul><li>Intensive emotional support needed </li></ul></ul>
  38. 38. Baclofen Withdrawal <ul><li>Severity of withdrawal varies </li></ul><ul><ul><li>Mild </li></ul></ul><ul><ul><ul><li>Minimal symptoms </li></ul></ul></ul><ul><ul><ul><li>Mild flu-like syndrome </li></ul></ul></ul><ul><ul><li>Moderate </li></ul></ul><ul><ul><ul><li>Increase in tone </li></ul></ul></ul><ul><ul><ul><li>Itching </li></ul></ul></ul><ul><ul><ul><li>Mild dysphoria </li></ul></ul></ul><ul><ul><li>Severe </li></ul></ul><ul><ul><ul><li>Continuous spasms </li></ul></ul></ul><ul><ul><ul><li>Severe pain </li></ul></ul></ul><ul><ul><ul><li>Delirium </li></ul></ul></ul><ul><ul><ul><li>Death </li></ul></ul></ul>
  39. 39. Symptoms of Underdose <ul><li>Pruritis without rash </li></ul><ul><li>Hypotension </li></ul><ul><li>Paresthesias </li></ul><ul><li>Fever </li></ul><ul><li>Altered mental status </li></ul>
  40. 40. Symptoms of Withdrawal <ul><li>Exaggerated rebound spasticity and muscle rigidity </li></ul><ul><li>Might be diagnosed in ER as seizures </li></ul><ul><li>Rhabdomyolysis </li></ul><ul><li>Multiple organ failure </li></ul><ul><li>May resemble </li></ul><ul><li>Autonomic dysreflexia </li></ul><ul><li>Sepsis </li></ul><ul><li>Malignant hyperthermia </li></ul><ul><li>Neuroleptic-malignant syndrome </li></ul>
  41. 41. Suggested Treatment <ul><li>Always assume it’s the Intrathecal Baclofen </li></ul><ul><li>Initiate life-sustaining measures if indicated </li></ul><ul><li>Thorough history </li></ul><ul><ul><li>Abrupt vs insidious onset </li></ul></ul><ul><ul><li>Presence of VPS </li></ul></ul><ul><ul><li>Recent refill/replacement/programming </li></ul></ul><ul><li>Start oral baclofen </li></ul><ul><ul><li>Each patient needs prescription at home </li></ul></ul><ul><ul><li>Remind family to take prescription on vacation </li></ul></ul>
  42. 42. Other Interventions <ul><ul><li>Benzodiazepines </li></ul></ul><ul><ul><ul><li>GABA agonists </li></ul></ul></ul><ul><ul><ul><li>IV or PO </li></ul></ul></ul><ul><ul><li>Cyproheptadine (serotonin antagonist)* </li></ul></ul><ul><ul><ul><li>Cyproheptadine 4-8 mg every 6 hours </li></ul></ul></ul><ul><ul><li>Lioresal Intrathecal via LP </li></ul></ul><ul><ul><ul><li>50-100 mcg bolus </li></ul></ul></ul><ul><ul><li>Lumbar infusion via bedside pump and percutaneous catheter </li></ul></ul><ul><ul><ul><li>Maintenance </li></ul></ul></ul><ul><ul><ul><li>Weaning ITB Therapy </li></ul></ul></ul><ul><li>*Meythaler JM, Roper JF, Brunner RC. Cyproheptadine for intrathecal baclofen withdrawal. Arch Phys Med Rehabil 2003; 84:638-642. </li></ul>
  43. 43. Pearls to Prevent Withdrawal <ul><li>Refill pump if suspect low reservoir </li></ul><ul><li>Compare actual vs expected aspirate </li></ul><ul><li>Verify concentration of last refill </li></ul><ul><li>Systems to ensure patients receive refills in a timely manner </li></ul><ul><li>Examine programming after pump replacement </li></ul><ul><li>Troubleshoot pump and catheter immediately upon suspicion of malfunction </li></ul><ul><li>Patients should have some oral baclofen available for emergency </li></ul>
  44. 44. Conclusions <ul><li>While spasticity management can be difficult, it may also improve patient’s quality of lift </li></ul><ul><li>Spasticity is not necessarily the enemy, but is part of a pattern of abnormal motor control </li></ul><ul><li>Choice of treatment depends on pattern of involvement </li></ul>
  45. 45. Contact <ul><li>For questions about this audio conference please contact Dr. George Wittenberg at </li></ul><ul><li>For any questions about the monthly GRECC Audio Conference Series please contact Tim Foley at or call (734) 222-4328 </li></ul><ul><li>To evaluate this conference for CE credit please obtain a ‘Satellite Registration’ form and a ‘Faculty Evaluation’ form from the Satellite Coordinator at you facility. The forms must be mailed to EES within 2 weeks of the broadcast </li></ul>