Dystonia and spasticity

2. 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

3. 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.

4. 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

5. 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

6. Pathophysiology
Central Nervous System (CNS) Tracts
--Spinothalamic
--Corticospinal
--Corticothalamic
--Corticocerebellar

7. 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

8. Assessing Muscle Tone
• History
• Physical examination
• Measurement tools

9. Key Questions in History
• Location?
• Time of day,
aggravating/alleviating
factors? (diary)
• Painful spasms?
• Functional limitations?
• Need for extensor tone
in legs for standing?

10. Physical Examination
• Standard
musculoskeletal
examination including
range of motion
(measured with a
goniometer)
• Standard neurologic
examination
• Clonus
• Ashworth scale

11. 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

12. Quantitative Evaluation
• Gait lab with surface
electromyography
(mostly research use)
• Fugl-Meyer test for
upper limb dexterity

13. 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

14. Spectrum of Care for
Management of Spasticity
Injection
Therapy
Neurosurgeries
Orthopedic
Treatments
Rehabilitation
Therapy
Prevent
Nociception
Intrathecal
Baclofen
(ITB™)
Therapy
Oral
Drugs
Patient

15. 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

16. Generalized Spasticity
Remove Nociceptive
Factors
Positioning Modalities
Stretch/Splint
Oral Medication
Intrathecal Medication
Myelotomy
Dorsal Rhizotomy

17. Oral Medications (1)
• Baclofen
• Binds GABA-b receptors
• SCI > brain
• Side effects—weakness, lethargy, rebound
seizures—if stopped abruptly
GABA = gamma-aminobutyric acid.

18. Oral Medications (2)
• Dantrolene
• Blocks Ca++ channels in muscle
• No CNS effects, but causes significant
weakness
• Hepatic dysfunction rare
CNS = central nervous system.

19. Oral Medications (3)
• Tizanidine
• Alpha-2 agonist
• Brain = SCI
• Side effects: lethargy, orthostasis, dry mouth

20. 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

21. 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.

22. 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

23. 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.

24. 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.

25. 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.

26. 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

27. Answer
B. Look for nociceptive causes for his
increased tone such as UTI or pressure
sore

28. Localized Spasticity
Botulinum Toxin A
Botox, Dysport
Botulinum Toxin B
Myobloc
Smaller Muscles
Shorter Acting
Tendon Surgery
Phenol
Alcohol
Larger Muscles
Semipermanent
Prolonged Stretch
Bracing/Splinting
Ultrasound
OnabotulinumtoxinA (Botox®), AbobotulinumtoxinA (Dysport®), RimabotulinumtoxinB (Myobloc®).

29. 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

30. 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,

31. 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.

32. 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.

33. 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.

34. 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

35. Shoulder
• Internal
rotation/adduction

36. Case #2
• 70-year-old woman with spastic
hemiparesis with painful right
shoulder, adductor, and internal
rotation contractures
• How would you treat?

37. 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

38. 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.

39. Elbow
• Flexion/pronation
• Muscles?
• Biceps (2-joint rule)
• Brachialis
• Brachioradialis
• Pronator teres

40. 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.

41. Hand
• Thumb in palm
• Muscle?
• Flexor pollicis longus

42. Lower Limb
• Hip flexion/adduction
• Muscles?
• Iliopsoas, rectus
femoris/add mag
• Knee flexion
• Muscles?
• Hamstrings
• Equinovarus
• Muscles?
• Gastrocnemius/soleus

43. 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.

44. 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.

45. 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.

46. 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.