Overview
• A component of the UMN syndromes – Traumatic SCI and SC disorders
– Brain Injuries
– Neurodegenerative diseases, CP
• Presents with varying degrees of clinical
problems that challenge patients, families
and health care professionals.
Definition
• A motor disorder characterized by a
velocity-dependent increase in the tonic
stretch reflexes (muscle tone) with
exaggerated phasic stretch reflexes
(tendon jerks, clonus) resulting from
hyperexcitability of the stretch reflex.
Lance 1980
Definition
• Increased resistance to passive stretch
which is… – Velocity-dependent
– Direction-dependent
– May have initial free motion
• “clasp-knife” phenomenon
• Positive and negative symptoms
Spasticity
• Positive Symptoms
– Hyperreflexia
– Clonus
– Co-contractions
– Postural abnormalities
– Disorder of voluntary
movements
– Increased muscle
stiffness
• Negative Symptoms
– Muscle weakness
– Incoordination
– Fatigue
– Pain
Spasticity
(When you have a spastic pt, you see…)
1. Intrinisic tonic spasticity • Increased tone
• Increased denervation hypersensitivity and changed muscle properties
2. Intrinsic phasic spasticity • Hyperreflexia and clonus
3. Extrinsic spasticity • Involuntary spasms in response to a perceived stimulus.
Pathophysiology
• Not fully understood – Suspect loss of supraspinal inhibitory and excitatory inputs
causes:
• Increased excitability – Hyperexcitability of interneurons and alpha motor neurons leads to
exaggerated stretch reflexes
– Denervation hypersensitivity
• Reduced inhibition – Loss of descending inhibitory pathways to antagonist muscle leads
to co-contraction
• Plasticity?
Benefits of Spasticity
• Stability in sitting/standing
• Maintains muscle bulk
• Increases venous return
• Improves cough
• Improves functional capabilities – ADL’s
– Mobility
Problems with
Spasticity
• Impacts function and QoL – Medical
• Pain, stiffness, spasms, positioning, contractures, skin breakdown, infections and ulcers
– Physical
• Mobility, ADLs, hygiene
– Psychosocial
• Cosmesis, recreational, sleep, mood, self esteem, sexual
– Vocational
Assessment of Spasticity
• History and physical
• Scales
– Ashworth, Modified Ashworth, VAS, SCATS, Penn Spasm Frequency, Tardieu scales
• Tests
– Biomechanical
– Pendulum Wartenberg and joint oscillation tests
– Electrophysiologic (mostly used in research)
– H-reflex, H/M ratio, F-wave, Surface EMG
Clinical Scales
• Ashworth Scale: ordinal scale for measuring musle tone
• Modified Ashworth Scale: defines the lower end of the scale by
adding the 1+
• SCATS: spinal cord assessment tool for spasticity
• VAS: visual analogue scale (subjective)
• Penn Spasm Frequency Score: ordinal scale for measuring leg
spasm frequency per day
Clinical Scales, Continued
• Tardieu Scale – interval scale for measuring stretch response reactions at
specific velocities
– modified scale became much more involved and added specific
positions and alignments and measured angles
– R2 (muscle length at rest)
– R1 (muscle length when catch occurs)
– difference b/w the two is key
» large difference means large dynamic component
» small difference means more fixed and possibly
contracted
Testing Positions
Upper Limb
To be tested in a sitting position, elbow flexed by 90°
at the recommended joint positions and velocities.
Shoulder
Horizontal Adductors V3
Vertical Adductors V3
Internal Rotators V3
Elbow
Flexors V2 Shoulder adducted
Extensors V3 Shoulder abducted
Pronators V3 Shoulder adducted
Supinators V3 Shoulder adducted
Wrist
Flexors V3
Extensors V3
Fingers Angle PII of digit III- MCP
Palmar Interossei V3 Wrist resting position
+ FDS
Lower Limb
To be tested in supine position, at recommended joint
positions and velocities
Hip
Extensors V3 Knee extended
Adductors V3 Knee extended
External Rotators V3 Knee flexed by 90
Internal Rotators V3 Knee flexed by 90
Knee
Extensors V2 Hip flexed by 30
Flexors V3 Hip flexed
Ankle
Plantarflexors V3 Knee flexed by 30
Ref: Boyd R, Graham K. Objective Measurement of clinical findings in the use of Botox type A for the
management of children with Cerebral Palsy. European Journal of Neurology 6(Supp 4) S23-35
Tardieu G, Rondont O, Mensch J, Dalloz J, Monfraix C, Tabary J. Responses electromyograhpiques a
l’etirement musculaire chez l’homme normal. Revue Neurologie + 97(1), 60-61
Gracies J, Marosszzeky J, Renton R, Sandaman J, Gandevia S, Burke D. Short term effects of dynamic
splints on the upper limb in hemiplegic patients. Archives of Physical Medicine and Rehabilitation, 81
1547-1555.
Modified Ashworth Scale
Score Criteria
0 No increase in tone
1 Slight increase in tone (catch & release at end of ROM)
1+ Slight increase in tone, manifested by a catch, followed by
minimal resistance through remainder (<1/2) ROM
2 Marked increase in tone through most of ROM, but affected
part easily moved
3 Considerable increase in tone; passive movement difficult
4 Affected part(s) rigid in flexion or extension
Quantitative Tests: Difficulties
• Static test for dynamic process
• Spasticity changes based on time of day and with many
other factors
• Test position usually not the position of function
• Poor correlation between scales
• Discrepancy between self-rated and clinical scores
• Decrease in score does not necessarily correlate with
improved function (weakness, muscle coordination are also
factors)
• Does not fully evaluate specific impact of spasticity in
limiting activity or participation
Questions to Ask before
choosing Treatment
• Does the spasticity cause pain?
• Is it leading to contracture?
• Does it interfere with function or sleep?
• Does it interfere with passive care?
• Does it have psychosocial consequences?
• Is cosmesis an issue?
• Is it interfering with proper brace fitting?
• Does it affect QoL?
Treatment Concepts
• Goals must be reality based and meaningful
– Decrease pain
– Increase function (i.e. mobility, ADL, QoL)
– Prevent contractures
• Decisions
– Severity
– Scope: local vs. regional
– Medical and cognitive status of patient
– Side effects!!
– Cost-benefit ratio
Distribution
• Focal such as flexed elbow, extended
toe, clonus
• Multifocal: several joints in the same
limb
• Regional: spastic diplegia (mostly legs
but some arms as seen in CP)
• Generalized: diffuse
Treatment Management
• Utilizing the most conservative approach – Least side effects and most cost effective
• Progression from simple to complex
interventions: – Remove noxious stimuli Rehabilitation therapy Oral
Rx Neurolysis/Muscle Paralysis Surgery
Rehabilitation Interventions
• Positioning on a consistent basis
– Bed, WC
• Modalities
– Heat, Cold, E-stim, Biofeedback
• Therapeutic exercise
– Stretching, Hydrotherapy
• Orthotics, bracing, serial casting
Serial Casting • Series of casts to reduce spasticity
– Increase soft tissue and/or muscle lengthening
• Can use Tardieu scale to know how much range one can achieve
• Go to end range then back off slightly to help improve tolerance to cast
• Remove first cast in 24 hours to check skin
• Subsequent casts every 2-3 days
• Can bivalve
• Stop when no improvement noted in 2 consecutive casts
• Can use injections first to augment stretch
Pharmacological Treatments
• Baclofen
• Benzodiazepines
• Alpha-2-agonists
• Dantrolene sodium
• Others – Cyproheptadine
– 4-aminopyridine
– TCAs
– Gabapentin
– Opioids
– Cannabis
Oral Baclofen
• GABA-b selective agonist
• Decreases release of excitatory transmitters
• First line agent for spinal spasticity
• Dosage 5-80mg/day as per the PDR
• Equivalent to diazepam in efficacy but less
sedation
• Avoid sudden withdrawal seizures,
hallucinations, rebound spasticity
Clinical Effects of Baclofen
• Decreases seen in:
– Spasms
– Hypertonia
– Clonus
– Pain
– Bladder issues
• Side effects:
– Sedation
– Weakness
– Nausea
– Ataxia
– Confusion
– Decreased seizure
threshold
– Weight gain
Benzodiazepines
• Binds to GABA-a receptors – Facilitates post-synaptic effects of GABA resulting in
enhanced pre-synaptic inhibition
• Effective in painful spasms
• Long and short acting meds
• Doses – Diazepam: start at 1-5mg BID, max 60mg/day
– Klonopin: 0.5mg-1mg at bed time for nocturnal spasms
Benzodiazepines
• Benefits
– Decreases
hyperreflexia
– Decreased painful
spasms
– Good for bedtime use
– Decreases anxiety
• Side effects
– Sedation
– Fatigue
– Weakness
– Confusion
– Can slow cog recovery
– Depression
– Ataxia
– Incoordination
– Potential for
dependency, abuse
Dantrolene Sodium
• Works directly on muscle – Prevents Ca release from SR
– Effects normal as well as spastic muscles (weakness)
– Less likely to cause sedation
• Indicated primarily in supraspinal spasticity
• Initial dose 25mg TID
• Max dose 100mg QID
• Monitor LFTs: – Before starting, during titration and after stable dose reached
Clonidine
• Transdermal delivery – Also oral
• Benefits – Decreased spasticity, clonus, hyperreflexia
– Change patch weekly
– May enhance coordination (mobility)
• Side effects – Hypotension
– Fatigue
– Decreased intellectual function
– Skin reaction
Tizanidine
• Peak plasma concentration in 1-2 hours
• Start at 1-2mg QHS
• Max dose 36mg/day
• Better tolerated in QID dosing
• Most common side effects: – Dry mouth, somnolence, dizziness
• Most serious side effects: – Hallucinations, elevated LFTs
• Monitor pts on oral contraceptives
• Contraindicated with Cipro or Luvox
Cyproheptadine
• Antihistamine, antiseratonergic and mild anticholinergic activity
• Blocks post-synaptic denervated supersensitive spinal receptors
• Benefits
– May enhance coordination, i.e. improved gait mechanics
• Side effects – Fatigue, weight gain, psychosis
• Dosage: – Initiate at 4mg QHS
– Up to 36mg/day
– Most common: 16mg/day in divided doses
Other Medications • Gabapentin
– Small studies in MS and SCI
– Improved spasticity with higher doses: 1200-3600 mg/day in divided
doses
• Pregabalin
• Cannabis
• Opioids
• 4-aminopyridine
• Alcohol
Injections
Benefits:
• Reversible
• Temporary
• Titratable
• Can be used with other therapies
• Local treatment w/o systemic side effects – i.e., no fatigue, memory effects, or slowed thought
• Restores balance between agonists and antagonists
Goals of Injections
• Diagnose contracture
• Facilitate therapy and function – Increase passive and active ADL’s
– Improve fitting of braces
– Ease of care
• Clinical benefits – Decreased pain
– Prevent or delay complications
– Reduce disfigurement
Anesthetics and
Chemodenervating Agents • Local anesthetics short-duration diagnostic block
(2-8 hours) – Lidocaine
– Bupivacaine
– Etidocaine
• Neurolysis long-duration (3-12 months) – Phenol (3-7% dilution)
– Alcohol (35-60% dilution)
• Neuromuscular junction block long-duration (3-6 months) – Botulinum toxin A
– Bolulinum toxin B
Alcohol/Phenol Injection • Dehydrated alcohol (35-60%)
• Phenol (3-7%)
• Clinical uses: – Motor nerve block
– Motor point block (IM injection)
• Localize injection site using electrical stimulator
• Immediate effects
• Variable duration of effect
• Most common nerves – Musculocutaneous, median, obturator, femoral, posterior tibial
• Complications – Pain, dysesthesias, tissue fibrosis
Alcohol/Phenol:
Adverse Effects
• Acute: brief burning/pressure during injection, inflammation, skin irritation and rare systemic side effects
• Subacute: if mixed sensorimotor nerve injected, can see pain and dysesthesias lasting several weeks; rare risk of soft tissue necrosis
• Chronic: induration, tender nodule formation, tissue fibrosis
Botulinum Neurotoxin
• 7 serotypes
– A to G
• Only types A and B are currently available
– Multiple products for type A
– Not interchangeable
– Different preparations
– Different side effect profiles
Botulinum Toxin
• Acts presynaptically to prevent the release
of ACh
• Onset of effect 24-72 hours
• Peaks at 2-6 weeks
• Average duration = 3 months
• Nerve sprouts and reinnervates
• Can be used in conjunction with other
treatments
Botulinum Toxin Injections
• Techniques – Motor point with EMG guidance
– E-stimulator
– Ultrasound
– Anatomic landmarks
• Adverse effects – Short intervals between injections and higher doses may
increase Ab formation
• Recommend: – Injection at 3 month intervals
When to turn to
Intrathecal Baclofen
• Spasticity not
responding to oral
meds
• Intolerable side
effects from oral
meds
• No alternatives left
except for surgery
SynchroMed Infusion System
• Pump – Infuses drug at programmed rate
• Catheter – Delivers drug to intrathecal (subarachnoid) space
• Programmer – Allows for precise dosing
– Easily adjustable dosing
Advantages of ITB Therapy
• Non-destructive
• Reversible
• Potential for fewer systemic side effects
• Programmable
• Dose can be titrated to optimal effect
Pharmacokinetics
• Intrathecal
– 600 mcg/day dose: 1.24 mcg/ml IT lumbar
concentration
– Lumbar to cervical concentration is 4:1 b/c of
molecular weight
– Half-life is 4-5 hours
• Oral
– 60mg dose: 0.024 mcg/ml IT lumbar concentration
– Half-life 3-4 hours
Post-Operative Phase
• Begin concentration at 500mcg/ml
• Can increase dose after 24 hours
– 10-15% per day as needed
• Maintenance dose = 300-1500 ug/day
Emergency Procedure:
Overdose
• Signs of overdose: seizures, sedation, weakness
• Maintain ABCs and provide supportive care
• If applicable, empty pump reservoir to stop drug flow and record amount withdrawn
• Physostigmine controversial for treating sedation or respiratory depression
• If LP is not contraindicated, withdraw 30-40ml CSF to decrease baclofen concentration – can be done via access port as well
Emergency Procedure:
Under Dose • Sign of rapid withdrawal: seizures, autonomic dysfunction,
hallucinations, rebound spasticity, itching
• Always make sure your patients have a supply handy of oral baclofen
(or benzo) should they pass their alarm date or miss refill appointment.
– may even need IV medications
• Treat seizures accordingly
• Supportive care
• Rapidly refill or reprogram pump
• Investigate pump for cause of possible failure
• Have meticulous system in place to avoid alarms
Surgical Treatments
• Neurodestructive
– Neurectomy
– Myelotomy
– Rhizotomy
– Cordectomy
– Selective dorsal
rhizotomy
• Orthopedic
– Tenotomy, Tendon lengthening, Myotomy, Tendon
transfers
SPLATT Procedure
• Split Anterior Tendon Transfer
• Treatment of equinovarus deformity of the
foot – Foot is PF (due to spastic GSC) and inverted and
supinated (due to spastic TA)
• Usually in conjunction with achilles tendon
lengthening
• Makes the TA a neutral inverter
Selective Dorsal Rhizotomy
• Procedure – Surgeon intraoperatively determines which rootlets
cause the spasticity
• Via EMG and careful dissection
– Selectively cuts these rootlets
• Two primary goals 1. Facilitate patient care: sitting, dressing, transfers
2. Improve function: walking