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Management of Spasticity

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Management of Spasticity Powered By Docstoc
					Spasticity Assessment and
Management for the Primary
Care Physician

       Eileen Donovan, MD
          Medical Director
  The Detroit Institute for Children
Spasticity - Definition
   A motor disorder characterized by a
    velocity-dependent increase in tonic
    stretch reflexes (muscle tone) with
    exaggerated DTRs, resulting from
    hyperexcitability of the stretch reflex, as
    one component of the UMN syndrome
               Lancet, 1980, from consensus meeting in
                Scottsdale, AZ in 1979
Spasticity
   Assessed by
       Deep tendon reflexes
       Passive mobilization
            Like love – you gotta feel it
            Movement through the full ROM should take
             less than one second
   Evaluated by
       Modified Ashworth Scale
       Tardieu Scale
Modified Ashworth Scale
   0 – No increase in muscle tone
   1 – Slight increase in tone; a catch and release at the
    end of the range when the affected part is moved
   1+ – Slight increase in tone; a catch, followed by
    minimal resistance through the remainder (less than
    half) of the range of motion
   2 – More marked increase in muscle tone through
    most of the range of motion, but affected part easily
    moved
   3 – Considerable increase in muscle tone, passive
    movement difficult
   4 – Affected part rigid in flexion or extension
       Not seen in cerebral palsy
                    Bohannon and Smith, 1987
Pathophysiology of spasticy
   Not well understood, despite
    considerable investigation
   Interruption of descending inhibitory
    pathways
Etiology of spasticity
   Cerebral palsy (CP)
   Traumatic brain injury
   Spinal cord injury
   Anoxic brain damage
   Neurodegenerative disorder
   Stroke
   Multiple sclerosis
     Impact of spasticity
   Decreased function and decreased activity
       Activities of Daily Living (bathing, dressing,
        toileting, transfers)
       Social isolation
       Bowel and bladder problems
       Contractures
       Orthopedic problems (scoliosis, dislocated
        hips)
Impact of spasticity
   Decreased function and decreased
    activity
       Skin breakdown (decubitus ulcers)
       Infection
       Osteopenia/osteoporosis
       DVT – rare before puberty
       Fatigue
       Nutritional compromise
Impact of spasticity
   Affects quality of life
       Mobility
       Hygiene
       Pain
       Self-care (or caregiver-provided care)
       Sleeping patterns
       Self-esteem
    Cerebral Palsy
   A disorder of posture and movement
    caused by a non-progressive lesion that
    affects the developing brain
   It is the most common motor disability of
    childhood
   Incidence 2-3/1000 live births
   700,000 children and adults with CP in US
Cerebral Palsy
   Although the brain lesion in not
    progressive, the musculoskeletal
    pathology is certainly progressive
    Musculoskeletal progression in CP

Static (CNS lesion)                   Spasticity and weakness
                                      Muscles don’t grow as
                                      fast as bones
                                      Fixed contracture
                                      Bony torsion
Progressive                           Joint instability
(Musculoskeletal                      Dislocation or
deformity)                            degenerative changes


      Graham HK, Eur J Neurol, 2001
Examination
   Spasticity in extremities – velocity
    dependent
   Brisk DTRs
   Clonus
   Upgoing plantar reflexes (Babinski)
   Synergistic movement patterns
   +/- Contractures
Rationale for treatment
   If spasticity interferes with
       Functioning
       Positioning
       Comfort
       Care
   If spasticity is not useful (ie: transfers)
   If treatment is expected to provide
    improvement
To treat or not to treat? (And
how to treat?)
   Chronicity – acute vs. chronic
   Severity
   Distribution – diffuse vs. focal
   Co-morbidities – seizures, cognition,
    contractures
To treat or not to treat? (And
how to treat?)
   Availability of care and support
       Dosing compliance
       ITB – pump refills every 3 months
   Potential for complications
   Drug side effects, drug interactions
   Previous treatments (and results)
   Cost
Spasticity management goals
   Decrease spasticity/tone
       Improve constipation?
   Improve ROM
       A relaxed muscle is not a longer muscle
   Decrease spasms/pain
       Improve sleep, improve quality of life
   Improve mobility
   Improve gait
   Decrease energy expenditure
Spasticity management goals
   Improve fit of orthoses
   Improve ease of care
   Improve hygiene
   Improve cosmesis/appearance
   Improve nutritional status by
    decreasing number of calories burned
   Postpone orthopedic surgery
Treatment options
   Therapy/Orthotics    least invasive
   Oral medications
   Chemodenervation
   Neurosurgery
   Orthopedic surgery   most invasive
       Treatment Options
                           General

             Therapy         Selective
             Orthotics       Dorsal
             Oral meds       Rhizotomy
             ITB
Reversible                                Permanent
             Chemo-          Orthopedic
             denervation     surgery


                           Focal
Effects of Therapy, Bracing and
Biomechanical Alignment on Spasticity




                         From Cusick
Oral medications
   Treat systemic spasticity, but have
    systemic side effects
   Benzodiazepines
   Baclofen
   Dantrolene sodium
   Tizanidine
   Clonidine
Benzodiazepines
   Diazepam (Valium), Clonazepam (Klonopin)
   Mechanism of action – potentiates pre-
    synaptic inhibitory effects of GABA-A
       Brain stem and spinal cord
   Side effects – sedation (most), weakness,
    hypotension, incoordination, confusion,
    depression, ataxia, GI symptoms
   Dependency and withdrawl possible
Oral Baclofen (Lioresal)
   Mechanism of action – GABA-B agonist
       Pre- and post-synaptic actions
   Side effects –sedation, lowers seizure
    threshold, weakness, hypotonia, ataxia
   May potentiate antihypertensives
   Sudden withdrawl can cause seizures,
    hallucinations, rebound spasticity
   Usually drug of first choice
Tizanidine (Zanaflex)
   Mechanism of action – alpha-2 agonist
       Blocks release of excitatory amino acids
        from spinal interneurons
   Obtain LFTs at baseline and at 1,3,6 mo
   Side effects – drowsiness, dizziness, dry
    mouth, orthostatic hypotension
   Often drug of first choice
Dantrolene sodium (Dantrium)
   Mechanism of action – reduces calcium
    release from the sarcoplasmic reticulum;
    uncouples excitation and contraction
   Obtain baseline serum LFTs
   Hepatotoxicity associated with long-term
    maximum dose, especially in women over 30
   Side effects – weakness (including respiratory
    muscles), lethargy, nausea, diarrhea,
    drowsiness
Clonidine (Catapres)
   Mechanism of action – alpha-2 agonist
   Available as a patch
   May allow lowered baclofen dose
   Helps with sleep disorders
Chemodenervation
   Injectable therapy which results in local
    muscle weakening
   Temporary and titratable

   Botulinum toxin
   Phenol or ethyl alcohol
Botulinum toxin (BTX)
   Produced by clostridium botulinum
   7 serotypes (A-G); A and B are
    approved for clinical use
       Botulinum Toxin A
            Botox, Dysport, Xeomin
       Botulinum Toxin B
            Myobloc, Neurobloc
   Used clinically since 1978
Botulinum toxin
   1989 – FDA approved for strabismus,
    blepharospasm, hemifacial spasm
   2000 – FDA approved for cervical dystonia,
    spasmodic dysphonia
   2002 – FDA approved for cosmesis
   2004 – FDA approved for excessive axillary
    hyperhidrosis
   2010 – FDA approved for spasticity in elbow,
    wrist and finger flexors/chronic migraine
Botulinum toxin
   Not FDA approved for treatment of
    spasticity in children
   Used “off-label” to treat spasticity in CP
   Other uses – torticollis, drooling,
    sweating (hands)
Mechanism of action
   Cleaves SNAP-25 protein
   Blocks the presynaptic release of
    acetylcholine at the neuro-muscular junction
    (NMJ)
   Most effective in NMJs that are most active
   The muscle can’t contract, so it is weakened
   Has some role at the muscle spindle level
Botulinum toxin
   Onset 24-72 hours, maximum effect at
    1-3 weeks
   Effects last for 3-6 months
   Creates a “window of opportunity”
       Nerve terminal sprouting
Botulinum toxin side effects
   Pain, bruising at injection site
   No anaphylactic reactions or deaths
    reported as direct result
       One death from anaphylaxis when mixed
        with lidocaine instead of saline
       LD50 is 3000 units (300 vials)
Botulinum toxin side effects
   Dose-dependent weakness
       Spread 2-3 cm, therefore may have
        weakness of uninjected muscles
   Dysphagia, dyspnea
   More involved kids have higher risk of
    side effects (therefore use lower dose)
       FDA investigation in 2008; 4 pediatric
        deaths over 8 yrs (vs 3200 from PCN)
Botulinum toxin side effects
   Urinary incontinence after LE injection
   Brachial plexopathy after SCM injection
   Generalized botulism-like symptoms
    rare
   Flu-like symptoms
   Contraindication – use of
    aminoglycosides
Antibody formation
   Prior to 1997, 17% of all focal dystonia
    patients develop neutralizing antibodies
   Reformulated in 1997; now very rare (1-2%)
    to develop antibodies
   To reduce risk, wait as long as possible
    between injections (at least 3 months)
   Xeomin has no protein therefore theoretically
    no risk of developing antibodies
Consensus statement on
pharmacotherapy for spasticity
   Botulinum toxin can be used as a single
    modality for focal spasticity or in
    conjunction with multisegmental
    management to help with specific areas
    of spasticity
               Tilton AH; J Child Neurol 2004
Patient selection
   Spasticity interferes with function, comfort,
    care
   Focal tone reduction will not diminish level of
    function
   No fixed contracture
       A relaxed muscle is not necessarily a longer
        muscle
       Adjunct to other therapies (ie: serial casting)
   Pain
Botulinum toxin
   Other things to consider
       EMG-guidance for forearm muscles,
        posterior tibialis, peroneals
       Ultrasound guidance considered “gold
        standard”
       If not using one of these for guidance, will
        miss the muscle:
            Gastrocnemius – 22%
            Forearm – 63%
            Posterior tibialis – 88%
                     Berwek S; Lancet, 2004   AACPDM, 2004
Botulinum toxin
   Don’t need to use anesthesia (vs
    alcohol blocks)
       Topical anesthetic
   Consider sedation for injections into
    salivary glands
Phenol/Ethyl Alcohol Injections
   Phenol 3-5%
   Ethyl alcohol 35-60%
   Motor nerve block or motor point block
       Obturator and musculocutaneous nerves
   Causes axonal protein denaturation
   Reqrowth of axons after a variable time
    period
       Results usually last 6-12 months
Phenol/Ethyl Alcohol Injections
   Adverse effects - Pain
       2-32%
       Mixed nerve most likely source
       Often several days after injection
       Burning paresthesia, exacerbated by touch
       Duration usually several weeks, but may
        be chronic
Phenol/Ethyl Alcohol Injections
   Adverse effects – Edema
       More common in lower extremities
       Usually resolves in 1-2 weeks
   Other things to consider
       Anesthesia risk
Surgical Treatments
   Intrathecal Baclofen Pump
   Selective Dorsal Rhizotomy
Intrathecal Baclofen Pump
   Implantable, programmable pump,
    controlled by telemetry
   Baclofen – GABA-B agonist
   Dosing significantly less that oral dose;
    most patients on 300-800 µg/day
   Medication stable in pump up to 90 days
   May have a role in preventing hip
    dislocation
Intrathecal baclofen pump
    Intrathecal Baclofen Pump
   Patient selection
       Severe, generalized spasticity
       Dystonia (at higher doses than for spasticity)
       Oral medications have proven ineffective
       Age 3-4, at least 30-35 pounds
       Good response to trial dose
       Patient and family have commitment to
        program
Intrathecal Baclofen Pump
   Precautions / Contraindications
       Impaired renal function
       Autonomic dysreflexia
       Psychotic disorder
       Use of depressants or alcohol
       History of uncontrolled seizures
Intrathecal Baclofen Pump
   Risks / Complications
       Infection
       Seizure risk if pump malfunctions
       Overdose potential
            Respiratory depression
            Loss of consciousness
            Reversible coma
            Death
       May see worsening scoliosis
Selective Dorsal Rhizotomy
   EMG guided sectioning of afferent nerve
    rootlets from L2-S2
   Interruption of reflex arc
   Often “unmasks” underlying weakness
Selective Dorsal Rhizotomy
   Patient selection
       Pure spasticity
       No dystonia or rigidity
       No trunkal hypotonia, good head control
       Best age 4-6 years old
       Good IQ, good cooperation
       Family supportive
Selective Dorsal Rhizotomy
   Exclusion criteria
       Spasticity of spinal origin
       Dystonia/Athetosis
       Rigidity
       Poor trunk control
       Severe weakness
 Anatomic distribution of CP




Quadriplegia (32%)   Diplegia (24%)   Hemiplegia (30%)
    Case 1: Spastic
    Quadriplegia
   3 yr old male with CP-spastic quad
   All 4 extremities spastic; hypotonic
    trunk
   Seizure disorder; uncontrolled and
    just started on ketogenic diet
   Hips subluxing, but no frank
    dislocation on x-ray
    Spasticity
    Management
   Baclofen – not my first choice given
    his uncontrolled seizures
   Zanaflex
   Consider Botulinum toxin for focal
    reduction at hip adductors and
    gastrocs
   Consider ITB when bigger
     Case 2:
     Spastic Diplegia
   8 yr old female with SD
   Ambulatory with walker
    (or sink)
   Seizures well managed on
    one medication
   Hip x-rays show coxa
    valga, shallow acetabuli
    but no subluxation
   Significant medial rotation
    in gait from femoral
    anteversion
        Spasticity
        Management
   Orthotics
   Correct rotational
    biomechanics at hip
       TheraTogs
       Kinesiotaping
   Oral baclofen or zanaflex
   Focal botulinum toxin for
    right wrist flexors, hip
    medial rotators, adductors?
   ITB/SDR candidate?
TheraTogs™
     Kinesiotaping®




   A rehabilitative taping method; long lasting
    (3-5 days); very versatile in its ability to re-
    educate the neuromuscular system, reduce
    pain and enhance performance
   www.kinesiotaping.com
        Case 3: Spastic Hemiplegia
   2 yr old female with spastic
    hemiplegia secondary to
    shaken baby syndrome at 3
    months
   Seizure disorder well managed
   Chronic problems with skin
    breakdown from brace
       ROM DF -5 degrees
   Right hand always fisted with
    thumb in palm; skin
    maceration
      Spasticity Management
   No systemic oral medications
   Botulinum toxin to finger
    flexors, thumb abductor
   Right hand splint
   Botulunim toxin to right
    gastrocnemius
   Serial casting to right ankle
   AFO
      Summary of Spasticity Treatments
Treatment             Pro                               Con
Therapy, Orthotics    Non-invasive                      Short lasting

Oral meds             Treats systemic spasticity,       Systemic side effects
                      temporary
Botulinum toxin       Focal spasticity, No anesthesia   Temporary (3-6 months)
                      risk, few side effects
Alcohol/Phenol block Focal spasticity, longer lasting   Anesthesia risk, risk of
                     than BTX (6-12 mo)                 dysesthesia
ITB                   Titratable, shown to help at-     Anesthesia risk, refill
                      risk hips                         pump every 90 days
SDR                   Permanent                         Anesthesia risk, not
                                                        titratable
Ortho surgery         “Permanent”                       Anesthesia risk, may need
                                                        to be repeated
Dystonia
   Movement disorder in which involuntary
    sustained or intermittent muscle
    contractions cause
       twisting and repetitive movements
       abnormal postures or
       both
   Often see co-contraction; athetoid
    movements of fingers, toes, tongue
    Dystonia
   Tone varies in response to quick stretch
   Measured with Barry-Albright Dystonia
    Scale (BAD scale)
   Often see co-contraction; athetoid
    movements of fingers, toes, tongue
   Like beauty–you know it when you see it
Dystonic Cerebral Palsy
   Dystonic movements may not be seen until 1
    yr old
   Used to be associated with kernicterus
   Pseudobulbar palsy- dysarthria, poor voice
    modulation, drooling
   Persistence of primitive reflexes
   70% have hearing loss
   25-33% have seizures
   Cognition usually unimpaired
Dystonic Cerebral Palsy
Dystonic Cerebral Palsy
Treatments for Dystonia
   Orthotics often
    difficult to wear
    because of constant
    movements
   TRAFOs give
    uniform pressure
    distribution
   ITB at higher doses
Medications for Dystonia
   Trihexyphenidyl (Artane)
       Mechanism of action – anticholinergic
       Originally used to treat Parkinson’s Disease
       Don’t use in patients with narrow angle
        glaucoma
       Side effects – dry mouth (50%), blurred
        vision, mild nausea or nervousness,
        Neuroleptic Malignant Syndrome
Contact me with questions
   Eileen Donovan, MD
   (313) 832-1100, ext. 516
   edonovan@detroitchildren.org