Spasticity Management – Dr. Manish Shah

Management of Spasticity
in Children
Manish N. Shah, MD
Pediatric Neuroscience Symposium 5/14/2016
Director of Pediatric Spasticity and Movement Disorder Surgery
Assistant Professor, Pediatric Neurosurgery
The University of Texas Medical School at Houston
Children’s Memorial Hermann Hospital
Mischer Neuroscience Institute
The University of Texas, MD Anderson Cancer Center
Financial disclosures
• None
Overview
• Introduction
• Cerebral Palsy (CP)
• Spasticity from CP
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Treatments
Intrathecal Baclofen
Selective Posterior Rhizotomy
Selective Dorsal Rhizotomy (T.S. Park Procedure)
• Indications
• Treatment Description
• Preop/Postop Video
• Questions
• Contact info
Cerebral Palsy
• Affects 1/500 births worldwide
• Cause is unknown
• Risks include prematurity, intraventricular
hemorrhage and low birth weight
• Has different forms
– Spastic (increased tone)
– Dyskinetic (variable tone)
• Dystonic
• Choreoathetoid/Hyperkinetic
– Ataxic (decreased tone)
– Mixed
Let’s talk about Spasticity
• Velocity-dependent abnormal stretch reflex
• Abnormal imbalance between excitation and
inhibition of the nervous system to the muscles
• This is why your patient’s muscles are rigid and
high in tone
BOTH AGONIST
INHIBITION
EXCITATION
AND ANTAGONIST
MUSCLES
CONTRACT AT THE
SAME TIME
Penn RD and Corcos DM, “Management of Spasticity by CNS Infusion Techniques”, Youmans, 2011
Spasticity Scales
Brunstrom-Hernandez and Tilton, “CP, Spasticity and Dystonia,” Youmans, 2011
Gross Motor Function Classification Scale
I – walks without limitations
II – walks with limitations
III – walks with a crutch/cane
IV – limited self mobility, needs walker
V – wheelchair-bound
http://www.cerebralpalsy.org.nz/about/cerebral-palsy/gross-motor-function-scale/
Albright L, “ITB for CP”, Youmans, 2011
Why the legs can be more affected
Periventricular Leukomalacia
Limbrick and Park, “Selective Dorsal Rhizotomy for Spastic Cerebral Palsy, Youmans, 2011
Treatments
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Oral Medications
Botulinum Toxin (Botox) Injections
Ablative Peripheral Neurotomies
Intrathecal Baclofen
Selective Dorsal Rhizotomy
Multidisciplinary
Approach!
Sindou and Mertens, “Ablative Surgery for Spasticity,” Youmans, 2011
Drug Mechanism of Action
GABAB receptor
GABAA receptor
Penn RD and Corcos DM, “Management of Spasticity by CNS Infusion Techniques”, Youmans, 2011
Oral Medications
• Baclofen/Tizanidine/Diazepam
• Pros:
– Noninvasive
• Cons:
– Many children cannot tolerate the side effects
• Confusion
• Sedation
• Potential toxicity
– Not effective in every patient
Botox Injections
• Pros:
– Target specific muscles
– Relaxation seen within days and peaks at a month
– Reversible
• Cons:
– Effects only last 3-4 months
– Need repeat injections
Ablative Peripheral Neurotomies
• Pros:
– Target specific muscles
– Relaxation seen immediately
– More for adults with fixed deformities
• Cons:
– Irreversible
– “Functional” rigidity may be eliminated
– Can have chronic pain when postganglionic sensory
nerves are cut
Intrathecal Baclofen (ITB)
• Spastic Quadriplegics (both arms and legs are
involved)
• Multiple orthopedic procedures
• Poor trunk control
• Unable to become independent walkers
Penn RD and Corcos DM, “Management of Spasticity by CNS Infusion Techniques”, Youmans, 2011
ITB Pump
SUBFASCIAL
PLACEMENT
Source: Medtronic.com
Intrathecal Baclofen Pump
• Pros:
– Reversible
– Good for spastic quadriparetics
• Cons:
– Expensive
– Needs frequent monitoring to ensure correct dose
– Life-threatening baclofen withdrawal syndrome
14 year experience - Milan (n=430)
9% overall infection rate
15% overall catheter complication rate
10% for first implantation
25% of pediatric patients had at least 1 complication, mean f/u = 8.8 years
Motta and Antonello, “Analysis of complications in 430 consecutive pediatric patients treated with intrathecal baclofen therapy: 14-year experience,” JNS Pediatrics, 2014
Selective Posterior Rhizotomy
• Multiple methods
– TS Park – 1 level lumbar laminectomy with EMG
testing; 75-80% of most spastic rootlets cut
– 6 level laminoplasty – L1-S1 +/- EMG testing
• “Equivalent” long lasting spasticity outcomes
• Risks of scoliosis only with 6 level laminoplasty
• Outcomes are dose-dependent on percentage
nerve rootlets sectioned (McLaughlin et al.,
2002)
McLaughlin 2002 Meta-analysis
McLaughlin Meta-analysis 2002
• The meta-analysis of 3 RCTs concludes that
SPR+PT > PT alone
• Is this meta-analysis truly comparing similar
operative procedures?
– Vancouver: 45% rootlet sectioned
• Preop exam and clinical testing
– Toronto: 41% rootlets sectioned
• EMG testing only to separate dorsal/ventral roots
– Seattle: 25% rootlets sectioned
• EMG testing
• What does one conclude from n=90?
Selective Dorsal Rhizotomy
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Spastic Diplegia and Spastic Hemiplegia patients
Children and adults age 2-40
Good trunk control
Can become independent walkers
Limbrick and Park, “Selective Dorsal Rhizotomy for Spastic Cerebral Palsy, Youmans, 2011
SDR – modified Park Procedure
• 1 level laminectomy at L1
• The dorsal roots around the spinal cord for the
lower extremities are tested and the most spastic
ones are cut
• Surgery takes 2.5 hours
• Four days in the hospital
• 6-12 months of intensive rehabilitation
SDR – Identification
Source: Park and Johnston, “Surgical techniques of selective dorsal
rhizotomy for spastic cerebral palsy: Technical note.” Neurosurg Focus
21 (2):E7, 2006
SDR – Selective Sectioning – 75% of nerve
rootlets
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EMG-Driven selective sectioning
Divide each nerve root into 3-5 rootlets
Test each rootlet for tetany threshold
Grade response of each rootlet at tetany
Cut 75% of the most spastic rootlets after
testing them all
Source: Park and Johnston, “Surgical techniques of selective dorsal rhizotomy for spastic cerebral palsy:
Technical note.” Neurosurg Focus 21 (2):E7, 2006
SDR
• Pros:
– Immediate reduction in spasticity
– Best option for spastic diplegics and hemiplegics
– Child can potentially walk independently
• Cons:
– Not reversible
– Commitment to therapy for at least 12 months
postoperatively
SDR Patient Video
Overall SDR Outcomes
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>3,500 patients at St. Louis Children’s
Excellent, permanent reduction in spasticity
Many are independent walkers
Minimal complications
– Spinal fluid leak (a few patients)
– Spinal fusion (1 patient)
Additional SDR Outcomes
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Decreased need for orthopedic surgeries
70-80% of hip deformities unchanged postop
Upper limb improvement
Cognitive/language improvement
Psychological improvement
Anecdotal Bowel/Bladder functional
improvement
TCSC SDR Outcomes
• All 5 patients have
had their spasticity
eliminated
• Reduced hospital stay
by 1 day
• No complications
• These children don’t
just walk, they run
and take karate!
SDR vs. ITB: Different Patients!
SDR
ITB
Low GMFCS (di-, hemi- or triplegia)
Spastic cerebral palsy
High GMFCS (quadriplegia)
Spasticity from tumor/trauma
Ambulatory Potential
Wheelchair bound
Minimal orthopedic intervention
Multiple tendon/muscle releases
Parental Commitment to Therapy
Geographical commitment/closeness to
academic neurosurgeon
Spasticity Summary
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Multiple treatment options
Careful patient selection
Need multi-disciplinary team
Need close follow up
Brain MRI in CP children shows dysfunction
Improved connections with
therapy and stem cells
Englander Z et al, Neuroimage 2015
Corticopyramidal Tract
Resting State MRI Analysis
Brain network analysis
Temporal Latency Analysis
Texas Comprehensive Spasticity Center
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Nivedita Thakur, MD – Peds Neurology
Glendaliz Bosques, MD – Peds PM&R
Mariana Kendrick, PT
Magdalena Jungman, OT
Zach Kelsey, OT
Marcia Kerr, RN
Paula Maldonado, MA
Reyna Balderaz, MA
Vianey Leang, RN
Pediatric Orthopedics
– Lindsay Crawford
– Alfred Mansour
– Shiraz Younas
Thanks!
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PEDIATRICIANS!!!
Children’s Memorial Hermann Hospital
Texas Comprehensive Spasticity Center
Pediatric Neurosurgery, University of
Texas Health Science Center at Houston
– Stephen Fletcher
– David Sandberg
• Mischer Neuroscience Institute
• Pediatric Neurosurgery, St. Louis Children’s
Hospital
Questions?
CONTACT INFORMATION:
Manish N. Shah, MD
6410 Fannin Street, Suite 950
Houston, TX 77030
Cell: 314-302-4171
Clinic: 832-325-7242
Fax: 713-512-2221
[email protected]