Neurosurgical Therapies for Managing Spasticity in Cerebral Palsy (CP

DR. KAUSTUBHDR. KAUSTUBH
DINDORKARDINDORKAR
NEUROSURGEONNEUROSURGEON

Neurosurgical Therapies for Spasticity (CP )Neurosurgical Therapies for Spasticity (CP )
 Complex problemComplex problem
 Very wide spectrum of presentationVery wide spectrum of presentation
 NSx uncommon in Indian practiceNSx uncommon in Indian practice
 Manifold reasons of less popularityManifold reasons of less popularity
 Need for counseling, education of patients ( caregivers),Need for counseling, education of patients ( caregivers),
physical therapists , pediatricians & orthopedicians.physical therapists , pediatricians & orthopedicians.

WHY TREATWHY TREAT
 Spasticity can be extremely debilitatingSpasticity can be extremely debilitating
and painful.and painful.
 Common treatments for spasticityCommon treatments for spasticity
include physical therapy, medicationsinclude physical therapy, medications
and surgery.and surgery.

WHEN TO TREATWHEN TO TREAT
Spasticity should not be treated just because stiffness is
present.
Most of the time spasticity is useful to assure
safe balance and for compensating loss of motor strength.
With these considerations in mind, spasticity should only be treated
when excess muscular tone leads to further functional losses, impairs
locomotion, or induces deformities, or chronic pain.
Surgery for spasticity should be considered as a second line treatment
after failure of medical therapies (i.e. physical, pharmacological and
Botulinum toxin injections).

MEDICAL TREATMENTMEDICAL TREATMENT
Drugs are sometimes used to control spasticity, particularly followingDrugs are sometimes used to control spasticity, particularly following
surgery.surgery.
The three medications that are used most often are diazepam, whichThe three medications that are used most often are diazepam, which
acts as a general relaxant of the brain and body; baclofen, which blocksacts as a general relaxant of the brain and body; baclofen, which blocks
signals sent from the spinal cord to contract the muscles; andsignals sent from the spinal cord to contract the muscles; and
dantrolene, which interferes with the process of muscle contraction.dantrolene, which interferes with the process of muscle contraction.
Given by mouth, these drugs can reduce spasticity for short periods, butGiven by mouth, these drugs can reduce spasticity for short periods, but
their value for long-term control of spasticity has not been clearlytheir value for long-term control of spasticity has not been clearly
demonstrated.demonstrated.
They may also trigger significant side effects, such as drowsiness, andThey may also trigger significant side effects, such as drowsiness, and
their long-term effects on the developing nervous system are largelytheir long-term effects on the developing nervous system are largely
unknown.unknown.

NeurosurgicalNeurosurgical
treatmentstreatments
 Not sought due to complexities , cost involvedNot sought due to complexities , cost involved
 Useful for patients at both ends of clinical spectrumUseful for patients at both ends of clinical spectrum
 Either for pure spastic diplegia or severe CP withEither for pure spastic diplegia or severe CP with
nursing problemsnursing problems
 Therapies addressed towards ‘managing’ spasticityTherapies addressed towards ‘managing’ spasticity
& dystonia& dystonia

TreatmentsTreatments
 Address the ‘root’ of the problemAddress the ‘root’ of the problem
 Advancements in surgical techniques andAdvancements in surgical techniques and
technologiestechnologies
 Are always complimentary or adjuncts with otherAre always complimentary or adjuncts with other
therapies – PT, Botox injections and orthopedictherapies – PT, Botox injections and orthopedic
surgeriessurgeries

treatmentstreatments
 Three main treatmentsThree main treatments
 Intrathecal Baclofen PumpIntrathecal Baclofen Pump
 Selective Dorsal RhizotomiesSelective Dorsal Rhizotomies
 Deep Brain StimulationDeep Brain Stimulation

BaclofenBaclofen
 Intrathecal BaclofenIntrathecal Baclofen
 Baclofen is a drug that helps reduce spasticity andBaclofen is a drug that helps reduce spasticity and
dystonia.dystonia.
 Taken orally, little Baclofen enters the spinal fluid,Taken orally, little Baclofen enters the spinal fluid,
spinal cord or brain.spinal cord or brain.
 If Baclofen is given directly into the spinal fluid, itIf Baclofen is given directly into the spinal fluid, it
soaks into the spinal cord and is far more effective,soaks into the spinal cord and is far more effective,
with far fewer side effects.with far fewer side effects.

Intrathecal Baclofen TherapyIntrathecal Baclofen Therapy
(ITB)(ITB)
 A programmable pump with a reservoir.A programmable pump with a reservoir.
 A clear, flexible silicone catheter; and a programming device comprise the deliveryA clear, flexible silicone catheter; and a programming device comprise the delivery
system for intrathecal baclofen therapysystem for intrathecal baclofen therapy
 Typically, candidates for ITB therapy have severe spasticity that does not respond toTypically, candidates for ITB therapy have severe spasticity that does not respond to
conservative treatment with medications or have intolerable side effects at therapeuticconservative treatment with medications or have intolerable side effects at therapeutic
doses.doses.
 The system is surgically implanted after the patient has responded favorably to a testThe system is surgically implanted after the patient has responded favorably to a test
dose of the intrathecally delivered medication.dose of the intrathecally delivered medication.
 The pump, which is implanted subdermally, is usually refilled on OPD basis after four- toThe pump, which is implanted subdermally, is usually refilled on OPD basis after four- to
eight-weeks depending on the capacity of the reservoir and the dosage of ITB that iseight-weeks depending on the capacity of the reservoir and the dosage of ITB that is
administered, and typically lasts for five or more years.administered, and typically lasts for five or more years.
 The usual starting dose is 25 μg per day, with titration to efficacy, up to a maximum doseThe usual starting dose is 25 μg per day, with titration to efficacy, up to a maximum dose
of 200+ μg per day.of 200+ μg per day.

ITB ScreeningITB Screening
 The tip of the catheter is generally placed through a lumbar puncture at the level of the conus
medullaris (T12–L1 vertebral levels) for paraplegic patients to modulate the muscular tone in both
inferior limbs.
 A trial of ITB is required before performing the surgical implantation of the pump to check on the
efficacy and absence of side-effects of the method. This test allows the surgeon to define
whether there is an appropriate dosage of intrathecal baclofen suppressing the excess of
spasticity without impairing the useful muscular tone necessary to stand and for ambulatory
patients to walk.
 These tests can be performed via bolus injections of baclofen through lumbar punctures when
just an “on-off” effect is checked. In the absence of a positive response, indicated by a two-point
reduction in Ashworth score 4 to 8 hours following drug administration, the bolus dose is
increased by 25 μg increments up to a maximum bolus of 100–150 μg.
 Once a positive response is observed without unacceptable loss of function, the patient is
considered to be a candidate for pump implantation.

Advantages of ITBAdvantages of ITB
 Simple SurgerySimple Surgery
 TRIAL of efficacy & Titration of doseTRIAL of efficacy & Titration of dose
possiblepossible
 ReversibleReversible
 Targets large muscle groups with relativelyTargets large muscle groups with relatively
small dosessmall doses

Disadvantages of ITBDisadvantages of ITB
 Costly – 2.5 – 3.0 LacsCostly – 2.5 – 3.0 Lacs
 CANNOT be used if ULs also involved dueCANNOT be used if ULs also involved due
to fear of respiratory depressionto fear of respiratory depression
 Drug refills every 6- 8 weeksDrug refills every 6- 8 weeks
 Infection during surgery – waste ofInfection during surgery – waste of
expenditureexpenditure

Selective Dorsal Rhizotomy (SDR)Selective Dorsal Rhizotomy (SDR)
 Selective RhizotomySelective Rhizotomy
 A rhizotomy is an operation in which a nerve or part of a nerve is intentionallyA rhizotomy is an operation in which a nerve or part of a nerve is intentionally
cut.cut.
 Lumbar rhizotomies are operations on the lower back to partially divide nervesLumbar rhizotomies are operations on the lower back to partially divide nerves
from the legs.from the legs.
 Selective lumbar rhizotomies are operations in which the neurosurgeonSelective lumbar rhizotomies are operations in which the neurosurgeon
divides the various nerves coming into the spine from the legs into severaldivides the various nerves coming into the spine from the legs into several
branches, tests each branch with an electrical stimulus, then cuts thebranches, tests each branch with an electrical stimulus, then cuts the
branches which give abnormal responses.branches which give abnormal responses.
 Debate as to whether selective lumbar rhizotomies give better results thanDebate as to whether selective lumbar rhizotomies give better results than
non - selective rhizotomies.non - selective rhizotomies.

AIM OF SDRAIM OF SDR
 The reason a child undergoes a selective
dorsal rhizotomy (SDR) is either to make a
physical therapist’s efforts more successful
by normalizing the muscle tone in an
extremity or to ease the burden of care
takers by eliminating spasticity that
complicates dressing, bathing, toileting and
positioning.

DECISION-MAKING
 The surgeon and therapist can then discuss these goals with
the family.
 Also important is an understanding of what type and amount of therapy
will be available for the child after the SDR.
 This surgery only decreases muscle tone. It does nothing to the
functioning of the targeted limb.
 In fact, it is not uncommon for a limb to transiently deteriorate in its
function after a SDR.
 It is therefore extremely important that the child have therapy
after a SDR.
 The surgery should not be done if therapy will not be available for the
child after the SDR.

DECISION CONCEPTS IN SDRDECISION CONCEPTS IN SDR
 Selective dorsal rhizotomy (SDR) only treats spasticity.
 If employed on a child with either dystonic cerebral palsy or
mixed cerebral palsy, there will be a treatment failure within
several years of the surgery.
 Consequently for these types of cerebral palsy (CP)
intrathecal baclofen is favored over SDR.
 Key to successfully using SDR on children with cerebral palsy
is knowledge on how to perform a good tone examination.
 First, a good history is taken.

DECISION FOR SDRDECISION FOR SDR
 Spastic children typically have a history of being born around 30 weeks
gestation.
 If the child was born at term, the overwhelming probability is that the child
does not have spasticity or has mixed cerebral palsy and will not be a good
candidate for an SDR.
 During the history taking, time is spent observing the child sitting in its
parent’s arms relaxed.
 If choreoathetoid or writhing finger movements are noted the child has either
dystonic CP or mixed CP and is not a good candidate for SDR. Similarly, if
the child cannot maintain an erect posture, i.e., has the so called floppy trunk,
then the child is not purely spastic and is not a good candidate for SDR

ExaminationExamination
 Observational gait analysis is very important if the child is ambulatory.
This part of the exam can precede the formal tone examination
 Typical features of a spastic gait pattern is persisting flexion at the hips with an
associated hyperlordosis, inward rotation of the hip joints and scissoring of the
legs (hyper-adduction of the hips with a resulting crossing of the advancing limb
in front of the limb in stance phase).
 The latter abnormality can be of such a severity that repetitive limb
advancement is blocked.
 At the knees there is difficulty with extension due to hamstring spasticity. This
results in a crouched gait with shortened stride length.
 At the ankles there is an equinovalgus deformity (heel is elevated off the
ground and rotated outwards at foot strike and during stance phase).
 When these deformities are present and there is good tone in the trunk and no
writhing in the fingers, then it can be assumed that pure spasticity is present.

Screening/Selection CriteriaScreening/Selection Criteria for SDRfor SDR
 Candidates for a rhizotomy are usually young (four to eightCandidates for a rhizotomy are usually young (four to eight
years old)years old)
 have relatively good leg strength, and do not have severe leghave relatively good leg strength, and do not have severe leg
contractures.contractures.
 The primary goal of surgery is often to improve walking.The primary goal of surgery is often to improve walking.
 Rhizotomy can be done at any age to facilitate care.Rhizotomy can be done at any age to facilitate care.
 Rhizotomies will relieve the spasticity but will not improveRhizotomies will relieve the spasticity but will not improve
contractures (shortening of muscles and tendons) that arecontractures (shortening of muscles and tendons) that are
already present, nor will they improve dystonia.already present, nor will they improve dystonia.

SurgerySurgery
 Rhizotomy surgery generally lasts about two to three hours.Rhizotomy surgery generally lasts about two to three hours.
 The procedure involves a midline incision about 3-4 inches long in theThe procedure involves a midline incision about 3-4 inches long in the
lumbar region. Muscles are separated away from the spine and the nervelumbar region. Muscles are separated away from the spine and the nerve
roots coming and going to the legs are exposed.roots coming and going to the legs are exposed.
 Each nerve root divided into 3-5 branches and is tested with specialEach nerve root divided into 3-5 branches and is tested with special
monitoring equipment to identify nerves that give abnormal responsesmonitoring equipment to identify nerves that give abnormal responses
when they are electrically stimulated.when they are electrically stimulated.
 The nerve roots that give abnormal responses are cut; usually 50- 60% ofThe nerve roots that give abnormal responses are cut; usually 50- 60% of
the top half of each nerve is divided.the top half of each nerve is divided.

SURGERYSURGERY
 At the time of the operation, theAt the time of the operation, the
neurosurgeon divides each of theneurosurgeon divides each of the
dorsal roots into 3-5 rootlets anddorsal roots into 3-5 rootlets and
stimulates each rootlet electrically.stimulates each rootlet electrically.
 By examiningBy examining electromyographicelectromyographic
(EMG) responses from muscles in(EMG) responses from muscles in
the lower extremities, the surgicalthe lower extremities, the surgical
team identifies the rootlets thatteam identifies the rootlets that
cause spasticity.cause spasticity.
 The abnormal rootlets areThe abnormal rootlets are
selectively cut, leaving the normalselectively cut, leaving the normal
rootlets intact.rootlets intact.
 This reduces messages from theThis reduces messages from the
muscle, resulting in a better balancemuscle, resulting in a better balance
of activities of nerve cells in theof activities of nerve cells in the
spinal cord, and thus reducesspinal cord, and thus reduces
spasticity.spasticity.

SURGERYSURGERY
After the sensory nerves are
exposed, each sensory nerve root is
divided into 3-5 rootlets.
Each rootlet is tested with EMG,
which records electrical patterns in
muscles. Rootlets are ranked from 1
(mild) to 4 (severe) for spasticity.
The severely abnormal rootlets are
cut. This technique is repeated for
rootlets between spinal nerves L2
and S2.
Half of the L1 dorsal root fibers are
cut without EMG testing.

Problems that arise after a SDR
 First, the family should be warned that the first few days will be
marked by the child being in severe pain.
 This is due to the fact that these children have hyperactive muscle reflex
circuits that are responsive to pain.
 The pain will cause their back’s musculature to tighten in spasm and this is
typically of such a degree as to render nearly all analgesics inadequate.
 Judicious use of muscle relaxants can break this pain–spasm
cycle, rendering the analgesics more effective.
 There is an increased incidence of urinary tract dysfunction in children with
cerebral palsy.
 2–4% of patients will experience a subdermatomal sensory loss.
 40% of children undergoing an SDR will experience dysesthesia in their lower
legs

ComplicationsComplications
 The dorsal rhizotomy is a long and complex neurosurgical procedure.The dorsal rhizotomy is a long and complex neurosurgical procedure.
 As in other major neurosurgical procedures, it presents some risks.As in other major neurosurgical procedures, it presents some risks.
Paralysis of the legs and bladder, impotence, and sensory loss areParalysis of the legs and bladder, impotence, and sensory loss are
the most serious complications.the most serious complications.
 Wound infection and meningitis are also possible, but they areWound infection and meningitis are also possible, but they are
usually controlled with antibiotics.usually controlled with antibiotics.
 Leakage of the spinal fluid through the wound is another risk.Leakage of the spinal fluid through the wound is another risk.
 Abnormal sensitivity of the skin on the feet and legs is relativelyAbnormal sensitivity of the skin on the feet and legs is relatively
common after SDR, but usually resolves within 6 weeks.common after SDR, but usually resolves within 6 weeks.
 There is no way to prevent the abnormal sensitivity in the feet.There is no way to prevent the abnormal sensitivity in the feet.
 Transient change in bladder control may occur, but this also resolvesTransient change in bladder control may occur, but this also resolves
within a few weekswithin a few weeks

Advantages Of SRZAdvantages Of SRZ
 Done in a properly selected patientDone in a properly selected patient
can be of great benefitcan be of great benefit
 Long term treatmentLong term treatment
 Can facilitate good response forCan facilitate good response for
therapytherapy

Disadvantages of SRZDisadvantages of SRZ
 Complex surgery – intra-op EMGComplex surgery – intra-op EMG
 Intra –op problems due to prolongedIntra –op problems due to prolonged
anesthesiaanesthesia
 Immediate post op problems of pain ,Immediate post op problems of pain ,
weakness, urinary retention.weakness, urinary retention.
 Long term follow up needed to rule outLong term follow up needed to rule out
development of back problems - listhesis,development of back problems - listhesis,
chronic back pain etcchronic back pain etc

Myths/FactsMyths/Facts
 MYTH: Selective rhizotomy is usually permanent but the effects sometimesMYTH: Selective rhizotomy is usually permanent but the effects sometimes
wear off.wear off.
FACT: Whenever children get significantly tighter a few months or yearsFACT: Whenever children get significantly tighter a few months or years
after rhizotomy, it is almost always because they have dystonia (which isafter rhizotomy, it is almost always because they have dystonia (which is
not improved by rhizotomy) rather than because their spasticity hasnot improved by rhizotomy) rather than because their spasticity has
returned.returned.
 MYTH: Rhizotomies have a high complication rate.MYTH: Rhizotomies have a high complication rate.
FACT: The complication rate is surprisingly low: 5-10%, lower than the rateFACT: The complication rate is surprisingly low: 5-10%, lower than the rate
of complications for insertion of baclofen pumps.of complications for insertion of baclofen pumps.

Deep Brain StimulationDeep Brain Stimulation
 Deep brain stimulation (DBS) is a method of treating dystonia and tremorDeep brain stimulation (DBS) is a method of treating dystonia and tremor
involving an operation in which thin blunt wires (electrodes) are surgicallyinvolving an operation in which thin blunt wires (electrodes) are surgically
implanted precisely into a small area deep in the brain.( Pallidal DBS )implanted precisely into a small area deep in the brain.( Pallidal DBS )
 If the abnormal movement affects one side of the body, one electrode is insertedIf the abnormal movement affects one side of the body, one electrode is inserted
(on the opposite side of the brain than the body is affected).(on the opposite side of the brain than the body is affected).
 If both sides of the body are affected, bilateral (both sides) electrodes areIf both sides of the body are affected, bilateral (both sides) electrodes are
inserted.inserted.
 The electrodes are tunneled under the skin down the neck and are connected toThe electrodes are tunneled under the skin down the neck and are connected to
an electrical stimulator unit than can be programmed with a computer to stimulatean electrical stimulator unit than can be programmed with a computer to stimulate
the area of the brain at the tip of the electrode.the area of the brain at the tip of the electrode.
 The idea behind DBS is that fast electrical stimulation (130 times a second)The idea behind DBS is that fast electrical stimulation (130 times a second)
interrupts the abnormal electrical circuit within the brain that is causing theinterrupts the abnormal electrical circuit within the brain that is causing the
abnormal movements.abnormal movements.

DBSDBS
 Target selection is vitalTarget selection is vital
 MRI guided surgeryMRI guided surgery
 Cost of implants 3- 4 Lacs for each sideCost of implants 3- 4 Lacs for each side
 Problems of surgery, anesthesia, infection andProblems of surgery, anesthesia, infection and
neuromodulation need to addressedneuromodulation need to addressed
 Experience with DBS is lessExperience with DBS is less

THANK YOUTHANK YOU
 Combined effort of parents , therapists, orthopedicians ,Combined effort of parents , therapists, orthopedicians ,
pediatricians & neurosurgeons.pediatricians & neurosurgeons.
 Team effortTeam effort
 Need for extensive counselingNeed for extensive counseling
 Treatment is an ongoing process , so strategic planning ofTreatment is an ongoing process , so strategic planning of
goals (physical, emotional and financial) is necessarygoals (physical, emotional and financial) is necessary
 Newer treatments should be offered only for patients fulfillingNewer treatments should be offered only for patients fulfilling
strict selection criteriastrict selection criteria

Neurosurgical Therapies for Managing Spasticity in Cerebral Palsy (CP

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