DR. KAUSTUBH DR. 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 debilitating Spasticity can be extremely debilitating and painful. and painful.

Common treatments for spasticity Common treatments for spasticity include physical therapy, medications include physical therapy, medications and surgery. and surgery.

WHEN TO TREATWHEN TO TREATSpasticity should not be treated just because stiffness is present.

Most of the time spasticity is useful to assuresafe 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 following Drugs are sometimes used to control spasticity, particularly following surgery. surgery.

The three medications that are used most often are diazepam, which The three medications that are used most often are diazepam, which acts as a general relaxant of the brain and body; baclofen, which blocks acts as a general relaxant of the brain and body; baclofen, which blocks signals sent from the spinal cord to contract the muscles; and signals 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, but Given by mouth, these drugs can reduce spasticity for short periods, but their value for long-term control of spasticity has not been clearly their value for long-term control of spasticity has not been clearly demonstrated. demonstrated.

They may also trigger significant side effects, such as drowsiness, and They may also trigger significant side effects, such as drowsiness, and their long-term effects on the developing nervous system are largely their long-term effects on the developing nervous system are largely unknown. unknown.

Neurosurgical Neurosurgical 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 with Either for pure spastic diplegia or severe CP with

nursing problemsnursing problems Therapies addressed towards ‘managing’ spasticity Therapies addressed towards ‘managing’ spasticity

& dystonia & dystonia

Neurosurgical Neurosurgical TreatmentsTreatments Address the ‘root’ of the problemAddress the ‘root’ of the problem

Advancements in surgical techniques and Advancements in surgical techniques and

technologiestechnologies

Are always complimentary or adjuncts with other Are always complimentary or adjuncts with other

therapies – PT, Botox injections and orthopedic therapies – PT, Botox injections and orthopedic

surgeries surgeries

Neurosurgical Neurosurgical 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 and Baclofen 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, it If 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 Therapy Intrathecal 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 delivery A clear, flexible silicone catheter; and a programming device comprise the delivery system for intrathecal baclofen therapy system for intrathecal baclofen therapy

Typically, candidates for ITB therapy have severe spasticity that does not respond to Typically, candidates for ITB therapy have severe spasticity that does not respond to conservative treatment with medications or have intolerable side effects at therapeutic conservative 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 test The 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- to The 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 is eight-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 dose The 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.

ITBITB

Advantages of ITBAdvantages of ITB

Simple SurgerySimple Surgery

TRIAL of efficacy & Titration of dose TRIAL of efficacy & Titration of dose possiblepossible

ReversibleReversible

Targets large muscle groups with relatively Targets large muscle groups with relatively small doses small doses

Disadvantages of ITBDisadvantages of ITB

Costly – 2.5 – 3.0 LacsCostly – 2.5 – 3.0 Lacs

CANNOT be used if ULs also involved due CANNOT be used if ULs also involved due to fear of respiratory depressionto fear of respiratory depression

Drug refills every 6- 8 weeks Drug refills every 6- 8 weeks

Infection during surgery – waste of Infection 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 intentionally A 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 nerves Lumbar 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 neurosurgeon Selective lumbar rhizotomies are operations in which the neurosurgeon divides the various nerves coming into the spine from the legs into several divides the various nerves coming into the spine from the legs into several branches, tests each branch with an electrical stimulus, then cuts the branches, 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 than Debate 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 eight Candidates for a rhizotomy are usually young (four to eight years old)years old)

have relatively good leg strength, and do not have severe leg have 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 improve Rhizotomies will relieve the spasticity but will not improve contractures (shortening of muscles and tendons) that are contractures (shortening of muscles and tendons) that are already present, nor will they improve dystonia. already present, nor will they improve dystonia.

AIMSAIMS

Surgery Surgery

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 the The procedure involves a midline incision about 3-4 inches long in the lumbar region. Muscles are separated away from the spine and the nerve lumbar 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 special Each nerve root divided into 3-5 branches and is tested with special monitoring equipment to identify nerves that give abnormal responses monitoring 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% of The 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, the At the time of the operation, the

neurosurgeon divides each of the neurosurgeon divides each of the dorsal roots into 3-5 rootlets and dorsal roots into 3-5 rootlets and stimulates each rootlet electrically.stimulates each rootlet electrically.

By examining By examining electromyographicelectromyographic (EMG) responses from muscles in (EMG) responses from muscles in the lower extremities, the surgical the lower extremities, the surgical team identifies the rootlets that team identifies the rootlets that cause spasticity. cause spasticity.

The abnormal rootlets are The abnormal rootlets are selectively cut, leaving the normal selectively cut, leaving the normal rootlets intact.rootlets intact.

This reduces messages from the This reduces messages from the muscle, resulting in a better balance muscle, resulting in a better balance of activities of nerve cells in the of activities of nerve cells in the spinal cord, and thus reduces spinal 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 are Paralysis 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 are Wound 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 relatively Abnormal 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 resolves Transient 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 patient Done in a properly selected patient can be of great benefitcan be of great benefit

Long term treatmentLong term treatment

Can facilitate good response for Can facilitate good response for therapy therapy

Disadvantages of SRZDisadvantages of SRZ Complex surgery – intra-op EMGComplex surgery – intra-op EMG

Intra –op problems due to prolonged Intra –op problems due to prolonged anesthesia anesthesia

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 out Long 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/Facts Myths/Facts

MYTH: Selective rhizotomy is usually permanent but the effects sometimes MYTH: Selective rhizotomy is usually permanent but the effects sometimes wear off.wear off.

FACT: Whenever children get significantly tighter a few months or years FACT: Whenever children get significantly tighter a few months or years after rhizotomy, it is almost always because they have dystonia (which is after rhizotomy, it is almost always because they have dystonia (which is not improved by rhizotomy) rather than because their spasticity has not 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 rate FACT: 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 Stimulation Deep Brain Stimulation

Deep brain stimulation (DBS) is a method of treating dystonia and tremor Deep brain stimulation (DBS) is a method of treating dystonia and tremor involving an operation in which thin blunt wires (electrodes) are surgically involving 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 inserted If 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 are If 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 to The 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 stimulate an 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 the interrupts the abnormal electrical circuit within the brain that is causing the abnormal movements. abnormal movements.

DBS DBS 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 and Problems 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 of Treatment 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 fulfilling Newer treatments should be offered only for patients fulfilling strict selection criteriastrict selection criteria