Spasticity in children

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Journal of Taibah University Medical Sciences (2012) xxx, xxx–xxx

JTUMED 22 No. of Pages 8

26 December 2012

Taibah University

Journal of Taibah University Medical Sciences

www.jtaibahunimedsc.netwww.sciencedirect.com

Editorial

Spasticity in children

Yasser Awaad, MD *, Tamer Rizk, MD

National Neurosciences Institute, King Fahad Medical City, Riyadh, Kingdom of Saudi Arabia

Received 1 July 2012; revised 12 November 2012; accepted 28 November 2012

Keywords

Baclofen;

Cerebral palsy;

Management;

Pediatric;

Spasticity

* Corresponding author. Addr

National Neurosciences Institu

59046, Riyadh 11525, Kingd

2889999x1423; fax: +966 1 28

E-mail address: yawaad@kfm

Peer review under responsibilit

Production an

1658-3612 ª 2012 Taibah Univ

http://dx.doi.org/10.1016/j.jtum

Please cite this article in p(2012), http://dx.doi.org/10

ess:Depa

te, King F

om of S

89999x13

c.edu.sa

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d hostin

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ress as:.1016/j.

Abstract Spasticity is a wide spectrum disease that affects all age groups. Spasticity in children

may have diverse etiologies; this article will focus on children with spasticity, most of whom have

diagnoses of cerebral palsy; as approximately two thirds of all cerebral palsy patients suffer from

spasticity. This review is meant to discuss various aspects of spasticity; nomenclature over succes-

sive decades, clinical manifestations; including clinical examination tips, etiology, pathogenesis and

pathophysiology were also high lightened. We are discussing well established, as well as other recent

measures of management spasticity in view of diagnostic, as well as therapeutic approaches; includ-

ing two clinical vignettes.ª 2012 Taibah University. Production and hosting by Elsevier Ltd. All rights reserved.

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Introduction

Spasticity may result from either diffuse or localized pathology

of the cerebral cortex, brain stem, or spinal cord; Cerebralpalsy is considered a main cause of spasticity in pediatric agegroup as 2/3 of all cerebral palsy patients suffer from spastic-

ity. Management of spasticity is challenging as it is importantto evaluate the advantages and disadvantages that the patientsgain from their spasticity so that treatment strategies and goalscan be identified. Prenatal care during pregnancy can avoid

premature labor; prevent cerebral palsy in infants with itsresulting spasticity. Despite major updates and innovations

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rtment of Pediatric Neurology,

ahad Medical City, P.O. Box

audi Arabia. Tel.: +966 1

91.

(Y. Awaad).

ah University.

g by Elsevier

roduction and hosting by Elsevier

12.004

Awaad Y., Rizk T. Spasticityjtumed.2012.12.004

in the field of treatment of spasticity, there is no specific diag-nostic test for spasticity.

Historical note and nomenclature

Treatment for spasticity was documented as early as the late

19th century, when surgeons Abbe and Bennet discussed thedecreasing tone in a spastic limb through sensory rhizotomies.Later, in 1898, the scientist Sherrington published experimentsin which the sensory roots of spastic cats were severed to re-

lieve spasticity. 2 The technique of sensory rhizotomies hasbeen improved on and continues to be used today as a treat-ment for patients with spasticity as does neuromuscular block-

age, a longstanding treatment, which has been used for over30 years.36

Clinical manifestations

Spasticity was defined by Lance as a ‘‘velocity-dependent in-

crease in tonic stretch reflexes (muscle tone) with exaggeratedtendon jerks, resulting from hyperexcitability of the stretch re-flex’’.57,25 Young further added characteristics of positive andnegative symptoms. Positive symptoms consist of exaggerated

Ltd. All rights reserved.

in children. Journal of Taibah University Medical Sciences

mailto:[email protected]

http://dx.doi.org/10.1016/j.jtumed.2012.12.004


http://www.sciencedirect.com/science/journal/16583612



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2 Y. Awaad, T. Rizk


26 December 2012

cutaneous reflexes, including nociceptive and flexor with-

drawal reflexes, autonomic hyperreflexia, dystonia, and con-tractures. Negative symptoms include paresis, lack ofdexterity, and fatigability.57

Although there are many possible causes of spasticity, this

article will focus on children with spasticity, most of whomhave diagnoses of cerebral palsy; approximately two thirdsof all cerebral palsy patients suffer from spasticity.5 A patient

with spastic cerebral palsy presents with muscle imbalance,stands with bent knees and legs tightly together, and in severecases, a scissors-type gait.26,3 The antigravity muscles are

predominantly affected with arms in a flexed and pronatedposition and legs in an extended and adducted position. Whenthe muscles are at rest they are flaccid to palpation and electro-

myographically silent.Examination should begin with the patient in a relaxed,

lying position with the head up and arms resting to the sidesbecause it is easier to determine the extent of spasticity in this

position. The examination should include tonic stretch reflexesby manual passive stretches, elicitation of tendon jerks and clo-nus in a relaxed position, and tonic and phasic stretch reflexes

carried out in a sitting position.The manual passive stretch maneuver is used to assess resis-

tance at different rates. A joint is passively moved while the

muscles corresponding to that joint are lengthened and short-ened. In cases of mild spasticity, the muscles will only resistwhen stretched at a high rate, whereas in cases of moderatespasticity, resistance is noticed at a slower rate and the clasp-

knife phenomenon may be exhibited. Movement of the musclemay be difficult to impossible in cases of severe spasticity.19

The muscle tone is graded according to the Ashworth scale,

a scale ranging from 1 to 5, in which resistance to the passivemuscle stretch is measured at various velocities.5

Tendon jerks are easier to elicit in spastic patients than in

patients with normal muscle tone, and reflex responses canbe achieved in muscles without well-defined tendons. Percus-sion of the tendon reveals hyperactive tendon jerks, especially

for the Achilles, patellar, biceps, and triceps tendons.58 OnEMG, the jerks show greater amplitudes than are normaland are followed by after-discharge of the motor units that isoften slightly longer lasting than normal. The size of tendon

jerks can be measured by either EMG response or by record-ings of mechanical events.

H-reflex (Hoffmann’s reflex) studies are electrically elicited

tendon jerks and are restricted mostly to the soleus and flexorcarpi radialis muscles in normal adults. In cases of upper mo-tor neuron lesions, the H-reflex may be elicited in muscles

where it is not normally seen, such as the intrinsic hand mus-cles, tibialis anterior, or peroneal muscles.58

Measurement of resistance to passive stretch, reduction in

the tonic vibration reflex, and reduction of the plantarwithdrawal reflex should also be evaluated.Motoneuronal over-activity should also be evaluated because any input tomotoneu-rons produces excessive and prolonged activity that can be

observed in the contractions of many limb muscles.58

The amount of function the patient derives from spasticitycan be evaluated by having the patient obtain and maintain

standing and seated positions. To determine the degree to whichthe hamstring tone is affecting the alignment of the pelvis andknees, have the child sit with feet straight in front. The patient

can sit in a chair to allow the examiner to assess trunk control.The side sit position exhibits a patient’s ability to maintain con-

Please cite this article in press as: Awaad Y., Rizk T. Spasticity(2012), http://dx.doi.org/10.1016/j.jtumed.2012.12.004

trol in an asymmetric position.1 Video cameras are often helpful

during evaluation as the patient’s movements can be recordedand compared against movements during and after treatment.

It is important to evaluate the advantages and disadvan-tages that the patient gains from their spasticity so that treat-

ment strategies and goals can be identified. Disadvantages mayinclude interference with activities of daily living, inhibition ofgood sleep, contractures, dislocations, skin breakdown, bowl

and bladder dysfunction, impairment of respiratory function,pain with stretching, and the masking of the return of volun-tary movement. However, patients may rely on a certain

amount of spasticity to function and the advantages theymay receive include maintaining muscle tone, supporting circu-latory function, assisting in activities of daily living, and pre-

venting the formation of deep vein thrombosis.

Clinical vignette

Patient A was a 5-year-old African–American boy with a his-tory of developmental delay and a diagnosis of cerebral palsyof the spastic-diplegic type. He first presented at 18 months

with severe spasticity in both lower extremities. The patientwalked on tip toes and had hip and knee flexion. There wassome scissoring of the legs. On examination, exaggerated deep

tendon reflexes were elicited, as were sustained clonus andbilateral Babinski sign. MRI of the brain showed findings thatmay be secondary to previous hypoxic injury, compatible with

cerebral palsy. Prior treatments included physical therapy,bilateral ankle-foot orthosis, serial casting, and oral baclofen.Before treatment with botulinum toxin injections, the patientwas a tip-toe walker. With treatment the patient’s gait has im-

proved; he is flat-footed and presently wears bilateral ankle-foot orthosis. His hygiene and positioning have also improvedand he returns every 6 months to 9 months for reinjection.

Patient B was a 7-year-old African–American boy with ahistory of cerebral palsy of the spastic-diplegic type. On pri-mary examination he presented with tightness of both ham-

strings and heel cords with the right more involved than theleft. The patient had good toe standing, especially on the rightside and good sitting balance with a kyphotic sacral-type sitting

due to the tight hamstring. He uses a walker to ambulate andwalks on tip toes. The EEG was abnormal, indicating the pres-ence of epileptiform activity from the left central parietal headregion and diffuse background disorganization, which indi-

cates underlying neuronal dysfunction. Treatments beforeintrathecal baclofen pump implantation included bilateral an-kle-foot orthoses, tendon releases, alcohol block, and botu-

linum toxin injections. Before treatment with intrathecalbaclofen the patient was dependent on a care giver and useda walker to ambulate. With the intrathecal baclofen pump

the patient has gained function, does not use a walker to ambu-late, and performs activities of daily living independently.

Etiology

Spasticity may result from either diffuse or localized pathology

of the cerebral cortex, brain stem, or spinal cord. Possiblecauses of such injuries include cerebral palsy, traumatic braininjury, stroke, multiple sclerosis, spinal cord trauma, or diseaseand anoxic insults. The neurologic localization of the lesion

causing spasticity may result in different clinical manifesta-



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Spasticity in children 3


26 December 2012

tions. Thus, it is important to consider whether the spasticity

results from cerebral pathology, whether it is diffuse or local-ized, or whether it is a result of spinal cord injury. Diffuse cere-bral injury or diseases would include anoxia, toxic, ormetabolic encephalopathies, whereas localized cerebral injury

would include tumor, abscess, cyst, arteriovenous malforma-tions, hemorrhage, or trauma. Spinal cord injury or diseasemay result as an insult to descending pathways by trauma,

inflammatory or demyelinating disease, degenerative disorders,or compression such as is caused by a tumor or cyst.19,26,5

Common causes of cerebral palsy in children that may result

in spasticity are prolonged second stage labor, fetal distress,cystic degeneration of the brain, prematurity, periventricularencephalomalacia, cortical abnormalities such as porenceph-

aly, or congenital malformations of gyri such as micropolygy-ria. The annual incidence of spinal cord injuries in the UnitedStates is estimated to be 30–40 new cases per million individu-als. About 3–5% of cases each year occur in children younger

than 15 years of age.45 The male-to-female ratio of patients is4:1 in the general population, but in younger age groups, theration is approximately 1.5:1.59

Pathogenesis and pathophysiology

There are many different types of spasticity. Because of this,more than one mechanism may be responsible for the distur-bance in muscle tone and the mechanisms may vary between

patients. The neuropathophysiologic processes involved inspasticity are complex and not fully understood, but there isa widely accepted hypothesis that spasticity depends on hyper-excitability of spinal alpha motor neurons, which is due to the

interruption of descending modulatory influences carried bythe corticospinal, vestibulospinal, and reticulospinal tractsand other possible tracts.24 Ia afferent fibers provide segmental

input from muscle spindles to alpha motor neuron pools. Theysynapse on segmental inhibitory interneurons that then inhibitalpha motor neurons innervating antagonist muscles in the Ia

reciprocal inhibition pathway. Ib afferents inhibit alpha motorneurons by way of the Golgi tendon organs via the Ib inhibi-tory interneuron in another pathway known as nonreciprocal

inhibition.57,24 Increased excitation of these afferents doesnot seem to be the cause of spasticity. Instead, evidence sup-ports that reduced reciprocal inhibition of antagonist motorneuron pools by Ia afferents, decreased presynaptic inhibition

of Ia afferents, and decreased nonreciprocal inhibition by Ibterminals are all possible pathophysiologic mechanisms ofspasticity.57 The pathophysiology of traumatic brain injury in-

volves a complex combination of forces that has been a subjectof substantial debate.21 On occasion, autonomic dysreflexiamay occur after an intramuscular injection, although this is rel-

atively rare.49 In some patients, autonomic dysreflexia may oc-cur even if the level of spinal injury is below T6.7,37 The use ofantihypertensive pharmacologic agents in treating spasticity is

unclear because randomized trials have not been performed.Nifedipine has been used in a bit-and-swallow technique; morerecently, captopril also has been found to be of benefit.22

Epidemiology

As stated before, spasticity is present in about two thirds of

cerebral palsy patients, and cerebral palsy affects anywhere


from 1.5 to 2.5 per 1000 live births in the United States.3

The number of spastic patients continues to increase due toan increased survival rate of premature births. Males and fe-males are equally affected.

Prevention

To prevent cerebral palsy; due to preterm labour; and, thus,

the resulting spasticity, it is important that mothers receiveprenatal care during pregnancy, that measures are taken toavoid premature labor, and that special consideration is given

to pregnancies involving multiple gestations. Early detectionand treatment of other causes of cerebral palsy, e.g. neurode-generative diseases may prevent the development of spasticity

as well as detect the underlying diseases that could result inbrain injury. If children have conditions that make them sus-ceptible to brain or spinal cord injury or both, safety measures

should be taken (i.e., helmets for patients who have frequentseizures).

Differential diagnosis

Spasticity can be confused with rigidity when a patient is beingevaluated. Stretching can distinguish rigidity from spasticity.

Rigidity will relax through repeated stretching of a muscle,whereas a spastic muscle will continue to increase in resistanceas the velocity of the stretch is increased.19,57

Diagnostic workup

There is no specific diagnostic test for spasticity. Testing can bedone to establish the presence of any lesions or brain or spinalcord injury. MRI of the brain can be performed to rule out

periventricular leukomalacia. A baseline EEG to establishunderlying seizure activity can also be done as well as basiclab studies. Neurophysiological studies, such as the H-reflexstudy, may be performed in patients with neurodegenerative

disease; an enzymatic assay should also be performed.

Prognosis and complications

Spasticity results in limited functional capacity and increasedinactivity. The sequelae of this inactivity may include decubiti,

cardiovascular problems, thrombophlebitis, respiratory infec-tions, fixed contractures, osteoporosis,33 bladder and bowelproblems, and social isolation. Ultimately, these consequences

of inactivity may lead to a further decrease in strength andfunction.25 The patient’s quality of life may be compromisedas spasticity has negative impacts on mobility, hygiene, self

care, sleeping patterns, self esteem, mood, and sexual function.

Management

Traditional treatments for spasticity include physical andoccupational therapy, in which the patient is stretched any-where from once daily to several times per day, but this has

only a limited effect on the patient’s spasticity. Rehabilitationtreatment options include casting, orthotics or splints,strengthening, electrical stimulation, practice of functional

tasks, sensory integration; muscle stretching, and targetedmuscle training.23 Within the scope of pediatric neurorehabil-



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4 Y. Awaad, T. Rizk


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itation, distinct diseases can produce specific complications.

These complications; however, can also occur in associationwith many disorders. For example, spasticity from injury tothe upper motor neuron unit can develop in many neurologicdisorders in children. Several of these complications, such as

autonomic dysreflexia, deep vein thrombosis, and heterotropicossification, can be severe and potentially life-threatening(Lazar, 1998; Umphred, 2001).20,18

Oral medications can be used to decrease spasticity; how-ever, many have unwanted side effects such as drowsiness, seda-tion, confusion, and fatigue. Benzodiazepines, such as

diazepam, are rarely used because of their strong sedating ef-fects. They result in enhanced presynaptic inhibition, but be-cause they are presumed to enhance the postsynaptic effects

of GABA, they can only work if the GABA-mediated processfunctions. Benzodiazepines have a long half-life and an activemetabolite. Benzodiazepine therapy is indicated in spinal cordinjury and multiple sclerosis with possible application in trau-

matic brain injury, cerebral palsy, and cerebrovascular acci-dent. Clinical effects include sedation and reduced anxiety,decreased resistance to passive range of motion, decreased

hyperreflexia, and reduction in painful spasms. Side effects ofall benzodiazepines include sedation, weakness, hypotension,gastrointestinal symptoms, memory impairment, incoordina-

tion, confusion, depression, and ataxia. Also, benzodiazepinesare controlled substances with the potential for dependency.Diazepam is the most widely used benzodiazepine for spasticitymanagement. The recommended initial dose is 250 mcg/kg

three times daily with a maximum dose of 60 mg daily (20 mgthree times daily). If nocturnal spasticity is the presenting prob-lem the patient should be started with a single dose at night.

Like benzodiazepines, baclofen works centrally. Baclofenbinds with GABA-B receptors on brain and spinal membranes,restricting calcium influx into presynaptic nerve terminals,

thereby reducing spasticity.25 The use of baclofen is indicatedwhen spasticity is of spinal origin. The clinical effects includedecreased resistance to passive range of motion, decrease in

hyperreflexia, and reduction in painful spasms and clonus.Unlike benzodiazepines and baclofen, dantrolene sodium

works peripherally at the level of the muscle fiber. It has no ef-fect on neuromuscular transmission, but works by acting di-

rectly on the skeletal muscle, hindering the release of calciumfrom the sarcoplasmic reticulum, thereby preventing the exci-tation-contraction coupling mechanism. This affects both

intrafusal and extrafusal fibers by decreasing the force of mus-cle contraction. However, this mechanism is not selective formuscles with increased tone, and the resulting generalized mus-

cle weakness may weaken respiratory muscles. The use of dan-trolene sodium is indicated in treating spasticity secondary tocerebrovascular accident, cerebral palsy, and has possible

applications for traumatic brain injury, spinal cord injury,and multiple sclerosis. Clinical effects of dantrolene sodium in-clude decreased resistance to passive range of motion, decreasein hyperreflexia and tone, and reduction in spasms and clonus.

Another group of oral medications used in spasticity man-agement includes clonidine and tizanidine, which are alpha2,nonadrenergic receptor agonists that release excitatory neuro-

transmitters and inhibit supraspinal facilitatory pathways.57,25

Tizanidine is an oral antispasticity agent that is selective indecreasing tone and spasm frequency in only spastic muscles,

eliminating the unwanted side effect of generalized muscleweakness. Tizanidine is reported to have reduced symptoms


of spasticity in patients with multiple sclerosis or spinal cord

injury and is well tolerated in most patients. It is an imidazo-line derivative similar to clonidine but without the cardiovas-cular effects when appropriately titrated. Tizanidine resultsin a direct reduction of excitatory amino acid release from

spinal interneurons and inhibits facilitatory caerulospinalpathways. Its peak effect occurs 1–2 h following administra-tion and its half-life is 2.5 h. The clinical effects of tizanidine

include reduced muscle tone, spasm frequency, and hyperre-flexia. Animal studies with tizanidine demonstrate antinocicep-tive activity under specific conditions with increased dose

titration.17,14,55,39 As with other antispasticity medications,the potential side effects of tizanidine are dose related andmay be mitigated by dosage titration. The potential side effects

include drowsiness, dry mouth, and dizziness. Literature sug-gests that tizanidine may be better tolerated than other anti-spasticity agents as measured by the global tolerance ratingscale.38 In placebo-controlled studies, tizanidine has been

shown to be effective in multiple sclerosis and spinal cord in-jury. It is also useful for spasticity of spinal pathology whenweakness is of concern. Tizanidine may also prove effective

in managing spasticity of cerebral origin.40

Secondary oral and systemic agents include tiagabine,cyproheptadine, clonidine, lamotrigine, gabapentin and carbi-

dopa-levodopa.27 Multiple medications have been recom-mended, of which the most recent addition is gabapentin.59

Other treatments include chemical neurolysis, in which thenerve conduction is impaired through the use of chemical

agents and therapeutic nerve block using phenol or alcohol.The goals of these treatments are to prevent muscle contrac-tures and improve the patient’s function. A common side effect

is that after the nerve is injected, alcohol levels measure abovethe legal limit in children. Other side effects include damage tosensory and motor nerves, pain at injection site, scarring, and

dysesthesias. To ensure the correct site, injection must be madeusing an electrical stimulator.57,25

Another treatment used alleviate spasticity in children with

cerebral palsy is rhizotomy. Studies have shown that perform-ing selective dorsal rhizotomy at a young age can reduce theneed for orthopedic surgery.11 Goals of rhizotomy are de-creased tone, increased mobility, and the facilitation of care

for the patient, however, the reduction in spasticity cannotbe predicted and sometimes results in excessive hypotonia.29

The procedure is very meticulous, requiring general anesthesia

and a neurophysiologist who must be present to identify whichnerve is to be severed.

Other neurosurgical approaches include peripheral neurec-

tomy, myelotomy, and dorsal column electrical stimulation.Orthopedic procedures are the most frequently performed

operations for spasticity. The targets of these operations are

muscles, tendons, or bones. Muscles may be denervated andtendons and muscles may be released, lengthened, or trans-ferred. The goals of surgery may include reducing spasticity,increasing range of motion, improving access for hygiene,

improving the ability to tolerate braces, or reducing pain.Orthopedic problems that may result from a spastic limb in-clude cubital or carpal tunnel syndrome, spontaneous fracture,

gait difficulties, dislocation of the hip or knee, and heterotopicossification.

The most common orthopedic procedure for the treatment

of spasticity is a contracture release. In this procedure, thetendon of a muscle that has a contracture is partially cut,



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26 December 2012

completely cut or lengthened. The joint is then positioned at a

more normal angle, and a cast is applied. Regrowth of the ten-don to a new length occurs over several weeks. Serial castingmay be used to gradually extend the joint. Following cast re-moval, physical therapy is used to strengthen the muscles

and improve range of motion.Spastic muscles in the shoulder, elbow, forearm, hands, and

legs may all be treated with tendon or muscle lengthening.

Spasticity in the shoulder muscles may cause abduction oradduction and internal rotation of the shoulder. Abduction re-sults in difficulties with balance, which then affects walking

and transferring, and adduction causes problems when reach-ing for an object or with hygiene and personal care. An oper-ation known as a slide procedure may be used to lengthen the

supraspinatus muscle in an abducted spastic shoulder. Withadducted shoulders, the surgeon can perform a release of allfour muscles that typically cause this deformity.

In an operation known as a tendon transfer, the orthopedic

surgeon moves a tendon from the spot at which it attaches tothe spastic muscle. With the tendon transferred to a differentsite, the muscle can no longer pull the joint into a deformed

position. In some situations, the transfer allows improvedfunction. In others, the joint retains passive but not activefunction. Ankle-balancing procedures are among the most

effective interventions.Osteotomy and arthrodesis involves operations on the bones

and are usually accompanied by operations to lengthen or splittendons to allow for fuller correction of the joint deformity.

Osteotomy can be used to correct a deformity that cannot befixed with other procedures. In an osteotomy, a small wedgeis removed from a bone to allow it to be repositioned or re-

shaped. A cast is applied while the bone heals in a more naturalposition. Osteotomy procedures are most commonly used tocorrect hip displacements and foot deformities. Arthrodesis is

a fusing together of bones that normally move independently.This fusion limits the ability of a spastic muscle to pull the jointinto an abnormal position. Arthrodesis procedures are per-

formed most often on the bones in the ankle and foot. In triplearthrodesis, the three joints of the foot are exposed (talocalca-neal (TC), talonavicular (TN), and calcaneocuboid (CC)joints), the cartilage is removed, and screws are inserted into

the bones, fixing the joints into position. With a short walkingcast in place for 6 weeks or until the bones have fully healed, thepatient may bear weight immediately after the operation.56 The

risks of developing a structural spinal deformity ranges from24% to 36% for scoliosis and is 50% for lordosis for an averageof 4–11 years after selective dorsal rhizotomy.54,30

Other principals include single event, multilevel surgery;surgery is delayed as long as possible (more than 6 years).Spasticity management is used as an adjunct to surgical inter-

vention.9

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Alternative medicine in spasticity management

Physical modalities include electrical stimulation and coldtemperature,10,42,47,34 acupuncture and homeopathicapproaches.28 herbs and hyperbaric oxygen,4,35,41,44,13 con-

straint induced training,52,15 the Adeli suit,50 conductive edu-cation, craniosacral, and manipulation and patterning.

Context therapy is a new intervention approach that

focuses on changing the task and the environment rather than


children’s impairments. It can be a viable treatment to achieve

parent-identified functional goals for children with cerebralpalsy.16

New treatments for spasticity, such as botulinum toxin typeA, have proved easier, more effective, and less painful for pa-

tients. First clinically introduced in the United States in theearly 1980s, botulinum toxin is a potent neurotoxin derivedfrom the anaerobic bacteria Clostridium botulinum, but when

used in treatment, no serious systemic toxin effects have beenreported.25 The medication is more costly than alcohol or phe-nol but the cost is offset by less physician time and the lack of

anesthesia. The formation of antibodies has been a concern,but this can be prevented by allowing 2–3 months betweeninjections. Botulinum toxin works by acting in the neuromus-

cular junction, preventing the release of acetylcholine, whichresults in functional denervation. It can be given withoutEMG and anesthesia, does not cause dysesthesias, and is nomore painful than an injection of saline solution. Effects are

local and last 3–4 months, or longer. It is contraindicated dur-ing pregnancy, lactation, in individuals with neuromusculardisorders (such as myasthenia gravis), in patients taking ami-

noglycosides, or in those who have a known allergy to thedrug. Adverse effects are not common and are usually associ-ated with the site of injection, such as bleeding, bruising, and

soreness or redness at the injection site, or diffusion to nearbymuscle groups. In patients that do not respond to botulinumtoxin, possible reasons should be considered before labelingthe patient as unresponsive. Reasons could be related to injec-

tion technique, improper toxin storage, or the patient’s indi-vidual characteristics. Overall, botulinum toxin has provenclinically to be effective, safe, and less painful than other inva-

sive therapies.25,32 Botulinum toxin is available in serotypes Aand B, which have different unit potencies, side-effect profiles,and dilution schedules. Both have been used in children with

cerebral palsy, although serotype A has been used more exten-sively. Dosing guidelines have been suggested for botulinumtoxin A for adult and pediatric patients. Adult recommenda-

tions are available for botulinum toxin B, but studies are ongo-ing for pediatric patients.53,48,46

Several studies have reported the successful use of botu-linum toxin A for the treatment of drooling in children with

cerebral palsy, using injection into the submandibular orparotid glands alone or in combination with other agents(Jongerius et al., 2001).31,8,51 In some studies, the beneficial ef-

fects have lasted for up to 4 months without serious side effectsor disturbances of oral function (Jongerius et al., 2001)31,51

It has been established that oral baclofen does not cross the

blood–brain barrier effectively and that higher doses of themedication result in serious side effects.25 Intrathecal baclofenresults in a greater decrease in spasticity by allowing higher

concentrations of baclofen in the cerebrospinal fluid at about1% the daily oral dosage.29 To be considered for intrathecalbaclofen pump placement, the patient must have severe lowerlimb spasticity that does not respond to other less-invasive

treatments. The patient must first be given a trial of 50 lg bac-lofen through a lumbar puncture or spinal catheter. If unre-sponsive, 75 lg can be tried after 24 h and a third trial of

100 lg can be tried 24 h after that, after which if the patientis still unresponsive he or she must be excluded from the treat-ment.25 Implantation lasts 1–2 h and the pump is easy to refill

subcutaneously. It is programmed by a computer-controlledradiotelemetry programmer that is linked to the pump’s inter-



514

515

516

517

518

519

520

521

522

523

524

525

526

527

528

529

6 Y. Awaad, T. Rizk


26 December 2012

nal computer and that selects the rate and pattern of baclofen

administration. Complications to intrathecal baclofen includehypersensitivity to baclofen, intolerance to the side effects ofbaclofen including drug tolerance, cerebrospinal fluid leakage,pump pocket seroma, hematoma, infection, and soft tissue ero-

sion. The objective of intrathecal baclofen is to individualizethe patient’s dose and infusion so that the lowest dose thatyields the greatest response can be achieved.57,25 In compari-

530

531

532

533

534

535

536

537

538

539

540

541

542

543

Table 2: Therapeutic interventions for spasticity.

Therapeutic intervention Mechanisms

Non-pharmacologic treatments

Physical and occupational therapy Stretching exercises anywhere from

several times per day

Rehabilitation program Splints, strengthening, electrical st

practice of functional tasks, sensor

muscle stretching, and targeted mu

Casting and orthosis Extend joint range diminished by

reduce an abnormal pattern by po

Selective posterior rhizotomy Balancing spinal cord-mediated fa

inhibitory control

Orthopedic surgery Corrects deformity induced by mus

involving muscles, tendons, or bon

Pharmacological treatments, oral medications

Benzodiazepine Increases the affinity of GABA for

receptors; inhibitory effect at both

and supraspinal levels

Dantrolene sodium Inhibits release of calcium from sa

reticulum in muscle; works periph

muscle fibers

Baclofen GABA agonist; binds at the GAB

restricts calcium influx into presyn

terminals in the spinal cord

Tizanidine Centrally acting alpha-2 noradrene

inhibits release of excitatory neuro

the spinal cord and supraspinally

Pharmacological treatments, chemodenervation

Alcohol/phenol block Nonselective proteolytic agents; se

denervation when injecting into m

muscles

Botulinum toxin injection High affinity and specificity to the

membranes of cholinergic motor n

Pharmacological treatments, other

Intrathecal baclofen pump Using a programmable implanted

can be delivered intrathecally

Table 1: Useful tests for diagnosis.

Test Use

Basic lab studies

(electrolytes, bone profile, etc.)

Metabolic derangement

Enzymatic assay

(Arylsulfatase, Neuraminadase, etc.)

Neurodegenerative disease

EEG Underlying seizure activity

NCS Neurodegenerative disease

(Leukodystrophies)

MRI of the brain Periventricular leukomalacia


son, intrathecal baclofen has less complications and side effects

than other treatments and more generalized results in bothcerebral and spinal spasticity, making intrathecal baclofenthe most effective current tool for the treatment of spasticityin non-ambulant individuals. A recent systematic review

showed that there was no evidence to support the clinical useof intrathecal baclofen in ambulant individuals with hyperto-nicity without further rigorous longitudinal studies.43

As a precaution, families are prescribed diazepam or diaze-pam rectal as well as oral baclofen to have at home. If there isevidence of withdrawal, one of these medications is adminis-

tered, and the patient is instructed to go immediately to theemergency department. Although aggressive use of benzodi-azepines and oral baclofen may be helpful, recognition and re-

turn to appropriate intrathecal baclofen dosage is essential forrapid recovery.6

The goals of and benefits to the patient are important whenconsidering the path of treatment. In some cases, function will

not return, but treatment can result in pain reduction and al-low easier management of patient care. Common goals areto decrease pain, prevent or decrease contractures, improve

ambulation, facilitate activities of daily living, facilitate reha-

Major points

once daily to Only a limited effect on the patient’s spasticity

imulation,

y integration,

scle training

Mainstays and cornerstones in spasticity

management; complications, such as autonomic

dysreflexia, deep vein thrombosis, and

heterotropic ossification, can be severe and

potentially life-threatening

hypertonicity;

sitioning

Temporary effect

cilitatory and Permanent effect; sometimes results in excessive

hypotonia

cle overactivity

es

In moderate to severe spasticity; permanent effect

GABA-A

the spinal cord

Short-term treatment; strong sedating effects

rcoplasmic

erally at the

Serious side effects; hepatotoxicity in 1% patients;

respiratory muscle weakness

A-B receptor;

aptic nerve

Rapidly absorbed after oral administration; levels

in the CSF are low because of low lipid solubility

rgic agonist;

transmitters in

Drowsiness, dry mouth, and dizziness; monitor

liver function

lective

otor nerves or

Damage to sensory and motor nerves; painful

dysesthesias

presynaptic

eurons

Recommended as effective treatment; no sensory

disturbance

pump, baclofen Severe, generalized spasticity; less complications

and side effects



544

545

546

547

548

549

550

551

552Q5

553

554

555

556

557

558

559

560

561

562

563

564

565

566

567

568

569

570

571

572

573

574

575

576

577

578

579

580

581

582

583

584

585

586

587

588

589Q6

590

591

592

593

594

595

596

597

598

599

600

601

602

603

604

605

606

607

608

609

610

611



26 December 2012

bilitation participation, save caregiver’s time, improve the ease

of care, and increase safety. Appropriate management choicesare based on therapeutic objectives. Physical and occupationaltherapists can play a key role in identifying these objectives.Treatments with the fewest side effects are usually given prior-

ity. Both the patient’s and the caregiver’s goals must be consid-ered.12

A summary of therapeutic interventions is provided in

Tables 1 and 2.

612

613

614

615

616

617

618

619

620

621

622

623

624

625

�16. Pregnancy

The patient with spasticity may expect to have a difficult preg-nancy and delivery as well as difficulty managing and caring

for an infant. Close medical follow up throughout pregnancyis recommended, hospitalization, immediate post natal care,etc.

Anesthesia

Not applicable.
626
627

628

629

630

631

632

633

634

635

636

637

638

639

640

641

642

643

644

645

646

647

648

649

650

651

652

653

654

655

656

657

658

659

660

661

662

663

664

665

666

667

668

669

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Spasticity in children