approach to the rehabilitation of spasticity and neuromuscular disorders in children

Neurol Clin N Am 21 (2003) 853–881

Approach to the rehabilitation ofspasticity and neuromuscular disorders

in children

Ann Henderson Tilton, MDChildren’s Hospital of New Orleans, 200 Henry Clay, New Orleans, LA 70118, USA

Spasticity and tone management

Spasticity affects the motor abilities of many children and adults. Thepublic often associates it with the diagnosis of cerebral palsy; however, it hasmultiple etiologies. Disruption of the upper motor neuron system may occurwith prematurity, stroke, infection, head injury, multiple sclerosis, traumaticbrain injury, spinal cord injury, or anoxic insult, among other etiologies.The specific pathology influences whether or not extrapyramidal findings,such as dystonia, may be seen as part of the clinical picture.

The definition of spasticity has been revised as the underlyingmechanisms have become elucidated and new theories put forth. Theclinical findings that best describe the children and adults affected with thissyndrome, however, have been consistent. The most often quoted definitionis Lance’s, from more than 20 years ago, and is still widely used [1]:‘‘Spasticity is a motor disorder characterized by velocity-dependent increasein tonic stretch reflex (muscle tone) with exaggerated deep tendon jerksresulting from hyperexcitability of the stretch reflexes, as one component ofthe upper motor neuron syndrome.’’ Initially there is an increased resistanceof a passive limb to externally imposed joint motion and then movement isagain allowed. Thus, with the catch and give, the descriptive term ‘‘claspedknife’’ was coined.

The original understanding of human spasticity evolved from animaldata. Sherrington’s seminal work in the late 1800s is now believed notdirectly applicable to human movement disorders [2]. The current path-ophysiology of spasticity is believed a long-term reduction of inhibitoryinput and hyperexcitability of the a motor neuron pool as a result of the loss

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854 A.H. Tilton / Neurol Clin N Am 21 (2003) 853–881

of presynaptic inhibition and inability to synthesize or transport theinhibitory neurotransmitter c-aminobutyric acid (GABA) to the anteriorhorn cells of the spinal cord. As a result, there is hyperexcitability of the Iamotor neurons and abnormal processing in the spinal cord of otherperipheral afferent input. Additionally there are primary changes in thespinal cord with shortening of the motor neuron dendrites and collateralsprouting of the dorsal root afferents believed to contribute [3–6].

As Mayer points out, the exaggerated motor responses of the spasticpatient originate from the way the spinal cord circuitry is influenced byseveral different inputs. These include proprioceptive, nociceptive, andextraceptive and the descending inputs from the suprasegmental areas [6].

The physical properties of muscle also shape motor function. Musclesand joints are influenced by the rheologic properties (plasticity andviscoelasticity) and the imbalance of the agonists and antagonists aboutthe joints. Chronic spasticity leads to changes in the muscle actin andmyosin filaments and changes in connective tissue elements. Fibrosis andatrophy follow [6–8]. The enhanced viscosity is what is experienced by theexaminer. These characteristics further contribute to impaired movementand contracture formation.

Clinical characteristics

The clinical characteristics that define spasticity are fairly uniform,offering the clinician a broad template for approaching assessment andmanagement, particularly important with the new treatment optionsavailable. These findings include hypertonia, clonus, and crossed adductorreflexes. What is not as well recognized, but responsible for significantdisability, is the associated weakness and loss of dexterity. UMNS includespositive and negative symptoms, and the movements in an individual withUMNS represent a combination of positive and negative symptoms and alsochanges in the physical properties of muscle. The positive symptoms—increased reflexes and tone—are those associated with disinhibition (therelease of the intact motor system from control). The negative symp-toms—lack of agility, fatigability, and associated weakness—are associatedwith the loss of cerebral input (disconnection of the lower motor neuronsystem from the upper motor neuron system) [3]. The positive symptoms arethe most amenable to intervention. Unless the increased tone is significantlyrestricting activity, however, the underlying weakness and dexterity remainsubstantial impediments for the individual.

Recent studies have delved into the well-recognized symptoms ofweakness and difficulty with individual muscle movements in children whohave early cortical injury. In childhood not only is there altered corticalinput but also central motor reorganization. Given that cortical spinalprojections are essential for fine motor coordination, it has been assumedthat there was a lack of cortical control of the spinal motor neurons

855A.H. Tilton / Neurol Clin N Am 21 (2003) 853–881

secondary to motor cortex damage and a reduction in projections to themotor neuron pool. Studies using transcranial magnetic stimulation haveverified that the cortical spinal projections from the damaged motor cortexto the distal motor upper limb muscles are sparse or absent [9]. The impacton cortical control explains some of the impediment to voluntary activity inaffected children [10,11].

Carr et al demonstrated that there is reorganization of the cortical spinalprojections. They established that patients who had obligatory mirrormovements and spastic hemiplegia had projections of the cortical spinaltract that emanate from the intact hemisphere to innervate the ipsilateraland the contralateral upper limb. This was found specifically in childrenwho had damage at less than 24 weeks’ gestation [9,12]. Also in childrenwho have spastic quadriparesis, the study revealed that reorganizationseems age dependent, with the earliest preterm infants the most affected. Theobligatory mirroring and resultant simultaneous activity of both hands withbilateral cortical projections has functional and rehabilitation implications.Although it may allow opening of the hemiplegic hand without stimulationcoming from the damaged hemisphere, it does interfere with bimanual tasks[13]. There are excellent reviews and correlation of the data with the rec-ognized movement disorders of the cerebral palsy [13].

Other issues that affect appropriate motor function include lack ofanticipatory motor control, agonist and antagonist muscle interactions, andpostural control [13]. From a therapeutic standpoint, children who havespasticity have difficulties with manipulative skills and adapting to variableloads to the fingers. This may be because of a lack of impaired sensoryfeedback or a lack of cortical control [14,15]. For the successful movement ofa limb, there must be reciprocal action between the agonist and antagonistmuscles with inhibition of the antagonists while the agonists are firing.Alternatively, there are appropriate co-contractions to stabilize the joints. Inchildren younger than age 5 and in those children who have spastic cerebralpalsy, the action is primarily co-contraction with rarely any reciprocal action.The co-contraction of the agonists and antagonists in spastic cerebral palsy isbelieved the result of a lack of reciprocal inhibition by the cortical spinalprojections [16,17]. Additionally, the patterns of postural control aredisrupted because of the abnormal co-contraction and lack of the appropriatedevelopmental progression [13,16,17].

What does thismean for the patientwho has spasticity? From a physiologicstandpoint, the patient rarely has a pure syndrome. Instead it is not un-common for aspects of dystonia in conjunction with spasticity. Associatedweakness usually is present with hypertonia, pathologically brisk reflexes,Babinski signs, and clonus. Spasms also may occur, which may causediscomfort. The untreated consequences of severe spasticity are contractures,pain, decreased mobility, hygiene difficulty, and sleep disturbances.

Before deciding to treat spasticity, it is critical to determine if thespasticity may be helping the patient’s voluntary movement. The antigravity


effects of the spasticity in the lower extremity may support the leg so thatambulation is possible, although visibly impaired. Decreasing spasticity inthe patient who uses it for strength actually results in diminished function.The author’s interventions at times take away perceived strength as the toneis removed and the paresis becomes more prominent. In a patient who hasunderlying strength in which spasticity is interfering with their motorcontrol, however, spasticity reduction may lead to considerable improve-ment. Likewise, an individual with multiple spasms or pain associated withspasticity benefits.

Evaluation

The evaluation of the patient who has spasticity should be individualizedand provided through an interdisciplinary team in order to approachmaximum functional outcomes and facilitate goals. The team representsseveral medical subspecialties, including child neurology, pediatric neuro-surgery, orthopedics, physiatry, and the child’s primary care physician. Theprimary physician is a critical component of the spasticity team. The alliedhealth specialties include physical therapy, occupation therapy, speechtherapy, psychology, and social service and nursing.

Several scales have been developed to measure spasticity, although theyfrequently do not reflect subtle, but important, functional gains. TheAshworth scale, which measures tone, provides a quick but broad as-sessment [18]. This ordinal point scale (1 normal to 5 rigid) may be ad-ministered in ambulatory or hospital settings. It does not address thefunctional capabilities of the child; it is more a direct measure of thetechnical limitations and cannot reflect the more subtle changes. Assessmenttools, such as the Tardieu scale [19], gross motor functional measure(GMFM) [20], and gross motor performance measure [21], can bettermeasure disability and functional impairments.

Developing a treatment plan

In the overall consideration of any treatment option, it is important toascertain if the spasticity is preventing function and whether or not it is thepositive or the negative symptoms that are the most disabling [22]. Alsonecessary is determining which options were tried in the past, the severity ofthe problem, and whether or not the problem involves only a regional area,such as the gastrocsoleus complex, or is multisegmental with generalizedspasticity.

The treatment plan should be customized with succinct, definable, andachievable therapeutic goals. All parties should agree on the goals, includingease of ambulation, increased daily living skills, facilitation of hygiene,contracture prevention, and improved abilitation. The author tries to usethe least invasive technique before advancing to more invasive modalities.


Rehabilitation therapy

The foundation of treatment is rehabilitation management. Withoutfamily commitment and an adequate therapy program, either home- orcenter-based, the best of techniques often are not successful. Rehabilitationtherapy focuses on maximizing function. This is accomplished with familyand patient education and includes appropriate range-of-motion andstretching techniques. Additional modalities include orthotics, casting, anddynamic bracing [23]. Therapists also aid in determining seating devices.

Studies that advocate for therapy alone have been called into question[24–27]. Reports indicate that therapy in isolation is minimally effective inthe moderately to severely impaired group. Cost also is a factor.

Pharmacologic therapy

Because of their systemic effects, pharmacologic therapies target eithergeneralized or regional spasticity. Diazepam and baclofen facilitate thepresynaptic effects of GABA and are believed to inhibit the polysynapticareas in the spinal cord, thus balancing some of the chronic disinhibition[28,29]. This action does reduce tone but often causes significant sedation.Dantrolene directly inhibits the muscle contractility and theoretically isa good choice because it has fewer cognitive effects [30,31]. The potential forhepatic effects must be weighed [32], however. Tizanidine, an a-2 agonist, iseffective for reducing spasticity, and the concomitant sedation can be usedfor relaxation at night. Overall, these medications reduce spasticity inchildren who have cerebral palsy [33–35]. Functionally, however, the changeoften is only minimal and the benefit may not be substantial enough towarrant the side effects of increased weakness and sedation [29,36].

Botulinum toxin

Botulinum toxin injections can be used for regional or focal managementof spasticity. The toxin inhibits the release of acetylcholine at the neuro-muscular junction by cleaving one or more members of the complex thatfuse acetylcholine vesicles to the plasma membrane at the nerve terminal.Botulinum toxin A and B provide chemical denervation and muscleparalysis on a temporary basis in selective muscle groups that are injected.Reviews of the pharmacology, dosage recommendations, and injectiondistribution are available [37,38]. With dosage limits of approximately 10to 12 U/kg up to approximately 400 U, multiple muscle groups cannot besimultaneously injected. Additionally, because of its reversibility it requiresreinjection approximately every 3 months.

Botulinum toxin effects are dose dependent. The main action is localizedto a 4- to 5-cm area with the clinical effect apparent in 48 hours and peakaction in 2 to 4 weeks. Duration is 3 to 4 months, although improvementsmay outlast the direct effect on the nerve terminal. Possible explanations for


these prolonged effects include improved balance of the muscle groups withthe over-stretched antagonists shortening and muscle lengthening in theagonists at the injection site [39].

Patients best suited for botulinum toxin injections demonstrate muscleimbalance with stronger spastic agonist muscles. Sufficient power in theantagonist muscles is necessary to offset weakened agonists. Fixed con-tractures and bony deformities do not adequately benefit from the re-laxation of muscle and are only amenable to orthopedic intervention, suchas casting or surgery. Patients in the acute rehabilitation phase of an injury,such as traumatic brain injury, also may benefit. If the increased tone isadequately addressed, then the orthopedic abnormalities are not magnified.

Botulinum toxin provides decreased tone and functional improvementin the injected muscle groups [40–44], with efficacy in upper and lowerextremities. The side effect profile is good, with a 6% incidence primarilyrelated to the discomfort of local injections [45,46].

Intrathecal baclofen

For patients who have multisegmental or more extensive spasticity, andfor whom oral medications are not effective or appropriate, intrathecalbaclofen (ITB) is a strong consideration. Baclofen is a presynaptic inhibitorthat activates the GABAB receptors. The Food and Drug Administration(FDA) has approved intrathecal use for spasticity of cerebral and spinalorigin. A continuous supply of medication is delivered by an infusion systemthat uses a catheter in the intrathecal space, a pump in the abdomen, andtelemetry programming.

The intrathecal dose required is (often or frequently) 0.3% to 0.5% of theoral route and is better tolerated in patients who have systemic side effectsfrom oral baclofen [47]. The rate, mode, and pattern of infusion may bemodified noninvasively to meet the patient’s needs. The patient may receivea 24-hour simple continuous infusion dose. If there are variable needsthroughout the day, however, then the infusion rate can be adjusted up to 10distinct rates in a more complex infusion pattern. A common pattern is toincrease the dose in the evening to aid sleep and reduce it in the morning tofacilitate transfers. The catheter can be placed in the high thoracic or lowercervical area to better address upper extremity spasticity and dystonia [48–50].

Appropriate candidates for intrathecal infusion should have an Ash-worth score of 3 or higher in the lower extremity and must have enoughbody mass at a minimum age to support the pump. Although these areestimated at 40 pounds and 4 years, younger age groups and patients withless weight have been successfully implanted. The development of subfascialplacements has contributed to this wider indication. Patients who benefitfrom ITB include those who have poor underlying strength whose extremitymovements are impeded by spasticity, nonambulatory spastic quadripareticswhose spasticity interferes with daily living skills, nonfunctional patients for


whom the goal is to enhance quality of caregiving, and patients who havedystonia or other movement disorders [51,52].

Before placement, the patient should have a positive response to a trialbolus dose of ITB. An adequate response is a 1-point decline in theAshworth scale for cerebral origin and a 2-point drop in patients who havespinal cord origin. Patients who have dystonia require higher dosing. Toprovide higher dosing, a trial with placement of a catheter and then a dailyinfusion of higher volumes over the course of 24 hours is required [48].Another criterion before placement of an ITB pump is assurance that thefamily is committed to the modality. The pump must be refilled typicallyevery 6 to 12 weeks, and refill is mandatory to avoid withdrawal.

Studies have demonstrated improvement in tone, spasm, pain [53], andquality of life [50,52,54–56]. Compared with oral therapies, ITB reducesintolerable side effects. ITB can be used in conjunction with other therapies.

There is potential for side effects from baclofen and the indwellingtechnology, including hypotonia, weakness, nausea and vomiting, andchanges in urinary or bowel function. The frequency of seizures in patientspreviously diagnosed with epilepsy does not seem to be influenced by the ITB.Device-related and surgery-related events include seroma, infection, andcatheter-related problems, including kinking, breakage, and dislodging [53].Serious medical issues are related to overdose and acute withdrawal of themedication [57,58].

Surgical management

In the past, surgical management was one of the few options in thetreatment of spasticity. Interventions, dominated by tendon releases andosteotomies, were primarily the domain of orthopedists.

Selective dorsal rhizotomySince 1987, selective dorsal rhizotomy (SDR) has been performed at

many centers. The original procedure preceded this date by many yearswhen nonselective rhizotomy was attempted, but because of unwantedsensory effects, was abandoned. Peacock and Staudt repopularized thetechnique using a selective approach [59].

The best candidates are children 3 to 7 years old who have spasticdiplegia, good trunk control, and isolated leg movements [60,61]. Theseindividuals typically have significant weakness after the procedure andrequire extensive therapy. If they have significant underlying weaknessbeforehand and the spasticity is removed, they do not have functionalimprovement. Originally, patients who had severe spastic quadriparesis wereconsidered for SDR; their suitability is now strongly debated with ITBconsidered the preferred modality.

The SDR procedure consists of lumbar laminectomy in which the dura isopened. Dorsal rootlets from S-1 to T-12 are separated. Each rootlet is then


stimulated and the responses are recorded electromyographically. Thoserootlets with an abnormal sustained or generalized response are thensurgically severed. Approximately 40% to 60% of the posterior rootlets aresevered [62]. Postrhizotomymanagement includes aggressive physical therapyprograms 4 to 5 times a week and occupational therapy once or twice a week.It usually takes 6 months of rehabilitation to reach the preoperative state.

Statistically significant improvement is documented with SDR. Ran-domized studies have demonstrate improvement in tone and motor function[63–65]. SDR may be the most efficacious in marginal ambulators.

Orthopedic interventionsChronic muscle imbalance may lead to contractures and other orthopedic

issues, such as scoliosis and hip dislocations. Tendon releases for fixedcontractures are a major part of the armamentarium in the treatment ofspasticity in childhood. Orthopedic surgery is most efficacious in skeletallymature individuals. One multilevel surgical procedure, as opposed tomultiple procedures with each growth spurt, is strongly advocated.

Summary

Spasticity treatment is best approached as a decision tree, in which bothphysiologic and chronologic factors play a role in decision making. Thefundamental approach begins with ongoing physical therapy and occupa-tional therapy to maximize range of motion and strengthening. This shouldbe family centered so that the families can provide daily therapy as opposedto a once- or twice-a-week formal session. An additional benefit of familyinvolvement is that family members rapidly can become advocates for theirchild.

Frequently, oral medications are instituted and if adequate to controlspasticity, continuation of medications is all that is needed. If instead, oralmedications do not provide adequate control of spasticity or they produceundesirable side effects, then further options must be explored. If spasticityis confined to a functional unit, either regional or local, then injectionof botulinum toxin is a rational choice. If instead it is multisegmentalspasticity, then ITB via an implantable pump may be a consideration. Ifthe tone abnormalities involve the lower extremities, particularly in ayounger child who has good underlying strength, then SDR should be con-sidered. If contractures are present, then a tendon lengthening is appropriate.Orthopedic surgical procedures certainly have a role in hip dislocations andscoliosis. All these interventions may be used separately or in conjunction,depending on the individual needs of the patient.

The child who has excessive drooling

Sialorrhea, or drooling, is unremarkable in a young child; however, itoften is a cause for embarrassment and social isolation in the older child and


young adult. Drooling beyond age 4 is considered pathologic and when itoccurs in conjunction with developmental impairment and disability it leadsto further social barriers [66]. When families come to a clinic requestingassistance, the treating physician is faced with finding an effective modalitynot associated with significant side effects.

The basis of sialorrhea

Pathologic drooling occurs in association with many neurologic deficits.Between 10% and 37% of children who have cerebral palsy have persistentdrooling [67,68]. Additionally, drooling may begin in adulthood after anacute neurologic deficit or with a degenerative neurologic disorder, such asParkinson’s disease or amyotrophic lateral sclerosis (ALS).

Sialorrhea is the involuntary flow of saliva from the mouth andassociated spillage. Drooling ranges from mild to profuse, when there isalmost continuous flow. The primary basis for drooling is believed to beinadequate oromotor control secondary to pharyngeal sensory deficit orcentral interruption of normal swallowing reflexes rather than excessivesalivary production [69–71]. Swallowing studies in patients who havecerebral palsy implicate three areas of difficulties: incomplete lip closure, lowsuction pressure, and prolonged delay between suck and propelled stage[72]. Whereas drooling may be viewed merely as an issue of cosmesis, thereare significant psychosocial and physical ramifications. Affected childrenand caregivers often report it as a major source of anxiety. Associateddifficulties include skin breakdown, cellulitis, and yeast infections. Also, life-threatening consequences secondary to salivary aspiration may occur whenthe saliva spills posteriorly into the hypopharynx [73].

Traditional treatments

Although many approaches have been used individually or in combina-tion to treat drooling, none has been universally successful [74]. Behavioralprograms, such as biofeedback and oral appliances, have been employed asa less invasive approach but they are not highly effective [75]. Anticholin-ergic drugs, such as glycopyrrolate, scopolamine, and benztropine, are takenorally or transdermally to dry the secretions [76]. The basis of anticho-linergic pharmacotherapy is to address the excessive parasympatheticstimulation that is mediated through the muscarinic receptor sites. Theanticholinergic drugs have little selectivity for the receptor sites, however.Thus the widespread systemic effects result in the significant associated sideeffects [76]. Studies show that 69% of patients taking glycopyrrolateexperience side effects with approximately 20% discontinuing the medica-tion [77]. Common side effects include constipation, irritability, and urinaryretention. Less common side effects are diarrhea and blurred vision[67,77,78].


More invasive methods include irradiation of the parotid gland, rarelyused because of secondary risk of malignancy. Surgical approaches includebilateral submandibular gland excision with parotid duct ligation, parotidor submandibular duct rerouting, and transtympanic neurectomy [75,79].These have had variable results and associated side effects. Potential long-term risks include the increased incidence of dental caries, salivary glandcalculi, and excessive dryness of the oral mucosa [80,81]. Successful outcomeis higher in this group, approaching two thirds for those undergoing surgery[81,82]. In persons studied who have severe drooling, up to 70% eventuallyare referred for surgical intervention. Unfortunately, surgical procedures inthese chronically ill children are not without risk and complication [82].

Botulinum toxin

Botulinum toxin A has been used as a treatment modality in manyneurologically based disorders, inclulidng spasticity, dystonia, migraines,pain, cosmesis, and ophthalmic and autonomic disorders [38,83]. Morerecently, botulinum toxinA’s ability to chemodenervate at the neuroglandularjunction has been recognized. It is used therapeutically in hyperhidrosis,Frey’s syndrome, and vasomotor rhinorrhea. Parotid sialoceles also have beensuccessfully treated in the author’s clinic [82,84,85]. Data in adults show thatthere is a reduction in saliva production and a resultant improvement indrooling. Preliminary human trials in neurologically impaired adults withParkinson’s disease or ALS corroborate the efficacy of the use of botulinumtoxin A in sialorrhea [86,87].

Initial studies in canines demonstrated that botulinum toxin A and Bsignificantly but temporarily decreased the submandibular salivary pro-duction through an anticholinergic rather than a direct toxic effect on theacinar cells of the gland [85]. Additionally they found that even withcomplete cholinergic blockage with atropine there was only a 76% reductionof submandibular production [88], implying that one fourth of the salivapotentially is mediated by noncholinergic transmitters, an important pointwhen there are concerns regarding xerostomia.

There are many actions of botulinum toxin A, the most prominent ofwhich is the anticholinergic effect at the neuromuscular junction. Nerveendings of the postganglionic parasympathetic neurons secrete acetylcho-line. The blockade of these neurons inhibits the stimulation to the salivaryglands. The anticholinergic effect at the glandular level seems the mechanismof action in this setting. The synthesis and storage of acetylcholine is notaffected by botulinum toxin; instead there is an inhibition of the release ofthe acetylcholine at the neuromuscular or neuroglandular junction. Theseeffects are temporary and thus reversible.

Although there has been a great deal of analysis of the neuromusculareffects, the glandular effects are less well studied. Botulinum toxin type A isbelieved to have greater anticholinergic effect at the neuromuscular junction,


whereas type B is believed to have a greater effect at the autonomic junction[85]. Botulinum toxin A and botulinum toxin B have FDA approval forclinical use but not for this specific application. The unit potency of thevarious commercial preparations differs markedly, making it essential tospecify the type and commercial preparation of toxin. Unless otherwisenoted, all units below refer to botulinum toxin A as BOTOX.

Duration of actionThe duration of clinical action of botulinum toxin A at the neuromus-

cular junction is approximately 3 to 4 months. A longer lasting effect is atthe glandular level. Posttreatment anhidrosis for more than 12 months hasbeen reported in patients who have gustatory sweating treated with type Atoxin [89].

EfficacySeveral studies have used botulinum toxin A as a treatment of drooling in

children who have cerebral palsy. Jongerius et al applied botulinum toxin Ain the submandibular glands. They originally chose the submandibularglands to the exclusion of the parotid glands to diminish ambient salivaryflow rate, but not to restrict saliva production when the child was eating.There was a reduction of approximately 50% to 60% in the three patientstested. No disturbances of oral function were observed [90]. Bothwell et alevaluated and injected nine patients with 5 units of botulinum toxin Ain each parotid gland. At week 4 all the patients had reduced droolingfrequency and 55% believed that the treatment was successful overall [68].

In the author’s study, a preliminary consideration was the dosage choicefor the treatment of sialorrhea [74]. There were no studies that previouslydefined an accurate dose-response curve, although general clinical guidelinesfor children who have neuromuscular difficulties had been generated [91].The first priority was safety of the children. Because of the concernregarding local diffusion, the study was two-staged, first injecting sub-mandibular glands with escalating dosages. The delivery of the botulinumtoxin was intraglandular with ultrasound guidance. Speech pathologistsprovided pre- and postinjection evaluations. Because of the ability ofbotulinum toxin at times to diffuse to a certain degree beyond fascia planes,the targeting of the medication was deemed important. Studies of thebiometry of the normal salivary glands were reviewed, as were techniques toproperly identify the salivary glands by ultrasound. The botulinum toxinwas delivered with ultrasound guidance into each submandibular gland asa single injection. Each parotid gland received two separate injections one inthe body of the gland with guidance and one in the tail of the gland withoutultrasound guidance.

Measurement of the saliva was a significant issue. Patients completedquestionnaires and drool quotients, defined as the amount of saliva leavingthe lip in a measured period of time. Techniques such as dental rolls were


not successful because of patient intolerance. Other studies have used ab-sorbent cotton rolls and bibs and also drool quotients and questionnaires.

The study was divided into two phases. Originally the submandibularglands were injected with a total dose of botulinum toxinA (Botoxmay not benecessary) ranging from 10 units to 30 units. Five patients from the first phaseand five additional patients then were advanced to a second phase involvingthe injection of the submandibular glands with the total dosage of 30 unitscombined with injection of the parotid glands. The total doses in the parotidglands ranged from 20 to 40 units. Although the original group with onlysubmandibular injections had amodest response, 90%of the patients injectedat both the submandibular and parotid glands showed improvement. Also, 9of the 10 caregivers believed there was an improvement and would allowreinjections. After the study, the nonresponding patient had parotid andsubmandibular glands removed and continued to have significant drooling.This was believed related to oromotor problems.

Overall, only mild side effects of dry mouth and chewing difficulties arereported in adult studies [68]. There are no reported side effects in childrenother than local discomfort. No dysphagia has been reported.

Summary

Sialorrhea is a social obstacle and a potential medical problem. There arefrequent requests by patients and families to improve the symptoms.Botulinum toxin is a valuable consideration.

Rehabilitation of neuromuscular disorders

Children who have neuromuscular disorders (NMDs) present with a widerange of symptoms, from a subtle delay in motor milestones to severe hypo-tonia and weakness within the first days of life. Clinically, any disorder ofthe motor unit (anterior horn cell, axon, enveloping myelin, neuromuscularjunction, primary muscle) may be responsible for a constellation of symp-toms, including weakness, hypotonia, muscle atrophy, and sensory loss.

Clinical characteristics help differentiate the disorders, but the impactof molecular genetics on the diagnosis of nerve and muscle disorders isdramatic. What once required invasive studies, such as electromyography,nerve conduction studies and muscle biopsy, often is replaced with adiagnostically specific genetic test. This has revolutionized the diagnosticcomponent of the continuum of the care of the child who has an NMD.Once the evaluation is complete and the diagnosis confirmed, however, thelong-term management and rehabilitation issues still must be addressed.

Rehabilitation planning

The comprehensive medical care of the patient who has complex NMDsis best coordinated through a multidisciplinary approach. Because of the


potential of multiple ongoing medical and psychosocial issues, this teamoften includes neurologists, pulmonologists, and orthopedists, with con-sultations from cardiologists, gastroenterologists, geneticists, physicaltherapists, occupational therapists, speech therapists, social workers, andpsychologists. Although the patient may not require the entire team, havingthe expertise available allows anticipatory and therapeutic interventionswhen necessary. Unfortunately, the primary care physician often is notincluded in planning care, but these physicians are the front line when anacute medical illness arises. A child who has an underlying NMD is athigher risk for difficulties with a routine illness.

The role of therapistsTherapists provide significant assistance with family education, support,

and monitoring the patient’s developmental and physical progression. If thefamily is taught appropriate exercises and passive range of motion inconjunction with the proper use of the orthotics, and if compliance is good,contractures can be prevented. Work in patients who have Duchennemuscular dystrophy (DMD) demonstrates that stretching in conjunctionwith early and consistent nighttime splinting is superior to stretching aloneto prevent contracture. Stretching alone was similar to the group thatreceived no treatment [92]. The therapist also may supervise other activitiesand exercises to increase endurance. For example, water aquatics are welltolerated, particularly in the weak child, who can move much more freelywith gravity removed. Additionally compliance is improved with morepleasurable activities.

There is debate as to what degree the child who has NMDs can benefitfrom an exercise program [93]. Using the sports model of increasingendurance by multiple repetitions and essentially ‘‘pushing’’ the patient maylead to significant problems and overwork weakness [94]. Patients canincrease their strength and endurance with a regimen that does not lead tocramping or exhaustion. Exercise programs in which there is contraction ofthe muscle without associated joint movements (isometrics) is preferred.Atrophic muscle requires only 20% to 30% of the patient’s maximumresistance to strengthen [94]. In the child who has DMD, active exerciseprolongs ambulation. Whereas the improvement was most significant earlyin the program, improvement over baseline was sustained [95]. All theseprograms must be supervised closely, particularly in a child who may havean associated cardiac or pulmonary abnormality.

Mobility aids and modifications and strategies for school and daily livingneeds are addressed by the therapists, who are instrumental in develop-ing the appropriate seating system for the child who loses the abilityto ambulate. Depending on age and ability, a custom wheelchair isrecommended. Electric wheelchairs provide increased independence. Theauthor has been successful with electric wheelchairs in children as young as3 years old.


Daily living skillsOccupational therapists are involved with upper extremity and fine motor

skills. They focus on strategies, environmental modifications, and equip-ment to meet the requirements for the activities of daily living (ADL).Adaptive equipment is helpful for allowing patients to feed themselves andbe independent longer. In order for patients to be successful with mobilearm supports for arm flexor weakness, however, head and trunk stability arenecessary. Additionally, strength is required to be 2 + or 3 in the upperextremities with preserved range of motion [96,97]. The impact of familysupport, or its absence, on the value of adaptive equipment should not beunderestimated.

Occupational therapists also may have special interests in feeding issues.They often work in conjunction with speech therapists to assess modifiedbarium swallows and address feeding disturbances.

Speech therapySpeech therapists help with evaluation and management of dysphagia

and possible aspiration. Modified barium swallows with the supervision ofthe feeding specialists from speech and occupational therapy provide in-formation regarding the safety and efficiency of feeding orally with vary-ing food textures. If there are concerns, then specific modifications arerecommended.

Social services and psychologySocial services provide support for the patient and serve as liaison

between patient and family and community resources. Knowledge of theservices and programs available is as pivotal as being an unrelentingadvocate. With the many stresses on family and patient, psychologic servicesare often welcome and necessary for providing counseling.

Clinical issues in neuromuscular rehabilitation

With the substantial potential for complicating factors in the re-habilitation care of the child who has an NMD, a sudden deterioration ina patient’s strength raises several considerations.

Has general nutrition deteriorated?Good nutritional status must be maintained. Complicating factors, such

as dysphagia, oral motor weakness, and dental changes, may occur eitherprimarily the result of the underlying illness or the catabolic state. Thecatabolic state may lead to further weakness, perpetuating the cycle.

Has there been deterioration in ventilation status?Patients may have significant weakness of their diaphragm and in-

tercostal muscles, leading to insufficient ventilation or restrictive lung


disease. It is more obvious in the child born with a severe myopathy orneuropathy; however, it can insidiously evolve in the less affected olderchild. Some of the indicators that this is occurring are frequent nighttimeawakening, exhaustion in the morning, and frequent headaches.

Are cardiac-related abnormalities progressing?Cardiomyopathy accompanies several of the NMDs. Cardiologists

address the diagnostics, such as the electrocardiogram, echocardiogram,and Holter monitors, and direct the therapeutic interventions. As the patientbecomes more disabled with the associated cardiomyopathy, congestiveheart failure may evolve, reducing endurance that may present as progres-sive weakness. Arrhythmias and cardiomyopathy may be a proximatecause of death.

Is there progressive disability associated with orthopedic issues?Scoliosis, disuse, decreased endurance, and contractures may be major

concerns, and the orthopedic members of the team should follow thechildren over time. Interventions often begin with simple orthotics, such asankle foot orthoses (AFOs), to help with distal weakness and resting nightsplints to help with potential contractures in the hamstrings. With time, themusculoskeletal changes associated with the given disorders may lead to theneed for surgical soft tissue releases and osteotomy. If surgery is required,rapid treatment and return to activity as soon as possible is critical.Particularly in DMD, keeping the child ambulatory as long as possible helpsprevent or delay contractures and scoliosis, which may otherwise progressrapidly. When the child is in a wheelchair, side supports may be tried toaddress scoliosis, but often this is not adequate. In children whose curves aremild but progressing, spinal fusion often is considered preemptively, toavoid the risk of surgery after the inevitable loss of ventilatory capacity.

Nutrition

Children who have NMDs in children are a complex group unique in theway they are assessed nutritionally and how nutrition is delivered. Althoughthe most common problem is chronic malnutrition, undernutrition andovernutrition may present difficulty in the patients’ care and can lead toexacerbation of weakness in NMDs.

Nutritional assessmentDocumentation of nutritional intake, nutritional needs, underlying

medical problems, physical examination, anthropometrics, and biochemicalmarkers all are part of an appropriate evaluation. An accurate 3-day caloriecount including fluid intake should be recorded. Often there is anoverestimate of calorie and fluid intake based on the duration of timerequired to feed the children. A child may claim satiety when he becomes


tired as a result of the effort required to feed orally, or food refusal mayoccur because of the child’s fear of attempting to eat a specific texture offood or liquid. As a result, caloric needs are not met, fluid intake often isinadequate, and constipation and the risk of renal stones are exacerbated.Additionally, there is evidence that there is accompanying suboptimalvitamin and mineral intake [98].

Assessment of needCalorie needs often are dramatically altered in children who have NMDs,

as they lack typical movements and have reduced lean body mass. Thesefactors make the classic measuring instruments difficult to apply.

Medical issuesOther complicating factors may lead to unusual losses, such as excessive

drooling of saliva. There are documented cases of 25% of the maintenancefluids lost as saliva in a 24-hour period [98]. Physical examination is in order,of course, and anthropometrics provide better understanding and offera baseline to monitor the patient’s weight.

NMDs in general lead to progressive loss of strength, reducing the abilityto exercise; thus, the discrepancy between intake and energy may leadto overnutrition. Likewise, difficulties with eating may lead to the morecommon concern of undernutrition [98].

Nutrition in spinal muscular atrophySpinal muscular atrophy (SMA) type 1 (infantile) and type 2 (late

infantile) are characterized by progressive muscle weakness that can leadto undernutrition. Patients have associated weakening of the respiratorymuscles leading to fatigability and daytime somnolence as a result ofhypoventilation. They also develop diminished oral motor function. Theeffect is reduced oral intake, with a subtle but inexorable evolution tomalnutrition.

A primary issue is dysphagia. Children who have SMA frequently havedifficulties with the oral manipulation of food [99]. In addition to frequentbulbar involvement, the children often demonstrate craniofacial abnormal-ities that affect their facial structures and their ability to move the mandible[99,100]. Silent aspiration is a well-known potential entity but often notrecognized. The modified barium swallow offers the opportunity to simulatenormal feeding postures and visualize the anatomy from the oropharynxto the esophagus. Feeding specialists, primarily speech therapists, andoccupational therapists are specifically trained to recognize subtle oralpharyngeal aspiration in addition to esophageal dysfunction and reflux.Treatment strategies include positioning, thickening liquids, adaptation ofthe nipple, oral motor stimulation, and nasogastric tube supplement [98].Nasogastric and gastrostomy tubes may be the best option for some patients


who are at significant risk for aspiration or who are unable to sustainthemselves orally.

Often these children require tracheostomy because of ventilatory needs andto facilitate suctioning of secretions. This is at times a double-edged sword.Although there may be a reduction in the aspiration of secretions, thetracheostomy can adversely effect the phase of swallowing in which the foodbolus passes through the pharynx. Additionally, an inflated tracheostomy cuffcan result in esophageal compression. Furthermore, a tracheostomy maydesensitize the larynx, so that the patient becomes unaware of aspiration.

Malnutrition also can have psychosocial impacts. Children and youngadults spend a significant amount of time outside the home in the schoolsetting. Reasons why there are obstacles to adequate nutrition in schoolinclude lack of support services, inappropriate food textures, and timeconstraints. Additionally the child may not want the assistance required tobe fed and may choose to not eat. Associated depression also can lead todecreased interest in food. Food is an aspect of parental nurturing andparents often feel guilty and distressed when told the child’s nutrition isdeteriorating.

Nutrition in Duchenne muscular dystrophyChildren who have DMD often exhibit overnutrition, particularly in the

younger age group. By age 13, 54% of patients are obese. Undernutritionoften follows over age 14 with 50% of boys at age 18 demonstratingundernutrition [101].

A specific weight chart has been developed for children who have DMD,taking into consideration the progressive loss of muscle mass. Contributingstudies note children by age 6 have only 50% of the predicted muscle massand by 16 years only 20% [102]. Additional studies further validate thischart [101].

Obesity has significant consequences leading to difficulty with respiratoryfunction, decreased mobility, and low self-esteem [103]. The causes ofobesity are debated and seem multifactorial. An imbalance between energyexpenditures and intake has been hypothesized. The significant muscle lossreduces the resting energy requirements [104]. Additionally the low use ofpostabsorptive fat contributes to the obesity [104]. Whereas aggressiveweight loss contributing to increased muscle breakdown is a concern, calorie-controlled diets for boys with muscular dystrophy do not cause any long-term problems. Based on plasma creatine kinase activity and plasma andurine creatine concentration, there does not seem to be any sustained loss ofmuscle protein [103].

Other nutritional issues frequently are seen. The dystrophinopathiesaffect the smooth muscle in the gastrointestinal tract with resultanthypomotility and acute gastric dilatation with distension [105]. Feedingproblems associated with orofacial musculature abnormalities includemacroglossia and difficulty with mastication and malocclusion [99].


Dysphagia with pharyngeal weakness and abnormal contractions also arepotential contributors [106].

Corticosteroids are used in the management of the child who has DMD.This medication contributes to adverse nutritional side effects includ-ing weight gain, osteoporosis, and glucose intolerance. A low-sodium, low-carbohydrate, protein-sparing diet is recommended.

The nutritional and pulmonary status of these patients is important whenconsidering orthopedic surgery. Additionally in the postoperative period, anadequate nutritional state is essential. There is significant metabolic stressafter surgery; healing is slowed in the malnourished child [98].

General considerations in nutrition deliveryIn a child who has NMDs, nutrition delivery is individualized and adapted

depending on the inherent difficulties with the underlying disorder. Myotonicdystrophy patients have abnormal swallowing and associatedmyotonia of themasseters, and facial weakness leads to difficulties with mastication. Patientswho have myasthenia gravis also have difficulties with dysphagia. The abilityto obtain adequate nutrition may be influenced by many factors, includingfatigue, weakness, food refusal, swallowing difficulties, and fear of aspiration.

When it is determined that the individual can no longer be fed orally, thena percutaneous endoscopic gastrostomy (PEG) tube is considered for long-term management. PEG tube placements are much less invasive than in thepast. Often they require only conscious sedation and can be placed witha complication rate of less than 5% [98].Gastroesophageal reflux exacerbatingafter placement of the PEG is a concern. On the contrary, the author’sexperience and other recent prospective studies support that gastroesophagealreflux improves with nutritional rehabilitation [107]. Thus, once the child hasbetter nutrition, a fundoplication may not be necessary.

Frequent constipation is associated with poor feeding. Constipation iscomplicated by muscular weakness, lack of activity, and limited fluid intake.This can be addressed through dietary changes, additional fiber, and pre-parations such as lactulose or mineral oil. Oil must be used cautiously inanyone with dysphagia.

The nutritional management of a child who has NMD is complex and haseffects on many organ systems already involved in the disorders. Thedevelopment of an appropriate plan for comprehensive management iscritical.

Pulmonary management of neuromuscular patients

The respiratory failure that often accompanies NMDs significantlyinfluences the rehabilitation program. The insidious course of respiratorycompromise often is unrecognized and can lead to fatigue, increasingweakness, drowsiness, difficulty sleeping with frequent arousals, morningheadaches, and increased respiratory rate [108,109]. In patients who have an


NMD, a high level of suspicionmust be maintained. Customary managementincludes close monitoring of children who are at risk for respiratorycompromise as a result of their NMDs [110]. Those individuals who arerelatively presymptomatic should be evaluated every 6 months. Vitalcapacities sitting and recumbent, positive and negative inspiratory functionalcapacity, and assisted and unassisted peak flows often are recommended. Aschildren fall into higher risk categories, more frequent evaluations follow[108].

Etiology and pathophysiologyChildren who have NMDs reach their peak vital capacity before the

usual age of 19 years. As a result, they have a lower total lung volume vitalcapacity. In children who have DMD, vital capacity reaches its maximumbetween 10 and 15 years of age. The difference between achieved and normalmaximum is believed to correlate with the severity of the disorder andpredicts subsequent rate of loss of vital capacity [111]. Additionally patientswho have NMD have, by virtue of their underlying muscle disorders, furtherrestriction because of muscle compromise [108,110]. The weakness-as-sociated bony problems and even difficulties resulting from orthotics orspinal rods further complicate the picture.

The lack of ability to take deep breaths leads to microatelectasis. This mayoccur rapidly, particularly in an acute illness. With chronic hypoventilationand chronic microatelectasis, lung tissue is lost, as is compliance of the chestwall [108,109,112]. Chronic atelectasis and weak muscles lead to severaluntoward events. If untreated, chronic alveolar hypoventilation can occur andlead to cor pulmonale [109]. Additionally, hypocapnia, which occurs whenvital capacity is less than 55% of normal, can result in decreased musclestrength [113]. Acute respiratory failure directly correlates with increasinghypocapnia [114].

As Bach et al emphasize, children who have NMDs have greater weaknessof the expiratory muscles than inspiratory muscles. The pulmonologist oftenuses pulmonary function tests tomeasure inspiratory and expiratory pressuresand follows these over time to assess any trend of deterioration. Peak coughflow (PCF) correlates with the expiratory muscle weakness. Those patientswho have vital capacities below 800 mL, persistent PCFs below 4.5 L, andrepeated history of pneumonias are at high risk for acute respiratory failure ifthey become ill [108]. If patients demonstrate less than 300L/minonPCF, theyhave significant difficulty managing their secretions. Additionally, when theolder child’s PCF, either assisted or unassisted, cannot exceed 160 L/min, anindwelling tracheostomy tube is recommended for long-term management[115].

Blood gas abnormalities initially are seen during rapid eye movementsleep with hypercapnia and then hypoxemia [116]. There is gradualprogression of these abnormalities with continued deterioration of the


central responsiveness to hypoxia and hypocapnia. When PaCO2 is greaterthan 55 mm Hg during the wake state, there is significant SaO2 desaturationduring sleep, often below 85% [108]. There are several factors thatexacerbate this abnormality, including medications such as steroids, mal-nutrition, and illness. Supplemental oxygen is not efficacious and in fact is anerror in these patients. Studies show that oxygen supplementation prolongsapnea and increases respiratory failure in children who have DMD [117].

Children who have SMA have several factors that contribute to theirrespiratory compromise. The chest wall often is very flexible with paradoxiccollapse and a resultant decrease in lung volume during inspiration. Inchildren who have milder types, the restriction of the wall is less of an issue.All these children have exacerbation of their problems with infection andoften are seen to ‘‘tip over and then need further support’’ [108].

Multiple respiratory aids can be recommended by the pulmonologist.Selection must be individualized for the patient based on age, motivation,and mental capacity and also the capabilities of the caregivers. With thepossibility of altering the natural course of the disorder by greatly extendingthe expected lifespan, it is imperative that the treating physicians discuss thelong-term medical and ethical issues [118]. Home ventilation is a majordecision and the full impact of this on the family and child needs to be fullyreviewed long before the decision is made.

Bach et al have reviewed the appropriate candidates for ventilatorysupplementation and the options available. These include mouthpieceintermittent positive pressure ventilation, nasal intermittent partial pressureventilation, or intermittent abdominal pressure ventilation, oronasal inter-faces, and tracheostomy. The approach and management have changed dra-matically with the increased availability of technology [108,119].

Orthopedic management

Progressive weakness and muscle imbalance lead to significant ortho-pedic problems in children who have NMDs. The orthopedic surgeon’sinvolvement in the multidisciplinary team is pivotal for addressing theseissues. There are excellent comprehensive reviews of the orthopedic manage-ment of NMDs [120,121]. Reviewing the abnormalities in representativediagnoses provides insight into the approach to other children who havesimilar clinical findings.

Duchenne muscular dystrophyEarly childhood.

In the child 6 years old and under, the role of the orthopedist primarily isto observe for early signs of contractures. Therapists and other teammembers should give parents proper instruction in stretching. Thehope is that the stretching may help delay contracture formation.


Contractures develop in DMD for several reasons, including imbal-ance of the agonist and antagonist muscles, asymmetric weakness, andmuscle fibrosis [120]. A pattern consistently develops:

Feet: The equinovarus deformity of the feet is the result of contracturesof the gastrocnemius, soleus, and posterior tibialis and weakness of theanterior tibialis and peroneal muscles. The therapy for this is passiverange of motion and nighttime splinting with AFOs.

Knees and hips: The contracture of the tensor fascia lata muscle andfibrosis leads to hip flexion and external rotation contractures. There isassociated weakness in the quadriceps and gluteus maximus. Thetherapy is general passive stretch and knee immobilizers used at night.These contractures are not well tolerated because they reduce lowerextremity stability.

Ankles: Mild equinus contracture occurs at the ankles. The patientsusually tolerate the contractures at the ankles because the knees fallinto hyperextension. This allows added stability to compensate for thequadriceps weakness.

Flexion at the knees, hips, and ankles requires additional strength in thequadriceps to stabilize the lower extremity, a problem compounded by theunderlying weakness. With progressive instability resulting from contractureand weakness, the patient loses the potential for ambulation. Contracturesof the upper extremities also occur; however, patients tolerate these com-paratively well.

Late childhood. During late childhood to early teens, increasing contrac-tures evolve in the lower extremities in conjunction with a progressive loss ofstrength. Parents begin to notice a loss of exercise endurance and increasedinstability. As the patient begins to fall more, this frequently becomesa school safety issue. In addition to stretching exercises, knee ankle footorthoses (KAFOs) are added to help stabilize the knee [122,123]. KAFOsare appropriate only if there is less than 10� of equinus contracture at theankle and less than 20� of knee flexion [120].

With the progressive weakness involving the upper and lower extremities,walkers and crutches have limited applicability and lightweight braces arebetter tolerated. If contractures are significant and the patient continues tobenefit from bracing, then soft tissue surgical releases are recommended[124]. The procedure should be done when the child’s ability to walk ismarginal or within a few weeks after independent ambulation is lost[123,125]. Some investigators argue benefits of even earlier surgical in-terventions [126,127]. The surgical approach in the proper patient isbelieved to extend ambulation and evidence supports that patients receivingsurgery maintain ambulation for an average of 1.25 years longer [128].

Surgical correction classically includes the fascia lata of the hips,lengthening of the Achilles tendons, transfer of the posterior tibialis into the


dorsum of the foot, and potentially hamstring lengthening [120]. Someinvestigators believe a less extensive approach avoiding the hip musculatureis equally effective [121]. Children should begin ambulating as soon aspossible after surgery, usually within 24 to 48 hours, with aggressivephysical therapy to assist them. Because patients have reduced ability touse a walker or crutches, long leg casts often are changed to long legbraces [120].

Mobility is important developmentally but ambulation is not the onlyalternative. Ambulating with long leg braces is not an energy-efficientmethod, so a wheelchair for longer distances and community use is useful.Although a standard wheelchair is necessary, the patient can regain sig-nificant independence with a motorized wheelchair. Chair inserts shouldbe customized for support but there is no data to indicate that forcingany particular sitting posture influences the development of scoliosis[120,129].

Adolescence. Usually by adolescence children who have DMD have lostindependent ambulation and require full-time wheelchair use. Orthopedicproblems rapidly develop. Often it is difficult for patients to wear normalshoes because of the progression of the foot deformity, but this is largelya cosmetic issue. Scoliosis, however, becomes a significant worry. Datasupports that nearly 100% of children have progressive scoliosis afterambulation ceases [129]. Although bracing the curve and modifying thewheelchair may provide better sitting stability, they will not alter the pro-gression of the scoliosis [130]. It often is recommended that if the sco-liosis curve exceeds 30�, a posterior spinal fusion is considered. Once thecurvature approaches 30� to 35�, progression is a certainty. There is debateas to whether or not the progressive deformity causes deterioration ofpulmonary function or if the spinal fusion prevents this deterioration[121,131]. In addition there are debates regarding the effect on longevity.With progressive scoliosis, however, there is difficulty with sitting because ofthe mechanics of the trunk over the pelvis. Another argument for earlysurgery is to take advantage of a more stable pulmonary and cardiac status,lessening the morbidity of surgery and hastening postsurgical rehabilitation.Side effects and complications of posterior spinal fusion are estimated ashigh as 30% [120].

Spinal muscular atrophy

The incidence of SMA is second to DMD among the inherited NMDs.As with other weakening conditions, the earlier onset of the clinicalsymptoms correlates with increased severity early in the course of thedisorder. There are three types of SMA. Patients who have type 1 are themost affected, often with severe involvement evident at birth. Type 2patients may be diagnosed early in life, usually between 6 months and


2 years, and never have the ability to walk. Type 3 patients are older than2 years at diagnosis and although impaired, are able to walk.

Patients who are nonambulatory benefit from range-of-motion therapybut in spite of this often develop contractures in the upper and lowerextremities. In nonambulatory type 2 children, hip subluxation and dis-locations may occur [121]. Children who develop functional walkingmay require orthotics because of their associated weakness. LightweightKAFOs are recommended to stabilize the knee and ankle [120].

Scoliosis also is seen and is progressive in these children. There is debatewhether or not thoracolumbosacral orthoses are helpful in preventing theprogression of the scoliosis and delaying spinal fusion. They frequently areused in children younger than 10, but their efficacy is not proved and mostinvestigators believe they do not prevent the inevitable progression of thecurve [132,133]. Although there are some reports that they help posture,they may be restrictive from a pulmonary standpoint and must be followedclosely. The same precautions and considerations are taken into accountwith a child who has SMA or neuropathic scoliosis as with a child who hasDMD. The relentlessly progressive scoliosis occurs without intervention andmay lead to ventilatory collapse. Again, the surgeries are not withoutcomplications, which must be taken into consideration. From a rehabilita-tion standpoint, the self-help skills often are dramatically altered aftersurgery because the posture suddenly is changed to a completely erect one.Therapy intervention is important in this setting.

Hereditary sensory neuropathies

Charcot-Marie-Toothdisease, or the hereditary sensory neuropathies, is anexample of demyelinating or axonal neuropathies. The associated difficultiesare primarily distal, and foot deformities are seen in the majority of thesepatients. The primary presentation is foot drop because of the peronealatrophy and the tibialis anterior weakness. Additionally, there may be a cavusfoot with hind foot abnormalities and difficulties at the ankle joint. Thesepatients often have discomfort and pain, particularly if they overuse theirmuscles. The approach to these patients often is conservative, but manycontinue to have significant pain. Orthopedically, an articulating AFO is thefirst step that may offer some improvement. The surgical approach, rangingfrom a posterior tibial tendon transfer to a triple arthrodesis, usually isreserved for the severe or symptomatic patient who cannot tolerate orthotics.The underlying calf weakness is what presumably leads to the frequentcomplaints of pain and cramping and this persists even after the surgery [120].

Patients who have Charcot-Marie-Tooth disorder may have an as-sociated hip dysplasia with acetabular dysplasia and subluxation of the hip.Typically the patient presents with a Trendelenburg gait. Although thereare several approaches to hip reconstruction, because of the increased riskfor avascular necrosis of the femoral head, a more conservative approach


usually is recommended. Additionally, 10% to 30% of patients who haveCharcot-Marie-Tooth disorder also may have scoliosis by their mid-teens.Guidelines to address this have been developed and include periodicevaluations, spinal bracing for curvature between 25� and 40� in theskeletally immature patient, and spinal fusion for aggressive curves [120].

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