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DystoniaMedical Research Foundation

CONTRIBUTORS
of The expertise the following individualscontributedsigrrifrcantly of to the development this publication.Sincerethanksto: A. Leland Albright, MD Children'sHospitalof Pittsburgh of Departrnent Neurosurgery Pittsburgh,Pennsylvania Jeffrey Arle, MD, PhD LaheyClinic of Deparnnent Neurosurgery Burlington, Massachusetts Joel Blumin, MD Hospital Pennsylvania of Department Otolaryngology Pennsylvania Philadelphia, Guy Bouvier, MD Notre Dame HosPital DeparEnentofSurgery St. Larnbert,Quebec,Canada Blair Ford' MD Medical ColumbiaPresbyterian NeurologicalInstitute Center, NewYork,NewYork Mmiit Sangheta, PhD Baylor Schoolof Medicine Department ofNeurology Houston,Texas Beniamin L. ll/alter, fuID Emory UniversitySchoolof Medicine Departrnent Neurology of Atlanta,Georgia

This booklet was graciously reviewed by Scientific Director Mahlon R. Delong, MD, SchoolofMedicine EmoryUniversity of Department NeurologY Atlanta.Georgia

This bookletwasfundedin part by a grant from the Medtronic Foundation.
Projects Coordinator Feeley, Editor/Special Writtenby Jessica
ADystonta Medical Research Foundation' Prinled 12/03 ' 500

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TlsI,n I.Introduction..

oF CoNTENTS ...2

.....3 I I . P e r i p h e r aS u r g e r i e s . . . l ....3 CervicalDystonia./SpasmodicTorticollis PeripheralDenervation Selective The BertrandProcedure: Rhizotomy M icrovascular Decompression SpasmodicDysphonia/Larytgeal Dystonia. . . . . . . . .1 and Reinnervation SelectiveLaryngealDenervation Thyroplasty ........10 Blepharospasm.... Myectomy Surgery ....12 GeneralizedDystonia&Hemidystonia.. Intrathecal Baclofen III. Brain Surgery: Lesioning Procedures & .....15 D e e p B r a i nS t i m u l a t i o n Lesioning Procedures: Pallidotomy & Thalamotomy Deep Brain Stimulation Comparing Lesioning & DBS Children & Brain Surgery IV.Conclusion.. V.Appendix.... Dystonia The Nervous System Anatomy Eyes Neck Larynx VI. Sources ........25 ........26

.......31

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greatattentionis being paid to the As research aboutdystoniaprogresses, for Surgicaltreatments role of surgicalinterventions alleviatingsymptoms. that do not respondto oral for dystoniamay be an option for cases Researchers activelv or toxin injections. are medications botulinum and refining currenttechniques collectinginformationaboutwhich patientsmay benefitthe most from surgicaltreatments. that can be appliedto all forms of Thereis no singlesurgicalprocedure for dystonia. Surgicalprocedures dystoniacanbe divided into two broad brain surgery andperipheral surgery. Peripheralsurgery categories: includesprocedures that targetpartsofthe body otherthan the brain. In both brain and peripheralprocedures, goal of surgeryis to intemrpt the the faulty communication betweenthe brain and muscles that causes involuntarymusclemovements. Surgeryintendsto treat symptoms and improvefunctionbut doesnot curethe underlyingcondition. Because dystoniais a chronicdisorder,the management symptomsis of an ongoing,lifelongprocess. Justasmedications botulinumtoxin and injections oftennot singularsolutions an individual'sdystonia, to are surgeryis one component the total management dystonia.Surgery of of doesnot necessarily eliminatethe needfor additionalforms of treatment. However,in many casessurgeryimprovcsquality of life and reduces the needfor medications botulinumtoxin. Like all surgicalinterventions. or with the risk of certaincomplioperations treatdystoniaare associated to cations. The patientselection process determining an individual is a candiif for datefor surgeryis deliberate and precise.Only a neurologistor neurosurgeonwho specializes movementdisorders can recommend surgeryfor in and medicalcenter, dystonia. The cost of surgeryvariesby procedure and coverage often on a case-by-case is basisfor Medicareand private insurance. The success lies ofany surgicalprocedure heavily in proper team,and the skill and artistryof diagnosis, experience the clinical of the the surseon.

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The symptomsof dystoniaoccur when musclesof the body receivefaulty from thebraincausing information themto contract involuntarily. These faulty rnessages mostcommonlyin a partof the braincalledthe originate basalganglia.Thesemessages conveyed are over brainpathways the to into the muscles nerves. via spinalcord and,from the spinalcord,extend Peripheral surgeries occur outsidethe brain and generallytargetthe nervesand musclesaffectedby the incorrectmessages specific from the are usedto treatfocal dystonia. brain.Peripheral surgeries generally An exceptionis intrathecal baclofen,which targetsthe spinalcord and is used However,for the purposeof this to treatgeneralized hemidystonia. or publication,a discussion intrathecal of baclofenis includedunderthe categoryof peripheralsurgeries. CERVICAL DYSTONIA/SPASMODIC TORTICOLLIS The Bertrand Procedure: Selective Peripheral Denervation peripheral denertation surgery for cervical dystonia is Selective commonly referredto as the Bertrondprocedure.In the 1970s,Dr. ClaudeBertrand,with the collaboration Dr. PedroMolina-Negro, of procedureas a peripheralapproach treat cervical developed this to dystonia.The term selectiverefersto the caretaken to identify the nrusclesof the neck affectedby dystonia,and the tenn denen'ation refersto cuttingthe nerves that supplythosemuscles. The purpose the of Bertrandprocedureis to reduceabnormalcontractions the affected in muscles severing nerves thesemuscles. by the to The goal of the procedure is to leave intact the supply ofnerves to unaffectedor less-affected muscles. This procedure tailoredto address uniqueneeds is the and symptoms of eachpatient.The initial approach often to denervate muscles is the causingthe mostprominentdystonicmovement, knowing that some

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residual movements may remainfrom lesser-affected muscles. the If results not sufficiently do alleviate symptoms. second procedure a may be performed. manycases, initial surgery enough significantly In the is to improvethe abnormal posture. More aggressive surgeries, which all in cervicalmuscles involvedin the dystonia denervated a single are in operation,may result in temporaryweakness the neck. in An essential parl of the procedure the pre-operative is evaluationto properlyidentifythe muscles involvedandro assess theprocedure if will benefitthe individual. Patients who may be eligible for the surgeryare observed clinically by the physicianandwith EMG equipment monitor to muscleactivity and pinpoint the musclesaffectedby the dystonia. One basic elementof the Bertrandprocedureis to cut rootletsof the spinalaccessory nerve,which Figure I supplystemocleidomastoid Rotatory Torticollis Laterocolis musclesin the neck, and to sparethe nervesto the trapezius muscle.The spinal accessory nerveis one of 12 cranialnerves thatoriginate in the brainstem, which is the junction of the brain and the Super ntecollis sup; Retrocottis spinalcord.A second elementof the Bertrand *'vL k^ procedure cutting the is posteriorrami (branch)of one or more spinalnerves along the cervical vertebrae. I n f e r i o r A n t e c o l l i s Inferior Retrocollis (This element the proce.) of /"dureis calledoosterior ramiseclonn'.) Spinalnerves \/<4 s1- \ are arrangedin pairs along the lengthof the spinalcord and supplymusclesand organs.Some research The six elemental forms of spasmodictorticollis.

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in who that the suggests the ramisectomy increases irnprovement persons havebecome resistant botulinumtoxin tlierapy. to patients this To dateover2.000ceruical dystonia haveundergone proceimprovement as manyas 887'o in of repofi significant dure.Somecenters with a rangeof Althoughthe procedure may benefitindividuals cases. the who may havethe bestresults from symptoms, categories patients of procedure individuals which: are in the Berlrand . . . . . . . Symptoms mainly affectthe neck havestabilized 3 years for Symptoms The headturnsto oneside(rotational torticollis) The headis tilted(laterocolis) (rotational The headturnsandis pulledbackwards torticolliswith retrocollis) superior The headtumsandtilts forward(rotationai torticoliiswith superior antecollis) The headis pulledback(superior retrocollis)

Dystoniain which the headturnsboth to the sideand eitherbackor r.r'lro Indivrduals respond botulitbru'ardmay havethe bestoutcome. to rnay num toxin therapy well as non-responders be eligible.The as procedure may alsobe an optionfor a smallnumberof patients witli generalized dystonia who havevery definedsymptoms the neck. in in at Sideeff-ects may includenumbness the backof the head,tightrress rernaining thesurgery site,sorne movements. difficulty swallolving, and lack of beneflt.Patients often able to so homeafler two or three are nightsin thehospital. procedure significantly havedemonstrated the Bertrand that can Studies improle the posture the neckw'itha betterrangeof nrotion.Physical of fbllowing the procedure very important preserve therapy is to rangeof motion.

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Rhizotomy Sincethe late 19thcentury,physicianshave attempted treatcervical to dystoniasurgicallyby cuttingthe spinalnervesthat supplythe contracting muscles.While the ramisectomyelementof the Bertrandprocedure involvesexcisingspeciticbranches the ceruical of spinalnerves nearthe muscle(away from the spinalcanaland spinalcord),pioneeringsurgeons initially attempted removethe nerveat the root (insidethe spinalcanal to near the spinalcord). The procedureofcutting a nerve at the root is called a rhizotomy. Each spinalnerve hastwo roots: a dorsal(posterior)sensoryroot and a ventral (anterior)motor root. The sensolyroot conveyssensoryinformajoints andskin to the spinalcord,andthemotor tion from the muscles, root conveys from the spinalcord to the muscles. signals Cuttingthe sensoryroots doesnot alter dystoniabut doeshelp spasticity. Cutting the motor roots-which meanscutting 85-95%of the root innervatinga dystonicmuscle-will effectively the denervate musclebut at the costof inducing signifi cantweakness. Ventralrhizotorny dystonia was usedwrdelybetween 1930s for the and the 1970s was often combined with a denervation the accessory and of nerve.By destroying nerveat the root, the effecton the muscleis the more generalized may causea greater degree weakness. The and of procedures results these wereoverwheluringly of disappointing and patients. in The ventral caused high incidence complications dystonia a of was eventually rhizotorny a treatment cervicaldystonia as fbr replaced in the 1970s the Berlrandprocedure. by to in However, select medicalcenters continue incorporate rhizotomies and researchers their surgical approaches cer-vical to dystonia, continue to explorethe effectsof severing nervesat differentlocations the alongthe in surgical optionsfor cervicalvertebrae orderto provideadditionaI patients. dorsalrhizotomies commonlydoneto treat are dystonia Selective spastic stiffness the limbs(diplegia). of

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Microv asc ular D ecompres sion Microvascular decompressionsurgery for cervical dystonia is based on the ideathat variousblood vessels compress and irritate someof the (particularlythe spinalaccessory cranialnerves nerve),resultingin dystonicsymptoms. This surgicalprocedure involvesrelocating blood the vessels without injuring the vessels, nerves,or muscles. The relocated blood vessels held in placewith small implants.Sectioning nerves are of may or may not be includedin the procedure. There is very little publisheddataaboutthis procedure, and it has beenlargely abandoned for dystoniapatients. Adverseeffectsinclude lack of benefit,cerebrospinal fluid leakage, and stroke.There is evidence suggest to that if a patient undergoes decompression surgeryand is not satisfiedwith the results,the partial removal of the occipital bone and scarringthat results from microvascular decompression may make it difficult for a subsequent surgeonto perform a safe and effective Bertrand procedure.

SPASMODTC DYSPHONIA/LARYNGEAL

DYSTONIA

Selective Laryngeal Denervotion and Reinnemation Selective laryngeal adduction denervation and reinnervation (SLAD/R) is a surgicalprocedure treat adductorspasmodic to dysphonia/ laryngealdystoniaby cutting (denervating) selected end branches ofthe recurrentlaryngealnerye,which is a branchof the vaguscranial nerve. The first attemptsto reducethe spasms spasmodic of dysphoniaby severing laryngealnervetook placein the 1970s. the Cutting the laryngeal nerveparalyzed musclescontrollingone sideof the larynx so that the the larynx could not contractexcessively. Early resultswere good,but symptoms reappeared manypatients. in pioneersin the field Subsequent soughtto improvethe procedure varying the methodby which the by nerve was separated from the muscle.Recurrence symptomsas well of as breathyvoice continuedto be a problemin many patients.

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of The elementthat distinguishes SLAD/R from previousincarnations the surgeryis that, after the recurrentlaryngealnerve is cut away from the muscles, nervestumpsare the thyroarytenoid and lateralcricoarytenoid with hookedup to anothernerve(reinnervated), that is not associated one the the dystonia.Supplyingthe musclewith anothernerveprevents problematicbranchof laryngealnervefrom growing back and reconnecting to the muscle.Preventing laryngealnervefrom communicating the to the the muscleprevents spasms from returningand helpsto changethe is closingforcesof the larynx. It is importantto notethat the procedure performed previousnerveoperations performed for bilaterally,unlike adductorSD. Because disorderoriginates the centrallyin the brain,it likely existsbilaterallyin the larynx. It is therefore logicalto treatboth sides. The procedure accomplished makingan incisionin the neckand is by then creatinga small window into the laryngealcartilageto expose the underlyingnervesand muscles.An operatingmicroscope often usedto is aid in identificationand suturingofthe tiny nervebranches. The procedure takesthreeor four hours to complete.Greatcare is takento preservethe back part of the cartilagethat protectsthe nerve branches to the breathing muscles. SLAD/R is bestsuitedfor individualswith spasmodic without a dysphonia tremor.It may be an option for personswho are not satisfiedwith botulinumtoxin treatments. More than two hundredpersons with spasmodic dysphoniahaveundergone SLAD/R over the courseof about l0 years.During the initial recoveryperiod,all patientsexperience temporary voice breathiness someexperience and swallowingdifficulty. These issues resolveover a few monthsand the patientis left with a nearnormalvoice, free of spasm.Studieshave indicatedthat as many as 8590Yoof patients very satisfiedwith the resultsof surgery and the are results.so far. havebeenlife lons.

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Thyroplasty to surgeries includea group of surgicaltechniques modif-v 'fhese thecartilage surrounding larynx. and the adjustable reversible procedures wedees or involvemanipulating cartilage implanting by the :-;hims hold the tissuein place. numberof variations this procedure to A of are currentlyusedand are effectivefor restoration the voice after of paralysis in changing pitchof the voice. or the I thyroplasty beenusedfor the abductor has varietyof spasmodic dysphonia.In this procedure, vocalcordsare broughtclosertogether the in hopesof decreasing effectof the abductor Results are the spasms. mixed,with somepatients getting goodreliefandothers havingminimal effect. lI thyroplastyis a procedure adductorspasmodic dysphonia that for involvesspreading vocalcordsapartby inserting shimthatprevents the a them from contactingeachother during the spasms that occur with this disorder. Although somepatientshavereportedgood relief of vocal strain, othersfeel the trade offto a breathvand weak voice is excessive. Researchers the US and abroadcontinueto investigate in thyroplasty procedures. The advantage is ofthese procedures that they are largely non-destructive do not alter the musclesor nerve supplyof the and larynx. They work throughadjustment biomechanics of aloneand are theoretically reversible, althoughin practicethe reversibilitymay be limited by scarring. -lype 'lype 'fiwroplas\,

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BLEPHAROSPASM Myectomy Surgical rem.val'f the eyelidand brow-squeezing muscles ref'errecl is to as a mvecromv procedure is usedto treatblepharospasm. and Ml,ectomv prevents muscles the surrounding eyesfrom beingstimulated the by removing muscle. the tsefore availability the of'botulinum toxin,mvectomv wasessentiall;the '[-he only treatment optionfor blepharospasm. introduction ot'botulinunr toxin injections r989benefited in manypersons with brepharospasm tf ereby changing population individuals the of eligiblefo. myeciorn,r-.. candidates mv'ectomv fbr became thosefor whom botulinum torin rsnot sufficient. Justas the patientselection changed, procedure trre itselfevorved. lnitially,theprocedure involved removing eyelid-squeezing all muscres in both upperand lower lids as well as the brow areaat one time. At the present time,the procedure tailoredto the needs the patients. is is of It mostcommonfor the surgeon removethe muscre the upper to in eyerids and brow (full upper mvectomy) and then re_evaluate neld fo. a the lower myecromy a later date. patientshearfasterwhen the procedure at is done in stages, someindividuarsdo not requirethe rower and myectomy. The full uppermyectomymay be doneentirelythroughan eyebrow incision. The incisionliesimmediately adjacent thebrowhairand to allowsaccess the upperlid orbicularis to muscle, partof the rowerlid and orbicularismuscleas well as the procerusand muscres the in "o^rguto. brow area.Most of the orbicularismuscleis removedduring the eyelid surgery. strip of densemuscleis left at the margin of the upper A eyelid to help maintainsomevoluntaryclosureand to protectthe eyelash roots.

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A limited upper myectomyis a partial upper myectomy. It is available for thoseindividualswho arebenefitingfrom botulinumtoxin but need something extra to restorefunction of the eyes.lt may be helpful for thosepatientswho haveapraxia(difficulty openingthe eyes)or for those (drooping who in addition blepharospasm ptosis lids).Partial to have removalof the orbicularismay subsequently decrease needfor the botulinumtoxin in thesepatients. limited myectomyis donethroughan A uppereyelidcrease incisionand involvesremovalof the orbicularis musclewithin the upper lid areaonly. Because there is lesstissueremoval than the full uppermyectomy, patientsrecoverin lesstime. A limited myectomyalsogivesmorepredictable cosmeticimprovement because lesstissueis removed.lt is not designed replacea full upper to myectomy. Most patients will still requirebotulinumtoxin injections following the limited myectomyprocedure. who havestoppedresponding botulinum toxin as well asthose Persons to rare individualswho fail to respond all may be eligible for myectomy. at Individual surgicalcenters havetreatedhundreds ofblepharospasm patientswith myectomy.Techniques for cosmeticsurgery suchas used sculpting the fat beneaththe brow and manipulating the placementof the brow, may be implemented provide a beneficialaesthetic well as to as functionalresult. Myectomy surgerycan be done under local or generalanesthesia. The healingprocess following a myectomymay take up to a year.ln most cases, patientsare able to keeptheir eyesopen immediatelyfollowing the (blood accuthe operation. However,considerable swelling,hematomas mulationin lid), lymphedema (tissuefluid), and bruisingmay be present early in the post-operative periodand preventcompleteeyelid opening. Cool compresses the first four to five daysfollowed by warm comin presses very helpful at settlingthe lid swellingand bruising. are There are numerouspotentialside effectsassociated with myectomy predictable surgerythat are and,to somedegree, occur in most patients. Numbness the forehead of regionoften occursand is usuallytemporary

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but may last a year or more. Loss of tissuevolume in the eyelid areamay occurwith the muscleremoval,but the improvedbrow, lid position,and eyelid wrinkling generallygivesan improvedcosmeticappeardecreased ance.Decreased eyelid closureoccursas a result of eyelid muscle removaland may requirethe needfor additionalartificial tearsand lubricatingointment. the eyelidswellingresolves, eyelidclosure As the improvesand the dry eye symptoms generallyimprove.Chronic lid swellingwhich may last six monthsor longerin somepatientscan be a chronicandtroublesome complication. Chroniclid swellingis much less severe and persistent the modernmyectomypracticesin which upper in and lower lid myectomies performedseparately. are lnfection,hematoma, brow hair loss,and abnormalpositioningof the lower lid canoccasionally occurbut are uncommon. Patientscontinue to improve in function as well as in appearance for about six months to a year after myectomy surgery.Reports have shown thatvisualdisability is improvedin approximately90Yo patients. of Some patientshave more improvementthan others.Touch-up proceduresare requiredin somecases, someindividualscontinueto requirebotuliand num toxin injections. GENERALALED DYSTONIA & HEMIDYSTONIA Intrathecal Baclofen: The Baclofen Pump Baclofen(Lioresal@)is a medicationintroducedin the late 1960sas a treatment spasticity. for I'he medicationis alsocommonlyusedto treat selectcases dystonia.Baclofenin the spinalfluid aroundthe brain and of spinalcord supplements body'ssupplyof a chemicalneurotransmitter the calledGABA, which relaxesmusclemovement. The drug may be given orally,but very high dosesmust often be usedto ensure that the drug safurates blood streamand reaches spinalfluid. High dosesof the the baclofenmay causeintolerableside effectssuchas muscleweakness andfatigue.A surgicallyimplanted baclofenpumpdeliversbaclofen directlyto the spinalfluid, and only very small dosesare needed. (The term intrathecal meansin the spinal fluid.)

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Intrathecal baclofen therapyis a non-destructive, ustable, adj treatment. and reversible hundred dystonia Several patients havebeentreated u ith intrathecal baclofen over the course ofabout l0 years. It hasbeenusedfor children with generalized andadults (bothprimaryand dystonia secondary hemidystonia t and u,horespond baclofen. to with Many persons treated intrathecal havea baclofen combination dystonia of and cerebralpalsy.lntrathecal baclofenmay be usedto treat dystoniaaffectingthe upper andlowerlimbs.

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if In orderto determine an individual eligible intrathis for I tt tt'q tlt ec'qI bcr ktf'ert c he/she will undergo a ecalbaclofen, hutdvare in body. test the screening to obserue body's response baclofen. response the oral drug rnaynecessitate to A to a screening to observe body'sresponse a small doseinjected test the to is usinga standard directlyinto thespinalfluid. Thernedication injected lumbarpuncture spinaltap.The screening procedure test involves or injection the medication followedby several hor.rrs observation. of of purnpwill Relaxation themuscles indicates an irnplanted that baclofen of likely be eff-ective. The effectsof the screening test are telnporaryand may lastseveral hoursafterthe injection.If a patientdoesnot respond a1 all to the screening test,a second testusingthe sameprocedure may be tried the next day or at a later date.

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use as Somephysicians a continuous intrathecal infusionof baclofen a method, sincemorepatients respond continuous infusionthan screening to screening a to singleinjection doses. theinfusion In technique, small inserted is into the spinalfluid andis connected an external catheter to pumpthat infuses baclofenin increasing doses over two to threeda_vs. Starti g intrathecal l of'cn n bac i therapynvolves surgical implanting ly' a '[-he pump in thc body. pro-{ramrnable deviceis usuallyplaced eitherto the rightor left of the bellv button,beneath skin and fat of the abdothe '['he purnpis connected a thin tLrbe is tunneled rnen. to that aroundthe sideof thebodyto the back. srnallneedle A introduces tubeto the the spinalcanal. Oncethesurgical incisions closed. pumpis adjusted are the computerized by a remote deviceto deliverthe amountof medication for appropriate the individual.The procedure takesone to two hours.and the hospitalstay may rangefrom four to sevendays.Modest improvementof symptoms may be noticeable beforethe individualis discharged from the hospital, and it may takesix months moreto achieve full or the extentof benefit. Regular maintenance a key component intrathecal is of therapy. baclofen Regular examsand physicaltherapymay be a component postoperaof tive care.Pumpsmustbe refilledeveryoneto four monthsin the physician's ofltce as a straightforward outpatient procedure. The pump is refilledby inserting thin needle a the through skin,into thepump.The frequency refillingthe pumpdepends the doserequired. necesof If on sary,the doctor may adjustthe delivery rateof the pump at the time of the refill by remotecontrol.The pump batterylastsapproximately seven years,depending how much medicineis programmed be delivered on to eachday.Before the batteryruns out, the pump will needto be replaced with a new pump througha surgicalprocedure. The cathetercan usually stay in placeand be reconnected the new pump. to Baclofenin the spinalfluid relaxesmuscles throughout body,and the appears especially effectivefor targetingdystoniain both the upperand lower half of the body.ITB may be more effectivefor treatingsecondary dystoniathanfor primarydystonia. t4

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Studieshave shown that intrathecalbaclofen can dramatically improve havereportedsignificant and quality of life. Somecenters symptoms in of However,like any surgery irnprovement as much as 85Yo patients. may also is the procedure not without risks.Hardwarecomplications arise including infection and catheterbreakageand disconnection.In a of small percentage cases, symptomsmay resumeor worsenwithin the decreased first year.The most common side effectsare constipation, musclecontrol,and drowsiness.

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The goals of brain surgery for personswith dystonia are to decrease increase musclespasms, mobility andfunction,and improvepain. There are currently two categoriesof brain surgery for dystonia: lesioning procedures, which involve selective destruction of targeted, abnormal brain tissue, and deep brain stimulation (DBS), which mimics with nonthe effectsof lesioningby manipulatingselectivebrain areas destructive electricalpulses. Although risks exist,casestudies haveshownthat both lesioningproceduresand DBS can result in markedimprovement dystoniawith of or minimal complications. Somepatientsare able to decrease altogether stopdrugtherapyfollowing surgery. Dystoniamost often originatesin a part of the brain called the basal ganglia which are involved in the coordinationand control of muscle movements. The basalgangliaare a group of structures that includethe globus pallidus (also called the pallidum), the thalamas, and the subthalamic nucleus.Lesioningprocedures dystoniausually targetthe for globuspallidusor the thalamus; the deepbrain stimulationusuallytargets globuspallidusor subthalamic nucleus. The globuspallidusis responsible for the outputof messages from the basalganglia.The recipientof this output is the thalamus. The subthalamic nucleusis a tiny structurelocated directly beneath thalamus the to and is connected the globuspallidus.

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a Different partsof the brain work togetherto help the body accomplish specifictask,suchas tappingthe foot. 1-hepartsof the brain communimesof catevia pathways individualbraincellsthattransmitchemical indrvidualwith dystonia,the pathways from one to the other.In an sages that facilitatethe movementof the foot are disruptedby abnormal activity.The goal of brain surgeryis to freeup the pathwaysso that the the function-in this case, brain andbody may accomplish intended movingthe foot. Brain surgerymay be performedunilaterally(on one sideof the brain) or bilaterally(on both sides).The effectsofsurgery occur on the side ofthe to body opposite the surgicalsite. brain surgeryfor dystonia To date,most personswho haveundergone for were treated generalized dystonia.However,individualswho may be or with focal, segmental, genereligible for brain surgeryincludepersons with significant, disablingsymptoms that do not respond alizeddystonia to Adults aswell as childrenwith primary satisfactorily othertherapies. and secondary dystoniamay be eligible. Basedon the limited availabledata,different categories patientsmay of responddifferently to brain surgery.Although casesof both secondary (includingtardivedystonia)andfocal dystonias may be eligible, dystonias with DYT-l generalized dystoniaare the it appears thoughpersons as ither lesioningor DBS. Studieshave bestcandidates brain surgery---e for improvementin DYT-l patientstreatedwith shownas much as 60-90%o with secondary hemidystonia may b9 eligible lesioning DBS. Patients or for brain surgery,though they may not benefit as much as thosewith DYT-l dystonia.Researchers examiningthe possibilitythat persons are with secondarydystonia may get greaterbenefit from lesioning or DBS to the thalamusrather than the globuspallidus. There is limited data about the long-term effects of each approach.Brain surgeryfor dystonia is an evolving science,and investigatorsare continually collectinginformation.

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Lesioning Procedures: Pollidotomy & Tholamotomy 'l'he practice lesioning patients partsof the brain in dystonia wasverv of comnlon the 1950s in since thattime it wasessentially at the and 1960s, procedures. practiced only available treatment severe for cases. These as 50 'vears In thc u'as ago,had mixed results. sonre cases inrprovement developed; in still other spectacular; othercases in and complications casesrepeated procedures By were necessary. the 1980s,brain surgery for dystoniahad fallen out of favor and was not widely practiced. However,the increased understanding the basisof movement of disorders suchas Parkinson's disease in and the success treatingit with surgical plus the development brain imagingtechnology, to a reapproaches, of led evaluationof surgeryas an option for patientswith dystonia. The procedure that involvescreatinga destructive lesionin the globus pallidus is called pallidotomy,and the procedure that involvescreatinga lesion in the thalamusis calledthalsmotorny.A permanent lesion is made in the brain tissueby heatingthe tip of an electrode and coagulating the intended tissue. When lesioningsurgeryis chosen, pallidotomyis now preferredover thalamotomy and providesa reasonable alternative pallidal DBS for to patientswho are averseto the cosmeticappearance the implanted of pulse generator do not want to be burdened repeated or by battery replacements. Bilateral pallidotomyhasshownan average 67-80%o of improvement the Burke-Fahn-Marsden in dystoniarating scalein patients with generalized generalized patientsrespondbetter dystonia.Primary than focal or secondary patients, dystonias. Parkinson's ln bilateral pallidotomies avoidedbecause are they cause hypophonia, quietingof a speech. This hasnot beenobserved dystoniapatients,and many in dystoniapatientshavehad bilateral pallidotomieswithout significant worseningof speech. Although thalamotomywas once the most common brain surgeryperformed for dystonia,it is now usedalmostexclusivelyin cases stable of hemidystonia, a very specificsite in the thalamusis targeted. and The

is increase the procedure performedunilaterally.Bilateralbrain surgeries and in risk of complications, bilateralthalamotomies particularareknown to often causespeechimpairment. The primary factor that distinguishesmodern lesioning proceduresfrom are thoseof 50 yearsago is that surgeons able to locatethe lesioning The following factorsmake it much easierfor the targetmore accurately. surgicalteamto locatethe targetwithin the brain,which is crucialto : reducing risk of complications the . Stereotaclrcs-Surgeons are able to target the precise area of the brain with a computerized, 3-dimensional scaleusingMRI and CAI scans. Microelectrode recording and brain mapping-The surgical team hasthe ability to listento the sounds brain cells firing messages of to one another.Cells in different parts of the brain fire at very specific ratesand in characteristicpatterns,and by listening to thesecells the surgeon knows exactly wherethe electrodeis within the brain. Severalrecordingtractsmay be necessary identiff the precise to target.

.

Once a physician has recommendedbrain surgery and pre-operative screening testsand preparations complete, basicplan of operation are the for pallidotomyandthalamotomy the same. are The individual is fitted with a headframe under generalor local anesthesia. brain is mapped The with imagingtechnology create blueprintfor planningand measuring a to the placement the electrode. of Under local anesthesia, electrode is the inserted througha smallhole in the skull into the brain.The brain itself doesnot feel pain, and the patientis awakeduring most of the procedure. The surgical team interactswith the patient throughoutthe procedure,and the patientprovidesfeedbackaboutsymptomsand how he/shefeels. recordingis usedto confirm the target.The mapping Microelectrode procedurealone may take up to severalhours. Once the target is defined, the surgeoninsertsthe thermal electrodeand createsa lesion. The thermal electrodeis removedand the procedureis complete.A bilateral l8

procedure may be done in a single surgeryor in two separate surgeries. lf a second target is to be lesioned, mappingprocedureis repeated the for that specifictarget.Most patientsare in the hospitalfor two or three days.IVledications may be temporarilyresumed, and after a short time the patientreturnsto the neurologistfor a follow-up exam. 'l'here is a smallbut realrisk of complications associated with lesioning. 'l'he mostserious incidence strokeor hemorrhage risk is a 1-2Yo of during the mappingphaseof the surgery. Also, the targetof the pallidotomy,the internal segment the globus of pallidus, located is right above optic the tract which may be damaged the electrodeis not targetedprecisely. if -l'here alsoexiststherisk thatthe pallidotomy will not improvethe symptoms.Howeveqthe procedure beenshownto dramatically has improve dystonia some in patients.

Deep Brain Stimulation Deepbrainstimulation (DBS) involves implanting stimulating electrodes into selected targetsin the brain in orderto mimic the effectsof lesioning. Surgeons began usingDBS in placeof lesioning Parkinson's for disease patients the mid-1990s. in DBS alsohasapplications tremorand pain. to WhereasDBS has beenusedto treat thousands personswith of Parkinson's procedure disease, the began beingapplied dystonia to only in the late 1990s. The resultsof more than 200 dystoniapatientshave been published casestudies medical journals. as in BilateralpallidalDBS produces significantbenefitin dystoniawith averageimprovements about 50-60%in the Burke-Fahn-Marsden of dystonia ratingscale. Someprimarygeneralized patients havebeen repoftedto haveup to 90% improvement. DBS has also beenperformed on persons with secondary dystonias, cervicaldystonia,segmental dystonia, andmyoclonicdystoniawith encouraging results.

l9

Figure 3 The complete DBS apparatus includes the DBS electrode. a connecting wire, and a pulsegenerator "brain (a.k.a. pacemaker" or stimulator)that contains a battery.The initialprocedure to implantDBS Deep brain stimulation horchrore in body. is identical that to of the pallidotomy andthalamotomy. and Oncethe braintargetis rnapped places DBS identified, instead creating lesion, surgeon of the a the electrode into the target. The wire and pulsegenerator may be irlplanted at the sametime as the electrode at a laterdate.The generator is or implanted The ri ire is then underthe collarbone in the abdomen. or (the tunneled tlie neck,behindthe ear.and to the siteof the electrode up patientis undelgeneral anesthesia this part of the procedure). fol The wire is connected the electrode. the incisions closed. to Most and are DBS procedures involvethe irnplantation two generators aredone of anc'l in two surgeries. is possible implantbothgenerators a single It to in patient. for lrnmedisurgery, this is a very dernanding but approach the the may terrporarily resume atelyaftertheoperation, patient uredications. The patientmay be discharged next day. the

is the Oncethe generator implanted, patientmustwait a week or two are This waiting periodis necessary to beforethe batteries activated. with thesurgery diminish. The allow the swellingthatnormallyoccurs to pulses into the brain usingpower DBS electrode electrical conveys produced the batteryin the generator. series visitsto the hospital A of by to the needs the individual. It arerequired adjustthe voltagesettings to of mav take severalweeks or monthsto achievethe correct settines.The

patientcan checkthe statusof the generator using a handhelddevicethat resembles TV remotecontrol. Using this device,the patientcan a is determineif the generator on or off, and can turn it back on in the (Certainphenomenon suchas eventthat it shutsdown unexpectedly. by magneticfields caused securitydevicesmay causethe batteryto temporarilystopworking.) The expectedlife spanof a batteryat a typical voltage is about3-5 years. At a very high voltage,the batterymay needto be replacedafter a year, at a very low voltage,perhapsup to sevenyears.Replacinga batterycan be done undergeneralor local anesthesia an outpatientprocedure. as Dystoniadoesnot respondto DBS in the samemanneras other movement disorders For example,persons do. treatedfor tremor will generally improvewithin seconds turning the generator In patientswith of on. dystonia,improvement may be delayedfor days,and weeksor months may passbeforethe full extentof the benefit is reached. DBS doesnot necessarily drug or botulinumtoxin eliminatethepossibilityof subsequent treatments. Sideeffectsare minimal, but no procedure without risks.The main risk is in DBS is a fatal hemorrhage. However 99-99.5%of patientsdo not have sigrificant bleeding.Despitevigorouseffortsto avoid it, infectionis a risk in approximately 2%o patients.lnfection can be seriousand warrant the of removalof the hardware.If this happens, may be possibleto re-implant it the hardwareonce the infection is treated.Hardware failure is also a concern,thoughthis is rare and precautions in place in the eventof are situationssuchas a batteryfailing. lt is estimated that in 5% of DBS procedures dystoniasomecomplicationmay arise,most of which can for be addressed without removingthe hardware. Although no longerconsidered "investigational" dystoniaby the Food for & Drug Administration,DBS is in its infancyas a treatment this for preliminaryresultsare quite positive,and the procedure disorder. The is expected evolve over time as more patientsare treatedand more data to is collected.

Comparing Lesioning & DBS haveshownthat both lesioningand DBS can dramaticallyimStudies provedystonia.Both approaches associated are with a small,but real, -fhere hasnot beena clinicalstudyto compare risk of complications. the procedures DBS. andthe advantages disadand results lesioning of and of vantages eachremain an open issue. procedures DBS havemanyelements commonincluding: and in Lesioning . . . . . criteria Patientselection Area of brain targeted Basicsurgicalprocedure Potentialfor profoundbenefitto eligiblepatients Risk ofcomplicationsincludinghemorrhage duringsurgeryhemiplegia or hemiparesis, impairments, sensory speech/language impairment

In both cases, chanceof benefitmust be weighedagainstthe risk of the of complications. two cases dystoniaare alike, and determining No the specificapproach treatment-in this caselesioningor DBS-must be to decidedafter carefuldiscussions amongthe patient,family members, neurologist and neurosurgeon. Of the dystoniapatients who areeligible for brain surgery more individufor als are currentlybeingrecommended DBS than pallidotomy.The pallidotomy,howeveqis by no meansan obsolete procedure. Unlessa patientis against havinghardware installedin his/herbody,the tendency is to try DBS beforeproceeding the pallidotomybecause to DBS is adjustable reversible. and Financialand geographical issues cannotbe overlooked. who Persons haveDBS must visit the doctorregularlyfor maintenance check-ups. Peoplewho live in remoteareas areasnot in proximity to a major or movement disordercentermay be at a disadvantage. Travelto and from the center-and the expense this travel-is a part of the ongoing of management requiredof DBS patients. 2.

lesioning in thereis Llecause creates permanent a change the brain tissue, complications such higherrisk of permanent duringthe surgery a slightl;difflrcultv. cerebral and hemorrhage. asswallowing difficult1,, speech DllS involves implantation hardware, of complications Because the ':ssociatedith theapparatus possible. * are including infection. erosion 'l-he the failure. risk of ilrrr-rugh skin.hardware breakage. stimulator and is irardu'are complications existsfor as longas the hardware implanted. or It rernains be seenwhetherthe pallidotomy DBS is moreeffective lo of and rnedical teamare the thanthe other.l-he experience the surgeon 'l'he of of lowestincidence inostimportant detenninants success risk. and proceoccursin majormedicalcenters that performthese corrrplications ofierr. should with a long-standing Jrrres Patients choose center a exper'iisein rnovenrent disorders a clinicalteamdevoted surgery and to fbr A and movement disorders. movement disorder neurologist a and dl'stonia shouldbe specially trainedin functional surgeryandan electrosurgeon physiologist An nursing shouldbe on stafffor brainmapping. experienced staffis alsoimportant. Patients both categories brain surgery of of may benefitfrom physical therapy and supportive followingthe procedure. therapy

Lesioning
Controlleddestruction Non-destructive

Few post-oprestrictions

Common-sense restrictions regarding activity; must avoid Hardwaremay be slightly visible beneathskin in some people

No cosmetic issues

B

Children & Brain Surgery are andDBS, alChildrenoverthe ageof seven eligiblefor lesioning waits.the lessbrainand skin growthwill occur the though longerone l]orvever. thereis linle dataavailable aboutlong-term afterdreoperation. lopmentmay affectthe hardware. of efl'ects DIIS and how a child's deve to can can bc takenduringsurgery ensurcthat the apparatus Steps the may accomnrodate chrld'sgrowth.Childrenand adolescents be at a from DBS because general rather slightlyhigherrisk of complications 1s localanesthesia used duringimplantation post-operatively and than in childrenaremorelikely to engage roughplay that may affectthe hardware. haveto be considered only as a last resort. Surgerydoesnot necessarily respond a less to Certainly,if an individualis satisfiedwith how symptoms toxin or medications, suchasbotulinum thereis no invasive treatment However, early needto consider brainsurgery. especially children, in qualityof life.The benefits brain maysignificantly improve intervention of surgery includemorethanimprovedmobility-a child's ability to function and comfortablyat school(bothacademically socially),to makefriends, and to be activeare importantfactorsto consider.In both childrenand adults,brain surgerycan drasticallyimprovepain,which is often a major quality of life. component a person's to

z+

IV. Coxcr,usroN
Having surgeryis a very significantstepfor an individual to take in the treatrnent ofdystonia. Ifyou are consideringsurgeryor ifsurgery has the beenrecommended you by a movementdisorderspecialist, followto with your doctors: ing questions may helpyou initiatediscussions . . . . . . . . . . . . . What is the nameof the operationand what doesthe namemean? for Why is this specificsurgeryappropriate my case? of What are the advantages having surgery? What benefitsmight I expect? What are the risks? Are their alternative What happensif I don't havethe surgery? treatments? Where can I get a secondopinion? What is the experience the medicalcenterand surgeon of with this procedure? Doesthe medicalteam publishthe resultsof surgicalcasestudies? Wherewill the surgerybe done? What kind of anesthetic will be used(generalor local)? How long is the recoveryand what rehabilitationis necessary? How much will the surgerycostand who will pay for it?

Surgicalprocedures may improvefunction and betterthe lives of patients who do not receiveadequate relief from medications and/orbotulinum toxin injections. patientwho is considering A surgerymust weigh the opportunityfor benefitandthe risk of complications. Carefuldiscussions with movementdisorderspecialists being as knowledgeable and as possibleaboutdystoniaand surgerymay aide in the consideration process.The DystoniaMedical Research Foundationcan provide namesand contactinformationof dystoniaspecialists. Until a cure for dystoniais achieved, researchers working diligently are toward developing treatment optionsto improvethe lives of affected individuals. Surgery an areaof research which vastprogress being is in is madeand in which the prospect developingmore effectivetreatments of promising. is tremendously

V.Appnuox
Dystonia disorderthat causes Dystoniais a neurologicalmovement musclesin the body to contractor spasminvoluntarily.The involuntarymusclecontracand patterned movements well as as tions causetwisting, repetitive, postures. abnormal but that Dystoniais not a singledisease a syndrome-a set of symptoms Some cannotbe attributedto a singlecausebut sharecommonelements. forms of dystoniamay affect a specific body area,suchas the neck, face,jaw, eyes,limbs, or vocal cords.When dystoniaaffectsa single body area,it is called/ocal dystonia.Focal dystonias includecervical oromandibular dystonia, writer's cramp,and dystonia, blepharospasm, dysphonia). Segmental dystoniaaffects laryngealdystonia(spasmodic lf body two or more adjacentbody areas. two or more non-adjacent areasare affected, the dystonia is termed multifocal. Generalized dystonia refers to dystonia that may affect the limbs, trunk, and other on major body areassimultaneously. When dystoniaonly affectsmuscles Although the outward one side of the body, it is calledhemidystonia. appearances the various forms of dystonia may appearvery different, of they all sharethe elementof repetitive,patterned, often twisting and involuntary musclemovements. Dystoniaaffectsmen.women,and childrenof all agesand backgrounds. Dystoniamay be geneticor causedby factorssuchas physicaltrauma, conditions. or exposure certainmedications, otherneurological to Dystoniais the third mostcommonmovementdisorderafter Parkinson's 250,000personsin North disease and tremor,affectingan estimated America.Nonetheless, dystoniais often misunderstood the public and by misdiagnosed medicaldoctors.Dystoniais neithera psychological by disorder, doesit affect intellect.Dystoniais not fatal, but it is a nor varying degrees disability andpain, from of chronicdisorderthat causes mild to severe. 25

Tlrc Nervous System & Brain The nervoussystemis dividedinto two parts:the centralnervoussystem (CNS) and the peripheral nervoussystem(PNS). The CNS consists the of brain and the spinalcord. The PNS consists the of nervesextendingfrom the spinalcord. Thesetwo systems are for responsible all bodily ranging from activities, heartrateand muscle rnovement emotions to and leaming. The brainis the most complex intricate and organin the humanbody. graymass The r.vrinkled thatmakes 80% of the up brainis called cerebrql the corler. This part is responsuclr siblefor activities as perceiving, thinking, and

Figure 4

CentralNen'ous Systenl Brain and Spinal Cord

PeripheralNenous

Cen'ical region Thoracic region

Lumbar reqion Sacral region

A na|om.t' of the rtet'vottr .i l s/er?

producingand understanding The cerebralcortex is divided into language. trvo sidesor "hemispheres"-the right and the left. Although both henrispheres appearidentical,they differ in purposeand firnction. Language. r e a s o n i n ga n d l o g i c c a p a b i l i t i e o r i g i n a t e n t h e l e f t s i d eo f t h e b r a i n , i s whereasappreciation shapes and texturesand artistic talentsoriginate in of the right side. The cerebral cortex is further divided into four sections or "lobes"-frontal, parietal,temporal,and occipital.The frontal lobe is involved in movement

Figure 5

Basal

ganglia of I e vi ew.

sncl related stnt(tttres ilrc brain--pt'ofi

B a s a lG a n g l i a G l o b u sP a l l i d u s Thalamus Substantia igra N Cerebellum

and decision-makin-s skillsl the parietallobe interprets touch, pain. and lobe is involved in hearingand memory; and the temperatllre: temporal the occipital lobe containsthe vision center. Beneaththe cerebralcortex lie structures that help us move, sleep,wake, breathe,smell. hear,see,taste.and eat. Dystonia affectsan area in this deep part of the brain Lrelieved re_er"rlate to lrovement calledthe basalean_slia. An i r n b a l a n c e t ' b r a i nc h e m i s t r .c a u s e sh e b a s a lg a n g l i at o s e r r d r r a p p r o p r i a t e o y t i messages the muscles.causingthem to contractand spasm iuvoluntarily. to Artatomy
Orbicularis oculi
L o1111u 1.11 1 >\ ,'

Figure 6

Eyes Theorbicularis oculimuscle the of encircles opening theeye socketand actsto closethe The corrugator eyelids. r.nuscle draw's eyebrollstogether the and wrinklesthe brorv. The procerus betrveen muscleis a facialrnuscle the eyebrows and down the nose.

Pr,,..-rur-;P
Y..i ,j*:{'

&
Ey'entttsclesin pro/ile

.t

J

28

Neck The stemocleidomastoid and trapezius muscles major are muscles the neck.The two in stenrocleidomastoid muscles are thick muscles eachside on ofthe neckthat act to bend, rotate,flex, and extendthe head.The trapeziusmuscle movesthe shoulder blades upward in a shrug.

Figure 7

-ilt*r.../
-: ,,/
S t e r n o ce i d o mas t o i d l

Trapezius

Anatomy of the neck.

Larynx The larynx or "voice box" is an organ in the neck that plays a crucial role in speakingand breathing.The framework of the larynx is madeup of the thyroid cartilage. The front portion of the thyroid cartilageis visible in somepeopleas the "Adam's apple." The vocal cords are locatedin the centerof the larynx. The thyroarytenoid muscleis responsible closing for the vocal cords,and the posteriorcricothyroidmuscleis responsible for openingthe vocal cords.
Figure8
Posterior cr i co a r y t e n o d i

Thyroarytenoid muscle

Lateral c r ic o a r y t e n o d i muscle

Inside larynr, viev'-from above

D

Larynx Figure 9

(continued)

Outer cartilage of larynx,angled viewfrom front

Figure l0

Cartilage , Thvroid of I .. cartilage \larynx Cricoidcartilage
t"i-'o:'

Stemocleidomastoid Trapezius Supraclavicular .. fossa

;;&,

ofthe neck. Surface

VI. Souncps:
''Surgery Panel" presentations 7d Worldwide DystoniaPatientSymposiurn 2"d at & I-'amily Symposium. November 8-10.2002. "tJpdateon Spasmodic Dysphonia"& "Surgical Options for Dystonia" Presentations at M id-Atlantic RegionalSymposium,May 12, 2003. Activa@ TherapyFact Sheet,Medtronic (2003) <http'//medtronic.com./ downloadabl les/dystoniaHDE-ActivaTherapyFactSheet.pdt> efi Be lnformed: Questions Ask Your Doctor BeforeYou Have Surgery, to AHCPR Publication #95-0027.January1995,Agency for Healthcare Policy and Research, Rockwell, lVlD < www.ahrq. gov/consumeri surgery. htm> Hyman-NewmanInstitutefor Neurology & NeurosurgeryBeth IsraelMedical Center, New York, NY <http://nyneurosurgery.org> I fB@ TherapyFact Sheet,Medtronic (2001) <http'/lmedtronic.conlidownloadablefiles/ ITBTherapyFS.pdD I- Albright, MJ Barry,DH Shafton,SF Ferson,Intrathecal baclofenfor generalized dystonia,Developmental Med Child Neurol 2001; 43:652-657 L Albright, lntrathecalBaclofenfor SevereSpasticity, Part I & 2. reprintedffom ExcepNovember 1996<www.medtronic.com> tional Parent. GS Berke,KE Blackwell,BR Genatt,AVerneil, KS Jackson, Sercarz, JA Selective larl'ngealdenervation-reinnervation:new surgicaltreatment adductorspasmodic A for dysphoni4 Ann Otol Minol Laryngol 1999;108:227-231 Berke GS and Blumin JH: Spasmodic Dysphonia:Therapeutic Options.Curr Opvt Otolaryngol.2000;8(6):509-53 I P Coubes, Roubertie, Vayssiere, Hemm, S Tuffery B Echenne. Frerebeau. A N S P Earlvonsetgeneralized dystonia:Neurosurgical treatment continuousbilateralstimulationof by the interbalglobus pallidus in l5 patients, Neurology,2000 (suppl.3),332.002(Abstract) E-Move reportsflom 7s IntemationalCongress Parkinson'sdisease of and Movement Disorders,November10-14,2002 (MovementDisorders2002: 17 (suppl. 5) P95I ; 5289290 B Ford, Surgeryfor Parkinson's Disease:AGuide for Patients, Families.& Caregivers, Parkinson's Disease Foundation(2003)

3l

SM Kitgore,H Bronte-Stewart, Outcomeof palladialsurgeryin primary vs, secondary (suppl7); 58, P05.l5l; ,4.395 dystoni4Neurology,2002 of A Lang, SurgicalTreatment Dystoni4 Dystonia3: Advancesin Neurology,Vol 78, 1998. J A Muenchau, Plamer,D DressleqN Quinn,A Lees,KP Bhati4 Selective peripheral denrvation with posterior primary ramisectomy is useful for botulinum toxin resistant patientswith cervical dystoni4 Neurology 1999; 52(suppl.2):M94 (Abstract) MY Oh, AAbosch, SH kim, AE Lang, AM Lozano, Longterm hardware-related complications of deep brain stimulation, Neurosurgery,2002;50 1268-1276(Abstract) T Taira, T Kobayashi, K Takahashi,T Hori, A new denervationfor idiopathic cervical dystoni4 J Neurosurg2002, Sep;97(2suppl):201-6(Abstract) RH Walker, DM Sope, FO Danisi, IM German, RR Goodman, MF Brin, Intrathecal baclofentherapyfor dystoni4 Neurology 1999;52 (suppl.2);4521 JL Vitek. M Evatt, IY Zhang, V Chockan, MR Delong, S Triche, RAE Bakay, Pallidotomyand deepbrain stimulationas a treatment dystoni4 Neurologl 1999; for 52(suppl.2):A294(Abstract)

Images
Figure l-l'arsy, D., Vitek, J., Lozano, A., Surgical Treatmentof Parkinsonb Diseaseand Other MovementDisorders,Totow4 NJ: HumanaPress,2003. Figure 2-Image provided courtesyof Medtronic. Figure 3-Image provided courtesyof Medtronic. Figure 4-Dystonia Medical Research Foundation,8-18 Guidebook,1994. Figure S-Henkel,J, "Parkinson'sDisease: New Treatments Slow Onslaughtof Symptoms," -FDl Corxumer: The lvlagazineof the U.S. Food & Drug Administration,Yol.32, No. 4. July-August1988. Figure 6-Gray, H., Anatomyof theHuman Body, Philadelphia: Lea & Febiger,l9l8; Bartleby.com, 2000. www.bartleby.com/07l. | | I 1212003. I Figure 74ray,H., Anatomltof theHyman Bod,, Philadelphia'.Lea&Febiger,l9l8; Bartleby.com, 2000. wwwbartlebv.com/ 07/. | | I 1212003. I Figure 8-Gray,H., Anatomyof the Human Body,Philadelphia: Lea & Febiger,l9l8; Bartleby. com,2000. www.bartteby.com/ I 07/. | | I 1212003. Figure9-Gray.H.,Anatomltof HumanBody,Philadelphia: the Lea&Febigeq l9l8; Bartlebv. com.2000. www.bartleby. com/I 07l. | | I 1212003 . Figure lO-Stark.F., Gray s Anatomy: A FacrFilled Coloring BooN Philadelphia: RunningPressBooks, I 991.
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