Selection of Treatment Modalities in Children With Spastic Cerebral Palsy

Paul Steinbok, B.Sc., M.B.B.S., F.R.C.S.(C) Neurosurg Focus. 2006;21(2) 2006 American Association of Neurological Surgeons Posted 01/26/200

Abstract and Introduction

Abstract !"e #ur#ose of t"is re#ort $as to outline t"e %arious o#tions currentl& used for treatment of s#astic cere'ral #als& ((P) and to discuss factors in%ol%ed in selecting t"e a##ro#riate treatment modalities for t"e indi%idual c"ild. )n a re%ie$ of t"e literature and "is #ersonal o'ser%ations* t"e aut"or #resents an outline of treatment o#tions and t"e criteria for using eac". !"era#eutic o#tions include t"e follo$ing+ #"&siot"era#&; occu#ational t"era#&; oral s#asmol&tic and antid&stonic drugs; 'otulinum to,in in-ections; ort"o#edic #rocedures; continuous infusion of intrat"ecal 'aclofen ()!.); selecti%e dorsal r"i/otom& (S01); and selecti%e #eri#"eral neurotom&. !"e most commonl& used neurosurgical #rocedures are )!. #um# #lacement and S01* and t"ese are discussed in t"e most detail. !"e aut"or2s #ersonal sc"ema for assessment of t"e c"ild to determine t"e nature of t"e "&#ertonia* t"e im#act of t"e "&#ertonia* and t"e a##ro#riate t"era#eutic inter%ention is #resented. !"ere are factors t"at "el# guide t"e o#timal treatment modalities for t"e c"ild $it" s#astic (P. !"e treatment of t"ese c"ildren is o#timi/ed in t"e setting of a multidisci#linar& team. Introduction Selection of t"e most a##ro#riate treatment modalit& for a #atient $it" s#astic (P re3uires t"at all #arties in%ol%ed* including t"e caregi%ers* understand and "a%e similar e,#ectations for $"at mig"t 'e ac"ie%ed '& inter%entions. !reatments ma& 'e used to relie%e "&#ertonia and/or im#ro%e t"e #atient2s range of mo%ement* 'ut t"ese outcomes can 'e considered a means to an end. !"e real goals of treatment are not sim#l& reduced tone or im#ro%ed range of mo%ement* 'ut also include im#ro%ed motor function* increased mo'ilit&* increased inde#endence* decreased discomfort* and easier care. !o ma4e t"e most a##ro#riate recommendations* t"e management team must 'e familiar $it" and #re#ared to discuss t"e #ros and cons of t"e %arious o#tions a%aila'le. ("ildren ma& 'e referred for management of t"eir 5s#astic5 (P* 'ut it is im#ortant to recogni/e t"at t"e la'el of 5s#asticit&5 a##lied to t"ese #atients ma& or ma& not 'e correct. (larification of t"e definitions of t"e %arious disorders t"at mig"t 'e encountered in c"ildren $it" so6called s#astic (P is im#ortant and "as 'een #u'lis"ed recentl& '& t"e !as4 Force on ("ild"ood 7otor 0isorders. 8 19 !"ese c"ildren "a%e "&#ertonia* $"ic" "as 'een defined '& t"e tas4 force sim#l& as 5a'normall& increased resistance to e,ternall& im#osed mo%ement a'out a -oint.5 )t ma& 'e caused '& s#asticit&* d&stonia* or rigidit&* indi%iduall& or in com'ination. )n c"ildren $it" (P* rigidit& as seen t&#icall& in #ar4insonian #atients occurs rarel&* if

e%er* so t"at in #ractice one "as to determine $"et"er t"e "&#ertonia is due to s#asticit&* d&stonia* or a com'ination of t"ese. According to Sanger* et al.* 8 19 2S#asticit&2 is defined as "&#ertonia in $"ic" 1 or 'ot" of t"e follo$ing signs are #resent+ 1) resistance to e,ternall& im#osed mo%ement increases $it" increasing s#eed of stretc" and %aries $it" t"e direction of -oint mo%ement* and/or 2) resistance to e,ternall& im#osed mo%ement rises ra#idl& a'o%e a t"res"old s#eed or -oint angle. !"e second criterion defines one as#ect of t"e s#astic catc"* $"ic" ma& 'e felt on e,amination. After t"e rise in resistance* t"ere ma& 'e a ra#id decrease in resistance if t"e e,ternall& im#osed force is maintained* #ro%o4ing t"e so6called clas#64nife res#onse. S#asticit& is #art of t"e u##er motor neuron s&ndrome and usuall& coe,ists $it" ot"er motor s&m#toms and signs. !"ese include "&#errefle,ia* clonus* refle, o%erflo$* a .a'ins4i res#onse* $ea4ness* and incoordination. Similar to earlier definitions*82:*2;9 in Sanger* et al.*8 19 d&stonia is defined as a 5mo%ement disorder in $"ic" in%oluntar& sustained or intermittent muscle contractions cause t$isting and re#etiti%e mo%ements* a'normal #ostures* or 'ot".5 0&stonia ma& cause "&#ertonia* 'ut it ma& also 'e #resent in t"e a'sence of "&#ertonia.

Management O tions
Nonneurosurgical o#tions for t"e treatment of so6called s#astic (P include t"e follo$ing* among ot"ers+ #"&siot"era#&; occu#ational t"era#&; oral s#asmol&tic and antid&stonic drugs; 'otulinum to,in in-ections; and ort"o#edic #rocedures suc" as tendon lengt"ening. Neurosurgical inter%entions* $"ic" are discussed more full& else$"ere in t"is issue* include #um# im#lantation for continuous infusion of )!.* and #rocedures suc" as S01 and SPN. !onneurosurgical O tions <ne of t"e ma-or #ro'lems encountered in t"e c"ild $it" s#astic (P is t"e de%elo#ment of -oint contractures caused '& im'alance 'et$een t"e s#astic agonists and t"e $ea4er antagonists. !"ese contractures most commonl& affect t"e an4le #lantar fle,ors* $"ic" ma& 'e #art of t"e e3uinus deformit&* and t"e "amstrings* leading to a 4nee fle,ion deformit&. Stretc"ing of t"e s#astic muscles ma& "el# #re%ent t"e de%elo#ment of contractures* 'ut to 'e most effecti%e* suc" stretc"ing needs to 'e done for at least 6 "ours dail&. 8=29 Stretc"ing e,ercises ma& 'e #erformed '& #"&siot"era#ists* $"o also teac" caregi%ers to do t"ese e,ercises re#eatedl&. <rt"otic de%ices ma& 'e "el#ful in 4ee#ing s#astic muscles stretc"ed for a more #rolonged #eriod. <ccu#ational t"era#ists assist t"e c"ild in o#timi/ing "is or "er a'ilit& to #erform A0>. !o ac"ie%e t"is* t"e needs of t"e c"ild $it" res#ect to eating* #ositioning* s#linting* and e3ui#ment are addressed. A num'er of drugs "a%e 'een used orall& to reduce s&m#toms in c"ildren $it" s#astic di#legic or 3uadri#legic (P.8:2*=;9 !"ese drugs include 'aclofen* dantrolene* clona/e#am* dia/e#am* and ti/anidine. .aclofen* $"ose c"emical name is :6 amino6?6(:6c"loro#"en&l)6'ut&ric acid* is a s&nt"etic @A.A agonist t"at acts selecti%el& on 'ot" #re6 and #osts&na#tic @A.A . rece#tors to reduce e,citator& s&na#tic transmission. .aclofen "as 'een used orall& since t"e earl& 1A 0s as an

antis#asmodic agent. !"e drug is a'sor'ed $ell after oral administration* 'ut "as lo$ li#id solu'ilit& and does not cross t"e 'lood6'rain 'arrier readil&. 8;*?A9 <ral 'aclofen "as not 'een #articularl& effecti%e in reducing s#asticit& of cere'ral origin* s#ecificall& in c"ildren $it" (P.8; *=:9 !"e %alue of oral 'aclofen "as 'een limited '& t"e common occurrence of sedation at t"e dose le%els needed for relief of s#asticit&. <t"er less common side effects "a%e included 'e"a%ioral c"anges* confusion* ata,ia* urinar& fre3uenc&* and insomnia. 8;*; 9 0antrolene in"i'its t"e release of calcium from t"e sarco#lasmic reticulum* and t"us $ea4ens muscles* t"ere'& reducing tone. 0ia/e#am and clona/e#am decrease s#asticit& '& increasing #res&na#tic in"i'ition in t"e s#inal cord* 'ut tend to cause unacce#ta'le sedation. !i/anidine is an B26adrenergic agonist t"at increases noradrenergic in"i'ition in t"e s#inal cord. !"is agent2s side effects include "&#otension* dro$siness* and muscle $ea4ness* $it" dro$siness 'eing t"e most common limiting #ro'lem. )n c"ildren in $"om it is a significant com#onent of t"e "&#ertonic (P* oral medications directed at t"e d&stonia ma& 'e used. )n c"ildren $it" d&stonia in $"om t"e su##osed (P is at&#ical or #rogressi%e* a trial of le%odo#a ma& 'e indicated* 'ecause occasionall& a #atient $ill "a%e le%odo#a6res#onsi%e d&stonia. 8;69 C"en t"e diagnosis is clearl& (P* 'aclofen* tri"e,&#"en&dil* clona/e#am* and tetra'ena/ine "a%e occasionall& 'een effecti%e in im#ro%ing d&stonia* 'ut more often t"an not t"ese medications are ineffecti%e. 86;9 )ntramuscular in-ections of 'otulinum to,in A "a%e 'een used since t"e 1A 0s for t"e treatment of stra'ismus* and more recentl& its use "as 'een e,#anded to a %ariet& of d&stonias and also s#asticit&* including t"at caused '& (P. 81:*::9 !"e effect of 'otulinum to,in is tem#orar&* lasting from 2 to 6 mont"s. )n (P* 'otulinum to,in "as 'een used #rimaril& to treat d&namic e3uinus and* to a lesser e,tent* "i# adductor s#asms. )n a num'er of o#en trials and also in 1(!s* im#ro%ement in s#asticit& and #ossi'le im#ro%ement in function at ? to 6 mont"s "a%e 'een indicated* 'ut t"ere is no information on t"e longer6term effecti%eness of t"is treatment.8:*::9 !"ere "a%e 'een fe$ side effects of 'otulinum to,in in-ections* and t"is* toget"er $it" t"e transient nature of t"e res#onses and t"e ease of administration* "as made it an increasingl& #o#ular inter%ention in (P. !"e ent"usiasm for t"e use of 'otulinum to,in s"ould 'e tem#ered '& t"e recognition t"at t"e e%idence for its effecti%eness in (P is $ea4. <rt"o#edic surger& most commonl& in%ol%es tendon6lengt"ening o#erations to tr& to restore 'alance once contractures "a%e occurred* or to #re%ent "i# su'lu,ation in c"ildren $it" se%ere "i# adductor s#asticit&. !"ese #rocedures $ea4en t"e muscles* and 'ecause t"e underl&ing s#asticit& "as not 'een treated* s"ortening of t"e muscles tends to occur as t"e c"ild gro$s* and re#eated tendon lengt"ening #rocedures ma& 'e necessar&. !endon transfers ma& 'e done to augment t"e strengt" of a $ea4 muscle grou#. )n t"e c"ild older t"an : &ears of age* femoral derotation osteotomies ma& 'e done to treat internal rotation of t"e lo$er lim's* and in t"e c"ild a##roac"ing t"e age of s4eletal maturit&* art"rodesis of t"e an4le can add sta'ilit& to t"e foot. )t is im#ortant to recogni/e t"at e%en if one is successful in relie%ing s#asticit& '& using some of t"e treatment modalities descri'ed later* man& of t"e aforementioned management tec"ni3ues ma& still 'e re3uired to o#timi/e t"e c"ild2s functioning. !eurosurgical Procedures "irected at S asticit#

!"e ma-or neurosurgical inter%entions used currentl& in t"e treatment of s#asticit& in (P are S01 and #um# im#lantation for continuous infusion of )!.. )t is t"oug"t t"at SPNs ma& also 'e a t"era#eutic o#tion. "orsal (Posterior) R$i%otom#. <ne of t"e common neurosurgical #rocedures for s#astic (P is t"e #artial lum'osacral dorsal r"i/otom&. !"e rationale for t"is #rocedure is t"at in#ut entering t"e s#inal cord t"roug" t"e #osterior roots "as a net e,citator& effect on t"e anterior roots* and t"at '& decreasing t"e in#ut from t"e dorsal roots one can reduce s#asticit&. !"is conce#t and t"e initial dorsal r"i/otom& #rocedure are a centur& old. !ransection of #osterior lum'ar and sacral ner%e roots for t"e treatment of lo$er6 lim' s#asticit& $as first re#orted in t"e earl& 1A00s '& Foerster* 82A9 $"o descri'ed a series of #atients in $"om total sectioning of t"e #osterior ner%e roots of >62 and >6 ? as $ell as >6; and S61 (s#aring >6:) $as #erformed. 1elief of s#asticit& in t"e ma-orit& of #atients $as noted. )n t"e 1A60s* @ros* et al.* 8??9 modified t"e original #rocedure descri'ed '& Foerster and #erformed #artial #osterior r"i/otomies* $it" nonselecti%e sectioning of =0D of eac" of t"e ner%e roots from >61 to S61. Furt"er refinements of t"e #artial r"i/otom& #rocedure "a%e follo$ed t$o different #at"s. <ne refinement $as a selecti%e #artial r"i/otom& #rocedure (5sectorial r"i/otom&5)* $"erein t"e e,tent of t"e surger& $as tailored to t"e clinical status of t"e indi%idual #atient.86 9 >o$er6lim' s#asticit& $as categori/ed clinicall& into 5disa'ling5 com#ared $it" 5'eneficial5 s#asticit&* and t"e goal of t"e modified #rocedure $as to relie%e* if #ossi'le* onl& s#asticit& t"at $as t"oug"t to 'e disa'ling. 0uring t"e #rocedure* electro#"&siological stimulation of t"e #osterior rootlets $as #erformed to identif& t"e muscle grou#s #rimaril& inner%ated '& eac" indi%idual #osterior ner%e rootlet. !"is allo$ed sectioning of rootlets feeding into muscle grou#s t"at $ere identified clinicall& as in%ol%ed $it" 5disa'ling5 s#asticit&* $"ile s#aring ner%e rootlets inner%ating muscle grou#s t"at $ere identified as #ro%iding 5'eneficial5 s#asticit&. A different a##roac"* originall& #ro#osed in 1A = '& Fasano* et al.* 8269 in%ol%ed t"e selection of t"e #osterior rootlets to 'e cut 'ased on t"e functional electro#"&siological results of intrao#erati%e electrical stimulation* and not on t"e clinical findings. )t $as t"oug"t t"at '& stimulating lum'osacral #osterior ner%e rootlets and recording res#onses from anterior roots and muscles* one could identif& t"e ner%e rootlets t"at $ere relati%el& more in%ol%ed in t"e maintenance of s#asticit& and #referentiall& cut t"em* t"us reducing s#asticit& $"ile maintaining ma,imal #reser%ation of neural tissue.82 9 !"e %alidit& of t"e conce#t underl&ing t"is electro#"&siological S01 and t"e im#ortance of electro#"&siological guidance for a good outcome "a%e 'een 3uestioned.81A*?:*:6*6:* =*=69 !"e original electro#"&siological criteria "a%e 'een modified* and t"e current criteria of in%ol%ement in s#asticit& include s#read of t"e res#onse contralaterall& and su#rasegmentall&* and an incremental or multi#"asic #attern in t"e res#onse to a ;06E/ tetanic stimulus. C"et"er t"ese ne$er criteria are %alid and "o$ im#ortant electro#"&siological guidance is for a good outcome are not 4no$n. !"ere "a%e 'een significant %ariations among centers in t"e $a& t"e #rocedure is done. 8 *=19 !"ere "as 'een a trend to$ard rel&ing less on t"e electro#"&siological res#onses and more on t"e clinical findings* and in man& centers a com'ination of t"e t$o is used. <utcomes after S01 "a%e 'een $ell c"aracteri/ed and re%ie$ed. 8 :9 !"ere is %er& strong e%idence t"at S01 results in im#ro%ements in lo$er6lim' s#asticit& and an increase in t"e range of mo%ement at t"e lo$er6lim' -oints $it" no loss of strengt". 82?*;0* :9

!"ere is a moderate degree of certaint& t"at t"ese im#ro%ements in t"e im#airment dimension are maintained u# to ; &ears after S01* and some $ea4er e%idence t"at t"e im#ro%ements are maintained e%en longer. 8 :9 )n am'ulator& #atients* t"ere is strong e%idence for increased stride lengt" after S01 and moderate e%idence for increased gait %elocit&. !"ere is a moderate degree of certaint& for im#ro%ed sitting a'ilit& in more t"an 0D of #atients and an im#ro%ed le%el of am'ulation in more t"an ;0D of #atients $"o $ere not alread& inde#endent am'ulators #rior to S01. !"ere is strong e%idence* including t"at from t"ree 1(!s* for an im#ro%ement in motor function after S01* as assessed $it" t"e @7F7 scale. 8? *:=6;0* A*= 9 Eo$e%er* in onl& t$o of t"e t"ree 1(!s $as t"e im#ro%ement in @7F7 scores statisticall& more t"an in a control grou# recei%ing #"&siot"era#& onl&. 8 A*= 9 )n t"e ot"er 1(!* t"ere $as no difference 'et$een t"e S01 and t"e control grou# $it" res#ect to @7F7 im#ro%ement alone.8:=9 )n a metaanal&sis of t"ese t"ree 1(!s* t"ere $as a small 'ut statisticall& significant im#ro%ement in t"e #atients undergoing S01 #lus #"&siot"era#& com#ared $it" t"ose recei%ing #"&siot"era#& onl&. 81;9 As one e,amines t"e im#act of S01 on t"e c"ild2s a'ilit& to engage in A0>* t"ere is good e%idence from #ros#ecti%e case series in $"ic" #atients $ere e%aluated using %alidated assessment tools (eit"er t"e Functional )nde#endence 7easure for ("ildren or t"e Pediatric F%aluation of 0isa'ilit& )n%entor&) t"at t"ere are im#ro%ements in self care and t"e #erformance of A0> after S01. 816*22*:;*;1*;;9 !"ese findings are su##orted '& t"ose in ot"er #ros#ecti%e case series in $"ic" less $ell6 %alidated outcome tools $ere used.8 :9 )n addition to t"e im#act of S01 on t"e lo$er lim's* t"ere "a%e 'een $ell6 documented su#rasegmental im#ro%ements. !"ese "a%e 'een demonstrated most con%incingl& for u##er6lim' function '& using a %alidated tool* namel& t"e Gualit& of H##er F,tremities S4ills !est8:;9 and t"e fine motor s4ills section of t"e Pea'od& 0e%elo#mental 7otor Scales test*8;29 to measure u##er6lim' function. !"ere is a single small stud& in $"ic" in%estigators "a%e suggested im#ro%ement in cogniti%e function.8209 !"e com#lications of S01 "a%e 'een fe$ in most series. 8?*=09 Hrinar& incontinence is #ro'a'l& t"e most trou'lesome #ro'lem. !ransient urinar& retention is fre3uent* $it" an incidence of 'et$een 1.2; and 2:D*821*26*60*=09 'ut #ermanent urinar& retention or incontinence is rare. !ransient d&sest"esias lasting u# to a fe$ $ee4s "a%e 'een re#orted in 2.; to :0D of #atients*8?*:A*=09 'ut t"e re#orted incidence of #ermanent "&#est"esia after S01 is %er& lo$* generall& %ar&ing from 0 to 6D in different series* and usuall& $it"out functional significance. 82*11*2=*=09 A concern often raised is $"et"er S01 $ill reduce function in c"ildren $"o are using t"eir s#asticit& to stand and $"o "a%e #oor underl&ing lo$er6lim' strengt". )n our e,#erience* no #atient2s condition $as $orsened in t"is manner* and t"is ma& 'e more of a t"eoretical t"an a #ractical concern. <ne of t"e concerns in c"ildren $it" significant s#asticit& affecting t"e "i#s is t"e com#lication of "i# su'lu,ation. Cit" res#ect to t"is com#lication* fe$er t"an 20D of #atients $orsened and t"e %ast ma-orit& $ere sta'le (:?6=0D) or actuall& s"o$ed im#ro%ement (A6?=D) in t"e amount of "i# su'lu,ation after S01. 8?;6? *;A9 !"e surgical #rocedure for S01 is #erformed eit"er at t"e le%el of t"e conus %ia one6 or t$o6le%el laminectomies* or at t"e le%el of t"e root e,it foramina %ia multile%el laminectomies or laminotomies* $it" re#lacement of t"e laminar fla#. A

concern regarding laminectomies or laminotomies in &oung c"ildren is t"e later de%elo#ment of 'ac4 #ain or s#inal deformit&. 8==9 .ac4 #ain occurring $ee4s to &ears after S01 "as 'een re#orted in : to D of #atients* 81*6?*=09 'ut it is unusual for t"is to 'e se%ere enoug" to interfere $it" A0> or to lead to "os#itali/ation. !"e issue of s#inal deformit& is more contentious. )t "as 'een recogni/ed for a long time t"at c"ildren $it" s#astic (P are at "ig"er ris4 of s#inal deformit&* #articularl& scoliosis* t"an t"e normal #o#ulation*81?9 and t"at nonam'ulator& #atients $it" s#astic 3uadri#legia constitute t"e #o#ulation at "ig"est ris4. )t "as 'een suggested in a num'er of recent re#orts t"at S01 #erformed %ia multile%el lamino#lasties or laminectomies ma& increase t"e incidence of t"oracolum'ar scoliosis* 4&#"osis* and "&#erlordosis in t"ese at6ris4 c"ildren $it" (P. 8?=* 6*=?9 !"e #ossi'le increased ris4 of s#inal deformit& after S01 is noted #rimaril& in nonam'ulator& #atients $it" s#astic 3uadri#legia*8 ?* 69 $"o currentl& tend to 'e treated $it" )!. rat"er t"an r"i/otomies. )t "as 'een suggested t"at t"is #otential com#lication can 'e reduced '& #erforming t"e #rocedure %ia one6 or t$o6le%el laminectomies at t"e le%el of t"e conus* 'ut t"ere is no e%idence at #resent to su##ort t"is "&#ot"esis. Pum Im lantation &or I'B. .aclofen reduces lim' s#asticit& '& its effect on @A.A. rece#tors* $"ic" are ma,imal in la&ers )) and ))) of t"e dorsal gra& matter of t"e s#inal cord.8669 As mentioned #re%iousl&* oral 'aclofen "as a limited effect on s#asticit& in (P* in #art 'ecause of an ina'ilit& to '&#ass t"e 'lood6'rain 'arrier and ac"ie%e a "ig" enoug" concentration at t"e rece#tor sites in t"e s#inal cord* and in #art 'ecause s&stemic administration also affects t"e central @A.A . rece#tors* $"ic" results in to,icit&. Cit" continuous s#inal )!. infusions* t"e concentrations of 'aclofen t&#icall& ac"ie%ed in t"e s#inal su'arac"noid fluid are 10 to =0 times "ig"er t"an t"ose #resent after oral administration* 8?A*;:9 leading to a muc" greater t"era#eutic effect. !"e t"era#eutic ratio is also im#ro%ed for at least t$o reasons+ first* t"e (SF concentration intracraniall& is less t"an intras#inall&* 8:?9 and second* t"e intracranial @A.A. rece#tors are not as close to t"e surface as t"e s#inal ones* and 'ecause t"e 'aclofen diffuses #oorl& into neural tissue* t"e concentration of 'aclofen in tissue at t"e intracranial rece#tor sites is relati%el& less t"an at t"e s#inal rece#tor sites.8;=9 )n addition to an effect on s#asticit&* 'aclofen also im#ro%es d&stonia. !"e site of action of 'aclofen in t"e treatment of d&stonia is t"oug"t to 'e su#ras#inal.8=9 !o ac"ie%e a clinicall& useful #rolonged effect* )!. must 'e gi%en '& continuous infusion t"roug" an im#lanted #um#. .efore im#lantation of t"e #um#* most #atients undergo a trial of 'olus doses of )!. to confirm a #ositi%e effect of t"e drug in reducing "&#ertonia. For #atients $it" generali/ed d&stonia* successful reduction of t"eir s&m#toms during t"e test #"ase ma& re3uire t"at t"e 'aclofen 'e gi%en intrat"ecall& t"roug" an e,ternal infusion #um#. !"e most commonl& used #um# is t"e #rogramma'le S&nc"romed de%ice (7edtronic* )nc.* 7innea#olis* 7N). !"is is a 'atter&6#o$ered #um# t"at can 'e ad-usted using a radiofre3uenc&6controlled e,ternal $and and #rogramming com#uter to deli%er %aria'le flo$ rates* continuous and/or 'olus infusions* or a set total dose. !"e #um# is im#lanted into a su'cutaneous or su'fascial #oc4et in t"e anterior a'dominal $all. A cat"eter is inserted into t"e lum'ar su'arac"noid s#ace at t"e >?6: or >:6; le%el* and is t"readed su#eriorl& to #lace t"e ti# at a##ro,imatel& !10612 for s#astic di#legia* (;6!2 for s#astic 3uadri#aresis* or (16: for generali/ed secondar& d&stonia. 8A9

!"e e%idence for t"e efficac& of continuous infusion of )!. in c"ildren $it" (P $as re%ie$ed in detail recentl&.81 9 !"ere is consistent e%idence from multi#le case re#orts* case series* 5N of 15 1(!s* and t$o dou'le6'lind #lace'o6controlled studies t"at a 'olus dose of )!. reduces s#asticit&* clonus* and s#asms. 81 9 )n retros#ecti%e and o#en #ros#ecti%e studies* continuous infusion of )!. "as 'een s"o$n to decrease lo$er6lim' s#asticit& in c"ildren $it" (P. !"ere ma& also 'e a reduction in u##er6lim' s#asticit&* 'ut t"e e%idence is not as strong. )m#ro%ements in am'ulation and A0> "a%e also 'een demonstrated in #ros#ecti%e studies* 'ut t"e e%idence is not as conclusi%e as it is for a reduction in s#asticit&. 8 *10*?19 7ore recentl&* continuous infusion of )!. "as 'een used for t"e treatment of "&#ertonic d&stonia in c"ildren. !"ere is good e%idence t"at )!. reduces t"e se%erit& of generali/ed d&stonia in #atients $it" d&stonic or s#astic/d&stonic (P. 8=9 !"e reduction in d&stonia $as associated $it" im#ro%ed 3ualit& of life and ease of care. (om#lications associated $it" )!. are common* and ma& 'e related to t"e drug* to local #ro'lems $it" t"e surgical #rocedure* or t"e "ard$are. !"e most common drug6related #ro'lem from )!. is mild sedation* $"ic" re3uires a decrease in t"e dose. >ess commonl& t"ere ma& 'e di//iness* 'lurred %ision* and slurred s#eec". 8;*619 )ncreased fre3uenc& or #reci#itation of sei/ures "as also 'een re#orted. 81=*:1*6A9 >ife6t"reatening o%erdoses* c"aracteri/ed '& se%ere res#irator& de#ression* coma* "&#otension* and 'rad&cardia* can also occur. )n #atients $it" sus#ected drug o%erdoses* t"e diagnosis is su##orted '& t"e findings of $ea4ness* flaccidit&* and arefle,ia in 'ot" lo$er and u##er lim's. Sudden $it"dra$al of )!. after long6term use* as mig"t occur $it" a 'loc4age to t"e s&stem or #um# failure* ma& result in "allucinations* sei/ures* confusion* #s&c"otic 'e"a%ior* re'ound se%ere s#asticit&* and "&#ert"ermia.8:0*: *6=* 29 )f t"e diagnosis is made earl&* oral 'aclofen or 'en/odia/e#ines ma& 'e gi%en* and oral dantrolene "as also 'een used to "el# #re%ent or treat "&#ert"ermia. 8?29 1einfusion of 'aclofen into t"e su'arac"noid s#ace as a 'olus t"roug" t"e #um# or '& lum'ar #uncture ma& re%erse t"e s&m#toms. )f it is decided to discontinue continuous )!. t"era#&* t"is s"ould 'e done graduall&* and t"e #atient s"ould recei%e oral 'aclofen. Failure of t"e #um# occurs in ; to 10D of cases* and re3uires its re#lacement. 81=*619 <t"er local #ro'lems related to t"e infusion s&stem include t"e follo$ing+ seromas around t"e #um#; erosion of t"e s4in o%er t"e de%ice; disconnection at a connector site; a 'rea4* 4in4ing* or dis#lacement of t"e intrat"ecal cat"eter; and lea4age of (SF at t"e cat"eter insertion site.8;*12*?09 Some of t"ese com#lications "a%e 'een reduced as surgeons "a%e gained e,#erience inserting t"e #um#s and $it" im#ro%ed cat"eter s&stems* #articularl& since t"e de%elo#ment of t"ic4er6$alled cat"eters.8629 !"e local #ro'lems tend to 'e aggra%ated in c"ildren. !"e #um#s are large and #rotrude mar4edl& in t"e anterior a'dominal $all* es#eciall& in &oung or %er& t"in c"ildren* alt"oug" t"is is less of a #ro'lem $it" su'fascial #lacement. )n c"ildren $it" s#astic 3uadri#legia* $"o are often restrained in a $"eelc"air and $"o tend to 'e t"in* t"e #rotruding #um# ma& 'e irritated re#eatedl& '& t"e restraints t"at are used* leading to a "ig"er incidence of local com#lications. As in an& surger& in%ol%ing im#lants* infection is a ma-or concern. >ocal infection of t"e $ound or su'cutaneous #um# #oc4et "as 'een re#orted in u# to 20D of #atients.8?0*;?9 Pum# erosion t"roug" t"e s4in and su'se3uent contamination of t"e de%ice is not unusual* es#eciall& in c"ildren. >ocal infections can sometimes 'e

eradicated $it" t"e use of s&stemic anti'iotic drugs* 'ut more often t"e #um# "as to 'e remo%ed. 7eningitis "as 'een documented* 'ot" during trials of re#eated intrat"ecal in-ections 'efore insertion of t"e #um# and after its im#lantation. (ontinuous )!. t"era#& re3uires long6term medical attention to refill t"e #um#* to ad-ust t"e dose to o'tain t"e desired effect* to re#lace t"e #um# $"en t"e 'atter& fails* and to address t"e %arious com#lications #re%iousl& mentioned. S"ould s#asticit& recur in a #atient recei%ing continuous )!.* t"e #ractitioner needs to determine $"et"er tolerance to t"e drug "as occurred or $"et"er t"ere is a #ro'lem $it" t"e deli%er& of t"e drug intrat"ecall&. Eence* #um# failure or some mec"anical #ro'lem $it" t"e s&stem "as to 'e ruled out. Finall&* t"e e,#ense in%ol%ed $it" t"e continuous infusion of )!. must 'e considered. !"e #um# itself costs more t"an I10*000 HS and "as to 'e re#laced #eriodicall&. )n addition* t"ere are ongoing costs associated $it" t"e refills and $it" management of t"e com#lications t"at ma& occur. 8 ;9

Assessment o& t$e C$ild (it$ S astic CP

!"e first issue in t"e assessment of t"e c"ild $it" (P $"o is referred for management of s#asticit& is to determine $"et"er t"e increased tone relates to s#asticit&* d&stonia* or 'ot"* and $"at is t"e relati%e contri'ution of eac" to t"e o%erall "&#ertonia. Ea%ing determined t"e nature of t"e "&#ertonia* t"e ne,t 3uestion is $"et"er it is t"e "&#ertonia or some ot"er #ro'lem* suc" as contractures* incoordination* or $ea4ness t"at is in"i'iting t"e c"ild2s functioning or t"e a'ilit& of t"e caregi%ers to loo4 after t"e c"ild. !"e motor disorder in c"ildren $it" "&#ertonic (P is com#le,* and is t"e result of a num'er of factors in addition to t"e effects of s#asticit& and/or d&stonia. Cea4ness and incoordination are common. !"ere usuall& is co6contraction of agonists and antagonists* e,cessi%e refle, s#read to ot"er muscle grou#s* and reduced selecti%e motor control (defined as im#airment of t"e a'ilit& to isolate t"e acti%ation of muscles in a selected #attern in res#onse to t"e demands of a %oluntar& #osture or mo%ement). 8 09 As t"e c"ild gets older* contractures and deformities of t"e e,tremities often de%elo#* #articularl& 4nee fle,or and an4le #lantar fle,or contractures. !"ere ma& 'e "i# dislocations* roc4er6'ottom feet* and s#inal deformit&* es#eciall& scoliosis* $"ic" ma& add to t"e motor d&sfunction and discomfort and to t"e difficult& in #ro%iding care. An element of im#aired sensor& #erce#tion #ro'a'l& adds to t"e im#airment of motor function as $ell. )t is im#ortant to find out from t"e #arents or ot"er caregi%ers $"at t"e& t"in4 is "olding t"e c"ild 'ac4 or ma4ing care difficult. )t is instructi%e to "ear "o$ t"e& t"in4 t"e c"ild $ould 'enefit if t"e "&#ertonia* 'ut not"ing else* in t"e lo$er or u##er lim's $as reduced. )f it is not t"oug"t t"at reducing "&#ertonia $ill im#ro%e function* t"e c"ild is not a candidate for a "&#ertonia6relie%ing #rocedure. )f t"e c"ild "as s#asticit& and mig"t 'enefit functionall& or $it" res#ect to t"e ease of recei%ing care if s#asticit& in multi#le muscle grou#s in t"e lo$er lim's $ere relie%ed* t"e #ractitioner $ill consider o#tions suc" as )!. or S01. !"e am'ulator& #otential must 'e assessed* and an attem#t is made to determine if reduction in lo$er6lim' s#asticit& mig"t 'e detrimental to t"e c"ild2s am'ulator& function* 'ecause t"e c"ild ma& 'e using t"e s#asticit& to su##ort $eig"t in t"e u#rig"t #osition. !"is is one of t"e most difficult issues. )n our e,#erience* c"ildren $"o

$al4 inde#endentl&* $it"out a $al4er or crutc"es* and &ounger c"ildren $"o cra$l on t"eir 4nees and arms "a%e enoug" underl&ing strengt" in t"e lo$er lim's so t"at t"e lessening of s#asticit& is not a concern. For t"e ot"er #atients* t"e c"ild2s a'ilit& to rise in a slo$* graded fas"ion from t"e s3uatting #osition is assessed. )f t"e c"ild is a'le to do t"is $it" t"e e,aminer su##orting t"e arms or u##er 'od& minimall&* t"e #ro'a'ilit& is "ig" t"at t"ere is ade3uate underl&ing 3uadrice#s strengt" to su##ort t"e c"ild2s $eig"t if t"e s#asticit& is reduced. )n t"e #atient $it" s#astic 3uadri#legia* in $"om am'ulation is not a realistic goal* t"e in%ol%ement of s#asticit& in su##orting t"e c"ild in t"e u#rig"t #osition is also a concern. 7an& of t"ese c"ildren* $"o get around in $"eelc"airs* use t"eir s#asticit& to assist in standing transfers. !"e& ma& 'e $orse functionall& if t"at s#asticit& is reduced and t"e& are no longer a'le to su##ort t"eir $eig"t. !"is ma& not a##ear to 'e a ma-or issue in t"e &oung c"ild $"o is easil& lifted* 'ut it attains increasing im#ortance as t"e c"ild gets older* "ea%ier* and more difficult to lift. At t"e com#letion of t"e assessment* it is critical to ascertain $"at t"e e,#ectations and "o#es of t"e #arents or ot"er #rimar& caregi%ers are and to ensure t"at t"e& are realistic. !"is #erce#tion s"ould 'e documented* 'ecause almost all #arents are see4ing a 5cure5 for t"eir c"ild2s motor #ro'lems* or if not* t"e& "o#e t"at t"eir c"ild $ill $al4* and man& "a%e unrealistic e,#ectations of t"e treatment t"at is #ro#osed. F%en if t"e c"ild im#ro%es functionall&* t"ese #arents ma& not 'e "a##& 'ecause "e or s"e does not meet t"eir e,#ectations. )n most cases* one can allo$ man& mont"s to #ass 'efore treating t"e "&#ertonia* and t"e #arents are usuall& ad%ised to ta4e some time to t"in4 a'out t"e suggested recommendations* es#eciall& if an o#eration is ad%ised. <ccasionall&* $"en t"e c"ild2s "i#s are dislocating 'ecause of e,cessi%e "i# a'ductor s#asticit&* a more urgent inter%ention is recommended; e%en t"en* "o$e%er* one ma& 'e a'le to tem#ori/e $it" a treatment suc" as intramuscular 'otulinum to,in. )f it is determined t"at treatment of t"e "&#ertonia is indicated* one t"en needs to decide $"at inter%ention $ould 'e most a##ro#riate. )n c"ildren in $"om t"e functionall& im#ortant s#asticit& is #redominantl& in one muscle grou# (suc" as t"e an4le #lantar fle,ors or "amstrings)* consideration is gi%en to 'otulinum to,in or an ort"o#edic tendon6lengt"ening #rocedure. )n some centers* suc" #atients could 'e considered for an SPN. F%en $"en t"e functionall& im#ortant s#asticit& affects one muscle grou#* t"ere is usuall& s#asticit& t"roug"out t"e lo$er lim's* and an argument can 'e made for #erforming a dorsal r"i/otom& or using )!. to decrease t"e lo$er6lim' s#asticit& more diffusel& in t"is #atient #o#ulation. Eo$e%er* $e o#t for a sim#ler or less destructi%e #rocedure if t"e functional results mig"t reasona'l& 'e e,#ected to 'e similar. )ntrat"ecal 'aclofen is t"e inter%ention of c"oice in c"ildren suffering from s#astic d&stonic 3uadri#legia from near dro$ning. 8129 )n #atients in $"om d&stonia rat"er t"an s#asticit& is in"i'iting function* )!. is indicated. 8=9 ("ildren $it" 3uadri#legia caused '& s#astic (P affecting t"e u##er lim's more t"an t"e lo$er ones* or c"ildren $it" se%ere o#ist"otonic #osturing* ma& 'e considered for )!. 'ecause it is #ossi'le to direct t"e su'arac"noid cat"eter to t"e cer%icot"oracic region and treat t"e "&#ertonia in 'ot" t"e u##er and lo$er lim's. 8A9

)ntrat"ecal 'aclofen is often #ro#osed in #reference to S01 in c"ildren $it" s#astic di#legic (P $"en t"ere is concern t"at s#asticit& is needed for standing or $al4ing su##ort.869 !"e argument is t"at t"e dose of 'aclofen is titrata'le* and in t"e $orst6 case scenario* if t"e result is unsatisfactor&* t"e treatment is re%ersi'le. !"is concern a'out t"e #ossi'le loss of a'ilit& to stand or $al4 ma& 'e focused on a #ro'lem t"at is more t"eoretical t"an real. <ur e,#erience "as 'een t"at t"ese #atients* a'out $"om $e "a%e agoni/ed* "a%e done $ell after S01* $it" no c"ild losing t"e a'ilit& to $al4 or stand after t"at #rocedure. Eence* our #reference "as 'een to recommend an S01 as o##osed to )!. in t"is situation. )n t"ese c"ildren $e $ill limit t"e e,tent of section of t"e dorsal rootlets t"at cause #rimaril& 3uadrice#s contraction $"en stimulated intrao#erati%el&. <t"er indications for )!. include di#legia in older #atients* #articularl& t"ose older t"an 16 &ears* and s#astic 3uadri#legia in c"ildren in $"om t"e lo$er6lim' s#asticit& is disa'ling or ma4ing care difficult. 869 !"ese c"ildren ma& also meet t"e criteria to 'e considered for S01* and t"ere are no studies in $"ic" t"ese t$o treatment modalities are com#ared in t"ese #o#ulations. )n t"ese situations* in $"ic" eit"er )!. or S01 mig"t 'e considered to 'e effecti%e and indicated o#tions* one "as to $or4 $it" t"e famil& to determine in eac" indi%idual circumstance $"at t"e #referred o#tion mig"t 'e. <ne must consider not onl& t"e #ure medical outcomes 'ut also t"e logistics of ac"ie%ing t"e #osto#erati%e care re3uired after S01 com#ared $it" t"at re3uired for management of continuous infusion of )!. and t"e costs in%ol%ed for t"ese o#tions. (om#ared $it" )!. t"era#&* S01 is a more in%asi%e #rocedure* 'ut it re3uires little long6term management* does not re3uire reo#eration* and does not re3uire re#eated %isits to a medical center for refills. For a famil& li%ing man& "ours a$a& from a medical center* t"e difficulties of re#eatedl& accessing medical care ma& 'e an im#ortant consideration.

Conclusions
!"e management of motor im#airment in t"e c"ild $it" "&#ertonic (P s"ould 'e multidisci#linar&* and usuall& in%ol%es a #"&siot"era#ist* occu#ational t"era#ist* de%elo#mental #ediatrician* ort"o#edic surgeon* and ort"otist* in addition to t"e neurosurgeon. !"e neurosurgeon is usuall& as4ed for an o#inion a'out t"e management of t"e s#asticit& and/or "&#ertonia. )t is im#ortant t"at "e/s"e a##reciates t"e %arious neurosurgical and nonneurosurgical inter%entions a%aila'le for t"e relief of s#asticit&* and recogni/es t"at t"e goal of treatment is not t"e relief of "&#ertonia #er se* 'ut t"e im#ro%ement of function* comfort* or care. !"e neurosurgeon s"ould $or4 as #art of a team* $"ic" includes t"e #arents or caregi%ers* to identif& t"e 'est management o#tion to o#timi/e t"e outcome. Furt"ermore* if a neurosurgical inter%ention directed at t"e relief of "&#ertonia is 'elie%ed to 'e indicated* t"e 'est outcome is ac"ie%ed $it" in#ut from #"&siot"era#ists* occu#ational t"era#ists* ort"otists* and ort"o#edic surgeons in t"e #osto#erati%e #eriod.

Re&erences
1. A''ott 1+ Sensor& r"i/otom& for t"e treatment of c"ild"ood s#asticit&. J ("ild Neurol 11+ S?66S:2* 1AA6 2. A''ott 1* Forem S>* Jo"ann 7+ Selecti%e #osterior r"i/otom& for t"e treatment of s#asticit&+ a re%ie$. ("ilds Ner% S&s ;?? 6?:6* 1A=A ?. A''ott 1* Jo"ann67ur#"& 7* S"imins4i67a"er !* Guartermain 0* Forem S>*

@old J!* et al+ Selecti%e dorsal r"i/otom&+ outcome and com#lications in treating s#astic cere'ral #als&. Neurosurger& ??+=;16=; * 1AA? :. Ade6Eall 1* 7oore A+ .otulinum to,in t&#e A in t"e treatment of lo$er lim' s#asticit& in cere'ral #als&. (oc"rane 0ata'ase S&st 1e%* 2001 ;. Al'rig"t A>+ .aclofen in t"e treatment of cere'ral #als&. J ("ild Neurol 11+ 6=?* 1AA6 6. Al'rig"t A>+ )ntrat"ecal 'aclofen in cere'ral #als& mo%ement disorders. J ("ild Neurol 11+ S2A6S?;* 1AA6 . Al'rig"t A>* .arron C.* Fasic4 7P* Polin4o P* Janos4& J+ (ontinuous intrat"ecal 'aclofen infusion for s#asticit& of cere'ral origin. JA7A 2 0+2: ;6 2: * 1AA? =. Al'rig"t A>* .arr& 7J* S"afton 0E* Ferson SS+ )ntrat"ecal 'aclofen for generali/ed d&stonia. 0e% 7ed ("ild Neurol :?+6;266; * 2001 A. Al'rig"t A>* !urner 7* Pattisa#u JK+ .est6#ractice surgical tec"ni3ues for intrat"ecal 'aclofen t"era#&. J Neurosurg 10: (: Su##l)+2??62?A* 2006 10. Almeida @>* (am#'ell SL* @irolami @>* Penn 10* (orcos 07+ 7ultidimensional assessment of motor function in a c"ild $it" cere'ral #als& follo$ing intrat"ecal administration of 'aclofen. P"&s !"er + ;16 6:* 1AA 11. Arens >J* Peacoc4 CJ* Peter J+ Selecti%e #osterior r"i/otom&+ a long6term follo$6u# stud&. ("ilds Ner% S&st ;+1:=61;2* 1A=A 12. Armstrong 1C* Stein'o4 P* (oc"rane 00* Lu'e S0* Fife SF* Farrell L+ )ntrat"ecall& administered 'aclofen for treatment of c"ildren $it" s#asticit& of cere'ral origin. J Neurosurg = :0A6:1:* 1AA 1?. .almer @A* 7acF$en @0+ !"e incidence and treatment of scoliosis in cere'ral #als&. J .one Joint Surg .r ;2+1?:61? * 1A 0 1:. .enti%oglio A1* Al'anese A+ .otulinum to,in in motor disorders. (urr <#in Neurol 12+:: 6:;6* 1AAA 1;. .-ornson LF* 7c>aug"lin JF+ !"e measurement of "ealt"6related 3ualit& of life (E1G>) in c"ildren $it" cere'ral #als&. Fur J Neurol = (Su##l ;)+1=?61A?* 2001 16. .loom LL* Na/ar @.+ Functional assessment follo$ing selecti%e #osterior r"i/otom& in s#astic cere'ral #als&. ("ilds Ner% S&s 10+=:6=6* 1AA: 1 . .utler (* (am#'ell S+ F%idence of t"e effects of intrat"ecal 'aclofen for s#astic and d&stonic cere'ral #als&. AA(P07 !reatment <utcomes (ommittee 1e%ie$ Panel. 0e% 7ed ("ild Neurol :2+6?:66:;* 2000 1=. (offe& 1J* (a"ill 0* Steers C* Par4 !S* <rdia J* 7e&t"aler J* et al+ )ntrat"ecal 'aclofen for intracta'le s#asticit& of s#inal origin+ results of a long6term multicenter stud&. J Neurosurg =+22662?2* 1AA? 1A. (o"en A1* Ce'ster E(+ Eo$ selecti%e is selecti%e #osterior r"i/otom&M Surg Neurol ?;+26 62 2* 1AA1 20. (raft S* Par4 !S* C"ite 0A* Sc"at/ J* Noet/el 7* Arnold S+ ("anges in cogniti%e #erformance in c"ildren $it" s#astic di#legic cere'ral #als& follo$ing selecti%e dorsal r"i/otom&. Pediatr Neurosurg 2?+6=6 ;* 1AA; 21. 0eletis K* Koduse4 0.* A''ott 1* F#stein FJ* !urndorf E+ )ntrao#erati%e monitoring of t"e dorsal sacral roots+ minimi/ing t"e ris4 of iatrogenic micturition disorders. Neurosurger& ?0+ 26 ;* 1AA2 22. 0udgeon .J* >i''& AL* 7c>aug"lin JF* Ea&s 17* .-ornson LF* 1o'erts !S+ Pros#ecti%e measurement of functional c"anges after selecti%e dorsal r"i/otom&. Arc" P"&s 7ed 1e"a'il ;+:66;?* 1AA: 2?. Fngs'erg J1* 1oss SA* Cagner J7* Par4 !S+ ("anges in "i# s#asticit& and strengt" follo$ing selecti%e dorsal r"i/otom& and #"&sical t"era#& for s#astic cere'ral #als&. 0e% 7ed ("ild Neurol ::+2206226* 2002

2:. Fa"n S+ (once#t and classification of d&stonia. Ad% Neurol ;016=* 1A== 2;. Fa"n S* .ressman S.* 7arsden (0+ (lassification of d&stonia* in Fa"n S* 7arsden (0* 0e>ong 7 (eds)+ 0&stonia ?. P"iladel#"ia+ >i##incott61a%en* 1AA=* Kol =* ## 1610 26. Fasano KA* .roggi @* .arolat61omana @* Sgua//i A+ Surgical treatment of s#asticit& in cere'ral #als&. ("ilds .rain :2=A6?0;* 1A = 2 . Fasano KA* .roggi @* Neme S+ )ntrao#erati%e electrical stimulation for functional #osterior r"i/otom&. Scand J 1e"a'il 7ed Su##l 1 +1:A61;:* 1A== 2=. Fasano KA* .roggi @* Neme S* >o 1usso @* Sgua//i A+ >ong6term results of #osterior functional r"i/otom&. Acta Neuroc"ir Su##l ?0+:?;6:?A* 1A=0 2A. Foerster <+ <n t"e indications and results of t"e e,cision of #osterior s#inal ner%e roots in man. Surg @&necol <'stetrics 16:6?6: :* 1A1? ?0. @ardner .* Jamous A* !edd& P* .ergstrom F* Cang 0* 1a%ic"andran @* et al+ )ntrat"ecal 'aclofen6a multicentre clinical com#arison of t"e 7edtronics Programma'le* (ordis Secor and (onstant )nfusion )nfusaid drug deli%er& s&stems. Para#legia ??+;;16;;:* 1AA; ?1. @ers/ten P(* Al'rig"t A>* .arr& 7J+ Fffect on am'ulation of continuous intrat"ecal 'aclofen infusion. Pediatr Neurosurg 2 +:06::* 1AA ?2. @ooc" J>* <'erg CA* @rams .* Card >A* Cal4er 7>+ (om#lications of intrat"ecal 'aclofen #um#s in c"ildren. Pediatr Neurosurg ?A+166* 200? ??. @ros (* <a4nine @* Kla"o%itc" .* Frere'eau P+ >a radicotomie selecti%e #osterioeure dans le traitment neuroc"irurgical de l2"&#ertonie #&ramidale. Neuroc"irurgie 1?+;0;6;1=* 1A6 ?:. Ea&s 17* 7c>aug"lin JF* .-ornson LF* Ste#"ens L* 1o'erts !S* Price 1+ Flectro#"&siological monitoring during selecti%e dorsal r"i/otom&* and s#asticit& and @7F7 #erformance. 0e% 7ed ("ild Neurol :0+2??62?=* 1AA= ?;. Eeim 1(* Par4 !S* Kogler @P* Laufman .A* Noet/el 7J* <rtman 71+ ("anges in "i# migration after selecti%e dorsal r"i/otom& for s#astic 3uadri#legia in cere'ral #als&. J Neurosurg =2+;6 6; 1* 1AA; ?6. Eicdonme/ !* Stein'o4 P* .eauc"am# 1* Sa$at/4& .+ Ei# -oint su'lu,ation after selecti%e dorsal r"i/otom& for s#astic cere'ral #als&. J Neurosurg 10? (1 Su##l)+10616* 200; ? . Eodg4inson )* .erard (* Jindric" 7>* Sindou 7* 7ertens P* .erard J+ Selecti%e dorsal r"i/otom& in c"ildren $it" cere'ral #als&. 1esults in 1= cases at one &ear #osto#erati%el&. Stereotact Funct Neurosurg 6A+2;A626 * 1AA ?=. Jo"nson 7.* @oldstein >* !"omas SS* Piatt J* Aiona 7* Sussman 7+ S#inal deformit& after selecti%e dorsal r"i/otom& in am'ulator& #atients $it" cere'ral #als&. J Pediatr <rt"o# 2:+;2A6;?6* 200: ?A. Lnuttson F* >ind'lom H* .eissinger 1>* 7artensson A+ Plasma and cere'ros#inal fluid le%els of 'aclofen (>ioresal) at o#timal t"era#eutic res#onses in s#astic #ara#aresis. J Neurol Sci 2?+: ?6:=:* 1A : :0. Lofler 7* Arturo >eis A+ Prolonged sei/ure acti%it& after 'aclofen $it"dra$al. Neurolog& :2+6A 66A=* 1AA2 :1. Lofler 7* Lronen'erg 7F* 1ifici (* Saltuari >* .auer @+ F#ile#tic sei/ures associated $it" intrat"ecal 'aclofen a##lication. Neurolog& ::+2;62 * 1AA: :2. Lrac" >F+ P"armacot"era#& of s#asticit&+ oral medications and intrat"ecal 'aclofen. J ("ild Neurol 16+?16?6* 2001 :?. Lroin JS* Ali A* Oor4 7* Penn 10+ !"e distri'ution of medication along t"e s#inal canal after c"ronic intrat"ecal administration. Neurosurger& ??+2266 2?0* 1AA? ::. >annin N* Sc"ein'erg A* (lar4 L+ AA(P07 s&stematic re%ie$ of t"e

effecti%eness of t"era#& for c"ildren $it" cere'ral #als& after 'otulinum to,in A in-ections. 0e% 7ed ("ild Neurol :=+;??6;?A* 2006 :;. >oe$en P* Stein'o4 P* Eolsti >* 7acLa& 7+ H##er e,tremit& #erformance and self6care s4ill c"anges $it" s#astic cere'ral #als& follo$ing selecti%e #osterior r"i/otom&. Pediatr Neurosurg 2A+1A161A=* 1AA= :6. >ogigian F>* Colins4& JS* Soriano S@* 7adsen J1* Scott 17+ E refle, studies in cere'ral #als& #atients undergoing #artial dorsal r"i/otom&. 7uscle Ner%e 1 +;?A6;:A* 1AA: : . 7andac .1* Eur%it/ FA* Nelson KS+ E&#ert"ermia associated $it" 'aclofen $it"dra$al and increased s#asticit&. Arc" P"& 7ed 1e"a'il :+A66A * 1AA? :=. 7c>aug"lin JF* .-ornson LF* Astle& SJ* @rau'ert (* Ea&s 17* 1o'erts !S* et al+ Selecti%e dorsal r"i/otom&+ efficac& and safet& in an in%estigator6 mas4ed randomi/ed clinical trial. 0e% 7ed ("ild Neurol :0+22062?2* 1AA= :A. 7c>aug"lin JF* .-ornson LF* Astle& SJ* Ea&s 17* Eoffinger SA* Armantrout FA* et al+ !"e role of selecti%e dorsal r"i/otom& in cere'ral #als&+ critical e%aluation of a #ros#ecti%e clinical series. 0e% 7ed ("ild Neurol ?6+ ;;6 6A* 1AA: ;0. 7ittal S* Farmer JP* Al6Atassi .* @i'is J* Lenned& F* @alli (* et al+ >ong6 term functional outcome after selecti%e #osterior r"i/otom&. J Neurosurg A +?1;6?2;* 2002 ;1. 7ittal S* Farmer JP* Al6Atassi .* 7ont#etit L* @er%ais N* Poulin (* et al+ Functional #erformance follo$ing selecti%e #osterior r"i/otom&+ long6term results determined using a %alidated e%aluati%e measure. J Neurosurg A +;106;1=* 2002 ;2. 7ittal S* Farmer JP* Al6Atassi .* 7ont#etit L* @er%ais N* Poulin (* et al+ )m#act of selecti%e #osterior r"i/otom& on fine motor s4ills. >ong6term results using a %alidated e%aluati%e measure. Pediatr Neurosurg ?6+1??61:1* 2002 ;?. 7Pller E+ !reatment of se%ere s#asticit&+ results of a multicenter trial conducted in @erman& in%ol%ing t"e intrat"ecal infusion of 'aclofen '& an im#lanta'le drug deli%er& s&stem* in >a44e JPCF* 0el"aas F7* 1utgers ACF (eds)+ Parenteral 0rug !"era#& in S#asticit& and Par4inson2s 0isease. >ancaster+ Part"enon Press* 1AA2* ## 10?611? ;:. 7uller E* Niers4i J* 0ralle 0* Lraus 0* 7utsc"ler F+ P"armaco4inetics of intrat"ecal 'aclofen* in 7uller E* Niers4i J* Penn 10 (eds)+ >ocal S#inal !"era#& of S#asticit&. .erlin+ S#ringer6Kerlag* 1A==* ## 22?6226 ;;. Nis"ida !* !"atc"er SC* 7art& @1+ Selecti%e #osterior r"i/otom& for c"ildren $it" cere'ral #als&+ a 6&ear e,#erience. ("ilds Ner% S&s 11+? :6?=0* 1AA; ;6. N&gaard !@* Caran SP* >e%ine 1A* Naini A.* ("utorian A7+ 0o#a6 res#onsi%e d&stonia simulating cere'ral #als&. Pediatr Neurol 11+2?662:0* 1AA: ; . <20onnell 7* Armstrong 1+ P"armacologic inter%entions for management of s#asticit& in cere'ral #als&. 7ental 1etardation and 0e%elo#mental 0isa'ilities 1esearc" 1e%ie$ ?20:6211* 1AA ;=. <c"s @A+ )ntrat"ecal 'aclofen. .aillieres (lin Neurol 2 ?6=6* 1AA? ;A. Par4 !S* Kogler @P* P"illi#s >E ))* Laufman .A* <rtman 71* 7c(lure S7* et al+ Fffects of selecti%e dorsal r"i/otom& for s#astic di#legia on "i# migration in cere'ral #als&. Pediatr Neurosurg 20+:?6:A* 1AA: 60. Peacoc4 CJ* Arens >J+ Selecti%e #osterior r"i/otom& for t"e relief of s#asticit& in cere'ral #als&. S Afr 7ed J 62+11A612:* 1A=2 61. Penn 10+ )ntrat"ecal 'aclofen for s#asticit& of s#inal origin+ se%en &ears of e,#erience. J Neurosurg +2?662:0* 1AA2 62. Penn 10* Oor4 77* Paice JA+ (at"eter s&stems for intrat"ecal drug deli%er&.

J Neurosurg =?+21;621 * 1AA; 6?. Peter J(* Arens >J+ Selecti%e #osterior lum'osacral r"i/otom& for t"e management of cere'ral #als& s#asticit&. A 106&ear e,#erience. S Afr 7ed J =?+ :;6 : * 1AA? 6:. Pollac4 7A+ >imited 'enefit of electro#"&siological studies during dorsal r"i/otom&. 7uscle Ner%e 1 +;;?6;;6* 1AA: 6;. Pran/atelli 71+ <ral #"armacot"era#& for t"e mo%ement disorders of cere'ral #als&. J ("ild Neurol 11 (Su##l 1)+ S1?6S22* 1AA6 66. Price @C* Cil4in @P* !urn'ull 7J* .o$er& 7@+ Are 'aclofen6sensiti%e @A.A. rece#tors #resent on #rimar& afferent terminals of t"e s#inal cordM Nature ?0 + 16 :* 1A=: 6 . Pri%at J7* .ene/ec" J* Frere'eau P* @ros (+ Sectorial #osterior r"i/otom&* a ne$ tec"ni3ue of surgical treatment for s#asticit&. Acta Neuroc"ir ?;+1=16 1A;* 1A 6 6=. 1ee%es 1L* Stol#6Smit" LA* ("risto#"erson 7C+ E&#ert"ermia* r"a'dom&ol&sis* and disseminated intra%ascular coagulation associated $it" 'aclofen #um# cat"eter failure. Arc" P"&s 7ed 1e"a'il A+?;?6?;6* 1AA= 6A. 1ifici (* Lofler 7* Lronen'erg 7* Lofler A* .ramanti P* Saltuari >+ )ntrat"ecal 'aclofen a##lication in #atients $it" su#ras#inal s#asticit& secondar& to se%ere traumatic 'rain in-ur&. Funct Neurol A+2A6?:* 1AA: 0. Sanger !0* ("en 0* 0elgado 71* @ae'ler6S#ira 0* Eallett 7* 7in4 JC+ 0efinition and classification of negati%e motor signs in c"ild"ood. Pediatrics* 2006 (in #ress) 1. Sanger !0* 0elgado 71* @ae'ler6S#ira 0* Eallett 7* 7in4 JC+ (lassification and definition of disorders causing "&#ertonia in c"ild"ood. Pediatrics 111+e=A6eA * 200? 2. Siegfried 1N* Jaco'son >* ("a'al (+ 0e%elo#ment of an acute $it"dra$al s&ndrome follo$ing t"e cessation of intrat"ecal 'aclofen in a #atient $it" s#asticit&. Anest"esiolog& 10:=610;0* 1AA2 ?. S#iegel 0A* >oder 1!* Alle& LA* 1o$le& S* @ut4nec"t S* Smit"6Crig"t 0>* et al+ S#inal deformit& follo$ing selecti%e dorsal r"i/otom&. J Pediatr <rt"o# 2:+?06?6* 200: :. Stein'o4 P+ <utcomes after selecti%e dorsal r"i/otom& for s#astic cere'ral #als&. ("ilds Ner% S&s 1 +161=* 2001 ;. Stein'o4 P* 0anes"%ar E* F%ans 0* Lestle J1+ (ost anal&sis of continuous intrat"ecal 'aclofen %ersus selecti%e functional #osterior r"i/otom& in t"e treatment of s#astic 3uadri#legia associated $it" cere'ral #als&. Pediatr Neurosurg 22+2;;626;* 1AA; 6. Stein'o4 P* Eicdonme/ !* Sa$at/4& .* .eauc"am# 1* Cic4en"eiser 0+ S#inal deformities after selecti%e dorsal r"i/otom& for s#astic cere'ral #als&. J Neurosurg 102 (: Su##l)?6?6? ?* 200; . Stein'o4 P* Lestle J1+ Kariation 'et$een centers in electro#"&siologic tec"ni3ues used in lum'osacral selecti%e dorsal r"i/otom& for s#astic cere'ral #als&. Pediatr Neurosurg 2;2??62?A* 1AA6 =. Stein'o4 P* Le&es 1* >angill >* (oc"rane 00+ !"e %alidit& of electro#"&siological criteria used in selecti%e functional #osterior r"i/otom& for treatment of s#astic cere'ral #als&. J Neurosurg =1+?;:6?61* 1AA: A. Stein'o4 P* 1einer A7* .eauc"am# 1* Armstrong 1C* (oc"rane 00* Lestle J+ A randomi/ed clinical trial to com#are selecti%e #osterior r"i/otom& #lus #"&siot"era#& $it" #"&siot"era#& alone in c"ildren $it" s#astic di#legic cere'ral #als&. 0e% 7ed ("ild Neurol ?A+1 =61=:* 1AA =0. Stein'o4 P* Sc"rag (+ (om#lications after selecti%e #osterior r"i/otom& for

s#asticit& in c"ildren $it" cere'ral #als&. Pediatr Neurosurg 2=+?006?1?* 1AA= =1. Stein'o4 P* Sgouros S+ )nternational 3uestionnaire of selecti%e dorsal r"i/otom& and intrat"ecal 'aclofen for s#astic cere'ral #als&. ("ilds Ner% S&s 1A+6?:* 200? =2. !ardieu (* >es#argot A* !a'ar& (* .ret 70+ For "o$ long must t"e soleus muscle 'e stretc"ed eac" da& to #re%ent contractureM 0e% 7ed ("ild Neurol ?0+?610* 1A== =?. !uri 7* Lalen K+ !"e ris4 of s#inal deformit& after selecti%e dorsal r"i/otom&. J Pediatr <rt"o# 20+10:610 * 2000 =:. Kargus6Adams JN* 7ic"aud >J* Linnett 0@* 7c7a"on 7A* (oo4 FF+ Fffects of oral 'aclofen on c"ildren $it" cere'ral #als&. 0e% 7ed ("ild Neurol :6+ = * 200: =;. Kerrotti A* @reco 1* S#alice A* ("iarelli F* )annetti P+ P"armacot"era#& of s#asticit& in c"ildren $it" cere'ral #als&. Pediatr Neurol ?:+166* 2006 =6. Ceiss )P* Sc"iff SJ+ 1efle, %aria'ilit& in selecti%e dorsal r"i/otom& J Neurosurg A+?:66?;?* 1AA? = . Crig"t FK* S"eil F7* 0ra4e J7* Cedge JE* Naumann S+ F%aluation of selecti%e dorsal r"i/otom& for t"e reduction of s#asticit& in cere'ral #als&+ a randomi/ed controlled trial. 0e% 7ed ("ild Neurolo :0+2?A62: * 1AA= ==. Oasuo4a S* Peterson* EA* 7ac(art&* (S+ )ncidence of s#inal column deformit& after multile%el laminectom& in c"ildren and adults. J Neurosurg ; +::16::;* 1A=2 Abbre)iation !otes A0> Q Acti%ities of 0ail& >i%ing; (P Q (ere'ral Pals&; @A.A Q R6Amino'ut&ric Acid; @7F7 Q @ross 7otor Function 7easure; )!. Q )ntrat"ecal .aclofen; 1(! Q 1andomi/ed (ontrolled !rial; S01 Q Selecti%e 0orsal 1"i/otom&; SPN Q Selecti%e Peri#"eral Neurotom&