S TO THE EDITOR

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The treatment of infantile spasms by paediatric neurologists in the UK and Ireland

SI R-lilfcrritile .spnsur.s (\tdiich cliaructerise West .syriclronie) Iiuve been the sirbject of a iiirriiber of reviews'. ' within the pcrst fe\t* years. predominaritly becoirse of achwices in their irivestigatio~i, classifctitiori arid tretrtrirerit-'. 4. Two recent reports hove sirrveyed the rn@ical trearrnerrt of sptisiiis Oy pmt(iatric rieirrologists in the UiiitetI Stotes' aj!d J [ I ~ I I P , where corticotro~~iri/rrtlreriocorticotro~~lric horriiorre (ACTH) crrid vitiiiiiin BdACTH \\*ere the drirgs of preferred choice respectively iri these t rrw corrntries.

A sinrilcir srrrve~ bvirs receritly irndertaken rrriiorig 41 comiil t i i i i t ptrerlitrtric tierimlogists in the Ujiited Kirigdoni tirid Irelarid. The siirvey w i . s

corirlircted by postal clirestionnri ire ,ihich asked the following: whetlier the cliriicitrii I I * ~ nioinly iiir-olved with treating spasiris. (idvising ori trentnierit. or both: crnd the preferred first-liiie drirg for treciting syniptorriutic sposrris and the preferretlfir:~t-lirie drirg for trecrting cry~~togenic/idiol~ntIiic spasms. Resportdents l i ~ d the optiori of renicriiiirig cuioIi.wioir.s. The cpre.stionrioires were tlistribirted in J d y I995 t ir id mitrlysed in Noveiiiher 1995.

Thirty eight coriipleted rlirestioiinrrir-es were r-etrrriied. represeiiting a resporise rate of 93%. N o otteiiipt w i s iiirule to con tcict the r h ree iioir -respondents. irr

vie,\- of the excellent iriititrl resporise. Nirieteeri clinicicrns (50%) stcited thnt they iiioirrly treated children with spasms, I I e&lly treated arid trdvised or1 treutiiieiit arid eight only provided trdvice 011 tremtnieiit. Tlrere was 110

obvioirs differerice between these three groirps. iri terms of the preferred firsr- lirie drugs. Tlierefore. the following Table shows the drugs offirst choice for all 38 clinicitiris.

TABLE First line drug for the aetiology of infantile spasms

Vigabatrin 29(769) I7(45%) ACTH 5( 13%) 14(37%) prednisonel 1(3%) 6( 1 5 8 ) betamethasone sodi urn 2(57r) 0 valproate pyridoxine I(3%) l ( 3 8 )

Tlrere were ( I iiirniber of cliriiciaris diose first-line drug depended rrpori the cretiology of die ir!foritile sptrsms; I 7 cliriiciaris (45%) stcited that they n-oirld rrse/recomiieiid \igcibatriri in the t rea ti i i en t of spasiiis irrespective of cretiology: 5 cliriiciaris ( 13%) sttired tlicrt they ~voultl ir.se/recowiinencIl ACTH irre.specti\*e of aetiology: arid 13 clinicicrns (34%) stcited thcit they worrld

irse/recoriir~ierirli vigabarrin for syniptorntrtic, mid A CTH for c~ptogeniclidiopothic spersiiis.

betweeri the use of vigahatriii tirid ACTH iii cqptogen ic/idiopath ic c i n d syriiptoriiatic spasms; one interpretcrtioii of this discr-rporicy is n r-rlirc1oiicr t o rise vipbotriii iri cr?tptogeiiic/iniopcrtliic spcrsins. The reasoiis fhr this [ire riot eiitirely clear: oiie explnriatioii (sirggested Dy ( I f ew of the resporideiits) wa.s the coIic'eiii tlicit these types of spcisni may represerit (111 isolated. transieiit mid age-related epileptic 'encei~licrloporliy ' wliicli is best trecitetl I>! a brief. firiitr arid 'proveri' coirrse of treotriient wliich is specificnlly tcrrgettiiig aii ' e i ice~~l~alo~~arl iy ' (i.e. steroids). Ho\vever, there i.? no coiiviricing evidence tlin t CI I I N ci r te 'er i cephci l o p tli y ' is responsible for e\*ery child with cnptogeiiic/iclioptitliic .sprisms. 111 nddiriori, there is iio reasori why \~igcibtitriri coiild riot tilso he used for ( I

siriiilar brief aricl finite period. Another iriicoiriirio~i, thoirgli

irriderstnii~ltrhle. coricerii e.vpre.s.sed in N

few c1irestioiinoirc.s. w m tlie lorig-terrii developiiientiil stlttris of irvirrits trecrted \t*itli vigdxrtriii. irrespective of spcrsiir- coritrol. There is little irrforwcrlion to . either co1ij7rrii or rejrte this co~icerii. Howvver. iri t iew of the relotii*ely short tiriie that tlie clrirg 1irr.s lweii rrsed ( I S .first- lirie treatiiierit for spcrsnis. preliniiiini? dnto ci re ~ I I C O i rrrig ir ig 7. This clen rl! reelirires jiirtiier r vcilirtrtion.

bet\c~een coirntries ~~r~~c l~~r i i i n t i r i t l~ reflect the fcrct h i t vignbatriii is riot cirrreritl! cr\wildde in the USA crnd Japa~i; ho~wver. there nicry he additioncil ~ ~ N S O I I J , siriiilur 1 0 tliose e.ipre.ssecl in the UK s i r r ~ ~ e y , c is well 0 s persisting (riltllo~rgh lulp,.o\~eI1) coi1c~'rIl.s aboirt the drug 's sufet! profile. I t is iiesei.tlie1es.s likely thtir were \igoOcitriri mwilable (t i t least in the USA). it \t*oiiItl be prescribed f . r cliildreri witli ir ferntile sp~isnis (persoiicil clcitri ). FiwcrllT. tliere is rrlso ir dige re1 ice of opir i ioii reg(/ rciing \igobntriri ( I S the iiiiricil t i ~ e ~ ~ i w i i r rrQithiri Errrope" '. c1iniciciri.s tind co1leagire.s ~ V i o

There is a c l e w discrepuncy

Tlie dij'fererit treatirierit reA' ' I I I 1 es

I ~*orrld like to tlicirik (ill those

contributed to this siirvrv orid who responded with sirch rilrcrity arid eiirhirsicrsiti.

RICHARD E APPLETON Consultant Paediatric Neurologi\t

Roold Dnlil EEG Unit, Roytil Liverpool Children's N H S Trirst. Alder Hey Liverpool L12 2AP h'~frrc.iicc~s I :Appleton RE. ( 1993) Infantile spasms. Arc/ri\.c.\ of

3. Chugani HT. (1995) Infantile spasms. Currrrif Opiriiori i r i N r i i w l o ~ ~ 8: 13944.

3. Chiron C. Dulac 0. Beaumont D. Palacips L. Pajot N. Mumford J. ( 1991 J Therapeutic trial of vigabatrin in refractory infantile spasms. Joirrrrtrl oJ Child rVuirm/o.cp 6: (wppl 3) SS2-9.

4. Applcton RE. Montiel-Vicsca F. ( 1993) Vigabatrin in infantile spasms - why add on? Ltriri~ci 341: 962.

5 . Bobclc GB. Bodensteincr JB. (1994) Thc treat- ment of infmtilc spasms by child ncurologists. Joirriid of C/ri/t/ NurrrfJ/o.q,v 9: 433-5.

6. Watanahe K. (1995) Medical trratment of West syndrome in Japan. Jorrr-rid of C/ii/d Nrrrro/og,v

7. Applcton RE. Nicolaidcs P. ivlonticl-Viesca F. ( 1995) Vigabatrin monotherapy for infantilc spasms. Epilcpsicr 36: (suppl 4) 37.

X. Vlck JSH. van dcr Heydcn AMHG. Ghijs A. Troost J. (1993) Vigabatrin in the treatment of infantile spasms. Nurtr~~~~etl i t i ir ics 24: 230- I .

9. Schmitt B. Wohlrab G. Boltshauser E. (1991) Vigabatrin in newly diagnosed inlhntile spasms. N~,rirr,fu,rrlirrrri(..s 25: 51.

Di.vrt/.sl* ill C/~i lc l /~o~~J 6Y: 6 14-8.

10: 143-7.

'Iodine and brain development'

SIR-Tlie wiiiot(itioii Iodirie mid Brairi Developriieiit (DMCN 37. 744- 748) by Peter OD Plitrrooh tirrd Kr\*iii J COIIIIOIIT is (111 e.rcelleiit reliew of er mijor topic in I i i i i ~ i ~ i ~ i rierrral dc Idopi i ie i i t. \\*titter i by cr I I tho rs 1 rho I i m ~ riicitle imdriiark cwitribirtioris i ; i

th is .field. Ho \ re 1.e r. th ei I' desc rip t ior I c$ the ririiirig of fetnl brcriii \vrlnercrbilir~ to iodine (rrrid tliercfore thTroid liorrriorie) tlrjicieric:\. r-eqirires (I repl!. All ohsci-vei-s ~ ~ o i r l d eigree \t*itli their stcrtemeiit tlitrt the f e t d riel-wits sFsterii riirry be ~wlriercrble t o lioririoiie restriction reailtirig jiorri the riitilfiriictioiiirig of both iiintei-rial a r i d fettrl thyroid gltrrrtls. m d that tliis is iniporto~it iri die cliriicnl s.vritlroiiie c.f eiicleniic cretininti (irieritcil rettrihtion. spastic-rigid niotor disorder. r r i i d detrf- iiiirtisra). This . ~ y ~ i d r - ~ ~ i i r is ty~icrrlly n i i r i h iiiore severe tlirrri spcmidic

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