differential diagnosis facial paralisis 1957

8/3/2019 Differential Diagnosis Facial Paralisis 1957

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1957;19;303-316PediatricsRichmond S. Paine

Report of Ten Cases Treated with CortisoneFACIAL PARALYSIS IN CHILDREN: Review of the Differential Diagnosis and

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Online ISSN: 1098-4275.Copyright 1957 by the American Academy of Pediatrics. All rights reserved. Print ISSN: 0031-4005.

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REV IEW ART ICLEFAC IA L PARA LYS IS IN CH ILDREN

Rev iew o f the D iffe ren tia l D iagnos is a nd R epo rt o f T enC ases T rea ted w ith C o rtis oneBy R ichm ond S . P a ine , M .D .

D epar tm en t o f P ed ia tr ics , H arvard M ed ica l Schoo l, an d the D epar tm en tfM edic ine o f the C h ild ren s M ed ica l C en te r, B os ton

(S ubm itted A pr il 9 , ac cep ted Ju ly 3 , 195 6 .)ADDRESS : 300 Longwood Avenu e , B oston 15 , M assach use tts .

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P ARALYS I S of the fac ia l ne rve is o ften re -fe rred to as B ells P a lsy in asm uch as

S ir C harles B e ll in 18211 described theco urse of th e fac ia l n erve and d ifferen tia tedits fun c tion from th at o f the trigem ina l.He a lso p re sen ted seve ra l ca se s o f fac ia lpara lys is, som e of th em assoc ia ted w ithtraum a o r in fec tion , an d o thers id io pa th ic .F ac ia l pa ra lys is is a sym ptom ra ther thana d isease and can be due to a w ide varie tyof underly ing causes . A rtic les rev iew in gthe sub jec t o f facia l pa ra lysis have beenpub l ished ( Park and W atk ins ,2 C ollie r,3Su lliv an and Sm ith ,4 Jam es and R ussellbu t th ese a re co ncerned p rin c ip ally w ithadu lts. Ex perience h as ind icated tha t a re -v iew of the sub jec t as it ap p lies to in fan tsan d ch ild ren m ay be o f in te res t and va lue .D iffe ren tial d iagn osis am ong the va rio uscauses o f fac ia l pa ra lys is w ill be d iscussed ,an d exp erien ce w ith 10 cases trea ted w ithcort i sone w ill b e presen ted .

ANATOMY OF THE FAC IA L NERVE(See F igu re I)

The m otor n uc leu s o f the fac ia l n e rve is bo -ca ted in the p on tine tegm en tum , near the sp in altrac t and n uc leu s of th e trigem ina l nerv e.F rom th e nuc leus, the axones ru n sligh tly up-ward , pas sin g ben eath th e floor of th e fo urthven tric le and fo rm a g enu around th e nu cleusof the ab ducens nerv e. T hey then em erge fromthe bra ins tem at the low er end of the po ns,iI itO the cereb ebbopon tine space . F rom therethe n erve runs upw ard an d fo rw ard to thein ternal au d ito r m ea tus , in clo se associatio nw ith the acous tic nerve and w ith the nervusin term ed ius , th rou gh wh ich v isc eral efferen t

fibe rs jo in the fac ial n erve . T he gen icu la tegan glion is fo und a t an acu te tu rn at the d is ta len d of the in ternab aud ito ry m ea tus ; the n ervefibe rs co ncerned in lac rim ation leave a t th ispo in t v ia the g rea te r superf ic ia l p e tro sa l ne rve .B eyond the gen icub ate gang lion , the fac ia l ne rveruns la te ra lly an d ch ie fly d ow nw ard , th roughthe fac ia l cana l, and g ives o ff a sm all b ranchto the staped ius m usc le. It then passes th roug hthe s ty bom asto id fo ram en , tu rns upw ard an dan te rio rly to p ass th rou gh th e p aro tid g lan dand d iv ide in the pes anse rinus in to its te rn -porab , z ygo rna tic , buccal, m and ibu la r, and cer-v ical b ranches , w hich inn erva te the m uscu la tu reof the face . V isce ra l e ffe ren t fibe rs concernedw ith sa liva tio n orig in ate in th e sup erio r sal i-va to ry n uc leu s, jo in the fac ia l ne rve in thenervu s in te rm ed ius , and pass in to the chord atym pan i nerv e, w hich leaves th e fac ia l ne rvew ith in the facia l cana l and p asses over thein ner su rface of the ear d rum , th rough th epe trous bo ne , and then jo ins the ling ua l b ran chof the m and ibu la r n erve to fina lly a ffec t thesec re to ry activ ity o f the sub ling ua l and sub -m ax illa ry g lands v ia a sy napse in the su bm axil-la rv gang lion . T he sensory neuron es of thefac ia l ne rve lie in the gen icu la te gang lion ,w here the ir sing le p rocesses d iv ide in to a T ,the cen tral en ds then p assin g v ia the nerv usin termedius int o the pon s n ear the p o in t o fex it o f the m oto r part o f th e fac ia l n e rve , andend in g a t the nuc leus of the trac tus so litar iu s,co nv eying taste sensa tion . Sensa tio ns of ta ste ,subserved b y these neu rones from the an te rio rtw o -th irds o f the to ngu e, are co nv eyed in th elingua l and cho rda tym pan i n erves and reachthe g en icu la te gang lion by the sam e rou te assa liva to rv im pu lses bu t in the reverse d irec tio n .Som e tas te sen sa tion fibe rs m ay pass from th echord a tvm pan i to the o tic g an g lion and th en

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-TA S TE S AL IVAT IO N LA C R IMAT IO N

304 PAI NE - FACI AL PARALYSI S I N CHI LDRENreturn i n the greater superf i ci al petrosal nerveto the geni culate gangl i on accordi ng to someauthori ti es. Some sal i vatory impul ses may con-cei vabl v f ol l ow a sim i l ar path i n reverse, w hi l eothers probabl y pass ori gi nal l y i n the gbosso-pharyngeal nerv e, f rom the petrosal gangl i onv ia an ex tracraniab anastomotic branch, to thefacial , upward through the stybomastoi d f ora-m e n , and thence into the chorda tympani . T hefacial nerve also transm i ts superf i ci al sensati onf r om p a r t o f the palate and posteri or nasalmucous membranes and, debatably , f r om p ar tof the ex ternal ea r n ear the ex ternal audi toryme a t u s ; the last-mentioned innervation i s inclose associati on w i th the vagus, i f i t i n f actexists. Final l y , propri ocepti ve sensation f romthe faci al muscles, but probabl y not deep sen-sation f rom the face i tsel f , i s thought to be con-veved b the faci al nerve.

A study of Figure 1 w i l l make i t apparentthat to some ex tent the anatom ical l oca-ti on of the lesi on causing a peripheral typeof f acial paral ysi s ( bearing in m ind that anuclear l esi on in the brai n stem producesthe per ipheral type of weakness) may bepredicted f rom classi f i cati on according topossible accompany ing def ects i n bacrima-ti on, sal i vati on f rom submax i l l ary and sub-

l i ngual glands, and taste. T he occurrenceof hyperacusis should place the lesion abovethe point of branching of the nerve to thestapedius muscle, but i s not general l ycl i ni cal ly useful because few chi l dren gi vea history of i t, and i t i s di f f icul t to ev aluateobjecti vely . D etai l s of the cl i ni cal f indi ngsin f aci al paral ysi s cl ue to l esi ons atariousanatom ical l ocations are gi ven in T able I .I n a chi l d past the age of 4 oryears, i tis f requently possible to test taste i n theusual manner used w i th adul ts, by hav inghim extend the tongue and then droppingsoluti ons f lavored sw eet, sal ty , sour, andbi tter on the anter ior porti on of the lef t orri ght side. Since young chi l dren cannotpoint to the correct answ er on a printedcard, as can adul ts, i t may be more sati s-factory , af ter f i rst explaining w hat i sn-tended, to say the w ords sw eet, sal ty , sour, and bi tter, and ask the chi ld tonod his head when one reaches the correctdescripti on. N eedless to say , the test mustbe completed w i thout the chi l d w i thdraw -ing the tongue into his mouth and spread-ing the substance to other taste areas. Evenw i th young patients who are not able to

FIG. 1. A natom ical arrangement of the faciale r v e . The course of the niotor f ibers isshown i n gray .Relati ve length and si ze have been somewhat distorted for cl ari ty and to perm i t l abel l i ng. There i s ananastonioti c connecti on f rom the faci al nerv e to the auri cul ar branch of the vagus; thi s l eaves the nervein the faci al canal , but has been om i tted f rom the draw ing. Possibl e secondary pathw ays f or taste and

sal i vati on are descri bed in the tex t, but have b2en om i tted f rom the draw ing for simpl i f i cati on.



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! / ) c a t i o Ft o f Le s i o n

Voluntaryifore-meat.t p / ) e rI a e

VoluntaryMo r e -me n t sLowerFare

Bmolwnai

Move-ments

Salivat ionLacr ima- Subma.r.

l i on and Sub-l ingual

i p e r -Taste OPUSlX

Reta inedlostLo s t

Lo s tL o s tL o s t

L o s t

RetainedL o s tL o s t

L o s t

RetainedRetainedUsual ly

l o s tL o s t

Reta inedRe t a i ne dUsual ly

l o s tL o s t

RetainedRetainedUsual ly

l o s tL o s t

A l , s e n tPresentP r e s en t

Presei l t

Lo s t L o s t R etained L os t L o st Prese n t

L o st L os t R etaine d L o st L ost A l) se i i t

L o s t L os t R etained R e tain ed Re ta ined A l , s e n t

S ariab le \ariah le lartlv R etaine d R e tain ed R etaine (l A l)se f ltl os t

R EV IEW A R T IC L E 305TABLE I

(ix i F IN n I N G S IN F. J .& L I A U A L Y S E S 1 )u E T O L E S IO N S O F V A R IO US A N A T O M I ( L O C A T IO N S

pra I I UC1 ea rNuc lea rh it racran ial (be tw een pons an d

jut . illl(l. meatus)lii ( 31 10 11 at or abo v e g en icu late L o st

g il o gl io nBe tw een gang l ion and n erv e to L o s t

stape( l iusB etw een sta pe d iu s and cho rda L o s t

t y i npan iB e tW ( P i l charlia ty n ipan i aiic l L o st

hraiicl i ingIi i J,tS ai iseri i ius

id en tif y a f lav o r, it m ay b e obv ious f romthe f ac ial ex pression that the patien t taste ssome th ing ( usually unp leasan t) and th isw ill ind icate re ten tio n o f taste . T aste sensa-tion w as lo st in 52 o f the case s o f B e llspalsy desc rib ed by Park and W atk in s2 sothat as an iso lated tes t, it is n o t o f d if f e r-en tial d iagn ostic v alu e. It is su pposed to bean un favorab le p ro gno stic s ign , ind icatinga le sion relativ e ly h igh in the f ac ial can al.E v en tu al com p le te reac tio n o fegenerat ionw ith late and on ly partial recov ery w as50% m ore com m on am ong patien ts w ith b ossof

It is po S S il)le to te st saliv ation quan tita-tiv e ly by the u se o f sm all co tton balls w h ichhav e l)een w eig hed . O ne o f a pair is p lacedagains t the p rev iously dried orif ice o f thesu bm ax illary or sub lingu al d uc t on e ithe rside and th en w eig hed again in its w e igh -in g bo ttle af ter saliv ation has been stim u-lated by p lac ing m ustard or a s im ilar sub -stan ce on the ton gue . B earing in m indth at the f low o f saliv a f rom the paro tidgland is con tro lled th rou gh the au ricub o -tem poral ne rv e , on e m u st co v er the orif ic eo f the p aro tid duc t o n each side w ith co t-to n w hich , how ev er, n eed n o t he w eig hed .Onl y a sev e ral-f o ld d if f e rence b etw een thetw o s ides is sign if ican t inhe cases o f su b -

m ax illary or sub lingu al saliv ation . T he te stis te chn ically d if f icu lt to perf o rm and no tespec ially use f u l, inasm uch as re ten tion orlo ss o f saliv ation parallels th at o f taste . Ate chn ique sim ilar to that u sed to m easuref low o f saliv a can b e u sed to m easu re rateo f lac rim atio n af te r stim u latio n by inh ala-tion o f am m on ia. It is o f som e im portan ce ,s in ce f acial p araly sis w ith lo ss o f lac rim a-tion on the af f e c ted s ide can re su lt f romsom e in tracran ial le s io ns and also , in rarecases , f rom tum o rs o f th e g reat superf ic ialpe tro sal n erv e near th e gen icu late gang lion ,as in the case reported by T rem b le an dPen f ield .l7 In th e great m ajority o f case s ,inc lud ing all o r prac tically all o f the id io -p ath ic gro up , lacrim ation is re tain ed . R e -assurance m ay be o b tained b y the ens -tom ary statem en t o f the patien ts m o therthat ep ipho ra ( ov erf low o f tears) w as n o tedat the onset o f th e f acial paraly sis b ecauseth e ey e rem ain ed open . If a c lear statem en to n th is po in t is o b tained , g rav im e tric m eas-urem en t o f lac rim ation is unn ecessary .

CLASS IF IC AT ION AND ET IO LOGY OFFAC IAL PARALYS IS

I t is obv ious that f ac ial p araly sis can re-su it f rom les ions any w h ere in th e courseo f th e f acial ne rv e, an d that the reus t



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3 0 6 PA I NE - F ACI AL PARALYS I S I N CHI LDREN

G . Idiopathic

be a w ide varie ty o f possible causes. Themore frequent causes o f cong enital as w e llas postnatally acquired fac ial paralys is areg iven in Table II.

Facial paralysis as a congenital anomalyse ldom occurs as an iso lated phenomenon,but usually in assoc iation w ith other ab-no rmabities , chie f ly neurobog ic . Moebius6in 1888 described a 35 -y ear-o ld man w ithcong enital bilateral paralysis o f the six thand seventh cranial nerves, paralys is o f the

TABLE I IETI0LOGI( CLAssIFIcATIoN OF FACIAL PARALYSIS

I . Congen i t a l (i.e ., present atbirth o r a t l e a s t n c te d5 0 0 1 1 afterward)

A . Congenital anomaly (Moebius syndrome6 )B . Trauma

1. Skull f racture. Intracranial hemorrhag eS. Pressure from forceps4. P r e ssu r e a ga in s t m a t er n a l sac r u m 7

I !. P ostna la lly A cq u ir edA. Trauma

1. Sk u l l fractures S u r g i c a l3. Accidental

B . I)iseases of the skull1. Osteomyelitis O ste ope tro sis ( Albe rs -S ch# {2 46 }nbe rg s dise as e)3. Idiopathic infantile hypercalcem ia (severe

type)8C. Intracranial causesI . Neoplasm s, absces ses o r other space -oc cupy-

ing lesians2. H ypertension93. Intracranial pressure of whatev er cause , in-

e ludi ng he mo rrh ag e1) . Extracranial mass le 8 ions (ineck)E . Infections

I . O t i t i s media w ith or w itho ut m asto iditis2. Poliomyelitis3. Men i n g i t i s4. Encephalitis5. G uillain-B arr#{23 3} syndrome6. Facial neuritis assoc iated w ith other in-

fe ctio us pro ce sse sa. C ephalic te tanu& #{ 17 6}b . T ri ch in o si s c. N eu rosyp hilis2d. Leprosy3e. Herpes z o st e r (R am sa y Hunt syiidron:e)4

F. Syndrome of fam ilial re lapsing facial paralys isI . O ccu r r in g as i s o l a t e d finding5. Associated w ith edema oflip or fac e (Me l-

kerssons syndrome)6

ex tenso r musc les o f the w rist, lo ng flexo rso f the fingers and thumbs and the extensorcarpi ulnaris , as w e ll as w ebbing o f secondand third fingers o f the right hand. Theterm Moebius syndrome is generally ap-plied to congenital bilateral six th andsev enth nerv e paraly sis , butn a broads e n s e inc ludes a number o f variants. Hen-derson7 in 1 939 tabulated 69 cases o fw hich only 14 had fac ial dipleg ia alone .Congenital paraly sis o f o ther cranial nerves,espec ially the s ix th, is the most frequentaccompanying anomaly , but many o thersmay occur. M ental defic iency , how ever, isinfrequent. Four of Hendersons cases hadunilateral facial paraly sis , and one had in-vo lvement o f the low er half o f the faceonly . W e hav e seen tw o patients w ith w hatis be lieved tobe congenital fac ial paralysiso f only one side of the face and in one o fthese , only the low er part o f the faceseemed to be affec ted. Such a situation isplausible if one cons iders that the upperhalf o f the face in man has a bilateralsupranuc lear cortical representation by thecorticobulbar trac t w hereas the low er halfo f the face is only contralaterably repre-sented and it is there fore possible that thepo rtio n o f the fac ial nuc leus concernedw ith the bow er half o f the face may be tosom e extent anatom ically discrete from thatconcerned w ith the upper half . In anyev ent, unilateral and low er fac ial paraly sesas congenital anomalies are rare and shouldalw ay s be v iew ed w ith suspic ion. The prin-c ipal differential diagnosis is from persistentfac ial paralysis due to pressure either byfo rceps or by the maternal pe lv is , althoughsuch cases usually recov er. Congenital fac ialparalysis may occur also in other combina-lio ns than those described by Henderson.It is the most common cranial nerve paraly -s is accompanying the ptery g ium co lli syn-drome of B onnev ie -U llrich and in one re -po rt w as found in 11 o f 17 7 cases, 1 o fthem unilateral.8 It may be fam ilial, andit may also be assoc iated w ith contrabateralpyram idal trac t signs (Trautman9 ); in suchcases differentiatio n from pontine tumor isimportant. Ford,2#{ 176 } among o thers, has re -cently raised the questio n whether con-



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REV IEW A RT IC LE 30 7

I t is w e ll know n tha t in p o liom ye litis ,

g en ita l facia l pa ra lysis is in fac t a co ng en italap las ia o f m usc le ra th er than absence o fthe fac ial n e rve nuc leu s as h as been gener-ab ly assum ed to be the case , and m ay becom parab le to D u ane s sy ndrom e in w hichth e ex terna l rec tus m u sc les o f th e eye arerep laced b y fib ro us co rd s. R ich ards2 re -ports th ree cases in w hich b iopsy of thefron ta lis m usc le sh ow ed to tal ab sence ofm usc le tissue . R a iny and Fow ler22 rep orteda 10 -w eek-o ld ch ild w ith facia l d ip leg iaw ho at necrop sy show ed d eg enera tion ofth e seven th nuc leus and fac ia l n erve fibe rsbu t no t to ta l ap la sia . O n the o the r hand ,m ost o f the scan ty necropsy m ate ria l seem sto in d icate absen ce of nu clea r ce lls a s w e llas of o th er cen tra l n ervo us system e lem en ts ,as f or example the case o f b 23 Th eprim ary d efect in th is situa tion can no t b es tated a t p resen t. T he en tity needs to bew ell kn ow n as it is a re lativ ely frequ en tone , a lthou gh a p atien ts h isto ry w ill o rd i-na rily sugges t w he th er th e situa tion is con-gen ita l o r p ostna ta liy acqu ired .

C ongen ita l fac ial pa raly sis w h ich isp re sen t a t b irth bu t su bsequ en tly clea rs , ha sg en erally been assum ed to be due to pres -su re from obste trica l fo rceps o n the faceand pes anse rinu s. Th e s tudy of H epn er ,7how ev e r, is ex trem e ly ch alleng ing an d in -d icates tha t m any , if n o t m os t, cases a rep rob ab ly d ue to p ressu re o f the in fan tsface ag ain st the m oth ers pe lv is , pa rticu la r lyth e sac ral p rom inence . H epn e r foun d tha tall o f 40 in fan ts w ith le f t fac ia l pa raly sisw ere bo rn from LO A o r LO T positio n w h ilethe s itu a tion w as reve rsed in the case o f16 w ith r igh t fac ia l p ara lysis . M o st frau -matic facia l pa ra lys is in the n ew born p eriodis due to pressu re on an d sw ellin g abou tthe fac ia l ne rv e in its ex trac ran ial cou rse .E xcep t fo r ca se s w ith obv ious lace ra tion s,n eu ro su rg ical in te rv en tion is no t necessa ry ,and recovery takes p lace in the grea t m a-jo rity . It shou ld be b orne in m in d th a t inthe n ew bo rn p eriod , as in la te r life , facia lpa raly sis m ay b e a sym p tom of in trac ran ia labno rm ality , m ost frequ en tly h em orrh ag ein th e case o f the n ew born .

A m ong ch ild ren w ith fac ia l p ara lysis ac -qu ired su bsequ en t to the new born period ,

the g rea t m ajo rity o f cases a re eith er as -soc iated w ith o titis m ed ia o r are id io pa th ic .S om e au tho rs re str ict the use o f th e te rmB ells p a lsy to th e id iop a th ic g rou p . S om ecases , how ever, a re d ue to traum a. F ac ia lpa ra lys is occas iona lly resu lts from os teo -m yelitis o f the sku ll, o r hy perosto sis com -pressing the fac ia l am ong o th er c ran ialne rves, such as o ccasion ally occu rs inA lbe rs -S chonbe rg s d isea se (osteope trosis)o r the seve re ty pe o f in fan tile h ype rca l-cem ia . 8

A num ber o f in trac ran ia l p roces se s, pa r-ticu la rly n eo p la sm s, abscesse s and o th erspace occupy ing lesio ns, can resu lt in fac ia lpa ra lys is. T hese on ly ra re ly cause iso la tedfac ial p ara lysis w itho u t o the r n eu ro log icsigns, becau se o f the p rox im ity o f o the rstru ctu res in th e po ns to the fac ia l ne rveand its nuc leus. T he m ost f requen t tum orsin the pos ter io r fo ssa are th e astrocy tom a ,m edu lbob las tom a , an d pon tine g liom a . T helast-m en tio ned tum or m ay cause th e fam il-ia r com b in ation of fac ia l pa ra lys is (usua llyw ith abd ucens para lys is as w ell) w ith con-tra la te ra l p yram ida l trac t s igns . Sup ra ten -to na l tum ors can a lso cause fac ia l pa ra ly sisand , th rough obscure pressu re m ech an ism s ,m ay a t tim es s im ulate the pe riph e ra l, o rlow er m o to r neu ron , type of w eakn essrath er th an the sup ranu clea r.24 26 In tra -cran ia l p re ssu re a lon e occasio na lly p rodu cesfac ial p ara lysis , bu t in vo lv em en t o f th esix th n e rve w ith its lon ge r in trac ran ia lco u rse is fa r comm oner. H ype rtensio n hasbeen rep orted as a cau se of fac ial pa lsy 9an d m ay do so b y a va rie ty o f m echan ism s,in c lud in g pres su re o f a v es se l ag ain st thene rve itse lf, o r h em orrh ag e, an eu ry sm , o rin trac ran ia l p re ssu re.

O f id en tifiab le in fe c tiou s cau ses o f fac ia lp ara lys is in ch ild ren , o titis m ed ia w ith orw ithou t m as to id itis is b y fa r th e comm on-est. T h e p ro gno sis is u sua lly felt to b e le ssfavorab le than in id iop a th ic case s, bu t them a jo rity recov er w ith p rom pt e ffec tivet rea tmen t of the in fec tion . R oen tgenog ram so f the m asto ids shou ld be taken in eve rycase o f o titis accom pan ied b y facia l p ara ly -sis.



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30 8 PA INE - FA C IA L PARA LY SIS IN CH ILD RENthe facia l n erve is o ften inv o lved to ge the rw ith o the r low er c ran ial ne rv es . T he p araby-s is is nuc lea r, and of the periph era l type .In som e ou tb reak s it ha s n o t in freq uen tlyoccu rred as an iso lated sig n o f b ubba rpo liom ye litis .2 7 Iso la ted fac ia l pa ra lysis m aya lso be du e to po liom yelitis even in theab sence of p beocy tos is in th e sp ina l flu id .T hree su ch cases w ere seen at th e C h il-d ren s M ed ica l C en ter du ring the ex tensiv e1 955 po liom yelitis ep idem ic , and co nfirm edby iso la tion of v iru s from the sto o l, ris e inan tibody tite r in the b loo d orbo th .28 W h ileit is u sua lly sta ted tha t c ran ia l n e rve para ly -ses in bu lbar po liom ye litis a lm os t in va riab lyd isappear if the pa tien t su rv ives , they m aybe perm anen t in an ap prec iab le num berand p ersistence of fac ia l pa ra lys is o f 10o tit o f 37 cases w as rep orted in on e se rie s .29

G uilla in -B a rr#{233 } synd rom e c la ssically be -g ins w ith symm etrica l flacc id p aralys is inthe ex trem ities , w ith v ariab le senso ry find -ings , and in m any cases symm etrica l c ran ialne rv e pa lsy , m o re o ften fac ia l d ip leg ia th anan y o th er . O th er synd rom es of po lyn eu ritisw ith fac ia l pa ra lys is have been reportedw ith o r w ith ou t the sp ina l f lu id f ind ings o felev ated pro te in in th e absen ce of cells3 #{176 }typ ica l o f G u illa in -B arr#{233} syndrom e, b u t itsh ou ld be bo rne in m ind tha t pa tho log ica llytyp ica l G uilla in -B arr# {233} sy ndrom e m ay o ccurw ith alm ost any sp ina l f lu id fin d ing .3 1

M o st o f the o the r in fec tious cau ses o ffacia l pa ra lys is lis ted in T ab le II a re read ilyiden tifiab le by the ir o the r sym ptom s o r labo -ratorv f ind in gs. F acia l pa ra lys is a sso cia tedw ith he rp es zos ter o f the ex te rn al ear o rau d ito ry m ea tus ( th e syn d rom e o f R am sayH un t, d ese rve s som e com m en t in sp ite o fthe re lativ e in frequ en cy of he rpes zoste rin ch ild ren . T h is sy ndrom e is supp osed tobe based on h erpes zoste r o f th e g en icuba tegang lion w ith the ves icu la r e rup tion inh esensory d istrib u tion of the facia l ne rve ab ou tthe ex te rna l au d ito ry m ea tu s. A s h as beenprev ious ly m en tio ned , it is a t beast deba t-ab le w h eth er th e fac ia l ne rv e h as ind eedsuch a senso ry d istr ibu tio n and , if it d oes,it is ce rta in ly in c lose assoc iatio n w ith thevagu s. D oub t w as cas t on the v alid ity o f

g en icu late h erpes by D enny-B row n32 in1944 w ith a ca se in w h ich at necro psy theg en icu la te g an g lion w as n o rm al bu t he rpe ticlesio ns w ere fo un d in the g an g lion o f thesecond cerv ical n erve . T here a re anasto -m o tic con nection s b etw een the facia l ne rveand sev era l o th er cran ia l ne rve s an d in -directly w ith the h igh e r ce rv ic al n erv es ,and h e rp e s zos te r o f th e ea r an d fac ia lpa ra lysis has been rep orted w ith inv o lve -m en t o f the fif th , eig h th , and ten th c ran ialn erv es as w e ll as the seco nd and th irdcerv ical .a3h5

M ore o bscu re repo rted causes o f fac ialpara lys is such as a case w ith im pac ted( no t nece ssarily in fec ted ) w isd om tee th ,# {1 76 }a re inev itab ly v iew ed w ith som e susp icio no f rep re sen ting co in cid en tal id iop ath icB e lls p alsy .

It is in th e ev alu ation an d ifferentiald iagn osis o f id iop a th ic facia l p ara lysis tha tthe ped ia tric ian faces m ost o f h is p ro l) lem s.Excep t fo r the cases found in assoc ia tio nw ith otitis m edia , the g rea t m a jo rity w illin fac t be id iop a th ic . Th e firs t qu estio n toansw er is w he th er the acu te fac ia l pa ra ly sisis o f the low er m oto r n eu ro n ( peripheral)type o r w he the r it is sup ranuc lea r in o rig in .It is u su ally sta ted tha t the low er m oto rn eu ron ty pe o f fac ia l p ara ly sis inv o lve s theen tire ha lf o f the face , w hereas the up perface is sp a red en tirely o r at lea st re la tive lyin sup ranu clea r pa ra lys is , an d tha trno-t ionab ( as o ppo sed to vo litio na l) m ov em en tso f the face a re rela tive ly spa red in supra -nu c lea r cases. Th is s tatem en t is g en era llyva lid , bu t one m ust bea r in m ind th at apa tien t w ith reco ve rin g p e riph era l fac ialp ara lysis m ay sh ow ea rlies t s ign s o f re -co ve ry in th e u ppe r face , leav ing pred om i-nan tly low er fac ial w eakn ess w hich m ig h tbe con fused w ith a sup ranu clea r le sio n .Fu rth er , lesio ns o f the face and pes anse ri-nu s a re like ly to in vo lv e on ly pa rt o f thed istribu tion of th e fac ia l n e rve . It has b eensta ted tha t in lep ro sy , th e upp er face isusua lly invo lv ed m o re than the low erace . 1

O ne m ust a lso bea r in m in d tha t ex tra-c ran ia l m ass le s io ns in th e n eck can co rn -p rom ise the fac ia l n erv e at o r near its ex it



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TABLE HI

30 9

CASES O F I S O L A T E D FA C IA L PA R A L Y S IS A T C I iI I n R E x sM E D IC A L C E N T E R , 1943- 5()

(auseSeason o f Onset

sumher Summer- Winier-of Ca ses F all Spring

Co n g en i t a lCo n g e n i t a l anoma lyBir th t rauma

Postna ta lSurg ica l traum aO th e r t r a u m aI n t r a c r a n i a l t u mo rE x t r a cr a n ia l t u m orhyper tensionPol iomye l i t i sO titis m ediaId iopath ic

with uppe r re sp . in -fee t . (h u t no t o ti-t is)

w i t h o u t u p p e r resp .infec t .

518

35

16 6 1019 10 9

( 9 ) ( 6 ) ( 3 )

( 10 ) (4) (6)

HO 1 8 1 9

a part o f the M elkersson sy ndrom e of re -cu rren t fac ia l p a raly sis w ith ed em a o f th eface and lip .

A ny con sidera tion of fac ial p a ra ly sis andits possib le m eans o f trea tm en t depends onan asse ssm en t o f the p rog nos is in u n trea tedcases. Th e pe rcen tage of reco ve ry is so h ighas to m ake eva lua tion of various m eans oftrea tm en t d ifficu lt, s ince the b a rge num bero f pa tien ts w h ich w ou ld be req u ired fo r acon tro lled s tudy are no t av a ilab le in m o stcen te rs . F u rth er , the p rogn osis is p ro bab lyb ette r in ch ild ren th an in adu lts and thed is ea se itse lf le ss frequen t, w h ich rend ersa sta tis tic a lly va lid stud y a lm o st im po ssib le .In y ea rs past, th e fo llow -up of ou r case s a tthe C h ild ren s M ed ica l C en te r w as o ftenun success fu l, and m any cases w ere lostfrom con tact. O f th ose fo llow ed , how eve r ,th e m a jo rity o f all g roup s recove red , ex -cep t th ose du e to cong en ita l an om a ly . T hereco ve ry ra te am ong cases du e to o titism ed ia w as som ew ha t les s th an in the id io -p ath ic g roup . P a rk an d W atk ins2 rep ortedcom ple te reco ve ry in 6 6% of th eir adu lts ,2 1% w ith go od resu lts , 9% w ith fair and 4%

REV IEW A RT ICLEf ron i tlw S ty lO m aS tO i(l fo ram en , and m ay in -voive o ther c ran ia l ne rves as w ell (IX , X ,X II) . S u ch a com b ina tion of pa lsie s is the re -fo re no t a lw ay s due to an in trac ran ia l le s io n .N eop lasm s, inc lud ing those o f the bym -phom a grou p , a re th e m ost freq uen t causeof th is situa tion , bu t ce rv ica l lym phaden itisalo ne m ay o ccas iona lly be resp ons ib le .

In the average case of a ch ild w ith fac ia lpa ra lysis n o t based on som e fa irly obv iou sexp lan ation , con side ra tion of the ana tom icfac to rs p rev io usly d iscu ssed w ill lead toth e conc lus ion tha t the les io n is p rob ab lyin the facia l cana l. E xam ina tion usua llyfa ils to y ie ld frn - th er d iagnos tic in fo rm ationan d the d iagnos is o fod io pa th ic fac ia lpara ly sis is m ade by ex c lu d ing o ther cau sesinsofar as possib le . O f 517 cases reportedl)y Park nc I \iV a tk ins,2 on ly 6% each w eredue to traum a and in fec tion , a sm all rium -her w ere due to tum o r and over 87% w erei(1 i01)a th ic , bu t th is se ries w as com po sedch ie fly o f adu lts. In ch ild ren the in fectiousgroup , particu la rly those w ith o titis m ed ia ,is propo rtio na te ly m o re num erou s, bu t theid io pa th ic cases s till con stitu te th e la rges ts ing le g rou p . T ab le III summ arizes theex p er ie n ce o f the C h ild ren s M ed ica l C en -te r o ve r a 8 -yea r p e riod end ing in 1950 .The seasona l in c id en ce is o f som e in te res t,inasm uch as a tim e-ho nored theory is th atB e lls p alsy is d ue to ed em a of the facia ln e r v e in th e cana l based o n exposure toco ld a ir. If th is w ere true , the grea t m a-jo rity o f cases o ugh t to occur during thew in te r un less the summ er cases cou ld beaccou n ted fo r by unrecog n ized p o liom ye-litis. T he ap prox im ate ly eq ua l seasona l in -ciden ce in th is and o ther s tu d ies a rg uesag a in st co ld as an e tiobog ic ag en t, a s doesth e fac t tha t one ra rely ob ta ins a conv in c in gh isto ry of ex posure , a ltho ugh in an o cca -sion al case it is too s tr ik in g no t to be ac-cep ted . It seem s m o re probab le tha t fac ia lpa ra lys is is u sua lly due to som e form ofn eu r it is o f th e fac ia l ne rve w h eth er tox ic ,ischem ic , o r ed em atou s. I t is w orthy ofn o te tha t id iopa th ic fac ia l p aralys is canbe recurren t ( 7% , Park and W atk ins2 ), andth a t recurren t fac ia l p alsy is no t necessar ily



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3 1 0 PA INE - FA CIA L PA RA L Y SIS IN CH IL DRENw i th poor or no recovery . O ther reports ofrecovery rates i ncl ude those of Col l i er3 of75 to 90% and better in the younger agegroup, M oor38 of 80 to 90%nd of B ierman39of 92% . Taverner40 reported complete re-covery in onl y 45 of 100ases, but compari -sons of thi s sort alw ays rai se the questionof how str i ctl y complete recovery i s inter-preted.

I n general , cases of B el l s palsy fol l owone of tw o qui te separate courses, as Jamesand Russel l 5 have emphasized. T he f i rstcourse is one of recovery w i thout W ableriandegeneration. This begins w i thin the periodrequi red f or a total electri cal reaction ofdegeneration to appear ( not less than 18days) and i s probabl y alw ays f ol low ed bycomplete or almost complete recovery , usu-al l y w i thi n a month s time or sl i ghtl y more.A second course involves a total reaction ofdegeneration w i th subsequent slow re-covery only by regrow th in a distal di rec-ti on. Such recovery i s never complete, re-qui res several months, and invariabl y in-eludes phenomena or m isdi rected regrow thof f i bers to other muscles than those w i thw hich they were or iginal l y connected. Thisphenomenon of m isdi rected regrow th w asprobabl y f i rst descri bed by L ipschi tz4 i n1906 and has been discussed in some detai lby Ford and W oodhabl42 and conf i rmed inmonkey experiments by Howe, T ow er andD uel . T he mani f estati ons of m isdi rectedregrow th in incomplete recovery f rom facialparaly si s are of several ty pes:

1. M ass action or overf l ow ef fect i n w hichattempted acti v i ty of one muscle groupresul ts i n acti v i ty of several di f f erent musclegroups.

2 . T ics, w hich may actual l y be a form ofmass acti on ef f ect,

3. T he syndrome of crocodi l e tears i nw hich af ter several months the presence off ood in the mouth or other gustatory stimul i( i ncluding sometimes the smel l of food) w i l lbe f ol l ow ed by lacrimation rather thansal i vati on. The anatom ic basis of thi s i splausible f rom a study of Figure 1. Theenti ty w as f i rst descri bed by B ogorod44 andhas been rev iew ed by Ford45 and M c-

Govern,46 w ho estimated that of the0%of f acial palsy cases w i thout complete re-covery, crocodi l e tears occurred in0% .

A symmetry of the bones of the skul l hasbeen reported in rats and rabbi ts47 fol l ow -ing uni l ateral section of one f acial nerveat bi r th. A caref ul study has not been donein man, but superf i cial l y at l east, facialpalsies acqui red postnatal l y w i th incom -plete recovery seem to be f ol l ow ed only bysof t ti ssue asymmetry . Congeni tal f acialparal yses are almost alw ays bi l ateral andtheref ore di f f i cul t to evaluate. A s empha-si zed by Park and W atk ins2 the electri calsigns of the reaction of degeneration areof considerable prognosti c value i f testedat 14 days af ter the onset. Park and W at-kins2 reported total reaction of degenera-ti on in 13% of thei r cases and parti al i n35% . A fai r or poor resul t w as obtained in13% of those w i th parti al reaction of de-generation and in 43% of those w i th totalreaction, w hich w as rarel y i f ever f ol l owedby compete recovery . I n the reaction ofdegeneration, the nerve ceases to respondto ei ther faradic or galvanic current. Themuscle does not respond to f aradic current(rapid al ternating current) , but i s more i r-ri table than normal l y to the galvanic cur-rent al though the anodal closing con-tracture i s greater than the cathodal closingcontracture or requi res f ewer m i l l i amperesf or i ts threshold, w hich is the reverse of thenormal si tuation. A slow verm icular re-sponse replaces the normal quick jerk ofthe muscle. B efore the reaction of degenera-ti on i s complete, how ever, the nerve w i l lhave lost only i ts response to f aradicstimulati on and w i l l respond to galvanicstimulati on in a sim i lar manner to thatdescr ibed f or the muscle. Testing for thi sphenomenon is moderatel y uncom fortableand di f f i cul t to carry out in young chi l drenbecause of thei r l ack of co-operation. Asimpler, i f more superf i cial , cri teri on i sw hether recovery i s observed to begin l )e-fore the time requi red for degeneration.Such apparent recovery beginning soonerthan 18 days has in our experience alw aysbeen fol l ow ed by complete or at l east



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REVI EW ART I CLE 311nearl y complete eventual recovery . I tw ould seem to indi cate thathe patientw i l l f ol low the course of primary recoveryrather than secondary recovery by re-growth.

TREATMENTI n the acute stage, several modal i ti es of

physical therapy are avai l able includinglocal heat, electri cal stimulati on, massage,spl i nti ng of the face and activ e exerci sesill f r on t o f a m i rror. A ccording to B ierman,19the f i nal recovery rate w as not much in-f l uenced by physiotherapy , but w i th dai l ytreatment, 68% w ere reported to recover in4 weeks and 90% i n 12 weeks, as comparedw i th 38 and 72% , respecti vely , i f t reat men twa s onl y gi ven tw i ce a w eek . Park andWatkins2 on the other hand, reported nodi f f erence in the percentage of recovery i ntreated as compared w i th untreated groups.The process of w ai ti ng out a case of f acialparal ysis i s alw ay s try i ng for both pati entand parents and the use of physiotherapy(l oes of f er the com f ort and satisf action thatthe doctor i s at l east try ing to do some-thi ng. W e have preferred, how ever, to ex -pl ai n the si tuati on qui te f rank l y to parentsand to try to prov ide the necessary moralsupport i n a more open manner. The prin-cipal speci f i c measure usual l y f ound neces-sary i s to place a drop of m ineral oi l orol i ve oi l in the af f ected eye at ni ght toprevent dry i ng. A moist chamber can heused but i s di f f i cul t to keep in place onyoung chi l dren. I n the day time the prob-bern i s less severe, except in very dusty cl i -mates. T he corneal ref l ex i s abol i shed be-cause i ts ef f erent arc i shrough the facialnerve, but there i so corneal anesthesia.The patient i s aw are of a foreign body onthe cornea and w i l l usual l y make an at-tempt to close the eyel i ds w i th his f i ngersor at l east protest to his mother.

One approach to treatment of facialparal ysis i s by surgical decompression ofthe f acial canal , w hich i s sti l l probablyf avored by the majori ty of otol ogi sts. I tsrati onale depends on the assumpti on thatthe paral y si s i s due to edema of the facial

nerve in i ts canal , w hatever the underl y ingcause of the edema may be. K etteI 48 re-ports macroscopic edema in 28 of 50 cases,and Caw thorne reports both m icroscopicand macroscopic edema. I t i s di f f i cul t toproduce stati sti cal ev idence on the value ofsurgi cal decompression of the canal be-cause of the high recovery rate in mostcases, and the tendency to select f or de-compression those cases w hich have beenfol l ow ed f or several w eeks and are notdoing wel l . T he logic seems inescapablethat i f substantial benef i t i s to be producedby decompression, i t should be done beforedegeneration has taken place ( namelyw i thin 18 days), and i t i s di f f i cul t to arguethat one ought to subject pati ents rou-ti nely to decompression i f one expects an80 to 90% recovery rate w i th no treatment.Considerable justi f i cati on can be made forattempti ng decompression on those pati entsshow ing no apparent recovery and a begin-f l i ng reaction of degeneration at perhaps 14days af ter the onset, but the questi on wouldremain unansw ered as to w hether the valueof decom pr essi on, if any , w ould be ma-teri al l y less i f undertaken so late in thecourse of the disease.

H istam ine as a means of treatment forf acial paral ysi s w as reported by Sk inner l land has some f oundation in theory , but hasnow been largely replaced by corti sonetreatment.

Corti sone has been used for several yearsf or treatment of f acial paral y si s, but i tsevaluation, in v iew of the high recoveryrate in untreated patients, i s di f f i cul t andnot def ini ti ve. I n 1953, Rothendlerl l re-ported seven patients treated, w i th successin si x , using relati v el y large doses of 300 to600 mg oral l y in adul ts. T averner53 studied26 patients qui te caref ul l y , al l of w homw ere over 12 years of age. They were se-lected on the basis of a duration of notl ess than 10 days f rom the onset and forthi s reason the resul ts may have been lessfavorable than m ight have been obtai nedw i th ear l i er treatment. T he paralysi s w asnot necessar i l y requi red to be complete inal l . T he recovery rate of 2 total recoveri es



11/16

TA BLE IV

Si d e o f % o i h(,.,e . IgC e x Paraly- ofNo. (yr) , On s e t A s s oc i a t e dSymptoms

Da y s ofT r e a t .

Erentual merit lie.Recovery fore lie.

gi n niF tyRrcorery

I 8 F 1 M ar e1 P R !. n o o t it is S SO m g 7 to t a l 42 9 F 1 J an n on e 10 75 m g 5 to ta l 1a 7 F 1 M ar ch n on e 4 75 m g 8 to ta l 5

Rema r k s

6 M L Ma y UR I , 1 4 d a y sbe fo re o nset7 4 F R Ju ly U R I,n ootitis

8 F R De c n o n e

9 3 M R J an q u e st ion ab leo t i t i ,

10 S F I. Feb n o n e

l( i( l( Ill a irda y be fore ( ) l 3SC t

g, z I re ous t r ea tn i en twi th tliian, , i,e

6 to ta l 1 54 98r; . S it7

15 to t a l K

8 to ta l unknown 16S4 a l mo s t4 t o t a l

10 to ta l 2 404 7 5 mg 7 t o t a l

a N on e o f t h e ca ses sh ow ed an y b eg in n in g r ecov er y a t t h e t im e f ir st seen . Pa t ien t s a lr ead y im p r ov in g a t in it ia l e x am in a t ion w er e n o tt re ated w ith cortison e an dare exc luded f r om th is ch ar t .

t N ot inclu ding in m ost ca se s aew ad d it ion a l d a y s o f sm alle r dos age f o r gr ad u a l w ith d r aw al.*5 Face n ow en t ir ely sy m m et r ica l ex cep t w ith ex t r em e lau gh in g or c r y in g .

31 2 PA INE - FA C IAL PAR ALY S IS IN CH ILDRENand 10 p artia l reco ve rie s am ong th ose casestrea ted w ith p lacebo , is n o t s ign if ican tlyd iffe ren t from tha t o f 4 to ta l an d 10 p artia lreco ver ies am ong the co rtiso ne trea tedgro up . T he m ean dura tion of para lys is w asapprox im ate ly th e sam e in the tw o g roup san d th e stu dy cast som e dub t o n th e v a lueo f co rtisone . N eve rth ele ss co rtisone trea t-m en t, in the d oses used , has p ro ved sa feif care fu lly w a tch ed , and since it m ay in -f luence th e recove ry ra te , e spec ia lly if u sedearly , it h as been ou r p rac tice to trea t a llid iop ath ic cases as p rom p tly as pos sib le ,un less th ey are a lread y show ing ev idenceof recov ery w ith in th e tim e p e riod req u iredfo r d egene ra tion of the ne rve . B ecause acon tro lled stu dy has no t been don e ow ingto the sm a ll n um ber o f case s av ailab le , o necan offe r o n ly an adm itted ly u nreliab lec lin ica l im pression as to the bene fit. O n ly12 id iop ath ic cases in th e un trea ted se riesfrom 1943 to 1950 at C h ild ren s M ed ica lC en te r w ere adequa te ly fo llow ed , an d 9of these recove red . T he de ta ils o f the treat-m en t o f th e pa tien ts from th is ho sp ita l a rep re sen ted in Tab le IV . I t w ill be n o ted th ata ll o f 10 pa tien ts treated w ith co rtiso nerecov e red . The re is no sta tis tic al p roo f,

bu t on ly the im p ressio n , tha t in m any casesrecove ry began so so on afte r in itia tio n ofco rtison e treatm en t a s to p rov e qu ite str ik -in g . F ig u re 2 show s C ase 4 of th is se r ieson th e seco nd d ay of p ara lysis w henm i-tially seen and aga in af te r 5 d ays of co rti-sone . O ur resu lts a re cons isten t w ith theim pression of T hom as,54 from his ow n pa-tients and rev iew o f o the rs in th e litera tu re,tha t in case s o f fac ial pa lsy trea ted w ithco rtison e, v ir tua lly fu ll recov ery in 14 day sis the ru le , com pared w ith 6 w eeks o r m orein those un treated cases w hich recove r .Thom as urg ed prom pt treatm en t (w ith in10 days o f on se t; re su lts are m uch le ss un i-fo rm if it is s ta rted la ter) and fu ll dosag eof 3 00 m g d a ily in adu lts . T h e ave rag e tim ereq u ire d fo r ea rlie st d etec tab le im prove -m en t, an d fo r fin al recov ery , in o ur pa -tien ts in T ab le IV a lso com pares favorab lyw ith f igu re s fo r ad u lts n o t trea ted w ithco rtison e.2 U nfo rtu na tely , no com parab leand re liab le tim e da ta a re av a ilab le fo run trea ted ch ild ren . It ha s been o ur p lan totreat ch ild ren w ith 7 5 to 1 50 m g of co rti-so ne da ily , d ep en d ing on age , fo r 1 w eek ,co n tinu ing fo r a secon d w eek , in an in -c rea sed d osage if n o reco ve ry h as seem ed

C A S E S OF ID IOPATH IC FA C IAL PA RA LYS IS TREATED w IT S! CORTISO NE AT C hILD REN S M EI)I(AL (EN TER

4 7 F R M ay n o n e5 3 F R Oc t n o n e

Du ration Du ra tiona t F irst C ortiaon e o f T rea t-J sit (dose ,day ) m cn tf(day s) (d ay.

7 5 m g3 1 5 0 m g

0 0 m g7 75m g

7 [100m g.1 so m gS ( 7 5mg10 0 m g

6 100mg

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12/16

REV IEW A RT IC LE 31 3

to tal e lec trica l reac tion of degene ra tion is

F ie . 2 . C ase 4 , a g irl f 7 yea rs, w ith id io path ic B ells pa lsy . (a)left) A ppearan ce when first seen , onSeCOn( l day of p ara lys is. (b ) r ig / i t ) S am e child af ter ( lay s of cortiso ne therapy . R eco very is no t y et

com p lete , b u t sub sequently b ecam e so .

to take ilace . O n such do sage , spec ia l atten -tion to ek c tro lv te ba lance, added po tassiumch lo ride , e tc ., a re o rd inarily no t necessa ry ,no r have w e used prophy lac tic an tib io tic sro u tin e ly . U se of regu la r tab le food w ith110 d(i(le (l sa lt, and th e urg ing o f 4 to 16OUI 1CCS of fr uit j uice da ily , have been ad-\iS (a (I . T he p ln seem s perfec tly su itab lefo r am l)u lato ry trea tm en t if ca re fu lly sup er-V iSC (l. O ne is na tu ra lly re luc tan t tose cor ti-SOlIC in the presence o f o titis m ed ia, l)u te ha\C do l le SO l tw o instances whereho recoveiy seem ed to he tak ing p lace ,af ter fir st a dm in is ter ing an tib io tic s fo rsevera l (lays p rio r to and during trea tm en t.B ecau se o f the con side rab le num ber o fcases o f fac ia l p aralys is w h ich are undoub t-em ily (b ile to p o liom yelitis, and because ofthe g en eral assum ption th at co rtiso ne iscon tra ind ica ted in po liom yelitis, on e isn a tu ra lly h es itan t abou t trea ting su pposedid io pa th ic facia l pa ra lys is w ith cortiso ne

during th e p o lio seaso n . In ep idem ic yea rs ,such a p lan m ay be con tra ind ica ted ; o the r-w ise it is p robab ly sa fe to use co rtison e ifpo liom yelitis has been ex c luded so fa r a sfea sib le , in c lud in g ex am ina tion of th esp in al flu id . W e have , how ever, seen th reepa tien ts w ith fac ia l pa ra lysis ( lu r ing th e1955 M assach use tts ep id em ic w h ich w eresu bsequ en tly id en tified as o liom yelitisafte r they had been trea ted w ith co rtison el)y o ther phy sic ian s. N one o f these th reesu ffe red any com plica tion or w orsen in g ofthe po liom yelitis , and n one h ad p erm anen tparalysis.

A fte r g iv ing d ue co nsid era tion to th ep ossib le p resen ce o f o titis m ed ia an d po lio -m yelitis, it has been our p rac tice to treatid iopa th ic cases o f facia l pa raly sis w ith o ralcor t isone acco rd ing to the p lan m en tion edabov e. If a t the end o f 1 4 to 1 8 d ay s th ereis no ap paren t recov ery , and a partia l o r



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31 4 PAI NE - FA C IA L PA RA LYS IS IN CH ILDRENfound , w e ob ta in o to log ic consu lta tionconce rn ing p ossib le su rg ica l decom p res-s io n of the cana l. In the presen t se riessin ce th e use o f co rtison e, recove ry has o c-cu rred so prom p tly in all ca ses tha t th isq uestion h as no t a risen .

S eve ra l fo rm s of la te trea tm en t o f p e r-sisting fac ia l pa ra lysis a re ava ilab le bu tth eir de ta iled d iscus sion is beyond thescope o f th is p ap er . A n erve g ra f t is o neof the m ajo r appro aches to the prob lem .A pparen tly any typ e of n erve , w h eth e r m o-to r o r senso ry , w he the r reve rsed or no t, anda lso even if degenera ted ( T ick le55) can beused , and th e gra ft p robab ly se rv es m ere lyto p ro v ide a b ette r ch an ne l fo r reg row th ina d is ta l d irec tion . T he c lassica l paper o fB a llan ce and D uel56 in932 , d escr ibedbo th graf t trea tm en t and a lso anas tom oticprocedure s using th e sp ina l accesso ry o rhypogbossa l ne rves. L ov e and C annon57 re -ported exce llen t resu lts in abou t 50%singanas tom osis , bu t recov ery w as never com -p le te and am ounted to no m ore than5%of norm a l func tion . In un de rtak in g su chprocedure s, one m ust cons ide r w he the r theeven tu al d eg ree o f reco ve ry w ill be g reate rthan if n a tu ra l reg row th is p e rm itted totak e p lace w ithou t in te rference , bu t itw ould seem reaso nab le to co nside r an as to -m o sis in cases w here th e degree o f recov erya fte r 3 m on th s is co nsid ered h igh ly un satis -fac to ry .

A varie ty o f p las tic su rg ica l p ro cedu re sus ing fasc ia l s lin gs, tan ta lum m esh ,58 e tc .,fo r la te repa ir o f th e d efo rm itie s o f pe r-s isten t fac ia l pa ra lysis h ave been desc ribed ,bu t the se a re g en e ra lly to be cons ide reda fter g row th has b een large ly com ple ted ,and scarce ly in the p ed iatr ic age g rou p .F ina lly , a lth ough the crocod ile tea rs syn -(Irom e is no t u sua lly su ff icien tly tro ub le -som e to w arran t con side ra tion of such aprocedure , the case o f G otte sfe ld an d Lea -v itt5 9 is o f in te re st. T hese au tho rs in jectedalcoh o l in to the sph en opa la tine gang lionof a 23 -y ea r-o ld m an w ith id io pa th ic B ellspa lsy and ab o lished the ep iphora. T h e croc-od ile tears re tu rned , how eve r, a fte r an in -te rva l o f abo u t 4% m onths.

S U M M A R YT he an atom y of th e fac ial ne rv e and th e

va rio us e tiobo g ic facto rs in bo th cong en italand acqu ired fac ial pa ra lys is in ch ild renh av e been rev iew ed . D iffe ren tial d iagn osisam ong the m ore im portan t o f these hasbeen d iscussed and an ev alu atio n of thep ossib le m e tho ds o f treatm en t a ttem pted .Th is eva lua tion is d iff icu lt because o f th eh igh reco ve ry ra te w ith ou t trea tm en t,w h ich m ay app roach 90 % in ch ild ren . Ifca re fu l study of a case o f fac ial pa raly sisin d icate s its id iop ath ic na tu re, p rom pttreatm en t w ith o ra l co rtison e is con side redin d icated . W hile it is d iff icu lt to e stab lishth at co rtiso ne greatly im prov ed an a lreadyh ig h reco ve ry rate , it seem s m o re c lear th a tthe av e rage reco ve ry tim e is favo rab ly in -f luenced , at lea st in adu lts and p ro bab lya lso in ch ild ren . C ortison e seem s th e trea t-m en t o f cho ice at th e presen t tim e an(l d iir-ing th e past 2 yea rs a t one ho sp ita l, a ll o f10 pa tien ts so treated recov e red . T h is hase lim ina ted the n eed fo r con side ra tion ofsu rg ica l decom p ressio n o f the fac ial can a l.W heth e r th is w ill rem a in tru e w ith large rn um bers o f case s rem a ins to b e seen .

R E F E R E N C E S1 . B e ll, S ir C . O n the nerv es: G iv ing an ac-

cou n t o f som e exp eriences on the ir s truc -tu re and fun ction , w hich lead s to a newarrang em ent of the sys tem s . Ph il. T r.,R oy . So c . L ondon , 3 :398 , 1 821 .

2 . P ark , H . W ., a nd W atk ins , A . L . : Fa cia lpara lys is; an a ly sis o f 500 cases. A rch .Phy s . Med . , 3 0 : 7 4 9 , 1949 .

3 . Co llie r, J. , Spi l lane , J. D ., an d B auw ens, P .:Sym pos ium : T rea tm en t o f fac ia l pa ra ly -sis . P roc . R oy . S oc . M ed ., 43 :74 6 , 1950 .

4 . S u llivan , J. A ., an d Sm ith , J. B ., T he o to -log ica l concep t o f B e lls p alsy and itst reatment . Ann . Oto l . Rh in . & Larvng .,5 9 : 1 1 4 8 , 1 9 5 0 .

5 . Jam es, J. A ., an d R ussell, \V . H . Bel l spals ; ae tio bogv , cl in ical c ou rse andtrea tm en t. L an cet, 2 :5 9 , 195 1 .

6 . M #{246}b ius , P . J. : U ber ang eborene dop-p else itige A bd ucens-F ac ia lis-L iihm ung .M #{252}nchen . m ed . W chn schr., 3 5 :91 , 18 55 .

7. H epner, W . R . : S om e observa tio ns on fa -c ia l pa res is in the new born in fan t: e tio l-ogv and inc idence . PED I A T R I C S , 8 : 4 9 4 ,1951 .



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REV IEW ART IC LE8 . Sch les inger, B . E ., B u tle r, N . R ., and B lack , 25 .J. A . : Severe type o f in fan tile hyp er-

calcemia. B rit. M . J. , 1 : 127 , 195 6 .9 . Grif f i th , J. Q . : F acia l n e rve para lysis as-

soc iated w ith hy perten sion . A rch .N euro l. & Psych ia t., 2 9 :1195 , 193 3 .

1 0 . W atk ins, A . L . : Fac ia l pa ra ly sis in cep ha lic 26 .te tanu s. A rch . N euro l. & P sych ia t., 41 :788 , 1 939 .

1 1 . M acand rew , M ., and D av is, E . : T rich in i-as is p resen ting w ith foo t d rop and fac ia l 27 .pa lsy . L ance t, 1 : 1 41 , 1948 .

12 . R ick les , G . A . : S yph ilitic perip hera l 28 .seven th nerve para lysis ; d iscuss io n o f 2 9 .c a se . J. N erv . & M ent. D is., 102 :3 76 ,1945 .

13 . M onrad -K ro hn , G . H . : T he C lin ica l E x-am ina tio n of th e N ervo us Sys tem . N ew 30 .Y ork , H oeber, 1947 .

14 . H un t, J. R . : O n herpe tic in f lamm ation o fth e gen icuba te gang lion ; a new syndrom eand its com p lica tions . J. N erv . & M ent. 31 .D is ., 34 :73 , 190 7 .

15 . Johnson , E ., and S toesse r, A . V . : R e lap singfac ial p aralys is in the sam e fam ily . A rch .P ed ia t., 5 4 :726 , 1937 .

16 . K ette l, K . : M ebkersso ns sy ndrom e; report 3 2 .of f ive ca ses w ith specia l re fe rence to thepa tho log ic o bserv ations . A rch . O to -barvn g ., 46 :3 41 , 19 47 .

17 . H enderson , J. L. : C ongen ital fac ia l d i-p leg ia sy ndrom e: C lin ica l featu res , pa - 33 .th obogy , and ae tio logy ; a rev iew of s ix ty -one cases . B ra in , 62 :381 , 1 939 .

18 . R ossi, E ., and C aflisch , A . : L e synd rom e dup terv g ium ; sta tus B onnev ie -U lir ich , dys-troph ia b rev ico lli cong en ita , syn drom e 34 .de T u rner e t a rth rom yod p lasia con-genita . H elve t. p aed iat. a cta , 6 :119 ,1951 .

19 . T rau tm ann , D . : B e itrag zur K asu is tik ko n- 35 .gen itab er F ac ialispa resen . Z tsch r . ges.N euro l. u . P sy ch iat., 10 0 :289 , 1 926 .

20 . Fo rd , F . R . : C ongen ita l fac ia l d ip leg ia , in 36 .Y ear B ook o f Ped ia tric s. C h icag o , Y r.Bk. Pub ., 195 2 , p . 347 . 37 .

21 . R ich ards, R . N . : T he M #{246}b ius syndrom e.J. Bone & Jo in t S urg ., 35A :4 37 , 1 953 .22 . R a in s, H ., and Fow ler, J. S . : C ongen ita l

fac ia l d ip leg ia du e to nuc lea r les ion .R ev . N euro l. & Psych ia t., I : 149 , 19 03 . 38 .

2 3 . H eubner, 0 . : U ber an geborenen K ernm an-ge l (in fan tile r K ernschw und , M oeb ius) .C harit#{23 3}-A nn ., 25 :211 , 190 0 . 39 .

2 4 . F riedm an , E . D . an d E lsberg , C . A . : So-c ie tv T ran sac tions: N ew Y ork A cad . o f 40 .M ed ., Sec . o f N euro lo gy and P sych iatry .A rch . N eu ro l. & Psych ia t., 22 :10 59 ,1 9 2 9 . 4 1 .

3 1 5

Mussio -Fourn ier , J. C ., Raw ak , F ., an dFischer , J. T . : P a raly sie fac iale du ty pep# {233}riph iq ue do rig ine cen trale assoc i# {233 }edes tro ub les hom o la t#{233} raux do rd re sen si-tif , pa r# {233 }tique , e t c# {233 }r#{233}be lleux .R ev .neuro l., 69 :277 , 1938 .

A llen , I. M . : F ac ia l pa ra lys is o f supra -nuc lea r type sim ula tin g tha t o f periph-e ra l o r ig in . N ew Zea lan d M .., 45 :434 ,1 9 4 6 .

S tap le ton , T .: Fac ia l p alsy an d po liom ye-litis . L ancet, 2 :51 0 , 19 49 .

K ib rick , S . : P erso na l comm unica tion .Z ellw ege r, H . U ., and G aba thu ler , E .:

Pr ognosis of palsies f r om cr an ial n er v einvo lvem ent in po liom yelitis . P E D I A T R I C S ,1 0 : 1 2 7 , 1 9 5 2 .

Taylo r, E . W ., and M cD on a ld , C . H . : Sy n-d rom e of po liom yelitis w ith fac ia l d i-p leg ia . A rch . N euro l. & Psych ia t., 27 :79 ,1932 .

H aym ak er , W ., and K ernoh an , J. W . : T heL andry -G uilla in -B arr#{233 } syn drom e; c lin ico -p a th o log ic rep ort o f 5 0 fata l cases andcritique o f lite ra tu re . M edic in e , 28 :59 ,1949 .

D enny-B row n , D ., A dam s, R . D ., and F itz -gera ld , P . J. : Pa tho log ic fea tu res o fher pes zost er ; a not e on gen icu lateherpes . A rch . N euro l. & Psych ia t., 5 1 :2 1 6 , 1 9 4 4 .

O N eill, H . : H erpes zoste r au ris (g en ie-uba te gang lio n itis) ; repo rt o f eig h t case sw ith p ho to g rap hs o f th e so -called syn-d rom e of R am say H un t. A rch . O to -la ryng . , 42 :309 , 194 5 .

Engstrom , H ., an d W oh lfa rt. C .Herpeszo ster o f th e seven th , e igh th , n in th , andten th cran ia l ne rves. A rch . N euro b .&P sych ia t., 6 2 :63 8 , 1 949 .

T sch iassny , K . : H erpes zos te r o ticus( R am -say H unts S nydrom e). A rch . O to laryng .,5 1 : 7 3 , 1 9 5 0 .

Siege l, A . : F ac ia l pa ra lys is; a case rep ort.U . S . Nay . M . B u ll., 4 0 :9 49 , 194 2 .

T rem b le, G . E . and P enfie ld , W .Opera-tiv e ex posu re o f th e facia l cana l w ithrem ova l o f a tum or of the g rea ter supe r-f ic ia l pe trosa l ne rve . A rch . O to la ryng .,2 3 :573 , 1 936 .

M oo r, F . B . D iagnos is and treatm en t o fid iopa th ic fac ial p a ra ly sis ( B ells P a lsy ) .J . A. M. A . , 1 5 7 : 1 5 9 9 , 1 9 5 5 .

B ierm an , W . : D iagn osis and treatm en t o fBells P a lsy . J.A .M .A ., 14 9 :253 , 19 52 .

Tave rne r, D . : B e lls P alsy ; a c lin ica l andebec trom yog raph ic stu dy . B ra in , 78 :209 ,1955 .

L ipsch itz, R . : B e itr# {228 }ge zu r L ehre vo n Fac i-



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3 1 6 PA INE - FA CIA L PA RA L Y SIS IN CH IL DRENal i slahmung nebst. B emerkungen zurFrage der N ervenregenerati on. M o-natsschr. Psychiat. u. N eurol ., 20:84,1906.

42. Ford, F. R., and W oodhal l , B . : Phenomenadue to m isdi recti on of regenerating f i bersof cranial , spinal , and autonom ic nerves;cl i ni cal observations. A rch. Surg., 36:4 8 0 , 1 9 3 8 .

43. H ow e, H . A ., Tow er, S. S., and Duel ,A . B . : Facial t i c i n relati on to injury ofthe faci al nerv e: an experimental study .A rch. N eurol . & Psychiat., 38:1190,1937.

4 4 . Bogorad, F. A . : D as Syndrome der K ro-kodi l str#{ 228} nen. V rach. di ebo., 11:1328,1928.

45. Ford, F. R . : Paroxy smal lacrimation duri ngeati ng as a sequel of f acial pal sy (syn-drome of crocodi l e tears) ; report of 4cases w i th possibl e i nterpretati on andcompari son w i th auri cul o-temporal syn-drome. A rch. N eurol . & Psychiat., 29:1 2 7 9 , 1 9 3 3 .

46. M cGovern, F. H . : Paroxysmal bacr imationduri ng eating fol l ow ing recovery f romfacial paral ysis; syndrome of crocodi l et ears . A m. J. Ophth., 23:1388, 1940.

47. W ashburn, S. L . : Ef f ect of f aci al paral ysi son the grow th of the skul l of rat andrabbi t. A nat. Rec., 94:163, 1946.

48. K ettel , K . : B el l s Pal sy ; pathology andsurgery ; a report concerning f i f ty pa-t ients w ho w ere operated on af ter themethod of B ablance and D uel . A rch.Otobary ngol ., 46:427, 1947.49. Caw thorne, T . : Role of surgery in the in-

vestigation and treatment of peri pheralf aci al pal sy . L ancet, 1 : 1219, 1952.

50. L oom is, C . L . H istam ine treatment ofBel l s palsy ; report of f i ve cases. A rch.Otolaryng., 52:948, 1950.

51. Sk inner, D . A . Use of hi stam ine in B el l sPal sy ; repor t of f i ve cases. Ohio StateM . J., 4 5 : 5 6 5 , 1 9 4 9 .

52. Rothendler , H . H . : B el l s Pal sy treatedw i th corti sone. A m . J. M . Sc., 225:358,1953.

53. T averner, D . : Corti sone treatment of B el l sPal sy . L ancet, 2:1052, 1954.

54. T homas, N I . H . T reatment of B el l s Palsw ith corti sone and other measures. N eu-rology , 5:882, 1955.

55. T ick le, T . G . The repair of f aci al paraly si sof oti t i c ori gi n. S. C l in. N orth A meri ca,28:438, 1948.

56. B al l ance, C., and D uel , A . B .Operativetreatment of f aci al pal sy by the introduc-ti on of nerve graf ts into the f al l opiancanal and b other i l i tratemporal meth-ods. A rch. Otobary ng., 15: 1, 1932.

57. L ove, J. C., and Cannon, B . W . : N erveanastomosi s i n the treatment of f aci alparaly si s; special considerati on of theeti obogi c role of total removal of tumorsof the acousti c nerve. A . M . A . A rch.S u r g . , 6 2 : 3 7 9 , 1 9 5 1 .

58. Sheehan, J. E. : Progress in correcti on off acial palsy w i th tantalum w i rendmesh. Surgery , 27: 122, 1950.

59. Gottesf eld, B . H ., and L eav i tt, F. H .: Crocodi l e tears treated by injecti oninto the sphenopabatine gangl ion. A rch.N eurol . & Psychiat., 47:314, 1942.



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1957;19;303-316PediatricsRichmond S. Paine

Report of Ten Cases Treated with CortisoneFACIAL PARALYSIS IN CHILDREN: Review of the Differential Diagnosis and

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differential diagnosis facial paralisis 1957

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