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PAIN MEDICINE ACASE-BASED LEARNINC SERIES

Headache and Facial Pain

PAINMEDICINE ACASE-BASED LEARNINGSERIES

HeadacheandFacialPain

STEVEND.WALDMAN,MD,JD

Elsevier 1600JohnF.KennedyBlvd. Ste1800 Philadelphia,PA19103-2899

PAINMEDICINE:ACASE-BASEDLEARNINGSERIES HEADACHEANDFACIALPAIN

Copyright © 2023byElsevier,Inc.Allrightsreserved

ISBN:978-0-323-83456-8

Allunnumberedfiguresare ©ShutterstockCh1#1786423649,Ch2#345961085,Ch3#1395637079, Ch4#1530602156,Ch5#520571458,Ch6#692205289,Ch7#340629941,Ch8#1419564083, Ch9#764131363,Ch10#1784454875,Ch11#645411031,Ch12#1498236224,Ch13#1772070902, Ch14#1084314392,Ch15#1209115066.

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Thisbookandtheindividualcontributionscontainedinitareprotectedundercopyrightbythe Publisher(otherthanasmaybenotedherein).

Notice

Practitionersandresearchersmustalwaysrelyontheirownexperienceandknowledgein evaluatingandusinganyinformation,methods,compoundsorexperimentsdescribedherein. Becauseofrapidadvancesinthemedicalsciences,inparticular,independentverificationof diagnosesanddrugdosagesshouldbemade.Tothefullestextentofthelaw,noresponsibility isassumedbyElsevier,authors,editorsorcontributorsforanyinjuryand/ordamageto personsorpropertyasamatterofproductsliability,negligenceorotherwise,orfromanyuse oroperationofanymethods,products,instructions,orideascontainedinthematerialherein.

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It’s Harder Than It Looks

MAKING THE CASE FOR CASE-BASED LEARNING

For sake of full disclosure, I was one of those guys. You know, the ones who wax poetic about how hard it is to teach our students how to do procedures. Let me tell you, teaching folks how to do epidurals on women in labor certainly takes its toll on the coronary arteries. It’ s true, I am amazing. . .I am great. . .I have nerves of steel. Yes, I could go on like this for hours. . .but you have heard it all before. But, it’ s again that time of year when our new students sit eagerly before us, full of hope and dreams. . .and that harsh reality comes slamming home. . .it is a lot harder to teach beginning medical students “doctoring” than it looks.

A few years ago, I was asked to teach first-year medical and physician assistant students how to take a history and perform a basic physical exam. In my mind, I thought, “This should be easy. . .no big deal,” I won ’t have to do much more than show up. After all, I was the guy who wrote that amazing book on physical diagnosis. After all, I had been teaching medical students, residents, and fellows how to do highly technical (and dangerous, I might add) interventional pain management procedures since right after the Civil War. Seriously, it was no big deal I could do it in my sleep with one arm tied behind my back blah blah blah.

For those of you who have had the privilege of teaching “doctoring,” you already know what I am going to say next. It’s harder than it looks! Let me repeat this to disabuse any of you who, like me, didn’t get it the first time. It is harder than it looks! I only had to meet with my first-year medical and physician assistant students a couple of times to get it through my thick skull: It really is harder than it looks. In case you are wondering, the reason that our students look back at us with those blank, confused, bored, and ultimately dismissive looks is simple: They lack context. That’ s right, they lack the context to understand what we are talking about.

It’ s really that simple. . .or hard. . .depending on your point of view or stubbornness, as the case may be. To understand why context is king, you have to look only as far as something as basic as the Review of Systems. The Review of Systems is about as basic as it gets, yet why is it so perplexing to our students? Context. I guess it should come as no surprise to anyone that the student is completely lost when you talk about let’ s say the “constitutional” portion of the Review of Systems, without the context of what a specific constitutional finding, say a fever or chills, might mean to a patient who is suffering from the acute onset of headaches. If you tell the student that you need to ask about fever, chills, and the other “constitutional” stuff and you take it no further, you might as well be talking about the

InternationalSpaceStation.Justsaveyourbreath;itmakesabsolutelynosenseto yourstudents.Yes,theywanttoplease,sotheywillmemorizetheelementsofthe ReviewofSystems,butthatisaboutasfarasitgoes.Ontheotherhand,ifyoupresentthecaseofJannettePatton,a28-year-oldfirst-yearmedicalresidentwitha feverandheadache,youcanseethelightsstarttocomeon.Bytheway,thisiswhat Jannettelookslike,andasyoucansee,Jannetteissickerthanadog.This,atitsmost basiclevel,iswhat Case-BasedLearning isallabout.

Iwouldliketotell youthat,smartguy thatIam,Iimmediatelysawthelight andbecameaconvert to Case-BasedLearning. Buttruthbetold,it wasCOVID-19that reallygotmethinkingabout Case-Based Learning.Beforethe COVID-19pandemic, Icouldjustdragthestudentsdowntothemed/surgwardsandwalkintoa patientroomandriff.Everyonewasawinner.Forthemostpart,thepatients lovedtoplayalongandthoughtitwascool.ThepatientandthebedsidewasallI neededtoprovidethecontextthatwasnecessarytoillustratewhatIwastrying toteach the “whyheadacheandfeverdon’tmix” kindofstuff.HadCOVID-19 notrudelydisruptedmyabilitytoteachatthebedside,Isuspectthatyouwould notbereadingthis Preface,asIwouldnothavehadtowriteit.Withinaveryfew daysaftertheCOVID-19pandemichit,mydaysofbedsideteachingdisappeared,butmystudentsstillneededcontext.Thisgotmefocusedonhowto providethecontexttheyneeded.Theanswerwas,ofcourse, Case-BasedLearning. Whatstartedasadesiretoprovidecontext becauseitreallywas harderthanit looked ledmetobeginworkonthiseight-volume Case-BasedLearning textbookseries.Whatyouwillfindwithinthesevolumesareabunchoffun,real-life casesthathelpmakeeachpatientcomealiveforthestudent.Thesecasesprovide thecontextualteachingpointsthatmakeiteasyfortheteachertoexplainwhy, whenJannette’schiefcomplaintis, “MyheadiskillingmeandI’vegotafever,” itis abigdeal.

Havefun!

Spring2021

Averyspecialthankstomyeditors,MichaelHouston,PhD,JeannineCarrado, andKarthikeyanMurthy,foralloftheirhardworkandperseveranceintheface ofdisaster.GreateditorssuchasMichael,Jeannine,andKarthikeyanmaketheir authorslookgreat,fortheynotonlyunderstandhowtobringtheThreeCsof greatwriting...Clarity 1 Consistency 1 Conciseness...totheauthor’swork,but unlikeme,theycanactuallypunctuateandspell!

P.S. ...Sorryforalltheellipses,guys!

13 BrendaBrown A66-Year-OldBookkeeperWithSevere, ShocklikeFacialPain174

14 TommyFlannagan A47-Year-OldMaleWithSevereEpisodic ThroatandEarPain192

15 HeatherShepard A52-Year-OldEditorWithAchingJawPain andaClickingSensation212

Index 231

1

RenaldoSaldana A58-Year-OldMaleWith

Left-SidedFacialPainandRash

LEARNINGOBJECTIVES

• Learnthecommoncausesoffacialpain.

• Learnthecommontypesofpainfulrashes.

• Developanunderstandingofvaricellazosterinfection.

• Learntheclinicalpresentationofshingles.

• Developanunderstandingofthetreatmentoptionsforshingles.

• Learntheappropriatetestingoptionstohelpdiagnoseshingles.

• Learntoidentifyredflagsinpatientswhopresentwithacutefacialpain.

• Developanunderstandingofpostherpeticneuralgia.

RenaldoSaldanaisa58y/owaiter withthechiefcomplaintof, “Myleft foreheadiskillingme.” Renaldowent ontosaythathewouldn’thavebotheredcominginjustforthepain,which hadbeenpresentforacoupleofdays, butwhenhedevelopedarashonhis leftforehead,hisbosstoldhimhe couldn’twaittablesandwouldhave towashdishesuntiltherashwent away.IaskedRenaldoifhehadanythinglikethishappenbefore.Heshookhis headandresponded, “Youknowme,Doc,Iamhappyandhealthy,butIam reallyworriedaboutthisrash.Thedamnforeheadpainwasbadenough,but whenIwokeupandsawthisrash,itreallyfreakedmeout!” Hecontinued, “Doc,thecrazythingisthattherashwasn’ttherewhenIwenttobed.IampositiveaboutthisbecauseIwenttolookinthebathroommirrortoseeifIcouldsee whymyforeheadwashurting,andtherewasnothingthere.Igetupthismorning,andIseeacoupleoflittleblistersovermyeye.Nowthedamnrashisspreadingandmybosswon’tletmework.I’mprettytough,butthisreallyhasme worriedbecauseifIdon’twork,Idon’teat.Theothercrazythingisithurts whenItrytocombmyhair.Whatisthatallabout?DoyouthinkIgotbitbyone ofthosebrownreclusespiders?”

IaskedRenaldoaboutanyantecedenttraumatotheforeheadandhejust shookhishead. “Doc,thiskindofsnuckuponme.LikeIsaid,atfirst,myforeheadbeganachingandthenIwokeupwiththiscrazyrash.But,likeIalsosaid, Igottawork.” IaskedRenaldowhatmadehispainworseandhesaid, “Anytime Iforgetandtouchmyforehead,itreallyhurts.” Headded, “Youknow,Doc,the othercrazythingisthatifthefaninmyroomblowsonmyforehead,Igetthese sharppains.Whatthehellisthatabout?”

IaskedRenaldotopointwithonefingertoshowmewhereithurtsthemost. Hepointedtotherashoverhislefteye,takingcarenottotouchthearea. “Doc, Ican’treallypointtooneplace.Itkindofhurtsallaroundmylefteyeandmyforehead,andanothercrazythingis,sometimesIfeellikemyhairhurts.” Iaskedif hehadanyfeverorchillsandheshookhisheadno.Ithenasked, “Whatabout steroids?Didyouevertakeanycortisoneordrugslikethat?” Renaldoagainshook hisheadno.Hedeniedanycancerorhumanimmunodeficiencyvirus(HIV). Renaldosaid, “Doc,youknowme,Iamhappyandhealthy,” butwithaworried look,headded, “Thisreallyhasmefreakedout.Ireallyneedyourhelp!”

Onphysicalexamination,Renaldowas afebrile.Hisrespirationswere18 andhispulsewas84andregular.His bloodpressure(BP)wasslightly

elevatedat144/88.Imadeanotetorecheckitagainbeforeheleftbecausehe wasprettyanxious.Hehadobviousvesi cularlesionsoverthelefteye.He hadnolesionsinhisearandbothhiseyeslookednormal.Hiscardiopulmonaryexaminationwasunremarkableot herthanthemildhypertension.His thyroidwasnormal.Hisabdominalexaminationrevealednoabnormal massororganomegaly.Therewasnoc ostovertebralangle(CVA)tenderness.Therewasnoperipheraledemaor adenopathy.Hislowbackexaminationwasunremarkable.Ididarectal exam,whichrevealednomassanda normalprostate.TheremainderofRenaldo ’ sphysicalexaminationwas withinnormallimits.

KeyClinicalPoints

THEHISTORY

’ Ahistoryofleftforeheadpain,whichoccurredpriortotheonsetof vesicularrash

’ Nohistoryofacutetrauma

’ Nohistoryofprevioussignificantfacialpain

’ Nofeverorchills

’ Acuteonsetofvesicularpaininthedistributionoftheleftophthalmic branchofthetrigeminalnerve(V1)followingtheonsetofforehead pain

’ Allodyniawhentheaffectedareaisblownonbyafan

THEPHYSICALEXAMINATION

’ Thepatientisafebrile

’ Obviousvesicularrashinthedistributionoftheleftophthalmicbranchof thetrigeminalnerve(V1)(seephotoofRenaldoSaldana)

’ Noauricularlesionsbilaterally

OTHERFINDINGSOFNOTE

’ SlightlyelevatedBP

’ Normalhead,eyes,ears,nose,throat(HEENT)examination

’ Normalcardiovascularexamination

’ Normalpulmonaryexamination

’ Normalabdominalexamination

’ Noperipheraledema

’ Normalprostateexamination

’ Noadenopathy

WhatTestsWouldYouLiketoOrder?

Thefollowingtestswereordered:

’ Completebloodcount

’ Chemistryprofile

’ Enzyme-linkedimmunosorbentassay(ELISA)testforHIV

TESTRESULTS

Alltestingwaswithinnormallimits.

ClinicalCorrelation—PuttingItAllTogether

Whatisthediagnosis?

’ Acuteherpeszosterofthefirstdivisionofthetrigeminalnerveonthe left

TheScienceBehindtheDiagnosis

ANATOMYOFTHETRIGEMINALNERVE

ThetrigeminalnerveisthefifthcranialnerveandisdenotedbytheRoman numeralV.Thetrigeminalnervehasthreedivisionsandprovidessensoryinnervationfortheforeheadandeye(V1,ophthalmic),cheek(V2,maxillary),and lowerfaceandjaw(V3,mandibular),aswellasmotorinnervationforthemusclesofmastication(Fig.1.1).Thefibersofthetrigeminalnerveariseinthetrigeminalnervenucleus,whichisthelargestofthecranialnervenuclei.Extending fromthemidbraintotheuppercervicalspinalcord,thetrigeminalnervenucleus isdividedintothreeparts:(1)themesencephalictrigeminalnucleus,which receivesproprioceptiveandmechanoreceptorfibersfromthemandibleand teeth;(2)themaintrigeminalnucleus,whichreceivesthemajorityofthetouch andpositionfibers;and(3)thespinaltrigeminalnucleus,whichreceivespain andtemperaturefibers.

Thesensoryfibersofthetrigeminalnerveexitthebrainstematthelevelofthe midponswithasmallermotorrootemergingfromthemidponsatthesame level.Theserootspassinaforwardandlateraldirectionintheposteriorcranial fossaacrosstheborderofthepetrousbone.Theythenenterarecesscalled Meckel’scave,whichisformedbyaninvaginationofthesurroundingdura materintothemiddlecranialfossa.Theduralpouchthatliesjustbehindtheganglioniscalledthetrigeminalcisternandcontainscerebrospinalfluid.

Thegasserianganglioniscanoeshaped,withthreesensorydivisions:(1)theophthalmicdivision(V1),whichexitsthecraniumviathesuperiororbitalfissure;(2)the

Fig.1.1 Thesensorydivisionsofthetrigeminalnerve.(FromWaldmanS. AtlasofInterventionalPain Management.ed.5.Philadelphia:Elsevier;2021[Fig.12.1].)

maxillarydivision(V2),whichexitsthecraniumviatheforamenrotundumintothe pterygopalatinefossa,whereittravelsanteriorlytoentertheinfraorbitalcanalto exitthroughtheinfraorbitalforamen;andthemandibulardivision(V3),whichexits thecraniumviatheforamenovaleanteriorconvexaspectoftheganglion(Fig.1.2). Asmallmotorrootjoinsthemandibulardivisionasitexitsthecranialcavityviathe foramenovale.Threemajorbranchesemergefromthetrigeminalganglion(see Fig.1.2).Eachbranchinnervatesadifferentdermatome.Eachbranchexitsthecraniumthroughadifferentsite.Thefirstdivision(V1;ophthalmicnerve)exitsthecraniumthroughthesuperiororbitalfissure,enteringtheorbittoinnervatetheglobe andskinintheareaabovetheeyeandforehead.

Theseconddivision(V2,maxillarynerve)exitsthrougharoundhole,theforamenrotundum,intoaspaceposteriortotheorbit,thepterygopalatinefossa.It thenreentersacanalrunninginferiortotheorbit,theinfraorbitalcanal,andexits throughasmallhole,theinfraorbitalforamen,toinnervatetheskinbelowthe eyeandabovethemouth.Thethirddivision(V3,mandibularnerve)exitsthe craniumthroughanovalhole,theforamenovale.Sensoryfibersofthethird

V1, Ophthalmic nerve
V2, Maxillary nerve
V3, Mandibular nerve
V1
V2 V3

V2, Maxillary division

V1, Ophthalmic division

Gasserian ganglion

Supratrochlear n.

Supraorbital n.

Infraorbital n.

Mental n.

V3, Mandibular division

Fig.1.2 Theanatomyofthegasserianganglionandthebranchesofthetrigeminalnerve.(From WaldmanS. AtlasofInterventionalPainManagement.ed.5.Philadelphia:Elsevier;2021[Fig.10.2].)

divisioneithertraveldirectlytotheirtargettissuesorreenterthementalcanalto innervatetheteeth,withtheterminalbranchesofthisdivisionexitinganteriorly viathementalforamentoprovidesensorycutaneousinnervationtotheskin overlyingthemandible.

CLINICALPRESENTATION

Herpeszosterisaninfectiousdiseasecausedbythevaricellazostervirus(VZV). PrimaryinfectionwithVZVinanonimmunehostmanifestsclinicallyasthe childhooddiseasechickenpox(varicella).Investigatorshavepostulatedthatduringthecourseofthisprimaryinfection,thevirusmigratestothedorsalrootor cranialganglia,whereitremainsdormantandproducesnoclinicallyevident disease.Insomeindividuals,thevirusreactivatesandtravelsalongthesensory pathwaysofthefirstdivisionofthetrigeminalnerve,whereitproducesthecharacteristicpainandskinlesionsofherpeszoster,orshingles.

Inf. alveolar n.
Lingual n.
Frontal n.

Whyreactivationoccursinsomeindividualsbutnotinothersisnotfully understood,butinvestigatorshavetheorizedthatadecreaseincell-mediated immunitymayplayanimportantroleintheevolutionofthisdiseasebyallowingthevirustomultiplyintheganglia,spreadtothecorrespondingsensory nerves,andproduceclinicaldisease.Patientswhoaresufferingfrommalignant disease(particularlylymphoma)orchronicdiseaseandthosereceivingimmunosuppressivetherapy(chemotherapy,steroids,radiation)aregenerallydebilitatedandthusaremuchmorelikelythanthehealthypopulationtodevelop acuteherpeszoster(Fig.1.3).Thesepatientsallhaveincommonadecreased cell-mediatedimmuneresponse,whichmayalsoexplainwhytheincidenceof shinglesincreasesdramaticallyinpatientsolderthan60yearsandisrelatively uncommoninthoseyoungerthan20years.

Thefirstdivisionofthetrigeminalnerveisthesecondmostcommonsitefor thedevelopmentofacuteherpeszosterafterthethoracicdermatomes.Rarely, thevirusattacksthegeniculateganglionandresultsinhearingloss,vesiclesin theear,andpain(Fig.1.4).ThisconstellationofsymptomsiscalledRamsay Huntsyndromeandmustbedistinguishedfromacuteherpeszosterinvolving thefirstdivisionofthetrigeminalnerve.

Fig.1.3 Lateralviewofapatientsufferingfromlymphomapoststemcelltransplantwithfaciallesions includesseverecrustingandoozinginaclearlydemarcateddermatomaldistributionalongtheright cranialnerveVdistributionwithassociatedrightfacialedema.(FromCheemaH,DiedrichA,KyneB, etal.Acaseoftri-segmentalcranialnerveVherpeszoster. IDCases.2019;18:e00642.ISSN22142509. https://doi.org/10.1016/j.idcr.2019.e00642, http://www.sciencedirect.com/science/article/pii/ S2214250919302811 [Fig.2].)

Fig.1.4 RamsayHuntsyndrome.(FromWaldmanS. AtlasofCommonPainSyndromes.ed.4. Philadelphia:Elsevier;2019[Fig.1.1].)

SIGNSANDSYMPTOMS

Asviralreactivationoccurs,ganglionitisandperipheralneuritiscausepainthat maybeaccompaniedbyflulikesymptoms.Thepaingenerallyprogressesfroma dull,achingsensationtodysestheticorneuriticpaininthedistributionofthefirst divisionofthetrigeminalnerve.Inmostpatients,thepainofacuteherpeszoster precedestheeruptionofrashby3to7days,andthisdelayoftenleadstoanerroneousdiagnosis(see “DifferentialDiagnosis”).However,inmostpatients,the clinicaldiagnosisofshinglesisreadilymadewhenthecharacteristicrashappears. Aswithchickenpox,therashofherpeszosterappearsincropsofmacularlesions thatrapidlyprogresstopapulesandthentovesicles.Eventually,thevesicles coalesce,andcrustingoccurs(Fig.1.5).Theaffectedareacanbeextremelypainful, andthepaintendstobeexacerbatedbyanymovementorcontact(e.g.,with clothingorsheets).Asthelesionsheal,thecrustfallsaway,leavingpinkscarsthat graduallybecomehypopigmentedandatrophic(Fig.1.6).

Fig.1.5 Acuteherpeszosterinvolvingtheophthalmicdivisionofthelefttrigeminalnerve.(From WaldmanSD. Painmanagement.Philadelphia:Elsevier;2007.)

Fig.1.6 Patientwithhealingherpeszosterintheseconddivisionofthetrigeminalnerve.(A)Patient presentation2weeksafteronsetofshingles.(B)Patientpresentation4weeksafteronsetofpain. (FromPaquinR,SusinL,WelchG,etal.Herpeszosterinvolvingtheseconddivisionofthetrigeminal nerve:casereportandliteraturereview. JEndodont.2017;43(9):1569 1573[Fig.3].)

<65 years of age

>65 years of age

>65 years

<65 years

Fig.1.7 Ageofpatientssufferingfromacuteherpeszoster.(DatafromWaldmanS. PainManagement. ed.2.Philadelphia:Saunders;2011.)

Inmostpatients,thehyperesthesiaandpainresolveastheskinlesionsheal. Insomepatients,however,painpersistsbeyondlesionhealing.Thiscommon andfearedcomplicationofacuteherpeszosteriscalledpostherpeticneuralgia, andolderpersonsareaffectedatahigherratethanthegeneralpopulationsufferingfromacuteherpeszoster(Fig.1.7).Thesymptomsofpostherpeticneuralgia canvaryfromamild,self-limitedconditiontoadebilitating,constantlyburning painthatisexacerbatedbylighttouch,movement,anxiety,ortemperature change.Thisunremittingpainmaybesoseverethatitcompletelydevastatesthe patient’slife;ultimately,itcanleadtosuicide.Toavoidthisdisastroussequelato ausuallybenign,self-limiteddisease,theclinicianmustuseallpossibletherapeuticeffortsinpatientswithacuteherpeszosterofthetrigeminalnerve. Ideally,preventionofacuteherpeszosterbyimmunizationwithZostrixshould beundertakeninallpatients60yearsofageandolder.

TESTING

Althoughinmostinstancesthediagnosisiseasilymadeonclinicalgrounds, confirmatorytestingisoccasionallyrequired.Suchtestingmaybedesirablein patientswithotherskinlesionsthatconfusetheclinicalpicture,suchasin patientswithacquiredimmunodeficiencysyndromewhoaresufferingfrom Kaposisarcoma.Insuchpatients,polymerasechainreactiontestingandimmunofluorescentantibodytestingcanrapidlyidentifyherpeszostervirusanddistinguishitfromherpessimplexinfections(Fig.1.8).Inuncomplicatedcases,the diagnosisofacuteherpeszostermaybestrengthenedbyobtainingaTzanck smearfromthebaseofafreshvesicle;thissmearrevealsmultinucleatedgiant cellsandeosinophilicinclusions(Fig.1.9).However,thisinexpensivebedside

Fig.1.8 DetectionofantivaricellazostervirusimmunoglobulinGbythefluorescentantibodyto membraneantigenassay.(A)Positiveresultand(B)negativecontrol.(FromSauerbreiA,FärberI, BrandstädtA,etal.Immunofluorescencetestforsensitivedetectionofvaricella-zostervirus-specificIgG:an alternativetofluorescentantibodytomembraneantigentest. JVirolMethods.2004;19(1):15 30[Fig.1].)

testdoesnothavetheabilitytodistinguishbetweenlesionscausedbytheVZV andherpessimplexinfections.

DIFFERENTIALDIAGNOSIS

Acarefulinitialevaluation,includingathoroughhistoryandphysicalexamination,isindicatedinallpatientssufferingfromacuteherpeszosterofthe

Fig.1.9 Tzancksmearshowingintranuclearinclusionbodieswithingiantmultinucleatedcell.MayGrünwaldGiemsastain;originalmagnification: 3 1000.(FromDurduM,BabaM,SeçkinD.Thevalue ofTzancksmeartestindiagnosisoferosive,vesicular,bullous,andpustularskinlesions. JAmAcad Dermatol.2008;59(6):958 964[Fig.1].ISSN0190-9622. https://doi.org/10.1016/j.jaad.2008.07.059, http://www.sciencedirect.com/science/article/pii/S0190962208010669)

trigeminalnerve.Thegoalistoruleoutoccultmalignantorsystemicdiseasethat mayberesponsibleforthepatient’simmunocompromisedstate.Apromptdiagnosisallowsearlyrecognitionofchangesinclinicalstatusthatmaypresagethe developmentofcomplications,includingmyelitisordisseminationofthedisease.Othercausesofpaininthedistributionofthefirstdivisionofthetrigeminal nerveincludetrigeminalneuralgia,sinusdisease,glaucoma,retro-orbitaltumor, inflammatorydisease(e.g.,Tolosa-Huntsyndrome),andintracranialdisease, includingtumor(Box1.1).

TREATMENT

Thetherapeuticchallengeinpatientspresentingwithacuteherpeszosterofthe trigeminalnerveistwofold:(1)theimmediatereliefofacutepainandsymptoms, and(2)thepreventionofcomplications,includingpostherpeticneuralgia.Most painspecialistsagreethattheearliertreatmentisinitiated,thelesslikelyitisthat postherpeticneuralgiawilldevelop.Further,becauseolderindividualsareatthe highestriskfordevelopingpostherpeticneuralgia,earlyandaggressivetreatmentofthisgroupofpatientsismandatory.

Nerveblock

Sympatheticneuralblockadewithlocalanestheticandsteroidthroughstellate ganglionblockisthetreatmentofchoicetorelievethesymptomsofacuteherpes

BOX1.1 ’ CausesofFacialPain

’ Trigeminalneuralgia

’ Atypicalfacialpain

’ Temporomandibularjointdysfunction

’ Temporalarteritis

’ Clusterheadache

’ Autonomictrigeminalcephalgias

’ Dentalabnormalities

’ Acuteherpeszoster

’ Trauma

’ Neoplasm

’ Infection

’ Diseasesoftheeye

’ Sinusdisease

’ Inflammatorydisorders(e.g.,Tolosa-Huntsyndrome)

’ Eaglesyndrome

’ Multiplesclerosis

’ Referredpain

’ Salivaryglanddisease

’ Vasculitis

’ Aneurysms

’ Glossopharyngealneuralia

’ Vidianneuralgia

’ Psychogenicdisorders

zosterofthetrigeminalnerve,aswellastopreventpostherpeticneuralgia.As vesicularcrustingoccurs,thesteroidmayalsoreduceneuralscarring. Sympatheticnerveblockisthoughttoachievethesegoalsbyblockingtheprofoundsympatheticstimulationcausedbyviralinflammationofthenerveand gasserianganglion.Ifuntreated,thissympathetichyperactivitycancauseischemiasecondarytodecreasedbloodflowoftheintraneuralcapillarybed.Ifthis ischemiaisallowedtopersist,endoneuraledemaforms,thusincreasingendoneuralpressureandcausingafurtherreductioninendoneuralbloodflow,with irreversiblenervedamage.

Thesesympatheticblocksshouldbecontinuedaggressivelyuntilthe patientispainfreeandshouldbereimplementedifthepainreturns.Failure tousesympatheticneuralblockadeimmediatelyandaggressively,especially inolderpatients,maysentencethepatienttoalifetimeofsufferingfrompostherpeticneuralgia.Occasionally,somepatientsdonotexperiencepainrelief fromstellateganglionblockbutdorespondtoblockadeofthetrigeminal nerve.

Opioidanalgesics

Opioidanalgesicscanbeusefultorelievetheachingpainthatiscommonduring theacutestagesofherpeszoster,whilesympatheticnerveblocksarebeing

implemented.Opioidsarelesseffectiveinrelievingneuriticpain,whichisalso common.Carefuladministrationofpotent,long-actingopioidanalgesics(e.g., oralmorphineelixir,methadone)onatime-contingentratherthananas-needed basismaybeabeneficialadjuncttothepainreliefprovidedbysympatheticneuralblockade.Becausemanypatientssufferingfromacuteherpeszosterareolder orhaveseveremultisystemdisease,closemonitoringforthepotentialside effectsofpotentopioidanalgesics(e.g.,confusionordizziness,whichmay causeapatienttofall)iswarranted.Dailydietaryfibersupplementationand milkofmagnesiashouldbestartedalongwithopioidanalgesicstoprevent constipation.

Adjuvantanalgesics

Theanticonvulsantgabapentinrepresentsafirst-linetreatmentfortheneuritic painofacuteherpeszosterofthetrigeminalnerve.Studiessuggestthatgabapentinmayalsohelppreventpostherpeticneuralgia.Treatmentwithgabapentin shouldbeginearlyinthecourseofthedisease;thisdrugmaybeusedconcurrentlywithneuralblockade,opioidanalgesics,andotheradjuvantanalgesics, includingantidepressants,ifcareistakentoavoidcentralnervoussystemside effects.Gabapentinisstartedatabedtimedoseof300mgandistitratedupward in300-mgincrementstoamaximumof3600mggivenindivideddoses,asside effectsallow.Pregabalinrepresentsareasonablealternativetogabapentinandis bettertoleratedinsomepatients.Pregabalinisstartedat50mgthreetimesaday andmaybetitratedupwardto100mgthreetimesadayassideeffectsallow. Becausepregabalinisexcretedprimarilybythekidneys,thedosageshouldbe decreasedinpatientswithcompromisedrenalfunction.

Carbamazepineshouldbeconsideredinpatientssufferingfromsevereneuriticpainwhofailtorespondtonerveblocksandgabapentin.Ifthisdrugis used,strictmonitoringofhematologicparametersisindicated,especiallyin patientsreceivingchemotherapyorradiationtherapy.Phenytoinmayalsobe beneficialtotreatneuriticpain,butitshouldnotbeusedinpatientswithlymphoma;thedrugmayinduceapseudolymphoma-likestatethatisdifficultto distinguishfromtheactuallymphoma.

Antidepressantsmayalsobeusefuladjunctsintheinitialtreatmentofpatients sufferingfromacuteherpeszoster.Onashort-termbasis,thesedrugshelpalleviatethesignificantsleepdisturbancethatiscommonlyseen.Inaddition,antidepressantsmaybevaluableinamelioratingtheneuriticcomponentofthepain, whichistreatedlesseffectivelywithopioidanalgesics.Afterseveralweeksof treatment,antidepressantsmayexertamood-elevatingeffect,whichmaybe desirableinsomepatients.Caremustbetakentoobservecloselyforcentralnervoussystemsideeffectsinthispatientpopulation.Inaddition,thesedrugsmay causeurinaryretentionandconstipation,whichmistakenlymaybeattributedto herpeszostermyelitis.

Antiviralagents

Afewantiviralagents,includingvalacyclovir,famciclovir,andacyclovir,can shortenthecourseofacuteherpeszosterandmayevenhelppreventthedevelopmentofpostherpeticneuralgia.Theyareprobablyusefulinattenuatingthe diseaseinimmunosuppressedpatients.Theseantiviralagentscanbeusedin conjunctionwiththeaforementionedtreatmentmodalities.Carefulmonitoring forsideeffectsismandatory.

Adjunctivetreatments

Theapplicationoficepackstothelesi onsofacuteherpeszostermayprovide reliefinsomepatients.Applicationofheatincreasespaininmostpatients, presumablybecauseoftheincreasedconductionofsmallfibers;however,itis beneficialinanoccasionalpatientand maybeworthtryingiftheapplicationof coldisineffective.Transcutaneouselectricalnervestimulationandvibration mayalsobeeffectiveinalimitednumberofpatients.Thefavorableriskto-benefitratioofthesemodalitiesma kesthemreasonablealternativesfor patientswhocannotorwillnotundergo sympatheticneuralblockadeorcannottoleratepharmacologicinterventions.

Topicalapplicationofaluminumsulfateasatepidsoakprovidesexcellent dryingofthecrustingandweepinglesionsofacuteherpeszoster,andmost patientsfindthesesoakssoothing.Zincoxideointmentmayalsobeusedasa protectiveagent,especiallyduringthehealingphase,whentemperaturesensitivityisaproblem.Disposablediaperscanbeusedasabsorbentpaddingtoprotecthealinglesionsfromcontactwithclothingandsheets.

HIGH-YIELDTAKEAWAYS

• Thepatientisexperiencingtheacuteonsetofleftforeheadpainfollowedbythe appearanceofvesiculareruptionsinthedistributionofthefirstdivisionofthe trigeminalnerve.

• Theonsetofpainwithoutantecedenttraumafollowedbythevesicularrashis theclassicpresentationofacuteherpeszoster.

• Therashofacuteherpeszosterwillfollowthedistributionofanerve.

• Immunocompromisedstates,includingoldage,predisposethepatienttothe developmentofacuteherpeszoster.

• PreventionofacuteherpeszosterwithuseofimmunizationwithZostrixis indicatedforpatientsovertheageof65.

• Aggressivetreatmentofacuteherpeszosterisindicatedtoavoidthe complicationofpostherpeticneuralgia.

SuggestedReadings

BandaranayakeT,ShawAC.Hostresistanceandimmuneaging. ClinGeriatrMed. 2016;32(3):415 432.

CoreyW,WaldmanSD,WaldmanRA.Painofocularandperiocularorigin. MedClin NAm.2013;97(2):293 307.ISSN0025-7125.

KansuL,YilmazI.Herpeszosteroticus(RamsayHuntsyndrome)inchildren:case reportandliteraturereview. IntJPediatrOtorhinolaryngol.2012;76(6):772 776.

LeeHL,YeoM,ChoiGH,etal.Clinicalcharacteristicsofheadacheorfacialpainprior tothedevelopmentofacuteherpeszosterofthehead. ClinNeurolNeurosurg 2017;152:90 94.

LeeHY,KimMG,ParkDC,etal.Zostersineherpetecausingfacialpalsy. AmJ Otolaryngol.2012;33(5):565 571.

O’ConnorKM,PaauwDS.Herpeszoster. MedClinNorthAm.2013;97(4):503 522. SchmaderK.Zosterherpes. ClinGeriatrMed.2016;32(3):539 553.

StaikovI,NeykovN,MarinovicB,etal.Herpeszosterasasystemicdisease. Clin Dermatol.2014;32(3):424 429.

YawnBP,WollanSt.PC,SauverJL,etal.Herpeszostereyecomplications:ratesand trends. MayoClinProc.2013;88(6):562 570.

StephanieEllison A32-Year-OldGraphic DesignerWithSevereThrobbing Left-SidedHeadaches

LEARNINGOBJECTIVES

• Learnthecommontypesofheadache.

• Developanunderstandingofclinicalpresentationofspecificheadachetypes.

• Learntoidentifyprodromeandaura.

• Developanunderstandingofthetreatmentofspecificheadachetypes.

• Developanunderstandingofthedifferentialdiagnosisofheadache.

• Developanunderstandingofthetreatmentoptionsforspecificheadachetypes.

• Learnhowtoidentifyfactorsthatcauseconcern.

StephanieEllisonisa24-year-old graphicdesignerwiththechiefcomplaintof, “Myheadisthrobbingand I’mgoingtothrowup.” Stephanie statedthatshehashadseveralheadachesamonthherentireadultlife, butoverthepastseveralweeks,she hasbeenhavingdebilitatingheadachesthataregettingworse.Shesaid thattheheadacheshavebeensobad thatshewaswaybehindonherworkandthatherboyfriendhadjustaboutquit speakingtoher.IaskedStephanieifshehadeverhadanythinglikethisbefore andshesaid, “I’vehadheadachessinceIstartedmyperiods,buttheyseemto havegottenworsesinceIstartedworkingfromhome.So,Idon’tknowwhatto do!Nothingmakestheseheadachesbetter.IknowwhenIamgoingtogetone, butDoctor,oncetheystart,Iamjustsickerthanadog.AllIcandoisgrabapan tothrowupinandgohideinadarkroom.” Toproveherpoint,shegrabbedthe wastebasketnexttotheexaminationtableandhadthedryheavesrightthere.By thetimeshefinished,tearswererunningdownherfaceandsheclosedhereyes andsobbed.

ItriedtohelpStephaniecalmdown,andaftershetriedtovomitacouple moretimes,Iaskedherifshehadidentifiedanythingthattriggeredherheadacheandsheimmediatelyanswered, “Myperiods.” Shecontinued, “IalsosometimesgetthemwhenmysleepismesseduporwhenIhavethestressofa deadline.” Iaskedherifshewaseverwokenupwithaheadache,andshesaid no,buttheyoftenoccurredinthemorning. “Theyalsogotworsewhenmygynecologistchangedmybirthcontrolpillsbecausemyinsurancechanged.” Iasked ifanyoneelseinherfamilyhadsimilarheadachesandshesaidthatbothher momandheraunthadthesameheadaches,buttheygotbetterwithmenopause. Iaskedifsheknewthatshewasgoingtogetaheadachebeforeitactuallystarted andshesaid, “Absolutely.It’sthecraziestthing.WhenIamgoingtohavea headache,itfeelslikeeverythinglookslikeitisinahigh-definitionmoviewith thesubwoofersturnedallthewayup.Ithenstarttogetreallysensitivetothe lightofmycomputerscreen,everythingistooloud,andanystrongodorsmake mewanttogag.TheothercrazythingisthatIcan’tstopyawning.Itdrivesmy boyfriendcrazy.IjustkeepyawningoverandoveragaineventhoughIamnot tired.Thenmyvisioninmylefteyegetsalljigglyandshimmeryandglitteryand IknowIamreallyintrouble.Istilldon’thaveanyheadache,butIknowthat thereisnogoingback.I’vetriedalloftheusualover-the-countermedications likeExcedrinMigraineandthoseImitrexshots,butoncetheeyethinggetsgoing,

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