Pseudotumoral cerebellitis with acute hydrocephalus as a manifestation of EBV infection

ProfessorDefebvreisascientificadvisorforApoPharma, LaboratoireAguettant,OrkynandNovartis.

ProfessorJourdainhasnothingtodisclose.

Appendix

A.

Supplementary

data

Supplementary data associated with this article can be found,intheonlineversion,atdoi:10.1016/j.neurol.2017.06.014.

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[1] ErmakDM,BowerDW,WoodJ,SinzEH,KothariMJ. Incorporatingsimulationtechnologyintoaneurology clerkship.JAmOsteopathAssoc2013;113(8):628–35.

[2] MalakootiMR,McBrideME,MobleyB,GoldsteinJL,AdlerMD, McGaghieWC.Masteryofstatusepilepticusmanagement viasimulation-basedlearningforpediatricsresidents.JGrad MedEduc2015;7(2):181–6.

[3] OutinH,BlancT,VinatierI,legrouped’experts.[Emergency andintensivecareunitmanagementofstatusepilepticusin adultpatientsandchildren(new-bornexcluded].RevNeurol (Paris)2009;165(4):297–305.

[4] LesageFX,BerjotS.Validityofoccupationalstress

assessmentusingavisualanaloguescale.OccupMed2011.

C.Moreaua,b,c,_* A.Delvala,b,d,e G.Groleza,b,c S.Moulina,c,d,e Y.Chena,c,f T.Lebouviera,c,f L.Defebvrea,c J.Poissyb,c M.Jourdainb,c a_{ServiceofNeurology,RogerSalengroHospital,LilleUniversity}

MedicalCenter,Lille,France

b_{PRESAGESimulationCenter,FacultyofMedicine,Lille-University,}

France

c_{HenriWarembourgFacultyofMedicine,Lille-University,Lille,}

France

d

ClinicalNeurophysiologyDepartment,NeurovascularIntensive CareUnitRogerSalengroHospital,LilleUniversityMedicalCenter, Lille,France

e

NeurovascularIntensiveCareUnit,RogerSalengroHospital,Lille UniversityMedicalCenter,Lille,France

f_{MemoryResourceandResearchCentre,NeurologyDepartment,}

RogerSalengroHospital,LilleUniversityHospital,Lille,France *Correspondingauthorat:ServicedeNeurologieetPathologiedu mouvement,PoˆledeNeurosciencesetappareillocomoteur, HopitalRogerSalengro,RueEmileLaisne,F-59037Lille,France. E-mailaddress:caroline.moreau@chru-lille.fr(C.Moreau) Availableonline13November2017

http://dx.doi.org/10.1016/j.neurol.2017.06.014

0035-3787/#2017ElsevierMassonSAS.Allrightsreserved.

Pseudotumoral

cerebellitis

with

acute

hydrocephalus

as

a

manifestation

of

EBV

infection

Abbreviations: CSF;cerebrospinalfluid CT;computedtomography EBV;Epsteinaˆs‘‘Barrvirus ICP;increasedcranialpressure MRI;magneticresonanceimaging PCR;polymerasechainreaction VZV;varicella-zostervirus

1.

Case

report

A7-year-oldboypresentedwitha5-dayhistoryofafebrile vomiting, during which time he also developed acute behavioral disorders and temporal headaches. The child hadpresentedwithaviralrashafortnightbeforetheonset ofsymptoms.

Physical examination revealedneck stiffness associated withnew-onsetbradycardia.Thepatientwasconscious,but wailing. Neurologicalexamination was otherwise unremar-kable.

Computedtomography(CT)ofthebrainshoweddilatation of the ventricular system associated with asymmetrical density between the two cerebellar hemispheres (Fig. 1). Fundoscopy was normal. Cerebrospinal fluid(CSF) analysis revealed47elements/mm3_{.T2-weightedbrainmagnetic}

reso-nanceimaging(MRI)confirmedthepresenceofahyperintense

Fig.1–BrainCTrevealsasymmetricaldensityofthetwo cerebellarhemispheres.

revue neurologique 174(2018) 66–74

swellingoftheleftcerebellarhemispherewithamasseffect on adjacent structures and, consequently, hydrocephalus (Fig. 2). Serological analysis revealed positivity for anti-Epsteinaˆs‘‘Barr virus (EBV) IgM; otherwise, autoimmune serologieswerenegative.

Patientmanagementconsistedofinsertionofanexternal ventricular shunt anda courseoforal prednisone.Clinical evolution was favorable and the shunt was removed. On follow-ups1monthand1yearlater,therewaspersistenceof astheniaandslightpsychomotorslowness.

Thisreportdescribesacaseofcerebellar meningoence-phalitis due to EBV that affected predominantly a single hemisphere,inducingedemaintheaffectedregionleading to hydrocephalus and increased cranial pressure (ICP). Neurological manifestations of EBV infections include meningoencephalomyelitis, cerebellitis and Guillainaˆs ‘‘-BarrA˜ # syndrome,andaccountforaround0.5%ofcases[1]

thatmainlyaffectyoungpatients.

Cerebellitis is a parainfectious disease characterized by cerebellar swelling and presenting with signs of mild cerebellar dysfunction. While the outcome is favorable in mostpatients,thediseasecan,inaminorityofcases,induce neurologicalsequelaeordeath[2,3].Onrareoccasions,itcan manifest as unilateral cerebellar swelling with obstructive hydrocephalus and signs of ICP, but without the life-threatening cerebellar-syndrome presentation known as aˆs

˜

hemicerebellitisaˆsTM_{,whichcaneasilybemistakenfora}

tumoralprocess[4].

The literature contains reports of 39 cases of acute hemicerebellitis[2,3]inadditiontoourpresentcase,andall oftheminvolvepatientsaged3aˆs_{‘‘18years.Thesereported} casesalsosuggestapredominanceofaffectedfemales,witha genderratioof2:3.Inaddition,56%ofcaseshadpresented

withaninfectiouseventweeksbeforedeveloping manifesta-tions ofhemicerebellitis. Symptoms at diseaseonset were: cerebellarsigns(67.5%);headache(60%);vomiting(45%);fever; cranial nerve involvement (20%); neck stiffness (17.5%); pyramidal syndrome (15%); fluctuations of consciousness (10%);andseizures(7.5%).However,nocerebellarsignswere evidentin32.5%ofcases.

Anetiologicalinfectiousagentwasidentifiedineightcases (20%), and included EBV (two cases), Salmonella, rotavirus, Coxiella burnetii, varicella-zoster virus (VZV; two cases) and influenzavirus.

Managementofcerebellitisdiffersaccordingtotheseverity oftheinitialpresentation.Thetherapeuticvalueofsteroidsis questioned, as the clinical course is often benign and sequelae-free [5]. Antiviral therapy appears to be a safe therapeuticoptionforallpatientspresentingwith encepha-litis symptoms, as the benefits outweigh the risks in potentially life-threatening conditions.Emergency manage-ment ismandatoryincasesofconsciousness deterioration suggestiveofbrain-steminvolvement.Temporarydrainingof hydrocephalusthroughexternalventriculardrainageisoften sufficient,asthepost-infectiousinflammatoryreactionoften resolvesrapidly,butposteriorfossadecompressionis indica-tedwhendrainagedoesnotimprovetheclinicalcondition[6]. In conclusion, infectious cerebellitis can manifest as a pseudotumoral processwithacutehydrocephalus. The pre-sentcaseaddstothecurrentlyavailableliteraturebybeing onlythe second caseattributable toEBVinfection,thereby highlightingthevariabilityofthepathogenicspectrumofthis viralagent,andraisingtheissueoftheimportanceofantiviral therapyforallacuteneurologicaldisorders.

Funding

Thisresearchdidnotreceiveanyspecificgrantfromfunding agenciesinthepublic,commercial,ornot-for-profitsectors.

Disclosure

of

interest

Theauthorsdeclarethattheyhavenocompetinginterest.

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[1] Kleinschmidt-DeMastersBK,GildenDH.Theexpanding spectrumofherpesvirusinfectionsofthenervoussystem. BrainPathol2001;11:440–51.

[2] Rodriguez-CruzPM,Janet-SignoretS,Miranda-HerreroMC, Barredo-ValderramaE,VA˜ ! zquez-LA˜pezM,Ruiz-MartA˜nY, etal.Acutehemicerebellitisinchildren:casereportand reviewofliterature.EurJPaediatrNeurol2013;17:447–53.

[3] CarcellerLechA˜nF,DuatRodriguezA,SirventCerdaSI, KhabraK,dePradaI,GarcA˜a-PeA˜asJJ,etal.Hemicerebellitis: reportofthreepaediatriccasesandreviewoftheliterature. EurJPaediatrNeurol2014;18:273–81.

Fig.2–BrainMRIshowsahyperintenseswellingintheleft cerebellarhemispherewithamasseffectonadjacent structuresleadingtohydrocephalus.

[4] Madrid-MadridA,Ariza-ArandaS,Martinez-AntonJ, Mora-RamirezMD,Delgado-MarquesMP.Hemicerebellitisversus cerebellartumour.RevNeurol2006;42:508–10.

[5] HA˜uslerM,RamaeckersVT,DoengesM,SchweizerK,Ritter K,SchaadeL.Neurologicalcomplicationsofacuteand persistentEpsteinaˆs‘‘Barrvirusinfectioninpaediatric patients.JMedVirol2002;68:253–63.

[6] deRibaupierreS,Meagher-VillemureK,VillemureJG, CottingJ,JeannetPY,PorchetF,etal.Theroleofposterior fossadecompressioninacutecerebellitis.ChildsNervSyst 2005;21:970–4. C.Geurtena,* G.DeBilderlingb M.-C.Nassognec J.-P.Missond M.Verghoteb a

UniversityDepartmentofPaediatrics,CentreHospitalierrA˜#gional delaCitadelle,LiA˜¨ge,Belgium

b_{DepartmentofPaediatrics,CHRNamur,Namur,Belgium} c_{UniversitA˜#catholiquedeLouvain,CliniquesUniversitairesSt-Luc,}

Brussels,Belgium

d_{UniversityDepartmentofPaediatrics,DivisionofNeuropaediatrics,}

CentreHospitalierrA˜#gionaldelaCitadelle,LiA˜¨ge,Belgium *Correspondingauthorat:Chenestre72a,4606Dalhem,LiA˜¨ ge, Belgium. E-mailaddress:c.geurten@gmail.com(C.Geurten) Received17November2016 Receivedinrevisedform10April2017 Accepted15June2017 Availableonline3November2017

http://dx.doi.org/10.1016/j.neurol.2017.06.015

0035-3787/#2017ElsevierMassonSAS.Allrightsreserved.

Guillain–Barre´ syndrome

due

to

hepatitis

E

1.

Case

report

A60-year-oldmanpresentedwithprogressiveweaknessand sensory symptoms. Ten days earlier, he had experienced diarrhea, nausea,severe asthenia and afever lasting for4 days,andlivercytolysisandsystemicinflammation.Fourdays later,heexperiencedsymmetricalparesthesiaofthefeetthat rapidlyextendedtothewholeofhislowerlimbsandhands. Progressiveweaknessdevelopedintheproximallowerlimbs. Hehadnofever.Neurologicalexaminationshowed predomi-nantlyproximalweakness(upperlimbs:distal,grade5/5and proximal, grade 3/5; lower limbs: distal, grade 4/5 and

proximal, grade 2/5). Diminished temperature and pain sensationtogetherwithhypopallesthesiawerenotedinthe lower limbs, as well as areflexia and ataxia. He had no breathingorswallowingdifficulties,andnoclinical manifes-tationssuggestiveofhepatitis.

Magnetic resonance imaging of the entire spine was normal.AnerveconductionstudyshowedincreasedF-wave latencyinthelowerlimbs.Arepeatstudydone2weekslater demonstrated segmental demyelinating neuropathy with prolonged distal latency, and trunk and root conduction blocksinthelowerlimbs,indicatingadiagnosisofGuillain– Barre´ syndrome(GBS).Standardbloodtestsshowedsevere liver cytolysis (Table 1). Cerebrospinal fluid (CSF) was characterized by albuminocytological dissociation with moderately elevated protein (0.53g/L; normal: 0.45g/L), and only 27 leukocytes (85% lymphocytes) were without oligoclonal bands.ThefirstCSFsamplewasnottestedfor hepatitisEvirus(HEV)RNA.However,serumwaspositivefor IgM antibodies to HEV and for HEV genotype 3c RNA. Abdominal ultrasonography showed hepatomegaly with steatosis.

ThechronologicallinkbetweenacuteHEVinfectionand GBS suggested acausal relationship. Intravenous immuno-globulin(0.4g/kg/day)wasgivenfor5days.Livercytolysisand clinical, electrophysiological and laboratory abnormalities improved spontaneously. Thepatientalsoregainedmuscle strength.AsecondCSFstudy2weeksaftersymptomonset showed protein elevation (0.67g/L), no leukocytes and the presenceofHEVRNA(59.9IU/mL).Onfollow-upevaluation4 months later, the patient had normal sensory and motor function, and all limbtendonreflexeswerepresent. Nerve conductionstudyresultswerealsoimproved.Liverfunction testswerenormal,andHEVRNAwasundetectable.Oneyear later,therewasnoevidenceofrelapse.

HEVinfectionisanemergingcauseofacutehepatitisin industrialized countries. Neurological complications have been reportedin associationwith HEV infection,including GBS[1–4].ArecentstudyfromTheNetherlandsshowedserum IgManti-HEVantibodiesin5%of201casesofGBScompared withonly0.5%of201healthycontrols[5].Thus,HEVinfection isanemergingtriggerforGBSinEurope.Inimmunocompetent patients with acute HEV infection and GBS, intravenous immunoglobulintherapy hasbeenconsistentlyfollowedby favorable outcomes [1–4]. In contrast, thetreatment isnot alwayseffectiveinimmunocompromisedpatientsorinthose withchronicHEVinfections[3].Furtherstudiesareneededto determinethe best therapeutic strategyforHEV-associated GBS.

Inconclusion,patientswithGBSandlivercytolysisshould beroutinelytestedforHEVinfection,assuchadiagnosiscan lead to more appropriate follow-up as it carries a risk of chronichepatitisthatmayrequiretreatment.

Consent

Writteninformedconsentwasobtainedfromthepatientfor publicationofthiscasereport.Acopyofthesignedconsent formisavailableforreviewbytheEditor.

Abbreviations: CSF, cerebrospinal fluid; GBS, Guillain–Barre´ syndrome;HEV,hepatitisEvirus

revue neurologique 174(2018) 66–74