Focal delayed post-chickenpox myelitis: Case report. A review and update 夽

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AnPediatr(Barc).2015;82(1):41---42

www.analesdepediatria.org

SCIENTIFIC LETTER

Focal delayed post-chickenpox myelitis: Case report. A review and update ^夽

Mielitis transversa focal posvaricelosa tardía: a propósito de un caso. Revisión y puesta al día

TotheEditor:

Transverse myelitis (TM) is a focal inflammation of the entire thicknessof the spinal cord.¹ Its incidence rate is 1---8/1000000inhabitants/year.² It is typically present in patients aged 0---2 and 5---7 years with symmetric pain, progressiveweakness, sensorydisturbances,and bowelor bladderdysfunction.^2,3

It is caused by an altered immune response and viral infection of the spinal cord that extends to the nerve fibres,leadingtohypoxaemia,ischaemia,inflammation,and demyelinisation.²

The differentialdiagnosis must ruleout a compressive myelopathybymeansofmagneticresonanceimaging(MRI).

After ruling out a compressive aetiology, a lumbar punc- ture is indicated to assess for noninflammatory causes in theabsenceofleukocytosis.²

Afullrecoveryoccursinone-thirdofthepatients,usu- allyspontaneously,andfactorsforapoorprognosisinclude older age, acute onset, a protracted course, presence of supraspinal symptoms, severe denervation, normal cere- brospinalfluid,anddelayedonsetofrecovery.³

Thispaperpresentsasingularcaseofdelayed-onsetpost- chickenpox TM, as well asan update onthe few existing publicationsonthesubject.

Thepatientwasa10-year-oldgirlpresentingwithafever of39^◦C,paresthesias,painintherightshoulder,cervicalgia, andlossofpowerandweaknessintheupperlimbs(ULs)last- ing24h.The examinationfoundproximalweaknessof the pectoralgirdle,withtheleftside(1/5)weakerthantheright (2/5),preserveddistalstrength(4/5),andurinaryretention requiring catheterisation for bladder drainage. Blood and urinecultures,serologytests,immunologytesting,andthe

夽 Please citethisarticleas:ArizaJiménez AB, MartínezAntón J,UrdaCardonaA.Mielitistransversafocalposvaricelosatardía:a propósitodeuncaso.Revisión ypuestaaldía.AnPediatr(Barc).

2015;82:41---42.

Figure 1 Thickening of the spinal cord in the segment betweenthebodiesoftheC3 andC4vertebrae, withahigh signalintensityfusiformlesion(T2)affectingtheanteriorand peripheralportionofthespinalcord.

cytochemicalstudy andoligoclonalbandsofthe CSFwere allnegative,exceptforpositivetestsforIgMandIgGanti- bodiestoVZV.Thelatterwasevidenceofapreviouslatent infection,andthemotherreportedthatthepatienthadhad chickenpoxa month and ahalf earlier, while PCR didnot detectVZVintheCSF.AT2-weightedMRIscanwithoutcon- trastruledoutabrainabnormalityandrevealedathickening ofthespinalcordandahyperechoicfusiformlesioninthe anteriorandperipheralportionofthecordbetweenC3and C4,findingscompatiblewithavaricellaaetiology(Fig.1).

The patientwastreatedwithbolus steroids (1g/day/5 days)and immunoglobulins(2g/kg/day/4days),withpro- gressive recovery of strength in the ULs (right, 4/5; left 2/5).Afterdischarge,athomethepatientcompletedphar- macologicaltreatmentbytaperingoffsteroidsandstarted rehabilitationsessions,progressingfavourably.

Transverse myelitis presents withabrupt onset of pro- gressiveweaknessand sensorydisturbance, usuallyin the lowerlimbs,^2,4contrarytowhathappenedinourcase,with weaknessintheULs.Anotheratypicalfeatureinourpatient was the presence of fever. A recent history of vaccina- tion or infection is frequent in children, although VZV is notacommon causeof TM inimmunocompetent children (0.01---0.3%).¹

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42 SCIENTIFICLETTER The diagnosis of post-chickenpox myelitis is made by

meansofserologicaltests,CSFanalysiswithVZVPCR,and MRI,whichtypicallyshowsinflammationofthespinalcord withfusiform-likehyperintensities onT2-weighted images withheterogeneousenhancement,andinvolvementofsev- eralvertebrallevels,asobservedinourpatient.^2,5

Webasedourdiagnosisontherecenthistoryofinfection by VZV andthe compatible clinical features andserology andMRIfindings.

AsfortheintervalbetweentheonsetofTMandchicken- pox,theliteraturedescribesthattheyoccursimultaneously or that TM manifests 1---2 weeks after the appearance of thepapulomacularrash,¹whileourpatientdevelopedTM6 weekslater,probablyduetoadelayed-typehypersensitivity cell-mediatedimmuneresponse.

There is no established treatment regime for post- chickenpoxTM,buttheliteratureshowsthatpatientsevolve favourablywithacyclovirandsteroids.^1,4,5Acyclovirwasnot usedinourpatientbecausethepreviousinfectioushistory wasunknown,asthemotherhadnotreporteditduringthe anamnesis.Physicaltherapyfollowingtheacuteepisodecan diminishneurologicdeficitsinthesepatients.²Thus,early initiationofthesetherapiesisimportantinreducingseque- lae.Recoveryusuallystartsafter10weeks,andcantakeas longas12---24months.^1,4

It is worth notingthat a history of vaccinationagainst varicellawasabsent, sowe must underscore theneed to vaccinateagainstthisvirusinordertoreducetheincidence ofchickenpoxanditscomplications.⁶

In conclusion, post-chickenpox TM is an uncommon complication in immunocompetent children and must be consideredin the differentialdiagnosis of patientswitha

history of varicella infection in the six weeks preceding theonsetofsymptoms.Magneticresonanceimagingofthe spinalcordandserologicaltestsandPCRforVZVareuseful toolsfordiagnosingthisdisease.Pharmacologicaltreatment andrehabilitationtherapymustbeinitiatedassoonaspos- sibletoreducethesequelae.

References

1.Y´ylmazS,KöseoðluK,YücelHKA.Transversemyelitiscausedby varicellazoster:casereports.BrazJInfectDis.2007;11:179---81.

2.KimMY,SuhES.Acaseofacutetransverse myelitis following chickenpox.KoreanJPediatr.2009;52:380---4.

3.ArroyoHA.Mielopatíasagudasnotraumáticasenni˜nosyadoles- centes.RevNeurol.2013;57Suppl.1:S129---38.

4.AslanA,KurugolZ,GokbenS.Acutetransversemyelitiscompli- catingbreakthroughvaricellainfection.PediatrInfectDisJ.2014 [Epubaheadofprint].

5.ChandP,IbrahimS,Zaidi SS,AmjadN.Earlyrecovery inpost varicellatransversemyelitis.JCollPhysSurgPak.2014;24Suppl.

2:S107---8.

6.García-EstévezDA.Cervicodorsalmyelitissecondarytoinfection byvaricella zostervirusinan immunocompetentpatient. Rev Neurol.2013;57:191---2.

A.B.ArizaJiménez^∗,J.MartínezAntón, A.UrdaCardona UnidaddeGestiónClínicadePediatría,HospitalRegional UniversitarioMaterno-Infantil,Málaga,Spain

∗Correspondingauthor.

E-mailaddress:[email protected] (A.B.ArizaJiménez).