RevBrasAnestesiol.2016;66(5):543---545
REVISTA
BRASILEIRA
DE
ANESTESIOLOGIA
PublicaçãoOficialdaSociedadeBrasileiradeAnestesiologiawww.sba.com.br
CLINICAL
INFORMATION
Anesthesia
in
a
patient
with
Stiff
Person
Syndrome
夽
Ozgur
Yagan
a,
Kadir
Özyilmaz
a,
Ahmet
Özmaden
a,
Özgür
Sayin
b,
Volkan
Hanci
c,∗aClinicofAnaesthesiologyandReanimation,OrduStateHospital,Ordu,Turkey bClinicofNeurosurgery,OrduStateHospital,Ordu,Turkey
cDepartmentofAnaesthesiologyandReanimation,C¸anakkaleOnsekizMartUniversity,C¸anakkale,Turkey
Received20April2012;accepted28February2013 Availableonline26October2013
KEYWORDS
Anesthesia; Totalintravenous anesthesia;
StiffPersonSyndrome
Abstract StiffPersonSyndrome(SPS),typifiedbyrigidityinmusclesofthetorsoand extrem-itiesandpainfulepisodicspasms,isarareautoimmune-basedneurologicaldisease.Herewe presentthesuccessfulendotrachealintubationandapplicationofTIVAwithoutmusclerelaxants onanSPSpatient.
A 46 years oldmale patient was operated with ASA-IIphysical statusbecause oflumber vertebral compression fracture. After induction ofanesthesiausing lidocaine, propofol and remifentaniltrachealintubationwascompletedeasilywithoutneuromuscularblockage. Anes-thesia wasmaintainedwithpropofol,remifentanilandO2/airmixture.Afteraproblem-free
intraoperativeperiodthepatientwasextubatedandsevendayslaterwasdischargedwalking withaid.
Thoughthemechanismisnotclearneuromuscularblockersandvolatileanestheticsmaycause prolongedhypotoniainpatientswithSPS.WethinktheTIVAtechnique,ageneralanesthetic practicewhichdoesnotrequireneuromuscularblockage,issuitableforthesepatients. ©2013SociedadeBrasileiradeAnestesiologia.Publishedby ElsevierEditoraLtda.Thisisan openaccessarticleundertheCCBY-NC-NDlicense( http://creativecommons.org/licenses/by-nc-nd/4.0/).
PALAVRAS-CHAVE
Anestesia; Anestesiavenosa total;
Síndromedapessoa rígida
Anestesiaempacientecomsíndromedapessoarígida
Resumo Asíndromedapessoarígida(SPR),caracterizadapelarigidezdosmúsculosdotronco eextremidadeseepisódiosdeespasmosdolorosos,éumadoenc¸aneurológicaautoimunerara. Apresentamosoocasodeintubac¸ãoendotraquealbem-sucedidaeaplicac¸ãodeAVTsem relax-antesmuscularesemumpacientecomSPR.
夽 ThiscasereportwaspresentedasaposteratTurkishAnesthesiologyandReanimationAssociation46thNationalCongressinAntalya,
Turkey,in2012.
∗Correspondingauthor.
E-mail:vhanci@gmail.com(V.Hanci).
http://dx.doi.org/10.1016/j.bjane.2013.02.004
544 O.Yaganetal.
Pacientedosexomasculino,46anosdeidade,estadofísicoASA-II,submetidoàcirurgia dev-idoàfraturaporcompressão dacoluna lombar.Apósainduc¸ãodaanestesiacomlidocaína, propofoleremifentanil,aintubac¸ãotraquealfoiconcluídacomfacilidade,sembloqueio neu-romuscular.A anestesiafoi mantidacompropofol,remifentanile misturadear/O2.Apóso
períodointraoperatórioquetranscorreusemintercorrências,opacientefoiextubadoe,sete diasdepois,recebeualta,deambulandocomajuda.
Emboraomecanismonãoestejaclaro,bloqueadoresneuromusculareseanestésicosvoláteis podemcausarhipotoniaprolongadaempacientescomSPR.AcreditamosqueatécnicadeAVT, umapráticadeanestesiageralquenãorequerbloqueioneuromuscular,éadequadaparaesses pacientes.
©2013SociedadeBrasileiradeAnestesiologia.PublicadoporElsevierEditoraLtda.Este ´eum artigoOpen Accesssobumalicenc¸aCCBY-NC-ND( http://creativecommons.org/licenses/by-nc-nd/4.0/).
Introduction
Stiff Person Syndrome (SPS), typified by rigidity in mus-cles of the torso and extremities and painful episodic spasms,isarareautoimmune-basedneurologicaldisease.1 Itis thoughtthat autoantibodiesthat attack theglutamic acid decarboxylase (GAD) enzyme necessary for phys-iopathologicsynthesisof GABAmay beresponsible.Inthe highercenters without the effect of GABAergic inhibitors hyperactivityof themotor neuronsystem maycause con-sequent progressive muscle rigidity.2 Treatment includes medicationtoincreaseGABAactivityand immunosuppress-ants.Duetosomeanestheticmedicationsinteractingwith GABAreceptors,anesthesiainSPSpatientsrequiresspecial attention.3 We present a successful endotracheal intuba-tionandTIVAapplicationwithoutmusclerelaxantsinanSPS patient.
Case
report
A 46-year-old male patient was diagnosed with SPS 7 yearspreviously. Treatment withdiazepam (15mgday−1),
baclofen (30mgday−1) and prednisolone (20mgday−1)
reduced symptoms but for the previous two months the patientcomplainedof increasedpainfulspasms especially inthelowerextremities.Thepatientcouldwalkwithhelp, but a thoracolumbar CT scanwas done toinvestigate an increaseinatwo-yearlowerbackpaincomplaint.Thescan revealedacompressionfractureatlumbar2---5levelandan operationwasplanned.Thepatient’shistoryonlyincluded hypertension.Physicalexaminationrevealedincreasedtone inthelowerextremities.Onconsultation withan internal expertpreoperative20mgprednisolone(singledose)imwas advised.Extramedicationusedincludedbisoprolol, lanso-prazole,tramadol,calciumandvitaminDsupplements.With thesefindingsthepatientwasaccepted asASA riskgroup II.
Other than these medications, premedication was not given.Coincidentwithstandard monitoring invasiveblood pressuremeasurementsweretaken.Inductionofanesthesia wasdonewithmidazolam0.1mgkg−1,lidocaine1.5mgkg−1,
propofol 2mgkg−1 and remifentanil 2mcgkg−1. Without
usinganeuromuscularblockerendotrachealintubationwas
easilyachievedandthelungswereventilatedwithanO2/air mix.Centralvenousandbladdercatheterswereemplaced. Anesthesiawasmaintainedby60---100mcgkg−1min−1
propo-foland0.1---0.8mcgkg−1min−1remifentanilinfusion.
Intra-operativehemodynamicsremainedstable,bolusanesthetics were not required and the patient was given 2 units of packed red blood cells for bleeding. Postoperative analgesia was intravenous 75mg tramadol and 25mg dexketoprofen. At the end of the operation after a problem-free extubation the patient was transferred to the surgical intensive care unit. With no follow-up prob-lems the patient was moved to the wards the following day and 7 days later was discharged walking with aid again.
Discussion
StiffPersonSyndrome(SPS)wasfirstdescribedbyMoersch andWoltmanin1956.4Whilethecauseisunknown,the pres-enceofGADantibodiesinthecerebrospinalfluid(60---70%) and coincidence withother autoimmune diseases such as diabetesmellitusandthyroiditissuggestsanimmunological basis. The syndrome is progressive, severe muscle rigid-ity and sudden onset spasms are common. It affects the lowerextremitiesandvertebraeofnearlyallpatients.The symptomsmaybetriggeredbypsychologicalstress,sudden sounds or visual warnings and touch. Autonomic symp-toms (tachycardia,hyperhidrosis, blood pressurechanges, constipation, urinary retention, etc.) accompany spasms. Thesyndromedevelopsinmiddle age.Itcommonlyoccurs with autoimmune diseases and cancer (as paraneoplastic syndrome).5 EMG shows that diazepame helps to reduce the agonist-antagonistsimultaneousmuscle contractions.6 Treatment may be with benzodiazepines (GABA-A recep-tor agonists) which increase cortical and spinal inhi-bition, baclofen (GABA-B receptor agonist) and similar GABAergic agents andsteroids, plasmapheresisor immun-globulinsforimmunemodulation.5,7,8Ourpatienthadbeen usingdiazepam,baclofenand prednisolonesincehis diag-nosisin2005.
AnesthesiainapatientwithStiffPersonSyndrome 545
thiopental,sufentanil, vecuroniumandisoflurane for gen-eral anesthetic in an operation toimplant an intrathecal baclofenpumpinanSPSpatient.Fivemonthslaterthesame procedurewascompletedwithoutanyproblemsunder gen-eralanesthetic without usingneuromuscular blockers and they advised avoiding the use of non-depolarising muscle relaxantsinSPSpatients.
Bouw et al.10 noted prolonged hypotonia after general anestheticinanSPSpatientoperatedonforcoloncarcinoma usingpropofol,sufentanil,atracuriumandisoflurane. Phar-macokinetic analysis showed that plasma concentrations of atracurium and sufentanil were well below therapeu-tic levels. They proposed that patients using baclofen in thepreoperative periodmayexperiencehypotonia dueto the effects of volatile anesthesiaagents on GABA recep-tors.
Obaraetal.11 presentedacasestudyofan SPSpatient whounderwentthymectomy,appendectomyand an endo-scopicsinusoperationwithinoneyear.Diazepam,propofol, thiopental, fentanil nitrous oxide, isoflurane and vecuro-niumwereusedfor general anesthetic.The patientwoke fromallthreeproceduresinashorttimewithnoproblems extubating. Howevertheydonotmentionwhether or not thepatientusedbaclofenorotherGABAergicagentsinthe preoperativeperiod.
LedowskiandRussell3usedTIVAwithoutneuromuscular blockers in an SPS patient undergoing an ENT opera-tion and noted that the patient was discharged with no complications.
Regional anesthesia has been successfully used in SPS patients.Shanthanna12usedcombinedspinal-epidural anes-thesiaforplannedtotalkneearthroplastyinanSPSpatient whileElkassabanyetal.13usedaparavertebralblockforan inguinal herniaoperation. However,theyemphasized that asneedle pain,fear andanxiety maytrigger spasms it is importantthatdetaileddisclosurebemadeinthe preoper-ativeperiodandsufficientsedationbeensuredduringthe procedure.
The literature shows that suitable conditions for tra-chealintubationundergeneralanestheticcanbeprovided withouttheuseofneuromuscularagents.14 Acombination ofhypnotic andopioiddrugs wasused.Thoughchest wall rigiditymaybelinkedtohighopioiddosesespeciallyin con-sciouspatients15weencounterednosuchproblemwithour patient.
Thoughthemechanismisuncleartheuseof neuromus-cularblockersandvolatileanestheticsmaycauseprolonged hypotonia in SPS patients. We believethe TIVA technique
providessuitablegeneralanesthesiainthesepatients with-outtheuseofneuromuscularblockers.
Conflicts
of
interest
Theauthorsdeclarenoconflictsofinterest.
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