Case32015:A60YearOldWomanwith

AbdominalPain,Dyspnea,andDiplopia
WilliamS.David,M.D.,Ph.D.,ElizabethS.Temin,M.D.,JessicaJ.Kraeft,M.D.,
andDavidC.Hooper,M.D.
PresentationofCase
Dr.KathyChuang(Neurology):A60yearoldwomanwasadmittedtothis
hospitalinmidwinterbecauseofabdominalpain,dyspnea,anddiplopia.
Thepatienthadahistoryofasthmaandgastroesophagealrefluxdisease.Shehad
beenwelluntilthedaybeforeadmission,whenshenoticedthathervoiceseemed
hoarseandthickwithincreasedsalivation.Thatafternoon,nausea,retching,and
worseningabdominalpaindeveloped,withassociatedshortnessofbreathsimilar
tothatcausedbyherasthma.Thatevening,shewastakenbyambulanceto
anotherhospital.
Onarrivalintheemergencydepartment,thepatientreportedsharp,constant,
diffuseabdominalpainthatsheratedat9onascaleof0to10(with10indicating
themostseverepain),aswellasthroattightnessanddysphagia.Onexamination,
shewasalert,uncomfortable,andillappearing.Thetemperaturewas36.8C,the
bloodpressure138/79mmHg,thepulse117beatsperminute,therespiratoryrate
18breathsperminute,andtheoxygensaturation96%whileshewasbreathing
ambientair.Theabdomenwassoftandnondistended,withsoftbowelsounds;
therewaspainonpalpationintheleftupperandbothlowerquadrants.The
remainderofthegeneralandneurologicexaminationswasreportedlynormal.
Theplateletcountwasnormal,aswerebloodlevelsoftotalprotein,albumin,
globulin,andlipaseandresultsofcoagulationandliverandrenalfunctiontests;
othertestresultsareshowninTable1.Urinalysisrevealedaspecificgravityof
1.020,apHof5.0,1+ketones,andtraceleukocyteesteraseandhemoglobinby
dipstick,aswellasbacteria,mucus,and1to4whitecellsand1to3redcellsper
highpowerfield.Achestradiographshowedlinearopacitiesatthelungbasesthat
wereconsistentwithsubsegmentalatelectasis.

Adiagnosisofpossibleallergicreactionwasmade;diphenhydraminehydro
chloride,ondansetron,morphinesulfate,famotidine,methylprednisolone,normal
saline,andepinephrinewereadministered.Anelectrocardiogramshowedsinus
tachycardiaatarateof106beatsperminute,withnonspecificSTsegmentandT
wavechanges.Radiographsofthesofttissueoftheneckandthecervicalspine
showedmultileveldegenerativediskandfacetjointdisease.Oxygen
supplementationwasadministeredbymeansofanasalcannulaatarateof2liters
perminute,andtheoxygensaturationroseto100%.Anoralanticholinergicand
antispasmodicsolution(atropinesulfate,hyoscyamine,phenobarbital,
scopolaminehydrobromide,andlidocaine)andanoralantacidsolution
(aluminumhydroxide,magnesiumhydroxide,and
simethicone)wereadministered.Withinminutes,thepatientwasreportedly
unabletodrinkthesecondhalfofthemixture.Computedtomography(CT)of
thehead,neck,spine,chest,abdomen,andpelvis,performedafterthe
administrationoforalandintravenouscontrastmaterial,reportedlyrevealed
findingssuggestiveofsmallbowelobstruction.Anasogastrictubewasplaced,
and250mlofliquidremoved.Thepatientreportedhorizontaldiplopia,whichhad
beendevelopingduringtheprevious2hours,andsoonafterhadincreasing
difficultyspeaking.Shewastransferredtothishospitalbyambulance,arrivingat
6:23a.m.,approximately9hoursafterpresentationtotheotherhospital.
Onarrival,thepatient,whowasrighthanded,communicatedprimarilybywriting.
Shereportedincreasingshortnessofbreathandafeelingofsuffocation.The
previousweek,shehadhadanupperrespiratoryinfectionwithacoughbutno
fever.Shehadahistoryofhyperlipidemia,asthma,andgastroesophagealreflux
disease.Medicationsathomewereatorvastatin,dexlansoprazole,montelukast,
andfluticasonesalmeterol.Shewasallergictosulfadrugsandlevofloxacin.She
drankalcoholinmoderationanddidnotsmokeoruseillicitdrugs.Shelivedwith
herhusband,workedinanoffice,andhadnotrecentlytraveled.Herfamilyhistory
includedhypertension,heartdisease,andcoloncancer;herhusbandwaswell.
Ontheinitialexamination,performedat6:40a.m.,thepatientwasalertand
oriented.Thebloodpressurewas125/77mmHg,thepulse122beatsperminute,
thetemperature36.8C,therespiratoryrate18breathsperminute,andthe
oxygensaturation96%whileshewasbreathingambientair.Theabdomenwas
soft,mildlydistended,andnontender.Onneurologicexamination,thepupilswere
equalandreactivetolight;theextraocularmusclemovementwasintact,without
nystagmus.Therewasbinoculardiplopiaatfardistances.Thepatientwas
unabletophonateclearlyorprotrudethetongue;othercranialnervefunctions,
motorfunction,andperipheralsensationwereintact.At7a.m.,thepatientwas

unabletoelevatethepalate,phonationwasworse,andtherewasweaknesson
abductionoftherighteye.
Theplateletcount,erythrocytesedimentationrate,urinalysis,andresultsof
coagulationandrenalandliverfunctiontestswerenormal,aswerebloodlevels
ofmagnesium,lacticacid,totalprotein,albumin,globulin,amylase,lipase,IgA,
Creactiveprotein,thyrotropin,andglycatedhemoglobin;othertestresultsare
showninTable1.Anelectrocardiogramshowedsinustachycardiaatrateof
123beatsperminute.Surgicalandneurologicconsultationswererequested.
Onarepeatexamination,performedbyneurol
ogyconsultantsat8:30a.m.,therewasbilateralptosisandweaknessoneye
abductionandelevation.Reflexeswerenormalandsymmetricthroughout.CTof
thehead,performedwithouttheadministrationofcontrastmaterial,showedlow
lyingcerebellartonsilswithcrowdingoftheforamenmagnum,withoutevidence
ofintracranialhemorrhage,infarct,ormasslesion.
Ipratropiumwasadministeredbynebulizer.Duringthenext2hours,intermittent
stridorandworseningweaknessoftheface,palate,andtonguedeveloped,andthe
dyspneaworsened.Threehoursafterarrival,fiberopticnasopharyngoscopy
revealedbilateralvocalcordparalysis,withthecordsfixedat1to2mmof
opposition,withoutedema.Supplementaloxygenwasadministered,andthe
tracheawasintubatedforairwayprotection.
Thepatientwasadmittedtotheneurologyintensivecareunit.Onexamination,
shewasalertandabletofollowcommandspromptly.Therewasbilateral
completeptosis,ophthalmoparesis,andanisocoria;theleftpupilwasovalshaped,
withaverticallongaxisof8mm,andwaspartiallyreactiveintheupperhalf,
andtherightpupilwas6mmindiameterandreactiveto4mm.Therewasmild
proximalweaknessofthearmsandlegs(withaMedicalResearchCouncilgrade
of4onascaleof0[paralysis]to5[normalstrength]);distalstrengthwas
preserved.Reflexeswerebriskthroughout,andbothplantarreflexesweref
lexor.
Adiagnostictestwasperformed.
ImagingStudies
Dr.JessicaJ.Kraeft:CToftheneck,abdomen,andpelviswasperformedatthe
otherhospital,andthefindingswerereinterpretedatthishospital.Imagesofthe
neck,obtainedaftertheadministrationoforalcontrastmaterial,showthatthe
esophagusisdilatedabovethelevelofthethoracicinlet,afindingconsistent

withgastroesophagealreflux(Fig.1A).Intheabdomen,thestomachismildly
dilated(Fig.1B);however,noabnormallydilatedbowelloopsarepresent,and
thereisnotransitionpointindicativeofabowelobstruction.Fecalizationofthe
contentsofdistalsmallbowelloopsissuggestiveofslowtransitthroughoutthe
gastrointestinaltract(Fig.1Cand1D).

AB

CD

Figure1.CTScansoftheNeckandAbdomen.Asagittalimageoftheneck(PanelA),obtainedaftertheadministratio

esophagusisdilated(arrows).Acoronalimageoftheabdomen(PanelB)showsthatthestomachismildlydilated(arrow
andD,respectively)showfecalizationofsmallbowelcontents(whiteovals).

AirwayManagementintheEmergencyDepartment
Dr.ElizabethS.Temin:Thepatientreportedshortnessofbreathandchoking.
Herlungswereclearonauscultation.Hermentalstatus,gagreflex,respiratory
rate,andoxygensaturationwerenormal.Therewasconcernattheotherhospital
aboutanallergicreaction,soweperformedfiberopticnasopharyngoscopy.We
foundnoedemabutdidfindevidenceoffullvocalcordparalysis.Wewere
concernedthatherprogressiveanddiffusemuscleweaknesscouldinvolveher
respiratorymuscles;weperformedanassessmentofinspiratorymusclestrength.
Hernegativeinspiratorypressurewas10cmofwater(normalrange,45
to120cmofwater,dependingonstartinglungvolume,sex,andage),whichmet
acriterionforintubation.Thedecisionwasmadetoperformrapidsequence
intubation1;anondepolarizingneuromuscularblockingdrugwasadministered
becauseofconcernaboutlifethreateninghyperkalemiainpatientswith
musclewasting,burns,musculardystrophy,orparalysis.2,3Thispatientwas
intubatedwithrocuroniumandetomidate,withoutanycomplications.
DifferentialDiagnosis
Dr.WilliamS.David:Iamawareofthediagnosis.Inthispatient,rapidly
progressive,generalizedweaknessdevelopedthatfirstaffectedtheocu
larandbulbarmusculatureandsubsequentlyaffectedappendicularand
respiratorymuscles,inconjunctionwithautonomicdysfunction.
Thedifferentialdiagnosisofacutegeneralizedweaknessinthispatient
encompassesprocesseslocalizedtoanyleveloftheneuraxis,includingthecentral
nervoussystem(cortexandbrainstem),anteriorhorncells,nerve,neuromuscular
junction,andmuscle.Thispatienthadapreservedsensorium,symmetric
weakness,andnormalfindingsonbrainimaging.Takentogether,thesefeaturesof
herpresentationmakeacentralcauseofacuteweaknessunlikely.Peripheral
causesofacutegeneralizedweaknessincludemotorneuronopathies,acute
acquiredpolyneuropathies,myopathies,andpresynapticandpostsynaptic
neuromusculartransmissiondisorders.
MotorNeuronopathies

Theabsenceoffeverandofahistoryofameningoencephalitisinthispatient
suggeststhatadiagnosisofpoliomyelitisorWestNilevirusisunlikely,although
WestNilevirushasbeenreportedtooccurintheabsenceofbothfeatures.West
NilevirusismosquitoborneandthusisunlikelytobetransmittedinDecemberin
NewEngland.Inaddition,paralysisassociatedwithWestNilevirusistypically
asymmetric,theoculobulbarmusculatureisnotusuallyinvolved,andrespiratory
failureisuncommon(occurringinapproximately10%ofcases).EnterovirusD68
infectionhasrecentlybeenimplicatedincasesofacuteflaccidparalysisin
children.4,5Inmostcases,respiratorydiseaseappearstoprecedetheonsetof
neurologicsymptoms.Thispatientsageandtheabsenceofanantecedent
respiratoryillnessmakethisdiagnosisunlikely.
AcuteAcquiredPolyneuropathies

ClassicGuillainBarrsyndromeisanunlikelycauseofthispatientsillness,
becauseaffectedpatientsusuallypresentwithanascendingparalysisand
paresthesias,andneitherfeaturewaspresentinthiscase.PatientswiththeMiller
Fishersyndrome(anatypicalvariantoftheGuillainBarrsyndrome)canpresent
withoculobulbarsymptoms;ataxiaandareflexiaaretypical,andneitherfeature
wasdescribedinthiscase.ThepharyngealcervicalbrachialvariantoftheGuil
lainBarrsyndromeinvolvestheextraocularmusclesinabout50%ofcases,but
thelegsareusuallyspared,thereflexesareattenuated,andtheevolu
tionofsymptomsisrelativelyslow(overaperiodof1to3weeks).
Acutemotoraxonalneuropathyisunlikelybecauseinmostcases,itcauses
distallypredominantweakness,doesnotinvolvethecranialnerves,andsparesthe
respiratorymuscles.Patientswithporphyricneuropathycanpresentwithacute
generalizedweakness,abdominalpain,andvomiting,butpsychiatricorother
symptomsassociatedwiththecentralnervoussystem,includinghallucinations,
confusion,anxiety,andseizures,mayoccur;inaddition,distalsensorylossis
oftenpresentanddiseaseprogressionislessfulminantthanitwasinthiscase.
Tickparalysisproducesanascendingpatternofweakness,butthispatient
presentedwithadescendingpattern.Inpatientswithtickparalysis,paresthesias
arecommonandthecranialnervesareusuallynotinvolved.Inaddition,tick
paralysisusuallyoccursinthespringandsummermonths,whenticksareactive,
butthispatientpresentedinmidwinter.Diphtheriacancausebulbarandlimb
weaknessandrespiratoryfailure,buttheabsenceofparesthesias,sensoryloss,and
hyporeflexiainthiscasemakesthisdiagnosisunlikely.Diphtheriaisalso
manifestedasdistallypredominantweakness,andsymptomswouldbeexpected
toevolvemoreslowlythandidthispatientssymptoms.Arsenicintoxicationcan

mimictheGuillainBarrsyndrome,butpatientsusuallypresentwithdistally
accentuatedweakness,burninginthehandsandfeet,andweeksofdisease
progressionfeaturesthatarenotconsistentwiththispatientspresentation.
Myopathies

Afewmyopathiescanbemanifestedasacutegeneralizedweakness,butin
general,thiscategoryofdiseasesisnotlikely.Disordersthatcauseperiodic
paralysistypicallysparetheoculobulbarandrespiratorymuscles,whichwere
prominentlyaffectedinthispatient.Inflammatorymyopathiesalsospareocular
andrespiratorymuscles,andthediseaseprogressionofinflammatorymyopathies
istypicallynotasrapidastheprogressionseeninthiscase.
NeuromuscularTransmissionDisorders

Disordersofneuromusculartransmission,includingbothpresynapticand
postsynapticdisorders,needtobeconsidered.Myastheniagravis,apostsynaptic
disorderofneuromusculartransmission,
Thenewenglandjournalofmedicine

CaseRecordsoftheMassachusettsGeneralHospital
Table2.ResultsofElectromyographicStudies.
StudyLeftMedianNerveLeftUlnarNerveLeftFibularNerveLeftTibialNerve
CompoundmuscleactionpotentialmV(normal
value)

2.4(>4.0)

2.2(>6.0)

Compoundmuscleactionpotential3.6(50)5.1(150)5.3(39)after10secofmaximalvoluntarycontractionmV(%i
Distalmotorlatencymsec(normalvalue)

4.1(<4.5)

2.9(<3.5)

11.9(>10)

Conductionvelocitym/sec57.1(>49)67.8(>49)56.7(>43)52.4(>43)(normalvalue)
RightMedianNerveLeftUlnarNerveLeftSuralNerve
SensorynerveactionpotentialV(normalvalue)

18.1(>12.0)

Peaklatencymsec(normalvalue)2.9(<3.2)2.5(<2.8)3.0(<4.0)

typicallydoesnotprogressasrapidlyasthispatientsillnessdidandisnot
associatedwithautonomicsymptoms,whichwereprominentinthiscase.The
LambertEatonmyasthenicsyndrome,apresynapticdisorder,cancauseweak

nessandautonomicdysfunctionbutusuallyhasasubacutecourse.Involvementof
thebulbarmusclesmayoccurbutisuncommon;therespiratorymusclesare
usuallyspared,andtheeyemusclesaretypicallyunaffected.Forthesereasons,
theLambertEatonmyasthenicsyndromeisanunlikelydiagnosisinthiscase.
Paralysisduetoaneuromusculartoxinofmarineorigin(e.g.,tetrodotoxinfrom
pufferfishandsaxitoxinfromshellfish)orduetosnakeorscorpionen
venomationisaremoteconsideration,butthispatientdidnothaveanyrelevant
exposures.
Botulismisatoxinmediateddiseasethatresultsinthepresynapticblockadeof
acetylcholinetransmissionacrosstheneuromuscularjunction.Patientstypically
presentwithblurredvision,ptosis,anddiplopia.Impairedbulbarfunctionis
manifestedasfacialweakness,dysarthria,anddysphagia.Descendingmuscle
weaknessaffectsheadcontrolandsubsequentlyarm,respiratory,andleg
musculature.Theweaknesstypicallyhasaproximaltodistalpatternandis
bilateral.Autonomicsymptoms,includingnausea,vomiting,ileus,poorpupil
reactivity,andalterationsinbloodpressureandheartrate,arecommon;the
gastrointestinalsymptomsmayprecedetheneurologicsymptoms.Intact
sensorium,preservedsensation,andabsenceoffeveraretypical.Resultsof
cerebrospinalfluid,blood,andurinetestsand
imagingstudiesareusuallynormal.Thispatientpresentedwithnearlyallthese
features,andthusbotulismisthemostlikelydiagnosisinthiscase.Thenextstep
inestablishingthediagnosisistoperformelectromyographicstudies.
Dr.WilliamS.DavidsDiagnosis
Botulism.
DiagnosticStudies
Electromyography

Dr.David:Electromyographywasperformed1hourafterthepatientarrivedin
theintensivecareunit,whichwasapproximately24hoursaftersymptomonset.
Theelectromyographicfindingshelptorefinethedifferentialdiagnosis(Table2).
Thereductionintheamplitudeofevokedcompoundmuscleactionpotentials
(CMAPs)indicatesinvolvementoftheperipheralnervoussystem.Therelatively
normalresultsofsensorynerveconductionstudiessuggestthatageneralized
neuropathicprocessisunlikely,andthepreservedlatencies,velocities,andF
waveresponsesspecificallysuggestthattheGuillainBarrsyndromeandits
variantsareunlikely.Theremainingpossibilitiesincludeamotorneuronopathy,

myopathy,orneuromusculartransmissiondisorder.Amotorneuronopathy
wouldnotbeexpectedtoresultinattenuationoftheCMAPamplitudewithinthe
first24hoursaftersymptomonset;rather,aprocessaffectingtheelectrical
excitabilityofmuscle(primarymyopathy)oraneuromuscularjunction
blockadewouldbeexpected.Needleelectromyographyofthreemuscles
revealedlowamplitude,briefdurationmotorunitpotentials,asignofeithera
primarymyopathyorasevereneuromusculartransmissiondisorder.
Inpresynapticneuromusculartransmissiondisorderssuchasbotulism,thereis
animpairmentofacetylcholinereleaseacrosstheneuromuscularjunction.Two
factorsinfluenceacetylcholinereleaseintothesynapticspace:theamountof
transmittervesiclesavailableforreleaseandtheintracellularcalcium
concentration,whichaffectstheprobabilityofrelease.Inpatientswithbotulism,
theintracellularcalciumconcentrationinfluencestheelectrodiagnosticfindings.
Calciumfacilitatesthefusionofthesynapticvesiclestothepresynaptic
membrane,promotingreleaseofacetylcholinemoleculesintothesynapticspace.
Therefore,thehighertheintracellularcalciumconcentration,thegreaterthe
probabilityofacetylcholinerelease.Whenanerveactionpotentialinvadesa
presynapticterminal,voltagedependentcalciumchannelsopen,allowingcalcium
toenterthecell.Thecalciumpromotesvesiclereleaseforapproximately100to
200msec,andthenthecalciumisremovedbycellularmechanisms.Withevery
stimulus,calciumentersthecell.Atsuchrapidratesofrepetitivestimulationas50
Hz(20msecbetweenstimuli),calciumentryexceedscalciumuptake,allowing
calciumtoaccumulatewithinthecell;thissubsequentlyincreasestheprobability
ofreleasewitheachsuccessivestimulus.Inpatientswithbotulism,impaired
releaseofvesiclesleadstoareductionintheamplitudeofevokedCMAPs.
However,delivering10secondintervalsofstimulationatarateof50Hzwill
improvevesiclerelease;anincreaseintheCMAPamplitudeisobserved.In
addition,deliveringasinglestimulusafter10secondsofmaximalvoluntary
contraction(whichmimics50Hzoftetanicstimulation)increasestheCMAPam
plitude.Bothofthesephenomenawereobservedinthispatient(Fig.2)andare
definingfeaturesofpresynapticneuromusculartransmissiondisorders,suchas
botulismandtheLambertEatonmyasthenicsyndrome.Theclinicalpresentation
inthiscaseismoreconsistentwithbotulism.
AdditionalDiagnosticTestingandManagement

Dr.DavidC.Hooper:TheMassachusettsDepartmentofPublicHealth(MDPH)
andtheCentersforDis
easeControlandPrevention(CDC)werecontactedonthedayofadmission

becauseofastrongclinicalsuspicionofbotulism.Serum,stool,andgastric
aspiratesamplesweresenttotheMDPHandtotheBiodefenseLaboratoryat
WadsworthCenterinAlbany,NewYork,fordiagnostictesting.Equine
heptavalentbotulinumantitoxin(typesAthroughG)wassentfromtheCDCand
administeredtothepatientearlyonthemorningofthesecondhospitalday.
Diagnostictestresultsfirstbecameavailableonthefourthhospitalday.
Polymerasechainreaction(PCR)assaysofstoolandgastricaspiratesamples
werepositiveforClostridiumbaratiitypeF.AstoolculturewaspositiveforC.
baratii,andamousetoxinassayofthestoolwaspositiveforC.botulinumtypeF;
acultureandamousetoxinassayofthegastricaspiratewerenegative.APCR
assayoftheserumwasnegativeforC.baratiitypeF,andPCRassaysofall
sampleswerenegativeforC.botulinumtypesA,B,E,andF.
TheMDPHperformedanepidemiologicinvestigation.Noneofthepatients
familymembershadsymptomsofbotulism,andthepatienthadnohistoryof
consuminghomecannedfoodsorotherfoodsassociatedwithahighriskofbotu
lism.Oninvestigationofthepatientshomeonthesecondhospitalday,nohigh
riskfoodswerefound.PCRassaysoffourfooditemsthatthepatientmayhave
consumedintheprevious48hours,accordingtoherhistoryoffoodintake,were
negativeforC.baratiiandC.botulinum.Nineadditionalfooditemsfromthe
patientshomewerecollected9dayslaterandwerealsonegativeforthesetox
ins.Thispatientmostlikelyhadadultintestinaltoxemia,arareformofbotulism
thatiscausedbycolonizationofthegutbytoxinogenicclostridiumbacteria.In
adultintestinaltoxemia,C.baratiiismorefrequentlythepathogenthanC.
botulinum.6
Themainstaysofbotulismmanagementarerapidclinicalrecognitionand
notificationofpublichealthauthorities,whichenablesthepromptinvestigation
ofthetoxinsourceandpossibleothercasesandthepromptdeliveryand
administrationofantitoxin.Theeffectivenessofantitoxinisgreatestifitisgiven
earlyaftertheonsetofneurologicsymptoms,ideallywithin24hours,andthus
antitoxinshouldbeadministered,afterconsultationwithpublichealthauthorities,
onthebasisoftheclinicaldiagnosisandwithoutawaitingtheresultsof
diagnostictesting.Antibiotictherapyhasnotbeenshowntoimproveneurologic
recoveryandisnotrecommended.Thereisno
Thenewenglandjournalofmedicine

CaseRecordsoftheMassachusettsGeneralHospital

Figure2.ElectromyographicStudies.Electromyographywasperformedapproximately24hoursaftersymptomonset.

andrecordingswereobtainedfromtheabductordigitiminimi(PanelA).Stimulationfor10secondsatarateof50Hzin
actionpotentials(CMAPs).(Thesweepspeedwas2msecperdivision,andthegainwas0.5mVperdivision.)Motorne
performed,andrecordingswereobtainedfromtheextensordigitorumbrevis(PanelB).Thetopthreetracingsshowthe
head,andabovethefibularhead,whichhaveconsistentamplitudesandnormallatenciesandconductionvelocities.The
ankleafter10secondsofmaximalvoluntarycontraction;thereisanincreaseintheCMAPamplitude,fromabaselinele
speedwas5msecperdivision,andthegainwas5mVperdivision.)Takentogether,thesefindingsaredefiningfeatures
nengljmed372;4nejm.orgJanuary22,2015371

TheNewEnglandJournalofMedicineDownloadedfromnejm.orgonFebruary4,2015.For
personaluseonly.Nootheruseswithoutpermission.Copyright2015MassachusettsMedical
Society.Allrightsreserved.
CaseRecordsoftheMassachusettsGeneralHospital

persontopersontransmissionofbotulism,andinthehealthcaresetting,patients
canbetreatedwithstandardprecautions.
HospitalCourse
Dr.MinjeeKim(Neurology):Thispatientsinitialhospitalcoursewasnotablefor
severegastroparesis,mildhypotension,andtachycardia,featuresconsistentwith
autonomicdysfunction.Sinceaslowrecoverywasanticipated,earlytracheostomy
andpercutaneousgastrostomywereperformedonthethirdhospitalday.Thefirst
signofimprovementwasnotedonthefourthhospitalday,whenshewasableto
slightlyabductbotheyesandthengraduallywasabletosmile,openhereyes,and
movehertongue.Sherecoveredhercoughreflexontheeighthhospitalday,and
bythen,herextraocularmusclestrengthwasnearlynormalexceptforrestricted
downgaze.Heranisocoric,fixedpupilsalsostartedshowingminimalresponseto
light.
Whilethepatientwasintheneurologyintensivecareunit,shecontinuedtohave
weaknessin
herproximallimbsandneck.Herhospitalcoursewasfurthercomplicatedby
EnterobactercloacaepneumoniaandC.difficilecolitis.
Dr.MaryW.Montgomery(InfectiousDiseases):Thepatientwasdischargedon
theninthhospitaldaytoarehabilitationhospital.Shehadcontinuedrapid
improvement,wasquicklyweanedfromtheventilator,andeventuallyhadthe
tracheostomyandfeedingtubesremoved.After1monthintherehabilitation
hospital,shewasdischargedhomeingoodcondition.Onemonthlater,her
conditionwasnearlybacktonormalexceptformildfatiguewithheavyexertion,
andshehadalreadyreturnedtowork.Atthe6monthfollowup,shewascom
pletelybacktoherbaseline.
FinalDiagnosis
Botulism(adultintestinaltoxemiaduetoClostridiumbaratiiinfection).
ThiscasewaspresentedatNeurologyGrandRounds.
Nopotentialconflictofinterestrelevanttothisarticlewasreported.

DisclosureformsprovidedbytheauthorsareavailablewiththefulltextofthisarticleatNEJM.org.

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