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Ростовский Государственный Медицинский Университет
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Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_186_библиотеки_им_акад_М_И_Перельмана

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confirmthediagnosis.Therearenoreliablebloodtests.Multidrugtherapywithdapsoneandrifampinis firstline;clofazimineisaddedforlepromatousdisease.Treatmentiseffective,butitmaytakeseveral yearsforthecutaneouslesionstofullyresolve.
Poliomyelitis
Asofthiswriting,thereareonlytworemainingpolio-endemiccountries:AfghanistanandPakistan. VaccinationhaseliminatedthediseasefromtheUnitedStatesandotherdevelopedcountries,andthe numberofcasesinlessdevelopedregionsisvanishingaswell.
Poliovirus(anenterovirusspecies)ismostoftenasymptomatic.Itcanalsocauseamildfebrileillness andasepticmeningitis.Farlessoften,itcanattackthemotorneuronsofthebrainstemandspinalcord, resultinginpoliomyelitis.Acute,asymmetricflaccidparalysisisthehallmarkofthedisease,often precededbymeningealsignsincludingneckstiffness,headache,andfever.CSFexaminationiscriticalfor diagnosisandwillshowanasepticmeningitisprofilewithamoderatepleocytosis,aswellasapositive polioviruscultureorPCR.Treatmentissupportive.
Approximatelytwo-thirdsofpatientswithpoliomyelitisareleftwithresidualdeficits.Althoughyou arelikelytoneverseeapatientwiththeacuteillness,patientswhocontractedpoliopriortowidespread vaccinationmaypresenttoyouwithprogressivefatigueandmuscleweakness,aconditionknownaspost- poliosyndrome.Typically,symptomsdevelopatleast15yearsaftertheacuteinfection.Thepathogenesis isnotfullyunderstood.Anelectromyography(EMG)willconfirmlowermotorneuroninvolvement,and treatmentissupportive.
Non-polioenteroviruses(suchasechovirusesandcoxsackievirus)andarboviruses(includingWest Nile,asmentionedabove)canalsocauseacuteflaccidparalysis,mimickingpoliomyelitis.
Botulism
BotulismiscausedbythebacteriumClostridiumbotulinum.Descendingparalysisandcranial neuropathies,oftenprecededbygastrointestinalsymptoms,aretheresultofblockadeofthepresynaptic acetylcholinereceptors.ThisdiseaseisdiscussedfurtherinChapter12(seepage316).
BrainAbscess
Brainabscessesareinfectiousinnature,buttheypresentmostoftenlikemasslesions,withheadache, seizures,andfocalneurologicdeficits.Thelocation,size,andrateofgrowthoftheabscesswill determinetheprecisesymptomatology.Feverispresentinonlyabout50%ofcases.Thedifferential diagnosisthereforeincludesbothCNSinfectionsandothermasslesions,includinghematomasand tumors.
Abrainabscesscandevelopeitherviahematogenousspreadfromadistantsiteofinfectionorasa resultofextensionfromacontiguoussourceofinfection(e.g.,sinusitis,mastoiditis,oradentalinfection). Operativeproceduresandheadtraumacanalsoberesponsible.Bacteriaarebyfarthemostcommon etiologicagents—staphylococcalspecies,streptococcalspecies,andenterobacteriaceaetopthelist—but otherpathogens,suchasfungi,mycobacteria,andparasitescanberesponsibleaswell,particularlyin immunocompromisedpatients.Forexample,HIVinfectionisamajorriskfactorforbrainabscesscaused byToxoplasmagondiiandMycobacteriumtuberculosis.
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Allpatientswithsuspectedbrainabscessshouldundergoimaging.MRIwithgadoliniumisthe preferredtestandisparticularlygoodatdistinguishingbrainabscessfrommalignancy.Bloodcultures shouldalwaysbesent,andlumbarpunctureforCSFculturesshouldbeperformedifnotcontraindicated owingtotheriskofelevatedintracranialpressureandsubsequentherniation.
MRIpostcontrastT1(A)andT2(B)sequencesshowingacerebralring-enhancingabscesswith significantsurroundingvasogenicedema.Whitearrowspointtotheabscess.ReprintedfromFarrellTA. Radiology101,5thEdition.Philadelphia:WoltersKluwer,2019.
Treatmentincludesantimicrobialtherapy,andthesooneritisinstitutedthebettertheoutcome.Typical coursesrangefrom4to8weeksofIVantibiotics.Stereotacticaspirationisalsooftenindicatedfor diagnosisanddrainage.
Potentialcomplicationsofbrainabscessesincludeseizures,hydrocephalus(particularlywithlesions intheposteriorfossa),andruptureintotheventricularsystem.Mortalityhasimprovedgreatlyinthepast fewyears—itisnowabout15%—andthemajorityofpatientsmakeagoodrecovery.
Box8.7SpinalEpiduralAbscess
Spinalepiduralabscessisrareandtheresultofhematogenousspread,neurosurgical procedures,orspinalinjection.Mostpatientshaveunderlyingriskfactors,notablydiabetes andIVdruguse.Staphylococcusaureusisthepathogenidentifiedmostoften.Fever,focal backpain,andneurologicdysfunction,includingsensorimotordeficitsandbowelandbladder dysfunction,canoccur.TreatmentincludesIVantibioticsand,inamajorityofcases,
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neurosurgicaldrainage.Mostpatientsdowell,butasmallnumberofpatientsendupwith somedegreeofparalysis.Mortalityislessthan10%.
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PostcontrastT1-weightedMRIshowingalargeepiduralabscesswithenhancementofthesurrounding wallcausingseveredisplacementandcompressionofthespinalcord.(ModifiedfromPetersonJJ. Berquist’sMusculoskeletalImagingCompanion.3rded.WoltersKluwer;2017.)
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Follow-uponYourPatient:Amirpresentedwithseveralhoursofheadache,fever,chills,
andphotophobia,andyourexaminationconfirmednuchalrigidity.Yoususpecthemighthave meningitisand,givendelaysingettinghimtothehospital,starthimimmediatelyonIV antibiotics(ceftriaxoneandvancomycin)anddexamethasoneevenbeforealumbarpuncture canbeperformed.TheCSFultimatelyshowsanincreasedopeningpressure,ahigh leukocytecountwithaneutrophilicpredominance,anelevatedprotein,andalowglucose. GramstainispositiveforStreptococcuspneumoniae.Heimprovesrapidlywithantibiotics andrecoverswithoutanyneurologiccomplications.
Younowknow:
1. Howtorecognizeanddiagnosebacterialmeningitis,atruemedicalemergency.
2. Thespecificfeaturesofbacterialmeningitisassociatedwiththemostcommonpathogens.
3. How CSF analysis can help you distinguishbacterial meningitisfrommeningitis due toviruses as well as fromotherinfectious and noninfectiouscauses.
4. Whentosuspectchronicmeningitis,whichcanresultfrominfection,malignancy,andautoimmune/inflammatorydisorders.
5. Whentosuspectencephalitis,andspecificfeaturesofherpesencephalitisandarthropod-borneencephalitis.
6. Theclinicalmanifestationsandmanagementofneurosyphilis,Lymedisease,COVID-19,andotherinfectionswithimportantneurologic manifestations.
7. Thefeaturesandtreatmentofbrainabscesses.
1
Manifestationsofbacterialmeningitisininfantscanbeparticularlynonspecificandcanincludebothhyper-andhypothermia,poorfeeding,
seizures,andabulgingfontanelle;neckstiffnessisuncommon.
2
NegativeGramstainsarecommoninpatientswithbacterialmeningitiswhohavealreadybeenonantibiotictherapyandinthosewith
listeriosisorgram-negativebacterialinfection.
3
Likemeningitis,encephalitisdoeshavenon-infectiousetiologies,includingautoimmmuneencephalitis(seepage248),whichwillbediscussed
lateron.
4
RemembertogiveIVacyclovirwithIVfluidstopreventacyclovir-inducedrenalinjuryfromcrystalformation.
5
KeepinmindthattheCSFVDRLtestlackssensitivity:whereasareactivetestisconfirmatory(mostofthetime),anegativetestdoesnot excludethediagnosis.TheFTA-ABStestistheopposite:sensitivebutnotspecific.PCRtestingoftheCSFisalsoavailable,butitisnot nearlysensitiveenoughtobewidelyused.
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9MultipleSclerosis(andOtherImmunologic DiseasesoftheCentralNervousSystem)
Inthischapter,youwilllearn:
1. Howtodiagnosemultiplesclerosis
2. Howtosortthroughthecomplicateddifferentialdiagnosisofmultiplesclerosis
3. How to treat exacerbations of multiple sclerosis and reduce the risk and severity of recurrentattacks
4. Thedifferentialdiagnosisofopticneuritis
5. How to diagnose and manage several less common but nevertheless important immunologicCNSdisorders
CASE9
YourPatient:Emma,apreviouslyhealthy33-year-oldairlinepilot,comestoseeyou
becauseofdoublevisionthathaslastedabout36hoursandthatoccurswhenevershelooks
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toherleft.Shewentontheinternet,lookedupthediagnosticpossibilities,andisconcerned thatshemighthavemultiplesclerosis.Sherecallsthatayearagosheexperienced numbnessinherleftlegthatlastedseveraldaysandthengraduallyresolvedjustas mysteriouslyasitappeared.Whatisthenextstepinyourmanagement?
Internuclearophthalmoplegia(INO).YourexaminationofEmmarevealstheeyefindingsshownhere. Thisisanexampleofinternuclearophthalmoplegia(INO),causedbyalesioninthemediallongitudinal fasciculus(MLF).TheMLFisatractoffibersinthebrainstemthatyokestogetherthenucleiofthe thirdandsixthcranialnervestoallowforconjugatehorizontalgaze.Forinstance,tolooktotheleft,the leftnucleusofthesixthcranialnervefires,resultinginabductionofthelefteyeand,viatheMLF, simultaneousadductionoftherighteye.
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IftheMLFisdamaged,asinthecaseofEmma(andasillustratedinthediagramabove),therighteye cannotadductbeyondthemidlinewhenshetriestolooktotheleft.Thelefteye,whichcanabduct, wouldshowmarkednystagmus.Aquicknoteonnaming:Emma’sINOwouldbecalledaRIGHTINO. It’sconfusingbecausetheMLFitselfcrossesthemidline,butconventiondictatesthatthelesionis namedaftertheeyethatfailstomovecompletely(inthiscase,therighteye).
Theimmunesystemisanimpressivebutimperfectconstructthatsometimesattackshealthyhostcells andtissueswhenitreallyshouldbebusytakingcareofforeigninvaderssuchasvirusesandbacteriaor eliminatingcancerouscellsbeforetheygetoutofcontrol.Thenervoussystemisnotexemptfromthissort ofmisguidedautoimmuneattack.Oneoftheseautoimmunediseases,multiplesclerosis(MS),isquite common,withaprevalenceinsomeregionsofgreaterthan100per100,000people.
MultipleSclerosis
Notthatlongago,thediagnosisofMSwasjustlyfeared.Althoughsomepatientsdidwelland experiencedlittleornodisability,manyothersgotworse—usuallyinfitsandstarts,sometimes relentlessly—andwentontodevelopdiffuseandoftendevastatingneurologicdeficitsanddisability. Littlecouldbedonetoalteritsnaturalcourse.Thisisnolongerthecase.Manymedications,allimmune modulatorsofonekindoranother,arenowavailable,andthese,combinedwithlifestyleinterventions andsymptomatictreatments,havesignificantlyalteredtheprognosisforthebetter.
Thegreatestchallengeoftenliesinmakingthediagnosis.ThediseasecanstrikeanywhereintheCNS andcanthereforemanifestitselfinmyriadways.Manypatientscometoseetheirhealthcareprovider withminorneurologiccomplaints,farlessdramaticthanEmmaabove,anditcanbedifficulttofigureout whoneedsanevaluationforMSandwhodoesnot.Itisimportantnottodismisstheseseemingly inconsequentialcomplaintswithoutacarefulhistoryandphysicalexamination,becauseearlydiagnosis andtreatmentofMScanslowtheprogressionofthediseaseandlimitdisability.
https://t.me/med1917
ThepathophysiologyofMSiscomplexandnotcompletelyunderstoodbutinmostcasesappearsto involveinflammationdirectedagainstthemyelinsheathwithintheCNS,leadingtodemyelinationand, ultimately,axonaldegeneration.Theaxonatthetop(A)showshealthymyelination,whereastheonebelow (B)hasbeendamagedbyMS.
SomeBasicBackgroundFacts
MSisthreetimesascommoninfemalesasinmales Itpresentsmostoftenbetweentheagesof20and50years
Awordtothewise—thesefirsttwofactsshouldnotbemisconstruedtomeanthatMSonlyoccursinyoungfemales;itcanand doesoccurinmenandcananddoesoccurinchildrenandolderpatientsaswell,justlessoften.
RiskfactorsforMSincludebothgeneticandenvironmentalfactors
Hundreds ofgeneticvariantshave beenidentifiedthatareassociatedwith anincreasedriskofMS.However, noonegeneor constellationofseveralgenesis sufficienttoaccount forthedisease;theinteractionbetweengeneticpredispositionandvarious environmentalfactorsappearstobeessential. Environmentalrisk factorsincludeobesity,smoking,priorinfectionwith EpsteinBarrvirus,andgeographicallocation.MShasa uniquegeographicdistribution,becoming morecommonas onemovesfartherfrom theequator(i.e.,athighlatitudes). Noone
https://t.me/med1917
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