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Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_186_библиотеки_им_акад_М_И_Перельмана

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Sjogrensyndrome)  CNS autoimmune diseases (multiple sclerosis, neuromyelitis optica, acute disseminated encephalomyelitis) Paraneoplastic (mostoftenassociated with antiHu andantiCRMP5 antibodies;more onthese in Chapter16)  Spontaneous (idiopathic; the cause of as many as 30% of cases remains unknown despite a comprehensiveevaluation)
DiagnosisrequiresanMRI,whichwillshowadiscretecontrastenhancinglesioninthecordspanning1 ormorevertebralsegments(ifthelesionspans3ormoresegments,itistermedlongitudinallyextensive transversemyelitis,orLETM2).TheCSFshould,inmostcases,beinflammatory,withpleocytosisand elevatedprotein.Aseriesofassaysforviralnucleicacid,specificantibodies,oligoclonalbands,and immunoglobulinsynthesisratesareperformedonboththeCSFandserumtofurtheridentifytheetiology.
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https://t.me/med1917
(A)Transversemyelitisinapatientwithmultiplesclerosisand(B)withneuromyelitisoptica.Arrowsare pointingatthehyperintensecordlesionsconsistentwithtransversemyelitis.(ReprintedfromLouisED, MayerSA,NobleJM.Merritt’sNeurology.14thed.WoltersKluwer;2021.)
Treatmentdependsontheunderlyingetiology,butasyoucanimagine,thisisnotalwaysimmediately apparentandtheworkupcantakeseveraldays.Whenthecauseisnotknownupfront,firstlinetreatment consistsofhighdoseIVcorticosteroids,whichshouldbegivenempiricallyandrapidlytoprevent progressivesymptoms.Plasmapheresis(PLEX)aswellasimmunosuppressiveagentssuchas cyclosporine,mycophenolate,andrituximabarealsousedforrefractorycases.
Toxic/MetabolicSpinalCordDisorders
VitaminB12deficiencycanresultindegenerationofthedorsolateralwhitematterspinalcolumns,a disorderknownassubacutecombineddegeneration.Thisconditionpresentswithagradually progressive,uppermotorneurontypeweakness(duetodegenerationofthecorticospinaltractsthatrunin thelateralwhitematter),paresthesias,andpotentiallydisablingsensoryataxia(duetoinvolvementofthe sensoryfibersthatruninthedorsalcolumn/mediallemniscustract).Anelevatedserummethylmalonic acidisamorereliablediagnostictestthanadecreasedserumB12.MRIcanshowhighT2signalinthe dorsolateralcolumnsofthespinalcord.AggressivetreatmentwithB12canstopprogressionofthe disease.Seepage281forareviewontheothersequelaeofB12deficiency.
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T2hyperintensities(whitearrows)involvingthedorsolateralspinalcolumnsonaxialMRI,consistentwith subacutecombineddegeneration.(ModifiedfromKumarA,SinghAK.Teachingneuroimage:invertedV signinsubacutecombineddegenerationofspinalcord.Neurology.2009;72(1):e4.)
Copperdeficiency,whichresultsmostcommonlyfrommalabsorptioninthesettingofgastrointestinal surgery(suchasgastricbypass)orzinctoxicity(zincinhibitscopperabsorption),canmimicsubacute combineddegeneration.Associatedanemia(whichcanbemicro,macro,ornormocytic,asopposedtothe megaloblasticmacrocyticanemiacausedbyB12deficiency)andleukopeniaarecommon.Treatmentis withcoppersupplementation.
Nitrousoxideisacommondrugofabuse,inlargepartbecauseitcanbeobtainedsoeasily.Itis,for example,usedtofillballoonsandasapropellantincannistersofwhippedcream.Wheninhaled,it createsasenseofeuphoria(itsuseforentertainmentgaverisetosocalledlaughingparties).However,it canalsocauseirreversibleinactivationofvitaminB12.Thismostoftenoccursafterchronicexposureto nitrousoxide,butitcanalsooccurquickly,evenafterasingleexposure,inpatientswithbaselinelow levelsofB12.TheresultingdorsolateralspinalcorddysfunctionisidenticaltothatcausedbyB12 deficiencyfromothercauses.Treatmentrequiresimmediatecessationofnitrousoxideexposureandhigh-
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doseB12supplementation.
LumbarSpinalStenosis
Lumbarspinalstenosis,ornarrowingofthebonylumbarspinalcanal,isnotprimarilyaspinalcord problem,butitisanincrediblycommoncauseofdisabilityintheelderlypopulationandonethatyouwill likelyencounterregardlessofwhatfieldofmedicineyouchoose.Itismostoftencausedbydegenerative disease(alsocalledspondylosis)affectingthevertebrae.Althoughassociateddischerniationand osteophyteformationcancompressthecord(iftheT12/L1vertebraeareaffected;remember,thecord endsatapproximatelyL1)orthecaudaequina,mostoftenitimpingesontheneuralforaminawherethe spinalrootsexit.
Radicularbackandlegpain3thatworsenswithstandingandwalkingandimproveswithsittingand leaningforward,knownasneurogenicclaudication,ischaracteristic.Itisthoughtthatwalkingand standingincreasethemetabolicdemandofnervesandnarrowthespacewithinthelumbarcanal,resulting inworseningischemiaandcompressionofalreadyinjurednerves.Bilateralbutoftenasymmetricsensory lossandweaknessofthelowerextremitiesarealsocommon.
Diagnosisrequiresimagingdemonstratingnarrowingofthelumbarspinalcanal(MRIisthegold standard)aswellascharacteristicclinicalsymptoms.Physicaltherapyandnonsteroidalantiinflammatory drugsarefirstlinetherapies;currentevidencedoesnotsupporttheutilityofepiduralsteroidinjections. Surgeryistypicallyreservedforthosewhodonotrespondtomedicalmanagementandmustbecarefully consideredonacasebycasebasis,asthereislimitedevidencedemonstratingclearbenefit.
Box10.5CervicalStenosis
Cervicalstenosis,likelumbarstenosis,ismostoftentheresultofdegenerativevertebral disease.Ittendstopresentgradually,withgaitdysfunctionand/orhandclumsiness;frank weaknessislesscommon.Diagnosisandmanagementaremuchthesameaswithlumbar stenosis.Degenerativethoracicdiseaseissignificantlylesscommonthaneithercervicalor lumbardisease.
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Yourpatient’sfollowup:Youareconcernedaboutspinalcordinjuryandimmediatelyorder
anMRIofthecervicalandthoracicspine.Youdon’tknowwhatexactlyisgoingon,butgiven Priya’sbilaterallowerextremitysymptoms,sensorylevel,andhyperreflexia,youhavebeen abletoquicklylocalizewhateveritistothespinalcord.Luckily,theimagingisdonequickly andshowsachunkyT2hyperintenselesionwithinthecordspanningseveralthoracic vertebrallevels.Sheisstartedonempirichighdosesteroidsandadmitted.Afewdayslater, heraquaporin4antibodycomesbackpositive,confirmingthediagnosisofneuromyelitis optica.SheundergoesonecourseofPLEXfollowinghersteroidtreatment,withsignificant improvementinhersymptoms,andisdischargedsoonafterwithclosefollowup.
Younowknow:
Whentosuspectacutecordcompression,andwhattodoaboutit. Howtodifferentiateanddiagnoseconusmedullarissyndromeandcaudaequinasyndrome. Howtorecognize,diagnose,andtreatlumbarspinalstenosis. The many potential etiologies of transverse myelitis, including infectious, systemic autoimmune, CNSautoimmune,andparaneoplasticcauses. The presentation and treatment of vitamin B12 deficiency and other toxic/metabolic causes of subacutecombineddegeneration.
1
Weneedtomakeanimportantdistinctionherebetweenfacialweakness,whichcannotbecausedbyaspinalcordlesion,andnumbness, whichcanbe.BecausethenucleiofCN5(whichprovidesensationtotheface)arehuge,spanningmostofthebrainstemintothecervical cord,cervicalspinelesionscancausefacialnumbness.Thus,althoughfaceweaknesscannotbeattributedtoaspinalcordlesion,face numbnesscan,albeitrarely.
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2
IfyourememberfromChapter9,cordlesionsfrommultiplesclerosistendtobesmall,or“shortsegment,”whereascordlesionsassociated withneuromyelitisopticatypicallyfitintothe“LETM”classification.
3
Radiculopathyreferstosymptomsattributedtonerverootcompression.Unlikelesionsconfinedtothespinalcord,whichdon’ttendtobe painful,nerverootcompressionhurts.Thepainistypicallydescribedassharpwithanalmostelectricquality,shootingdownwhichevernerve isaffected(formoreinformation,takealookatBox11.8intheupcomingchapteronPeripheralNeuropathies).
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ThePeripheralNeuropathiesand AmyotrophicLateralSclerosis
Inthischapter,youwilllearn:
1. That amyotrophic lateral sclerosis (ALS) presents with weakness characterized by both upperandlowermotorneuronfindings
2. That lengthdependent sensorimotor neuropathy is extremely common and has multiple causes,butitcanoftenbeeasilydiagnosedwithabasicneurologicexaminationandsome simplelaboratorytests
3. WhentosuspectGuillainBarresyndrome,andtheimportanceofexpeditingevaluationand treatment
4. How centering your diagnosis of peripheral neuropathy around a few key points—acute versus chronic, motor versus sensory, symmetric versus asymmetric—will allow you to figureoutwhodoesanddoesnotneedtobeevaluatedandtreatedquickly
5. Thatmononeuropathies,suchascarpaltunnelsyndrome,oftenresultfromcompressionor injury,andhowtheyshouldbemanaged
6. Allthedifferentwaysthatdiabetescanwreakhavocontheperipheralnervoussystem
CASE11
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YourPatient:Allen,a50yearoldlawyer,presentswithnumbnessandtinglinginhisfeetfor
thepastyear.Hehasnoothersymptoms,andhispastmedicalhistoryisbenign.Heisonno medications.Heismildlyoverweight.Yourneurologicexaminationconfirmsdecreased sensationtovibrationinthelowerextremitiesaswellasdiminishedanklereflexes.Heis concernedthathemighthavediabetes—bothhisparentshavetype2diabetes—buthis fastingglucoseis96mg/dLandhishemoglobinA1cis5.6%,bothnormalvalues.Whatare yournextstepsinhisevaluation?
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Itseemsasifthecentralnervoussystemgetsalltheattention.Itis,afterall,theessenceofwhoweare. Butdon’tbetooquicktowriteofftheperipheralnervoussystem(PNS).Withoutit,theCNSwouldbe prettyhelpless.Wewouldn’tbeabletosensetheworldaroundus,feedanddressourselves,orpound awayatacomputerkeyboardtowritethesesentences.
Thischapterisdevotedtotheperipheralneuropathies,andtherearealotofthem.However,youwill discoverthattheirevaluationisstraightforward.Whenconfrontedwithapatientinwhomyoususpecta peripheralneuropathy,alwaysaskyourselfifthesymptomsare:
Acuteorchronic? Symmetricorasymmetric? Predominantlysensoryormotor?Andfinally, Localizabletooneormultiplenerves?
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