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

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☆
DefiningMS
ClinicalSignsandSymptoms
HowtoMaketheDiagnosis
DifferentialDiagnosis
ClinicalCourse
Treatment
Prognosis
MSandPregnancy AutoimmuneEncephalitis:OneOtherImmunologicCNSDiseaseYouShouldKnow
Presentation
Diagnosis
Treatment
CASE9:FOLLOW-UP
Chapter10
TheSpinalCord
CASE10
BasicAnatomy AcuteSpinalCordCompression
Causes
ClinicalPresentation
Examination
TriageandEvaluation SpinalCordSyndromes
Brown-SequardSyndrome
AnteriorandCentralCordSyndromes
ConusMedullarisandCaudaEquinaSyndromes TransverseMyelitis Toxic/MetabolicSpinalCordDisorders
LumbarSpinalStenosis
CASE10:FOLLOW-UP
Chapter11
ThePeripheralNeuropathiesandAmyotrophicLateralSclerosis
CASE11
AmyotrophicLateralSclerosis(ALS) PeripheralNeuropathies:AnOverview Polyneuropathies
Length-DependentSensorimotorPolyneuropathies
InflammatoryDemyelinatingPolyneuropathies(Guillain-BarreSyndrome)
SmallFiberNeuropathy MononeuritisMultiplex
Plexopathies
BrachialPlexopathies
LumbosacralPlexopathies Mononeuropathies
CarpalTunnelSyndrome
UlnarNeuropathy
PeronealandTibialNeuropathies
BellPalsy
MeralgiaParesthetica
CASE11:FOLLOW-UP
Chapter12
DiseasesoftheMusclesandtheNeuromuscularJunction
CASE12
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FiguringOuttheCauseofWeakness
CausesofWeakness—AnAnatomicApproach
CausesofMyopathy DiseasesoftheNeuromuscularJunction
MyastheniaGravis
OtherDiseasesoftheNeuromuscularJunction Myopathies
TheInflammatoryMyopathies
NoninflammatoryMyopathies
CASE12:FOLLOW-UP
Chapter13
ParkinsonDiseaseandOtherMovementDisorders
CASE13
ParkinsonDisease
Etiology
ClinicalPresentation
Diagnosis
Treatment
Prognosis DifferentialDiagnosis:DisordersThatCanMimicParkinsonDisease
Tremor
TheAtypicalParkinsonianSyndromes
SecondaryParkinsonism OtherMovementDisorders
ChoreiformDisordersandHuntingtonDisease
TardiveDyskinesia
Dystonia
Myoclonus
TicDisordersandTouretteSyndrome
Sleep-RelatedMovementDisorders
CASE13:FOLLOW-UP
Chapter14
NeurocriticalCare
CASE14
CerebralEdema ElevatedIntracranialPressure
Physiology
CausesandPresentationofElevatedICP
ManagementofElevatedICP HerniationSyndromes
BrainDeath
CASE14:FOLLOW-UP
Chapter15
AlteredMentalStatus
CASE15
EncephalopathyVersusAphasia NeurologicCausesofAMS Non-NeurologicCausesofAMS AMS:EvaluationinaNutshell
CASE15:FOLLOW-UP
Chapter16
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Neuro-Oncology
CASE16
AQuickWordonMassLesions PrimaryBrainTumors
GlialTumors
NeuronalTumors
OtherPrimaryBrainTumors Cysts ParaneoplasticSyndromes
CASE16:FOLLOW-UP
Chapter17
GeneticDiseasesandSyndromes
CASE17
LysosomalStorageDiseases GlycogenStorageDiseases(GSDs) NeurocutaneousDiseases MitochondrialDiseases
CASE17:FOLLOW-UP
Chapter18
TheCranialNerves
CASE18
CranialNerveBasics
BrainstemReflexes CommonCranialNervePathology
VisualFieldDefects
OculomotorPalsies(Third,FourthandSixthNervePalsies)
Facial(Seventh)NervePalsy
MultipleCranialNervePalsies
CASE18:FOLLOW-UP
Chapter19
IsThatAllThereIs?
Index
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1Let’sGetStarted
Inthischapter,youwilllearn:
1. The basicanatomyof the nervoussystem in a streamlinedand straightforward way that youcanapplydirectlytoclinicalpractice
2. Howtotakeausefulneurologichistory
3. The essentials of theneurologic examination, and what each test can tell you about the localization(i.e.,theanatomy)ofthepatient’sproblem
4. Thetoolstohelpyouinthediagnosisandmanagementofneurologicissues;theseinclude imaging (computed tomography [CT], magnetic resonanceimaging [MRI]andsomeother tests youmaynot be as familiar with), lumbar puncture, electroencephalography (EEG), nerveconductiontests(NCS),andelectromyography(EMG)
CASE1
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YourPatient:Hailey,a30-year-oldwomanwithnopastmedicalhistory,comestothe
emergencydepartmentcomplainingofaworseningheadache.Shehasalonghistoryof headachesbuttheytypicallyresolvewithibuprofenandagoodnight’ssleep.Thisheadache, however,haspersistedformorethan7daysandnothingseemstomakeitbetter.Hervitals arestableandherneurologicexaminationisnormal.Sheisgivenmetoclopramideand ketorolacandwithinanhourfeelssignificantlybetter.Yourattendingasksyouifyouthink she’sreadyfordischarge.What’syouranswer—isHaileyreadytoreturnhome?
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Introduction
Bythetimeyouhavefinishedthischapter,youwillbeexhausted(therearenotwowaysaboutit;thereis alotofmaterialtolearn)butreadytotakeontheworldofneurologicdisease!
Inabroadsense,alldiseaseisexperiencedthroughthenervoussystem.Physicalpain,emotionalpain —really,anykindofsensation,goodorbad—issensed,transmitted,andperceivedbytheneurologic system.Ourthoughts,feelings,andmemoriesareneurologicaswell,andwhentheygoawryyoucan pointyourfingeratthebrainastheresponsibleparty.
It’sallinyourhead,whetherit’smentalconfusionoraskinnedknee.
However,whatwemeanwhenwespeakofneurologicdisease,andwhatyouexpectedwhenyou pickedupthisbook,isanydisorderwheretheprimarypathologyresidesinthebrain,spinalcord,or peripheralnerves(theneuromuscularjunctionandmusclesareincludedinthedomainofneurologyas well,andwewon’toverlookthem).Andthereareplentyoftheseconditionstokeepusbusyforthenext severalhundredpages!
Neuroanatomy:TheBasics
Beforewegoanyfurther,let’swalkthroughthebasicstructureofthenervoussystem.Thenervoussystem
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canbeconceivedofasbeingdividedintothreeparts:thecentral,peripheral,andautonomicnervous systems.
1. Thecentralnervoussystem(CNS)includesthebrain,brainstem,andspinalcord.Allareprotectedbyboneandthreethinmembranes calledthemeninges:thedura,arachnoid,andpia.Mostofthebrainisalsoprotectedbytheblood-brainbarrier,atermthatrefers toa special propertyofthecerebralbloodvesselsthatlimitsthepassageofmanypotentiallyharmful substances,suchascirculating hormones,toxins,andpathogens,fromthebloodintotheextracellularfluidofthebrain.
2. The peripheral nervous system(PNS) includesall theneurons andganglia (i.e.,groupsof nerve cellbodies) thatlie outside of the brainandspinalcord.Thecranialnerves,withtheexceptionoftheolfactory(CN1)andopticnerve(CN2),arepartofthePNS.Unlike theCNS,thePNSisnotprotectedbybone,meninges,ortheblood-brainbarrierandisthereforemorevulnerabletoinjury.ThePNSis dividedintosomaticandautonomicdivisions.
a.
The somatic division consists of sensory and motor neurons. Specifically, we speak of sensory afferents (afferent means conducting inward), which receive and pass on sensory information from the outside world, and motor efferents (efferent meaningconductingoutward), whichenable voluntarymovement.These neuronsarealsoresponsiblefordeeptendon reflexes (seepage26).
b.
Theautonomicdivision ispart of the overall autonomic nervous system (see below) andincludes both the sympathetic and parasympatheticnerves. The sympathetic nerves use the neurotransmitters acetylcholine andnorepinephrineto communicate, whereas the parasympathetic nerves use onlyacetylcholine. These twosystems typicallyfunctionin oppositiontoeachother, triggering an event or response that is countered by the other system. The sympathetic nerves mediate the ‘fight or flight’ response(forexample,pupillarydilationtoallowmorelighttoentertheeye;bronchodilationtopromote oxygenexchange;and vasoconstriction to divert blood flow away from the GI tract and skin and redirect it to the skeletal muscles). The parasympathetic nerves mediate the ‘rest and digest’ response (for example, pupillary constriction; bronchoconstriction; and vasodilationtotheGItractandskin).
3. Theautonomicnervoussystemregulates visceralfunctionssuchas digestion, temperature,heart rate,andbloodpressure. It ismade up ofcomponents ofboth thePNS (the autonomic division,as above)andCNS(includingstructureswe haven’tdiscussedyet—the insularcortex, brainstem,andhypothalamus, amongothers).Theautonomic nervous systemalsocontains the entericnervous system, whichintegrates informationabout thegastrointestinal(GI)tractandprovides output tocontrolandcoordinateGItract functionsand localbloodflow.
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Themeninges.Theduraistheoutermostandtoughestlayerthatliesdirectlybeneaththeskull.The arachnoid,athinner,spiderymembrane,isseparatedfromthedurabyapotentialspace;inotherwords, undernormalconditions,theduraandarachnoidaretouchingoneanother,butunderpathologicconditions (asubduralbleed,forinstance),theycanbecomeseparatedasthespacefillswithfluid.Thepiaisthe innermostandmostdelicatelayer;itclingstothegyri(thefolds)ofthebrain.Cerebrospinalfluid(CSF) flowsbetweenthearachnoidandpiainthesubarachnoidspace.Together,thearachnoidandpiaare knownastheleptomeninges.
Box1.1
Thebrain—evenyours(evenours!)—isincontrovertiblyamazing.Thehumanbrainweighs about3poundsandhastheconsistencyandappearanceoftofu.Estimatesvary,butthe averagebrainhasaround100billionneuronswithtrillionsofconnectionsamongthem.Our wholelifeislivedinsideourbrain.Everythingweperceiveabouttheworldaroundusis nothingbutapanoplyofelectricalsignalswhizzingfromneurontoneuron.Thesky,afterall, isn’treally“blue”inthewaywemightbetemptedtothinkofblue—photonsaren’tintrinsically blue—rather,theyhaveawavelengththatappearsbluetousbecauseofthewaythebrain interpretstheelectricalsignalsgeneratedbyourlookingatthesky.Nordosoundwaves intrinsicallysoundlikeBeyoncéorBeethoven.Andwhatisathoughtorafeelingora memoryotherthanawayofintegratingandinterpretingelectrochemicalcurrentsracing throughourcerebralcortex?It’sprettyincrediblewhenyoustoptothinkaboutit.
Thereisalotofcomplexanatomythatgoesintoeachofthesesystems.Butratherthanburdenyouwith
allofitatonce,wewillgiveyouthebasicsnowandfillinmanyofthedetailslaterasweneedthem.
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Thenervoussystem.
BrainAnatomy101Thebrainconsistsofthecerebrum,thebrainstem,andthecerebellum.The cerebrumisdividedintotwohemispheres(leftandright),whichcommunicateelectricallyacrossaband ofnervefiberscalledthecorpuscallosum.Therearefourlobesoneachside:thefrontal,parietal, temporal,andoccipitallobes.Theoutermostlayerofthecerebrum,thecerebralcortex,containsgray matter—thecellbodiesoftheneurons—andtheinteriorconsistsofwhitematter—thenervefibers,or axons.Thestructuresthatliedeepwithinthebrain(includingthethalamusandthebasalganglia)arealso graymatter.Thebrainstemconsistsofthemidbrain,pons,andmedulla.Thecerebellumliesposteriorto thecerebrumandbrainstem.
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