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Ростовский Государственный Медицинский Университет

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Книга_186_библиотеки_им_акад_М_И_Перельмана

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AsignificantnumberofpatientsadmittedtotheICUwilldevelopmyopathy.

DefinitivediagnosiscanbemadebyEMG,butoftentheclinicalsetting,routineneurologic examination,andstandardlaboratorytestingaresufficienttoruleoutothercorrectablecausesand establishCIM/CIPasthelikelyculpritintheseveryillpatients.

Thereisnospecifictreatment.Thebestapproachistotrytopreventthedevelopmentofthese symptomswithearlymobilization,physicaltherapy,andadequatenutrition,buttheevidencesupporting thesemodalitiesisnotasrobustaswewouldlike.

Patientsusuallyrecoverwithinweekstomonthsofdischarge,butabouthalfwillhavesomedegreeof

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persistentweakness.

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Follow-uponYourPatient:Carol’sbasicneurologicexaminationisnormal,butbecause

yoususpectaneuromuscularjunctiondisorderbasedonthesymptomsshehasreportedto you,youdoathoroughevaluationandnotedifficultywithsustainedupgazeandfatigable shoulderweaknesswithrepeatedstrengthtesting.ShetestspositiveforAChRantibodies. Carolhasmyastheniagravis.Treatmentwithanacetylcholinesteraseinhibitorand immunosuppressivetherapyrelievesmuchofhersymptomatology.

Younowknow:

1. Howtosortoutneurogenic,neuromuscular,andmyopathiccausesofweakness

2. Thediagnosisandmanagementofthemostcommondisorderoftheneuromuscularjunction,myastheniagravis

3. Howtoevaluatepatientswithprimarymyopathies,anddiscriminatebetweeninflammatoryandnoninflammatorycauses

4. Themanycausesofnoninflammatorymyopathy,amongthemdrug-inducedmyopathies,endocrinemyopathies,andinheritedmyopathies

5. Howtorecognizeandevaluatepatientswithrhabdomyolysis

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13ParkinsonDiseaseandOtherMovement Disorders

Inthischapter,youwilllearn:

1. HowtorecognizeandtreatParkinsondisease

2. Howtodiagnosethedifferenttypesoftremor

3. HowtodistinguishParkinsondiseasefromotherhypokineticmovementdisorders;inother words,fromdiseasesassociatedwithpartialorcompletelossofmovement

4. How to recognize and treat the common hyperkinetic movement disorders, that is, diseasesassociatedwithinvoluntarymovementssuchasmyoclonusandchorea

CASE13

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YourPatient:Suzanne,a71-year-oldfamilypracticenurse,comestoyourofficeandasks

youtohelpherwithatremorinherrighthandthathasgraduallybecomequitebothersome. Oneglanceatherslow,shufflinggaittellsyousomethingiswrong.Whatarethenextsteps inherevaluationandmanagement?

ParkinsonDisease

AfterAlzheimerdisease,Parkinsondisease(PD)isthemostcommonneurodegenerativedisorder, affectingmorethan6millionpeopleworldwide,anditsprevalenceisincreasingrapidly.Whateverfield ofmedicineyouchoosetopursue,youwillhavepatientswithPD.Althoughcurrenttreatmentdoesnot alterthenaturalhistoryofPD,byrecognizingthediseaseandinstitutingtreatmentearlyonyoucanhelp patientsavoidunnecessarytestingandgreatlyimprovetheirqualityoflife.

Box13.1

WhyistheprevalenceofParkinsondiseaserising?Partlythisisduetotheagingpopulation andimproveddiagnosis,particularlyofpatientsintheearlystagesofthedisease.However, thereisalsoconcernthatenvironmentalexposures,suchaspesticides,herbicides,heavy metals,andvariousindustrialsolvents,maybeplayingaroleaswell.

Onequickpearl:YouprobablythinkofPDasadiseaseoftheelderly,andagecertainlyisamajorrisk factor.Itaffectsonlyabout40/100,000peoplebetweentheagesof40and50years,whereasitaffects over1000/100,000peoplebetweentheagesof70and79yearsandover2000/100,000overtheageof 80years.However,becauseitcanoccurinyoungadults,don’tbetooquicktodismissseeminglybenign neurologiccomplaints,suchastremor(seebelow),inyoungerpatientswithoutfirstperformingacareful evaluation.

RiskfactorsbesidesageincludeafamilyhistoryofPDandtheenvironmentalfactorsmentionedinBox

13.1above.FamilialformsofPDarerare,andmostcasesappeartobesporadic.Thejuryisstillouton whetherrepetitiveheadtraumaisariskfactorforPD.Depressionhasbeencitedasapossibleriskfactor, butthisassociationmayonlyrepresenttheoverlapoftwocommondisorders.

Etiology

TheunderlyingcauseofPDisnotknown.Thepathologyinvolvesthelossofprimarilydopaminergic neuronswithinthesubstantianigra(apartofthebasalganglialocatedinthemidbrain),aswellasthe destructionofneurons,bothdopaminergicandotherwise,inotherareasofthebrain.Lewybodies,which areeosinophiliccytoplasmicinclusionbodiescontainingtheproteinalphasynuclein,canbefoundinthe affectedregionsofthebrain.Theroleofalphasynucleininthehealthybrainisnotwellunderstood,butit isbelievedthatitcanbetoxictonervecellswhenpresentinaberrantconformations.

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(A)TheprimarylocusofpathologyinPDisthesubstantianigra,apartofthebasalganglialocatedinthe midbrain.(B)NeuropathologyfromapatientwithPDshowingaLewybody(blackarrow)withinthe substantianigra.Lewybodiesareoftensurroundedbyathin,clearhalo.(B,reprintedfromRubinE, ReisnerH.EssentialsofRubin’sPathology.6thed.WoltersKluwer;2013.)

ClinicalPresentation

PDcausesfourclassicphysicalsignsthataretheresultofinvolvementoftheextrapyramidalmotor system,thepartofthemotorsysteminvolvedinmodulationandregulationofmovement:

Tremor Rigidity Bradykinesia Posturalinstability

Ifyoulikemnemonics,tryTRAP(yes,werecognizethatthereisnoAinthelistabove,butneverdoubt theingenuityofneurologistswho,inthiscase,substituteakinesiafortheoftenmoreaccurate bradykinesia).

Box13.2TheExtrapyramidalMotorSystem

Theterm“extrapyramidal”distinguishesthispartofthemotorsystemfromthepyramidal system.Thetractsofthepyramidalsystem(thecorticospinalandcorticobulbartracts)begin inthemotorcortexanddescendtotheirtargetsthroughthemedullarypyramids(hencethe name).Theextrapyramidalsystemiseverythingelsethatimpactsmovement,andincludes neuronswithinthebasalgangliaandcerebellum.Ingeneral,thepyramidalsystemcauses voluntarymovement,whereastheextrapyramidalsystemcausesinvoluntarymovement, indirectlyregulatingandmodulatingtheactivityofthepyramidalsystem.Seepage18fora morecomprehensivereviewofmotorsystemanatomy.

ThetremorofPDisclassicallydescribedasa“pillrolling”tremor.Itisprimarilyarestingtremor, mosteasilyseenintheupperextremities,butitcanalsobepostural(i.e.,mostevidentwhenthearmsare outstretched).TremoristhepresentingsymptominthemajorityofpatientswithPD.Ittendstobegin unilaterallyandthenspreadscontralaterallyoveracourseofmonthstoyears.

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TherestingtremorofPDismostoftenevidentasapillrollingtremorinthehand,wherethefingersand thumbappeartoberollingapillbetweenthem.

Rigidityreferstoresistancetopassivemovementofanextremity.InpatientswithPD,rigidityisoften, butnotalways,feltonexaminationascogwheeling,ajerkystop-and-start(ratherthanasmooth) resistancetomotion.Contralateralactivationmaneuvers(suchasinstructingthepatienttorapidlyopen andclosetheirunaffectedhand)canhelpbringoutcogwheelrigidityonexamination,especiallyinmild cases.

Box13.3Hypertonia

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