Amyotrophic-Lateral-Sclerosis-(ALS)

AmyotrophicLateralSclerosis(ALS)Winter2009AlsoknowasLouGehrig'sDisease•NamedaftertheNewYorkYankeesbaseballstarwhoplayedfirstbaseandwasdiagnosedwiththediseasein1939anddiedin1941butduetopopularbeliefthisdiseasehadbeendiscoveredalmost100yearsearlierHistory•Wasdiscoveredin1850byEnglishscientistAugustusWallerwhodidn’tknowhehaddiscoveredALSbutsawnervefibersthathadextremeatrophy•But19yearslateraFrenchneurologistJean-MartinCharcottookcreditforitinpublishingthediseaseinascientificjournalObjectives•Whatisthisdisease?•SignsandSymptoms•ProbablecausesofALS•FormsofALS•DiagnosisofALS•MedicationsyouwillseeforALSpatients•Prognosis(outcome)•StatsforALSWhatisthisdisease?•aprogressiveneurodegenerativedisease“isaconditioninwhichcellsofthebrainandspinalcordarelost.”•Thisaffectsmostlymotorneurons”thecellsthatcontrolneededvoluntarymuscleactivitysuchasspeaking,skeletalmusclemovement,breathing,andswallowing.”WhatisALScont…•Whenthemotorneuronseventuallydie,theabilityofthebraintocontrolmusclemovementislost.Causingparalysisofessentialbodysystems.•Whenmusclesnolongerreceivethemessagesfromthemotorneuronsthattheyrequiretofunction,themusclesbegintoatrophy(becomesmaller).SignsandSymptoms•Earlysymptoms•Increasingmuscleweakness“especiallyinthearmsandlegs”•Difficultyspeaking•Troubleswallowing•Problemswithbreathing•Twitching•Crampingofmuscles(mostlyhandsandfeet)Signsandsymptomscont…•LateSymptoms•Extrememuscleatrophy•Reflexesthatareslowtonon-existent•Excessivedrooling•Babinski'ssign:“thebigtoedorsiflexesandtheothertoesfanout“•Increasedspasticity(musclerigidity)•Weightloss•Choking•CardiacarrestduetorespiratoryarrestusuallyresultingindeathProbableCausesofALS•Freeradicals.TheinheritedformofALSofteninvolvesamutationinageneresponsibleforproducingastrongantioxidantenzymethatprotectsyourcellsfromdamagecausedbyfreeradicals—thebyproductsofoxygenmetabolism.•Glutamate.PeoplewhohaveALStypicallyhavehigherthannormallevelsofglutamate,achemicalmessengerinthebrain,intheirspinalfluid.Toomuchglutamateisknowntobetoxictosomenervecells.ProbableCausesofALScont…•Autoimmuneresponses.Sometimes,aperson'simmunesystembeginsattackingsomeofhisorherbody'sownnormalcells,andscientistshavespeculatedthatsuchantibodiesmaytriggertheprocessthatresultsinALS.•ThusALSisnotcontagious.FormsofALS•Sporadic-themostcommonformintheUnitedStates-90to95%ofallcases.•Familial-genetic“accountsforasmallamountofcasesintheUnitedStates”-5to10%ofallcases.•Guamanian-anextremelyhighincidenceofALSwasobservedinGuam1950's.DuetotoxinsinthewaterandfoodHowtodiagnose•x-rays•MRI•Bloodandurinestudies•muscleandnervebiopsy•electomyography(EMG)•nerveconductionvelocity(NCV)•spinaltap•EvenwithallthistechnologyALSisextremelydifficulttodiagnose.ThisisbecausemanydiseasesmimicsignsofALSCommonMEDS•MostcommonlyseenisriluzoleRilutek©isaimedatsymptomaticreliefbasicallymaintainingoptimalqualityoflife.•Sidenote:thereisnodruginteractionstoanyofthedrugsweuseasparamedics.•Medicalmarijuana•Baclofenordiazepammaybeusedtocontrolspasticity•Trihexyphenidyloramitriptylinemaybeprescribedforpeoplewithproblemsswallowingtheirownsaliva.MedsCont….•O2andgastrictubesmaybeprescribedaswellduetotroublebreathingandchoking•ComingSoonduetoStemcellresearchmaybeacure?Prognosis•Usuallyendupneedingtogotoacarehomeorhavehospice•Stillafteralltheseyearspatientsafterdiagnosisonlyusuallylive3yearsSTATS•BasedonU.S.populationstudies,alittleover5,600peopleintheU.S.arediagnosedwithALSeachyear(approximately15newcasesaday.)•MostpeoplewhodevelopALSarebetweentheagesof40and70•ALSis20%morecommoninmenthaninwomen.•93%ofpatientsareCaucasian.MORESTATS•HalfofallpeopleaffectedwithALSliveatleastthreeormoreyearsafterdiagnosis.AllstatsarecourtesyofStudiesdonebyUniversityofMarylandReferences•Recentadvancesinamyotrophiclateralsclerosis.CurrentOpinioninNeurology13/4:397–405•PlayingdefenseagainstLouGehrig'sDisease“VerlagsgruppeGeorgvonHoltzbrinckScientificAmerican,(November2007).,pp.86–93.•“Amyotrophiclateralsclerosis:theoriesandtherapies”.RowlandLJournalofNeurologicalSciences.1994;31(169):126–127Refcont…••als.mdausa.org••••