CaseReport

RecoveryPhaseRehabilitationforAtypicalAmyotrophicLateralSclerosis:ACasewithLumbarVertebralFractureComplications

HiroshiMaeda１,２,ShogoToyama＊３,KoshiroSawada１,NorihideItoh４

SuzuyoOhashi１,TomoyukiIto５,AkikoSagara１,TakumiIkeda１

YujiArai５,YasuoMikami１andToshikazuKubo１,３,４,５

１DepartmentofRehabilitationMedicine,

KyotoPrefecturalUniversityofMedicineGraduateSchoolofMedicalScience２DepartmentofRehabilitation,KurashikiRehabilitationHospital

３DepartmentofOrthopaedics,KyotoPrefecturalUniversityofMedicineGraduateSchoolofMedicalScience４DepartmentofAdvancedRehabilitationMedicine,

KyotoPrefecturalUniversityofMedicineGraduateSchoolofMedicalScience,５DepartmentofSportsandPara-SportsMedicine,

KyotoPrefecturalUniversityofMedicineGraduateSchoolofMedicalScience

Abstract:Amyotrophiclateralsclerosisisarapidlyprogressivedisorderinvolvingdegenerationofmotorneurons.Excessiveexerciseimpactcanactuallydecreasemusclestrength.Wewouldliketoreportoursuccesswithapatientinwhomhome-basedconvalescencehadbeendifficultduetoalumbarvertebralfracture.Shewasabletoreturnhomeafterappropriateadjustments.Thepatientwasa64-year-oldwomanwhobecameawareofdiminishingmusclestrengthinherextremities12yearspreviouslyandwasdiagnosedwithamyotrophiclateralsclerosis.Shehadfallenathomeandsufferedalumbarvertebralfracture.Afteramonthofinpatienttherapyatanotherhospital,shewastransferredtoourinstitution.Shetiredeasilyandcouldnotperformmusclestrengthtraining,soherprogramwasadjustedsothatwalking,whichwasnotasexhaustingforher,wasthefocusofthetraining.Gradually,herfatiguebegantolessen,andshewasabletoundergomusclestrengthtraining.Ultimately,shebecameindependentenoughtoconductheractivitiesathome,allowinghertobedischargedhome.Evenincasessuchasourswherethepatientinitiallyfindsthetrainingtoochallenging,implementingalternativeexercisessuchastheuseofrepetitivelowimpacttrainingcanleadtoimprovementsinapatient’sactivitiesofdailyliving.

KeyWords:Amyotrophic lateral sclerosis, Lumbar vertebral fracture,Rehabilitation.

Rehabilitationforamyotrophiclateralsclerosis 407J.KyotoPref.Univ.Med.125（6），407～414，2016．

Received:January29,2016.Accepted:May9,2016＊CorrespondencetoShogoToyama465Kajii-cho,Kawaramachi-Hirokoji,Kamigyo-ku,Kyoto,602-8566,Japanshogo.toyama@gmail.com

Introduction

Amyotrophiclateralsclerosis（ALS）isarefractorydiseaseofunknowncausewherebothupperandlowermotorneuronsdegenerate,leadingtoprogressivemuscleweakness. Motorneurondegenerationhasbeenreportedtohalvethenumberofmotorunitsinjusthalfayear１）,leadingtoprogressivelimbmuscleweaknessandbulbarparalysis.Byapproximately3yearsafteronset,manypatientsdevelopwalkingdifficultiesanddysphasia,andultimately,manyofthepatientsdieofrespiratoryfailure.Elisabettaetal.reportedthatthe1-year/5-year/10-yearsurvivalratesin483casesofALSwere76.2％/23.4％/11.8％,respectively２）,andthatthereareslowlyprogressingcaseswheresurvivalbeyond10yearsispossible.Activitiesofdailyliving（ADL）arecompromisedasaresultofALSmakinghomeconvalescencedifficult,sorehabilitationmustbecarriedoutinaninpatientsetting.However,excessiveexerciseloadsduringrehabilitationcanactuallyleadtooverworkweaknessandmayevenhastentheprogressionofALS３）.Inaddition,theprimarydiseasemayprogressduringhospitalizationsothateventually,insteadofimprovedADLfunctioning,thepatientcannolongerbedischargedhome.Wewouldliketoreportourexperiencewithapatientwhohadatypicalslowly-progressiveALSthatwascomplicatedbyalumbarvertebralfractureinwhomweinstitutedrecoverystagerehabilitation.Byadjustingherexercisetreatmentappropriately,overworkweaknesswasavoidedandthepatientwasdischargedhome.

CaseReport

A64-year-oldwoman

PastmedicalhistoryBreastcancer

Familyhistory

HeryoungerbrotherwasdiagnosedwithALS.

Socialbackground

Sheliveswithherhusbandandeldestson.Beforetheinjury,shewasabletowalkfor20metersindoorsusingawheeledwalkerorbyholdingontowalls,whileoutdoorsshewasabletowalkforabout100mwithherhusband’shelp.Ashersymptomsprogressed,sheoftenfelleveninsideherhome.IntermsofADLs,sherequiredassistanceinshampooing,bathing,anddressing,andherhusbandtookoverthehousework.ShewasdesignatedasrequiringLong-Termcarelevel2,andalthoughherhomehadbeenrenovatedforherneeds,therewerestillareaswithleveldifferencesbutwithnorailings,suchastheentranceway,andneitherthetoiletnorbathroomhadrailings.

Historyofpresentillness

Thepatientbecameawareofdiminishingmusclestrengthinherextremitiesin2002,andwasdiagnosedwithALSbyaneurologist.Becauseherdiseaseprogressedslowly,shereceivedelectrophysiologicaltestinginordertoruleoutotherdiagnoses.OnEMG,giantspikeswereobservedforthetongueandfortheupperandlowerextremities

408 Maedaetal.

broadly,andonlysomefibrillationpotentialswereobservedincontrast.Thesefindingswereinterpretedasadisorderofanteriorhorncells,anditmeantthattherewerelowermotorneurondisordersinherupperandlowerextremities.Fortendonreflexes,otherthanareducedlowerjawreflexandpatellartendonreflexhyperactivity,deeptendonreflexesatothersiteswerenormal.Babinskireflexwasnegative.AlthoughthelowermotorneurondisorderwasfoundbyEMG,uppermotorneurondisorderfindingswerelimitedtophysicalfindings.AccordingtotherevisedElEscorialcriteria４）,shewasdiagnosedclinicallywithpossibleALS.Asdiseaseprogressionwasslowerthanthetypicalcase,herswasconsideredtobeacaseofatypicalALS.Lungfunctiontestswereconductedatthesametime:FVCwas1.96l,％VCwas71.3％,andsheexhibitedarestrictivediseasepattern.InSeptember2012,shefellwhileadjustingabuilt-infanandinjuredherselfathome.Severedorsal

lumbarpainmadeitdifficultforhertomoveherbody,andshewastakentoanemergencyhospital.ShewasdiagnosedwithanL1vertebralfractureandwashospitalizedfortreatment.Althoughconservativetreatmentwithahardcorsetwasinstituted,shecouldnolongerbedischargedhomeduetofurtherdecreasesinADLfunction,soinOctoberofthesameyear,shewastransferredtoourhospitalinordertoundergoinpatientrehabilitationtraining.

Statusonadmissiontoourinstitution

Noapparentimpairmentofmentalfunctionwasnoted.Milddysarthria,aswellastongueatrophyandfasciculations,wasnoted．Signsoflimbmuscularatrophyandmuscularweaknesswerepresentwitha2/5scoreonamanualmuscletest（MMT）oftheshoulderjointmusclesand3/5scoreinallothermusclestestedintheupperlimb.Inthelowerlimbs,thehipgirdlemusclewas2/5,thekneeextensorwas4/5,andplantardorsiflexionwas2/5（Table1）.Gripstrengthswere:right-5.1kgf;left-＜5kgf.Therewashyperreflexiaofthepatellartendon.Therewasnoobservationofsensoryimpairment,bladderorbowelincontinence,eyemovementdisorder,orpressureulcers.Uppermotorneuronsignsandlowermotorneuronsignswerepresent,andthepatientmetthediagnosticcriteriaforpossibleALS.Therewerehardlyanycomplaintsoflumbarpain,andshecouldmovetoherwheelchairwithassistance.

Rehabilitationforamyotrophiclateralsclerosis 409

Table1.MMTatadmissionandatdischarge

SpO2atrestonroomairwas95％.HerscoresontheSimpleTestforEvaluatingHandFunction（STEF）were71pointsontherightand85pointsontheleft（standardcutoffpointsat60yearsofage:88points）.Atadmission,herfunctionalindependencemeasures（FIM）werealldecreasedwiththeexceptionofsphinctercontrolandself-care,whilethemotorscoresubtotalwas61pointsandcognitivesubtotalwas35points.Inthelumbarspinex-raylateralviewatthetimeofadmission,34％collapsewasnotedintheanteriorcolumnofL1.Calluswasalreadyformed,andhercourseoftreatmentwasuncomplicated（Fig.1）.Duetohermilddysphagia,weusedathickenerinadministeringavideofluoroscopicswallowingstudy（VFSS）.Sheexhibitedslightdefectsinchewing,transport,andbolusformation,butaninduceddelayoftheswallowingreflexwasnotobserved.Laryngealelevationwasmildlydefective,andmildpharyngealresiduewasalsodetected.Byaddingathickeningagenttothebariummealsolutionweassuredtherewasnoaspiration.

Issues

Thispatient'spotentialissueswereasfollows.Functionaldisordersincluded:（1）muscleweakness（2）decreasedendurance;and（3）finger

dexterityimpairment.Decreasedfunctioningwasnotedin:（1）difficultywalking;and（2）decreasedADLability.Socialdisadvantageswere:（1）homeenvironment;and（2）homeconvalescencedifficulties.

Treatmentprotocol

Physiotherapyincludedmusclestrengthtrainingandwalkingtraining,whileoccupationaltherapyfocusedonADLtraining.Whiletakingcaretoavoidmusclefatigueduetooveruse,musclestrengthwascheckedonthedayaftertrainingtoadjusttrainingintensityaccordingly.Treatmentgoalsweretowalkindoorswithmodifiedindependence,andperformallADL（otherthanbathing）withmodifiedindependencesothatshecouldbedischargedhome.

410 Maedaetal.

Fig.1.Radiographyoflumbarspine.（A）Acompressionfractureofthe1stlumbarvertebrawasseenonadmission.（B）Callusformationatthefracturesitewasseenatdischarge.

CourseofTreatment

Itwasnecessaryforourpatienttocontinuewearingthebraceforherlumbarcompressionfracture.Initially,just50repetitionsofstandingandsittingexercisesledtoresidualfatigue.Duetothisresidualfatigueandcomplaintsofshortnessofbreath,musclestrengthtrainingcouldnotbecarriedoutthefollowingday.Shewasabletoperformwalkingtrainingwithfewcomplaints.Inordertomaintainacertaindegreeoftraining,thetreatmentprotocolwaschangedsothatrehabilitationtrainingwouldfocusonwalkingexercises.Afterabout3weeks,thepatient’sresilienceimprovedandwithfewercomplaintsofshortnessofbreath,shebecameabletoperformmusclestrengthtrainingwith0.5kgweightsdoing2setsof10repetitionseach.Therewasnoprogressionofherlowerbackpainorcollapsedvertebraviasimplex-ray.Becauseboneunionwasobserved,thecorsetwasdiscontinuedthreemonthsafterherinitialinjury.Hercomplaintsofshortnessofbreathdisappeared,anditwaspossibletoinitiateanaggressivemusclestrengthtrainingprogram.Ultimately,musclestrengthmeasurementsofleftshoulderjointflexionandabduction,bilateralhipjointabduction,andrightankledorsiflexionimproved

（Table1）.Gripstrengthincreasedfrom5.1kgfto6.7kgfintherighthandbutthelefthandremainedatlessthan5kgfforthedurationofherhospitalstay.Sheeventuallycouldwalkalonefor100musingawalkerwheeler,andwithhelpfromherhusband,shecouldevenwalkfor50mwithaT-cane.STEFdeterioratedfrom72pointsto56pointsontherightand85pointsto64pointsontheleft,indetailedmentionevaluationitemsusingsmallthings,suchasapinmainlydecreased.ButtherewasnoimpactonherADLperformance.FIM motoritemsincludedgrooming,dressing,toileting,transfers,locomotion,andstairclimbing,whilehertotalscoreimprovedfrom61to95points（Table2）.Herhomeenvironmentwasalsoimprovedbyplacinghandrailsattheentranceway,toilet,andbathroom,andhavingachievedmodifiedindependenceinADLfunctionathome,shewasdischargedhomeonDay86ofhospitalization.

Rehabilitationforamyotrophiclateralsclerosis 411

Table2.MotorFIMScores

Discussion

IncreasedmuscleweaknessrelatedtooverusecanoccurinALSaswellasinthepost-poliosyndrome,andthisresultsfromexcessiveloadinginmuscleswithreducednervefunctionduetolostcollateralbrancheswithinnervefibers５）６）.Byattemptingtoincreasebaselinemusclestrengththroughstrengthtraining,itispossibletoexacerbatethenervedegeneration６）.ADLfunctionsmay,however,improveifmusclestrengtheningexercisesareimplementedwhiletakingcaretoavoidoverworkweakness３）７）.Recentreportshavefoundexercisetherapycanimprovefunctioninganddelaydiseaseprogression,thusalleviatingtheburdenofneededcaretaking８）,andmorepapershavereportedimplementingoutpatientexercisetherapy９）１０）.Unfortunately,thereisnoconsensusontheoptimalexerciseintensity,cliniciansdebatewhetherexercisetherapyshouldbeenforcedoravoided,andtheefficacyofexercisehasnotyetbeenestablished.Ifthediseaseprogressesrapidly,notonlyistherenohopeofimprovingactivitiesofdailyliving（ADL）performance,butevenifthepatientishospitalizedlong-termforrehabilitation,thepatient’sADLfunctionmaydeteriorateduringthattimeduetoALSprogression,makingitdifficultforthepatienttobedischargedhome.Ourpatienthadatypicalslowly-progressiveALSthatwascomplicatedbyalumbarcompressionfracture,andprimarydiseaseprogressionduringhospitalizationwaslimited.Bycarefuladjustmentofexercisetherapy,itwaspossibletoachieveimprovementsinADLfunctionwhichallowedthispatienttobedischargedhome.Inlumbarvertebralfractures,reportssuggestthatcomparedtoinpatienttherapy,outpatient

therapymaydecreasetheriskofcompromisingADLfunction１１）.Thereforeinhospitalizedpatients,itisnecessarytoinitiateearlymobilizationandaggressiveexercisetominimizedecreasesinADLduetodisuse.Inthiscase,inadditiontothelumbarvertebralfracture,thepatienthadalreadysufferedadecrease

inADLperformanceduetoALSitselfbeforetheinjury,makinghomerecuperationdifficultandrequiringhospitalizationduringtheacutephase.Inadditiontothemilddecreaseinrespiratoryfunction,asexemplifiedoriginallyby95％SpO２atrest,itwasnecessaryforourpatienttowearabraceforthetreatmentofherlumbarvertebralfracture.Shecomplainedofshortnessofbreathafterevenlightexercise.Thisisaslowlyprogressingcasewherethepatientwasalreadymorethan10yearspastALSonset.ThatisthereasonwhythecontributionofALStoADLdeclineatthetimethepatientwastransferredtoourrehabilitationhospitalwasconsideredminor.DecreasesinADLfunctioningthathadbeennotedwhenthepatientwastransferredtoourhospitalwereprimarilytheresultofinactivityduringhospitalizationinanacutecareinstitutionforthefracture.Furthermore,learnednonuse,duetoinactivityanddecreasedresilience１２）１３）,ledtodisusewhichcompromisedmovementsthatshouldhavebeenpossible.ExercisetherapyforALSincludesmoderateresistancetraining,andtherearereportsthat

improvedmusclestrengthandendurancecanbeachievedwithsuchmoderateresistancetraining９）１０）,andsoinitiallythepatienthadbeenscheduledformusclestrengthtrainingwhichincludedresistancetraining.Nevertheless,restrictionofthoracicmovementduetothepatient'scorsetresultedinshortnessofbreath,whichpreventedusfromcompletingourscheduleofmusclestrengthtraining.Becausetheadmissionx-rayshowedmildcallusformationandcollapse,painwasnotalimitingfactorforrehabilitation,andwewereabletoremovethebraceatanearlystage.Forthatreasontherewasanoriginaldeteriorationofrespiratoryfunction,howeverwebelieveditpossibletoincreasetheamount

412 Maedaetal.

oftraining.Thus,theplanwaschangedtoonewithrepeatedwalkingexercisethatthepatientfoundlesstiringandwhichledtoimprovedendurance.Asaresult,itwasnowpossibleforhighfrequencymusclestrengthtrainingtohaveanimpact.Functionalimpairmentanddisabilitywereimprovedbytraining,andourpatientwasabletobedischargedhomewithinthe90-daydeadlineofheradmissiontoourconvalescentrehabilitationfacility.Wedidnotnoteanysignsofoverwork,suchasfatigue,musclepain,orsorenessafterexercise.

STEFdeterioratedinthisperiod,incontrasttoimprovedfunction,butwedidnotconsiderrehabilitationordiseaseprogressionasacause.Weconsiderthismaybefrom‘learned-nonuse’inthehand.Usually,ALSrehabilitationtargetslargejoints１４）,suchastheshoulder,knee,orankle,andadaptiveequipmentisappliedforhandimpairment.Handexercisesmighthavebeeninsufficientinourcase.Wemainlyusedexercisehoursformuscletraining,aswehadgreaterconcernforADL.EvenifthepatienthaddevelopedlearnednonuseduetoatypicalALScomplicatedbylumbar

compressionfractures,iftheALSprogressionisslow,withrepeatedlowimpacttraining,itwasthoughtthatitwouldbepossibletobreakfreefromtheviciouscycleoflearnednonuseandtoimproveADLperformance.ConflictsofInterest:Noneexist

Rehabilitationforamyotrophiclateralsclerosis 413

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References

〈和文抄録〉

腰椎圧迫骨折を合併した非典型的筋萎縮性側索硬化症に回復期リハビリテーションを行った1例

前田 博士１,２，遠山 将吾＊３，沢田 光思郎１，伊藤 慎英４，大橋 鈴世 1，伊藤 倫之５，相良 亜木子１,池田 巧１，

新井 祐志５,三上 靖夫１,久保 俊一１,３,４,５

１京都府立医科大学大学院医学研究科リハビリテーション医学２倉敷リハビリテーション病院リハビリテーション科

３京都府立医科大学大学院医学研究科運動器機能再生外科学（整形外科学教室）４京都府立医科大学大学院医学研究科リハビリテーション先進医療開発講座

５京都府立医科大学大学院医学研究科スポーツ・障がい者スポーツ医学

筋萎縮性側索硬化症は発症後平均約3年で呼吸不全から死に至る，急速進行性の運動ニューロン変性疾患である．自宅療養の継続にリハビリテーションは有効とされるが，過剰な運動負荷は筋力の低下を招くことも知られており，疾患の進行に配慮した訓練内容の設定が必要である．また入院を余儀なくされると，疾患自体の進行により退院困難になることがある．腰椎圧迫骨折の受傷により入院し退院が困難であったが，リハビリテーションの工夫により自宅退院が可能になった1例を報告する．64歳女性．12年前に四肢の筋力低下を自覚し，筋萎縮性側索硬化症と診断され，日常生活では夫の介助を要していた．自宅で転倒して腰椎圧迫骨折を受傷し，他院で1ヵ月間入院加療をうけた後，当院に転院した．疲労感のため筋力増強訓練が行えず，低負荷の歩行訓練を中心に行うよう訓練内容を変更した．次第に疲労感は軽減し筋力増強訓練が可能になり，最終的に動作が自立したため，自宅への退院が可能になった．本症例のように訓練に困難をともなう筋萎縮性側索硬化症の症例であっても，低負荷訓練を高頻度に行うなど訓練内容の工夫で，日常生活活動が改善することがあり，積極的な介入が必要と考えた．

キーワード：筋萎縮性側索硬化症，腰椎圧迫骨折，リハビリテーション．

414 Maedaetal.