North star PT Manual MSY2017 - Muscular …...• North Star Ambulatory Assessment (NSAA) –...

Updated Physiotherapy Manual Version 1.0 March 2017

PhysiotherapyManualNorthStarNetwork

Version2.0

May2017

Updated Physiotherapy Manual Version 2.0 May 2017

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TableofContents

1. WHICHTESTSSHOULDICOMPLETE? 4

2. OUTCOMEMEASURES 4

3. EQUIPMENTREQUIREDFOREVALUATIONS 4

4. SUGGESTEDORDEROFEVALUATIONS 6

5. GENERALGUIDELINESFOREVALUATIONPROCEDURE 7

6. PULMONARYFUNCTIONTESTING 7

FVC/FEV1MANOEUVRE 8OPEN-CIRCUITSYSTEM(FVC/FEV1):DEFAULTTECHNIQUE 8CLOSEDCIRCUITSYSTEM(CCS):TOBEATTEMPTEDIFSUBJECTHASPROBLEMSWITHOPENCIRCUIT 9PEAKCOUGHFLOW(PCF) 10SUMMARYOFSPIROMETRYTESTINSTRUCTIONS 11LISTOFABBREVIATIONS 12SPECIFICSPIROMETRYSTANDARDS: 12

7. NORTHSTARAMBULATORYASSESSMENT(NSAA)–VERSION2.0 13

TESTITEM1:STANDING 15TESTITEM2:WALK 16TESTITEM3:STANDUPFROMCHAIR 17TESTITEM4&5:STANDONONELEGRIGHT&LEFT 18TESTITEM6&8:CLIMBBOXSTEPRIGHT&LEFT 19TESTITEM7&9:DESCENDBOXSTEPRIGHT&LEFT 20TESTITEM10:LIFTSHEADFROMSUPINE 21TESTITEM11:GETSTOSITTING 22TESTITEM12:RISEFROMFLOOR 23TESTITEM13:STANDSONHEELS 24TESTITEM14:JUMP 25TESTITEM15&16:HOPRIGHT&LEFTLEG 26TESTITEM17:RUN(10METRES) 27NSAABRIEFTESTDETAIL 28

8. TIMEDGRADEDFUNCTIONALACTIVITIES 30

RISEFROMFLOOR 3010-METRERUN/WALK 31

9. TIMED4-STAIRCLIMBANDDESCEND 32

CONDUCTINGTHE4STAIRCLIMB–ASCEND 32CONDUCTINGTHE4STAIRCLIMBDESCEND 33


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10. PERFORMANCEOFTHEUPPERLIMB(PUL2.0)SCALE 35

INTRODUCTION 35EQUIPMENTREQUIRED 35GENERALINSTRUCTIONS 35CONDUCTINGTHEASSESSMENT 37A.ENTRYITEM–NOTINCLUDEDINTOTALSCORE–ADAPTEDBROOKESCALE 381. SHOULDERABDUCTIONARMSABOVEHEAD(SCOREDFROMENTRYITEMA) 392. RAISEBOTHARMSTOSHOULDERHEIGHT–(ELBOWSATSHOULDERHEIGHT) 403. SHOULDERFLEXIONTOSHOULDERHEIGHT(NOWEIGHTS) 414. SHOULDERFLEXIONTOSHOULDERHEIGHTWITH500G 415. SHOULDERFLEXIONABOVESHOULDERWITH500GWEIGHT 436. SHOULDERFLEXIONABOVESHOULDERWITH1KGWEIGHT 437. HAND(S)TOMOUTH 458. HANDSTOTABLEFROMLAP 469. MOVEWEIGHTONTABLE100G 4710. MOVEWEIGHTONTABLE500G 4711. MOVEWEIGHTONTABLE1KG 4712. LIFTHEAVYCANDIAGONALLY 4813. STACKOFTHREEHEAVYCANS 4914. STACKOFFIVEHEAVYCANS 4915. REMOVELIDFROMCONTAINER 5116. TEARINGPAPER 5117. TRACINGAPATH 5218. PUSHONLIGHT 5319. SUPINATION 5420. PICKUPCOINS 5521. PLACINGFINGERONNUMBERDIAGRAM 5622. PICKUP10GWEIGHTFINGERPINCH 57

11. EGENKLASSIFIKATIONSCALE1-17ITEMS 58

TESTDESCRIPTION 58TESTINGGUIDELINES 58NSAATOEK2-BRIDGINGITEM 59EK1:ABILITYTOUSEWHEELCHAIR 59EK2:ABILITYTOTRANSFERFROMWHEELCHAIR 59EK3:ABILITYTOSTAND 60EK4:ABILITYTOBALANCEINTHEWHEELCHAIR(MUSTBEOBSERVED) 60EK5:ABILITYTOMOVETHEARMS(MUSTBEOBSERVED) 60EK6:ABILITYTOUSETHEHANDSANDARMSFOREATING 61EK7:ABILITYTOTURNINBED 61EK8:ABILITYTOCOUGH 61EK9:ABILITYTOSPEAK 62EK10:PHYSICALWELL-BEING 62EK11:DAYTIMEFATIGUE 62EK12:HEADCONTROL 63


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EK13:ABILITYTOCONTROLAJOYSTICK 63EK14:FOODTEXTURES 63EK15:EATINGAMEAL 64EK16:SWALLOWING 64EK17:HANDFUNCTION 64

12. RANGEOFMOVEMENT 65

GENERALGUIDELINES 65ANKLEDORSIFLEXION 66HIPEXTENSION 67BETTERIFLIMBISDROPPEDOVEREDGEOFBEDTOACHIEVEFULLCHECKOFRANGE 67KNEEEXTENSION 68ITB 69SHOULDERELEVATIONTHROUGHFLEXION 70ELBOWEXTENSION 71FOREARMSUPINATION 72WRISTEXTENSION 73LONGFINGEREXTENSION 74

13. MANUALMUSCLETESTING 75

14. APPENDIX1 77


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1. WHICHTESTSSHOULDICOMPLETE?

Thisdependsonyourtimelimitations,facilities,experienceandavailableequipment.

2. OUTCOMEMEASURES

• PulmonaryFunctionTests:

o ForcedVitalCapacity(FVCandFEV1),o PeakCoughFlow

• NorthStarAmbulatoryAssessment(NSAA)–Version2.0

Includingtimedrisefromfloorand10mwalk/run

• Foursstairclimbanddescend–TimedFunctionTests• PerformanceofUpperLimbforDMD2.0(PUL)• EganKlassifikationScaleVersion2• RangeofMotionMeasurement• SuggestedMMTscale–detailsnotcurrentlyincluded

3. EQUIPMENTREQUIREDFOREVALUATIONS

PulmonaryFunctiontests

• Spirometer,hardwareandsoftwareasnecessarytomeasureFVCandPCF• ConsumablesforFVCincludingfiltersandmasks

• NorthStarAmbulatoryAssessment(NSAA)§ 15cmstep§ Heightadjustableplinthorstandardchairwithoutarms§ Stopwatch§ 12metrespacefortimedwalk/run

Functional4stairclimbanddescend

• Setof4standardsteps

PerformanceoftheUpperLimb2.0(PUL)

• Selectionofmetricweights10g,100g,200g,500gand1000g.• Smallcoins:1euro,10pence,quarterorsimilar


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• Pencil(standardHBpencil)• Plasticcup(vendingcup)• PlainA4paper• Ziploccontainer(8ounce)• Pushlightbutton• Numberpad(endofworksheet)• Tracingdiagram(endofworksheet)• Plastic(orpaper)tableclothmarkedwithcircles(Appendix1)• 5heavy(full)cans

RangeofMotion

Longarmgoniometer


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4. SUGGESTEDORDEROFEVALUATIONS

Ambulant

• PulmonaryFunctionTests(FVCandFEV1)• NSAA• Timedtestsincludingfourstairs• PerformanceofUpperLimb2.0(PUL)ifmodifiedBrookScaleislessthan6(topscore)• RangeofMotion–keyjointsincluded

Non-ambulant

• RespiratoryFunctionTests(FVC,FEV1andPCF)

• EganKlassifikation2

• PerformanceofUpperLimb2.0(PUL)includingmodifiedBrookScale–ItemA

• RangeofMotion–keyjointsincluded

• MMTasrequired

Updated Physiotherapy Manual Version 1.0 March 2017

5. GENERALGUIDELINESFOREVALUATIONPROCEDURE

The testing environment should be the same each time an assessment is repeated. As far aspossibleitshouldbedistractionfree.ClinicalEvaluatorsExperienceandtraininginthesetestsisrecommended.Ideally,thesameevaluatorshouldtestthesameparticipantoneachoccasion.WesuggestyoudonotlookbackonaprevioustestuntilyouhavecompletedtheassessmentsNSAAisperformedbarefoot.ClothingShortsandt-shirtsarerecommended.Socksandshoesshouldberemovedforfunctionalevaluations.

6. PulmonaryFunctionTesting

NOTE:ClinicalEvaluatorwillusethespirometeravailableatsite.

PulmonaryFunctionTests:

o ForcedVitalCapacity(FVC)o Forcedexpiratoryvolumeinonesecond(FEV1)o PeakCoughFlow(PCF)

GeneralTestGuidelinesforRespiratoryFunctionTests

1. Calibration of spirometer should be performed on a regular basis according to localguidelines

2. Theparticipantshouldsitcomfortablyasuprightaspossibleinachair,feetsupported.3. Loosentightclothing4. Theevaluatorexplainsanddemonstratesthetesttechniquetotheparticipant.5. The participant should follow the wording of instructions closely and practice the

manoeuvreuntilthecorrecttechniquecanbedemonstrated.6. Anoseclipshouldbeplacedoverthenosebeforebeginningeachattempt(ifavailable)7. The test should be repeated at least three times making sure that the participant has

recoveredbetweenattempts.


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8. If at each attempt there is an improvement in the results or if there is difficulty gainingcomplianceorwithunderstanding,furthertestscanbeconducted(maximumof6-8)untiltheparticipanthasachievedhisbestresult.

9. Theparticipantcanplacethemouthpieceinhisownmouthunlessheisunabletoreachhismouthwithhishandsorflexeshisheadandshoulderstoreachthetube. Inthiscasetheassessorshouldplacethetubeintotheparticipant’smouth.

NOTE:Thedefaulttechniqueistheopencircuitwithamouthpiece.Closedcircuitormasktobeusedifopencircuitisunsuccessful1.Iftheparticipanthasdifficultywiththetechniquesitisacceptabletoplacethetubeinthe

mouthbefore thebeginningof inspiration (closedcircuit).This shouldbedocumented inthecomments.

2.Whereatightsealaroundthemouthpiececannotbeachievedafacemaskmaybeused.Thisshouldbedocumentedinthecomments.

3.Theparticipantshouldnotbeallowedtoflexforwardexcessivelyduringexpiration.4.Incentivesoftwareisnotallowed.

FVC/FEV1Manoeuvre

Open-circuitSystem(FVC/FEV1):Defaulttechnique


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• Havetheparticipantassumethecorrectposture,feetwellsupportedonthefloor• Attachnoseclip• Askthesubjecttotakeabigbreathin• Placemouthpieceinmouthandcloselipsaroundthemouthpiece• Askthesubjecttoblowoutashardandasfastforaslongastheycan• They should exhalemaximally to residual volume until nomore air can be expelled

whilemaintaininganuprightposture• TrytoobtaingoodqualityFVCmanoeuvrewithagoodplateauattheendofexpiration• Repeatforaminimumofthreemanoeuvres;nomorethaneight,coachingvigorously• Checktestrepeatabilityandperformmoremanoeuvresasnecessary

ClosedCircuitSystem(CCS):Tobeattemptedifsubjecthasproblemswithopencircuit

• Havethesubjectassumethecorrectposture(seatedandupright)• Explainthetest“putthemouthpieceinyourmouthandcloseyourlips,breathinand

outnormally,thenhaveadeepbreathinandblowtheairoutthroughyourmouthashardandasfastasyoucan”

• Attachnoseclipandinstructtheparticipanttoplacethemouthpieceintotheirmouthandcloselipsaroundthemouthpiece.

• Performtidalbreathing

• Coachtheparticipantto“Takeadeepbreathin”• Coachtheparticipantto“Blastitout!”toexhaleasfastastheycan• Coach the participant to exhale as long as they can to obtain a plateau “Keep

blowing”…• Repeatinstructionsifnecessary,coachingvigorously• Repeat for a minimum of three manoeuvres, no more than 6 - 8 manoeuvres are

permitted


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Peak Cough Flow (PCF)

•Usetheadaptertoattachthemasktothebacterial/viralfilter

• Ensurethesubjectissittingupstraight,feetsupported• Askthesubjecttakeinadeepbreath.• Holdthemaskfirmlyoverthesubject’snoseandmouth• Theevaluatorshouldholdthemaskwithonehandandputtheotherhandonthebackofthe

headtoprovidestability.• Makesuretherearenoairleaks• Askthesubjecttocoughashardastheycan• Repeatforaminimumofthreemanoeuvres;nomorethansix,coachingvigorously• Checktestrepeatabilityandperformmoremanoeuvresasnecessary• AmericanThoracicSociety(ATS)CriteriaATScriteriaareprovidedtogiveaguidelinetowards

determiningavalidtestataspecificvisit.Theyarenotrequiredandifyoufeelabestefforthasbeenachievedthenthelargestvalueshouldbeselectedandnottheonebasedonrepeatability

• Criteriaforsatisfactoryspirometry

Theparticipantshouldbecoachedtogivetheir“best”effortswiththegoalofexhalingfor:

• ≥3sinchildrenaged<10yrs.• ≥6sinsubjectsaged≥10yrs.• Childrenundertheageof5arenotexpectedtoexhaleforlongerthan1second

(thoughmanywillbeableto)• 3acceptableefforts• Goodstartoftest• Arapidwelldefinedpeak• Satisfactoryendoftestcriteria• Freefromartefact• Nocoughingduringthefirstsecondofexhalation• Noglottisclosurethatinfluencesthemeasurement• Noearlyterminationorcut-off• Noeffortthatisnotmaximalthroughout• Noleak


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• Noobstructedmouthpiece

CriteriaforRepeatability• 2RepeatableEfforts• IftheFVCis>1.0Litres:-ThetwolargestvaluesFVCmustbewithin0.150Lofeach

other-ThetwolargestvaluesFEV1mustbewithin0.150Lofeachother• IftheFVCis=<1.0Litres:-ThetwolargestvaluesFVCmustbewithin0.100Lofeach

other-ThetwolargestvaluesFEV1mustbewithin0.100Lofeachother

• Checktestacceptabilityandrepeatabilityandperformmoremanoeuvresasnecessary:

• HOWEVER:IFATScriteriaarenotmetyoumayproceedifyouthinkthebesteffortwasgained.Ifnotrecordthisonyourworksheet.

SummaryofSpirometryTestInstructions

Followgeneraltestguidelinesasabove.

Ensuretheparticipantisrested.

ForFVC,theevaluatoraskstheparticipanttobreathoutasmuchaspossibleandthentakeasbigabreathinaspossible,placesthemouthpieceinthemouth,andthenaskstheparticipanttoblasttheairoutthroughthemouthashardandforaslongastheycan.Theassessorshouldverballyencouragetheparticipanttokeepgoingforaslongaspossible.Repeatatleastthreetimesbutnomorethaneighttogetthreeacceptable/repeatableefforts.Themouthpiececanbeputinthemouth by the participant but if this is not possible to do accurately then the evaluator shouldplace the mouthpiece. Ideally 3-6 seconds of plateau should be achieved but it is up to thediscretionoftheevaluatortodetermineifthetestisvalidandabesteffortfortheparticipant.

It isup to theclinicalevaluator todeterminewhether theparticipanthasachievedat leastonegood blow that represents their best effort. (You may accept a result that falls below ATSstandards).


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Listofabbreviations

ATS/ERSAmericanThoracicSociety/EuropeanRespiratorySociety.UsedinreferencetotheATS/ERS2005guidelinesontheStandardizationofSpirometry.

CRA ClinicalResearchAssociate(or‘monitor’).

DOB DateofBirth.

FEV1ForcedExpiratoryVolumein1second.Thevolumeofairthatisexhaledinthefirstsecondofaforcedmanoeuver.

FVCForcedVitalCapacity.TheVitalCapacityasdeterminedthroughaforcedmanoeuver.Thevolumeofairexpiredoverthecourseofaforced,maximalexhalationfromTotalLungCapacity.

ID Identification.

PFT PulmonaryFunctionTest.

SIV SiteInitiationVisit.

SpecificSpirometryStandards:

• Forfurtherinformation,seeMilleretal.(2005)StandardizationofSpirometry,EurRespirJ,26,319-338at:

• http://www.thoracic.org/statements/resources/pfet/PFT2.pdf


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7. NorthStarAmbulatoryAssessment(NSAA)–Version2.0

TestDescription

TheNSAAisa17-itemscalethatgradesperformanceofvariousfunctionalskillsonascalefrom0(unable),1(completesindependentlybutwithmodifications),and2(completedwithoutcompensation).

NOTE:Theorderhasbeenmodifiedtoenablemoreefficientconductoftheassessment.

a. ClimbandDescendstairs:climbboxstepisperformedstepupright,stepdownright,stepupleft,stepdownleft.

b. Liftsheadisperformedbefore‘getstositting’and‘risefromfloor’toavoidfatigue.

TestGuidelines

• Performanceofuprightactivitiesmustnotbecompletedifalowerlimbinjuryexists.However,thesubjectmayperformtheheadliftandgets-to-sittingitemsifheisable.Provide‘comments’onthesourceworksheet.

• CompletetheNSAAintheorderprovided(Pleasenotethishasbeenmodifiedfromprevioustrialsforasmootheradministration).

• DoNOTuseamat.

• Pleasenotethetimeinsecondsroundedtothenearesttenthofasecondforalltimedtests(eg,risefromfloorand10m‘run’).

• Generally,activitiesaregradedinthefollowingmanner:

Scoreof2=‘Normal’–noobviousmodificationofactivity

Scoreof1=Modifiedmethodbutachievesgoalwithnophysicalassistance

Scoreof0=Unabletoachievegoalindependently

• Ifyouthinkthesubjectiscapableofabetterperformance,duetonon-complianceorimprovedunderstandingoftask,itisacceptabletoaskthesubjecttorepeattheitemandre-scoreifappropriate.

• Ifunsureiftheperformanceonaparticularitemmeetsthehighercriteria,givethesubjectthelowerscore.


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Set-Up

• Markoffa10-metrecourseinalong,quiethallwayorroom.Providea‘finish’lineatapproximately11metrestoensurethesubjectmoves‘through’the10-metreline,ratherthanslowingdownpriortofinishing.

• Ensureaclearfloorspace,measuringapproximately10feetx12feet,isavailableforremainingassessments(i.e.,risefromfloor).


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ConductingtheTest

Testitem1:Standing

Startingposition

Feetmustbenofurtherthan10cmapartandheelsonthegroundifpossible.Armsbysides.

Instruction Canyoustanduptallformeforaslongasyoucanandasstillasyoucanforthreesecondswithyourheelsflatontheground?

Scoringdetail Whencountingto3–Use“And1,and2,and3”sothatthreesecondsisachievedonthewordof3.Score2-Minimumcountof3seconds.

Activity 2 1 0

Standing Standsupright,still,symmetrical,withoutcompensation(heelsflatandhipsinneutralrotation)forminimumcountof3seconds

Standsstillbutwithcompensation(e.g.ontoesorwithlegsabductedorwithbottomstuckout/hipflexion,etc)forminimumcountof3seconds

Cannotstandstillorcannotstandindependently,needssupport(evenminimal)

Photographs/

Notes

Score2-Symmetricalheelsflat

Score1–Asymmetricorcompensation


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Testitem2:WalkStartingposition Standing.Observewalkforatleast10stepsinbothsagittalandcoronal

planes.

Instruction CanyouwalkfromAtoB(statetoandwherefrom–approximately8-10feet)forme?

Scoringdetail

Score1-Ifthepatientgenerallytoewalksbutoccasionallygetsheelsflat,orcanonrequest,theymustscore1

Activity 2 1 0

Walk Walksconsistentlywithheel-toeorflat-footedgaitpattern

Persistentorhabitualtoewalker,unabletoheel-toeconsistently

Lossofindependentambulation–mayuseknee-ankle-footorthosis(KAFO)orwalkshortdistanceswithassistance

Score1–habitualtoewalker


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Testitem3:StandupfromchairStartingposition

Sittinginchair,benchorplinthwitharmsfoldedacrosschestwithhishandupbyhisshouldersandwithfeetabletoreachfloororsupportedonsecurebox.Adjusttheseatheightsothatthehipsandkneesareata90ºanglefromthefloor.Feetnomorethanshoulderwidthapart.

Instruction Canyoustandupfromthechairkeepingyourarmsfolded?

Scoringdetail

Asize-appropriatechair(withoutarm-rests)orheightadjustableplinthmustbeused.Alternatively,aboxstepunderthefeettoachievethecorrectstartingpositionmaybeused.

Score2-Armsmustbekeptcrossedwiththehandsupbytheshouldersthroughouttheactivitytoscore2

Activity 2 1 0

Standupfromchair

Abletostandupkeepingarmsfolded

Withhelpfromthighs/pushonchair/proneturnoraltersstartingpositionbywideningbase

Unable

Photographs/notes

Score2–keepsarmsfoldedandstandsupwithoutwideningbase

Score1-Needstwohandstopushand/or

Widenedbase

Score0-Unabletorisefromstartingposition

Score0-Needstochangebodyposition;and/orhipsextendedtoslideforwardfromchair(not90/90)


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Testitem4&5:StandononelegRight&LeftStartingposition

Standing.

Instruction Showmehowlongyoucanstandonyourright/leftlegwithyourarmsatyourside.

Count“And1,and2,and3”

Scoringdetail

Score2–Minimumcountof3secondsrequired

Activity 2 1 0

Standononeleg

Abletostandinarelaxedmanner(nofixation)foracountof3seconds

Standsbuteithermomentarilyorwithtrunkside-flexionorneedsfixatione.g.bythighsadducted

Unable

Photographs/notes

Score2–Relaxedandnofixation

Score1-Hookslegbehindstandingleg

Score1-Posturalcompensation/leaningtoside

Score0-Ifneedstoholdontoatableorpersonforsupport


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Testitem6&8:ClimbboxstepRight&LeftStartingposition

Standinginfrontofboxstep,toesnomorethan20cm(~8in)awayfromboxstep.

Instruction Canyoustepontothetopoftheboxusingyourright/leftleg.

Scoringdetail Boxstepmustbe15cmhigh.Score1:Iftheyneedahandforbalanceonly–evaluatorshandshouldbekeptrelativelyhigh.Score1:Iftheyuseahandontheirthigh.Ifindoubtwhetherthisisusedforbalanceorsupportofferthemyourhandandrepeattest.Score0:Iftheyputweightthroughtheevaluatorshand(withoneortwohands)orunabletoclimbstep

Theyshouldnothavetheirhandsintheirpocket.

Activity 2 1 0

Climbsboxstep

Facesstep–nosupportneeded

Goesupsideways/rotatestrunk/circumductshiporneedshandforbalance

Unabletoperformindependently

Photographs/notes

Score1–Needshandforbalanceonly

Score2–Facesstep

Score1-Rotatestrunkoruseshandonthigh


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Testitem7&9:DescendboxstepRight&LeftStartingposition

Standingonboxstep

Instruction Canyoustepdownfromtheboxusingyourright(orleft)leg?

Scoringdetail Boxstepmustbe15cmhigh.

Score1:Iftheyneedahandforbalanceonly–evaluatorshandshouldbekeptrelativelyhigh.Iftheyuseahandontheirthigh.Ifindoubtwhetherthisisusedforbalanceorsupportofferthemyourhandandrepeattest.Usesmethodthatavoidsflexingsupportingknee(oneontheboxstep)Score0:Iftheyputweightthroughtheevaluatorshand(withoneortwohands)orunabletodescendstep

Theyshouldnothavetheirhandsintheirpocket.

Activity 2 1 0

Descendsboxstep

Facesforward,stepsdowncontrollingweight-bearingleg.Nosupportneeded

Sideways,skipsdownorneedshandforbalance

Unablewithoutmorethanminimalsupport,orrequireshandsforsupport

Photographs/notes

Score2–facesforward

Score1–Needshandforbalance.Noweighttakenthroughhand.

Score1–Turningtosidetodescend


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Testitem10:LiftsheadfromsupineStartingposition

Supineonaplinthorfloor,armsfoldedacrosschestandthehandsupbytheshoulders.Nopillowmustbeused.

Instruction Canyouliftyourheadandtouchyourchintoyourchestkeepingyourarmsfolded?

Scoringdetail

Askpatienttokeeparmscrossedoverchestduringtheactivitytoavoidself-assistance.

Activity 2 1 0

Liftsheadfromsupine

Insupine,fullneckflexion,headmustbeliftedinmid-line.Chinmovestowardschest

Headisliftedthroughsideflexion,partialneckflexion,orwithprotraction.

Unable.Noclearanceofheadfromsurface

Photographs/Notes

Score2–Chinonchest

Score1-Didn’tachievefullrangeofflexion


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Testitem11:GetstosittingStartingposition

Startingpositionsupineonthefloor,orlargeplinthwitharmsbyside.Nopillowmustbeusedunderhead.

Instruction Canyougetfromlyingintositting?

Scoringdetail Aimistomoveintolongsittingratherthansittingovertheedgeofaplinth.Useofonehandorarmisacceptabletoachieveascoreof2.

Score1ifpatientturnsintoproneortowardsthefloortoworktheirwayintosittingorifusestwoarms.Alsoscoreaoneifthechilduseslegmomentum/rockingtogettositting.

Activity 2 1 0

Getstositting Startsinsupine–mayuseonehand/armtopushup

Usestwoarms/pullsonlegsorturnstowardsfloororusesmomentum/rocking

Unable

Photographs

/notes

Score2-Onlyneedsonearmtositup

Score1-Pullswithbothhandsonlegs


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Testitem12:RisefromfloorStartingposition

Startingpositionsupinewitharmsbysides,legsstraight.Nopillowtobeused.

Instruction • Givethefollowingverbalinstructionstothepatient:• “WhenIsayGOcanyougetupasfastasyoucanandstandupstraight

withyourarmsbyyoursidelikeasoldierusingaslittlesupportaspossible”.Givethecommand“Ready,Set,GO!”andstartthestopwatchwhensaying“GO”.

• Stopthetimerwhenthepatientassumesanuprightpositionwithhisarmsbyhisside.

Scoringdetail/Diagram

Activitymustbeattemptedwithoutuseoffurnitureinthefirstinstance.Score1:Ifthepatientgetstostandingindependentlyofanyfurniturebutdemonstratesanypartofthemaneuverdescribedbelow.ComponentsofGower’smanoeuvre:Turnstowardsthefloor(intoafour-pointkneelingpositionorrollstoprone).Placeshandsonthefloortoassistrisingfromfloorandwalkshandsbackintowardshim.Usesoneorbotharmstopushuponlegstoachieveuprightstanding.Largebaseofsupportbyabductinghipsandextendingknees.

Activity 2 1 0

Risefromfloor NoevidenceofGower’smaneuver.

Exhibitsatleastoneofthecomponentsdescribedabove–inparticularrollstowardsfloor,and/orusehand(s)onlegs

(a)NEEDStouseexternalsupportobjecte.g.chair,wall–stillrecordtimeOR(b)Unable(timemustbeenteredasN/Aifthepatientscores0)

Photographs/Note

Score2-Doesn’troll.Only1handonfloortomoveintositting

Score1–Rollstoprone

Score1-Useshandonthigh

Score0–Useschair


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Testitem13:StandsonheelsStartingposition Standingonthefloor.Noshoestobeworn.

Instruction Canyoustandonyourheels?Count‘And1,and2,and3.”

Scoringdetail Score2:Mustclearbothfeetatthesametimeusingcleardorsiflexion

Score1:Ifbothforefeetarelifted-allmetatarsalheadsoffloorevenifinversionisevidentorcleardorsiflexionofonefoot.

Score0:Ifusesinversionwithlateralborder(fifthmetatarsal)offootstillonthegroundorifjustliftstoes.

Activity 2 1 0

Standonheels Bothfeetatthesametime,clearlystandingonheelsonly(acceptabletomoveafewstepstokeepbalance)forcountof3

Raisesforefootonbothfeet–allmetatarsalheadsoffground–orclearlydorsiflexesonefootonly

Unable

Photograph/Notes

Score2–Bothfeetraised

Score1-Bothfeetraisedbutonlyforefeet

Score0–Unabletoraiseevenforefeet


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Testitem14:JumpStartingposition

Standingonthefloor,feetcomfortablyclosetogether

Instruction Howhighcanyoujump?

Scoringdetail Wantheight,notforwardmovement.Smallamountofforwardmovementacceptable

Activity 2 1 0

Jump Bothfeetatthesametime,clearthegroundsimultaneously

Onefootaftertheother(skip)ordoesnotfullyclearbothfeetatthesametime

Unable

Photographs/Notes

Score2–Clearsfloor

Score1-Onestepaftertheother(skip)

Score0-Feetdonotleavefloor


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Testitem15&16:HopRight&LeftlegStartingposition

Startingpositionstandingonflooronright/leftleg.

Instruction Canyouhoponyourright/leftleg?

Scoringdetail Score2:Needsobviousfloorclearance.Mustlandononefoot.Ifleavesfloorbutlandswithtwofeetthisisnotahop.

Activity 2 1 0

Hop Clearsforefootandheelofffloor

AbletobendkneeANDraiseheel,nofloorclearance

Unableoronlyraisesheel

Photographs/Notes

Score2–Clearsfootofffloor

Score1-Bendskneebutfootdoesnotleavefloor


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Testitem17:Run(10metres)Startingposition

Standing

Instruction Givethefollowingverbalinstructionstothepatient:“WhenIsayGO,yougoasfastasyousafelycanallthewaytome.Ifyoucanrun,thenrun.Ifyoucan’tthenwalktomeasfastasyoucan.”(Encouragechildtorunpastthe10-metremark)bystandingatleastonemetrebehindtheline.Givethecommand“Ready,Set–GO”.Continuouslyencouragethepatientuntilhecrossesthefinishline.

Startthetimerwhenyousay“GO”.Stopthetimerwhenthesecondfootclearsthefinishline.

Scoring

detail

Astraight12-mwalkwaymustbeclearlymarkedat10-minaquietdepartmentorcorridor.Astopwatchmustbeusedtotimethewalk.Theymustself-selectspeedafterbeingaskedtogo‘asfastastheycan’.Score1-‘Duchennejog’-notatruerun(Thereisadoublesupportphase),butitisfasterthanawalk.Characterisedbyexcessiveuseofarms,trunkrotation,substantial’waddle’.Noreal‘push-off’.

Activity 2 1 0

Run Bothfeetofftheground(nodoublestancephaseduringrunning)

‘Duchennejog’orfastwalk

Walk(Noacceleration)

Photographs/Notes

Score2-Bothfeetofftheground

Definite‘run’

Score1-Picksupspeedbutwaddlesandhasonefootincontactwithflooratalltimes


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NSAABriefTestDetailActivity 2 1 0 Score

1.Stand Standsupright,stillandsymmetrically,withoutcompensation(withheelsflatandlegsinneutral)forminimumcountof3seconds

Standsstillbutwithsomedegreeofcompensation(e.g.ontoesorwithlegsabductedorwithbottomstuckout)forminimumcountof3seconds

Cannotstandstillorindependently,needssupport(evenminimal)

2.Walk Walkswithheel-toeorflat-footedgaitpattern

Persistentorhabitualtoewalker,unabletoheel-toeconsistently

Lossofindependentambulation–mayuseKAFOsorwalkshortdistanceswithassistance

3.Standupfromchair

Keepingarmsfolded.Startingposition90ºhipsandknees,feetonfloor/supportedonaboxstep.

Withhelpfromthighs/pushonchair/proneturnoraltersstartingpositionbywideningbase.

Unable

4.Standononeleg–right

Abletostanduprightinarelaxedmanner(nofixation)forcountof3seconds


Unable

5.Standononeleg-left

Abletostanduprightinarelaxedmanner(nofixation)forcountof3seconds


Unable

6.Climbboxstep-right



Unable

7.Descendboxstep–right



Unable

8.Climbboxstep-left



Unable

9.Descendboxstep-left



Unable

10.Lifts Insupine,headmustbeliftedinmid-line.Chin

Headisliftedbutthroughsideflexionorwithnoneck

Unable


29

Activity 2 1 0 Scorehead movestowardschest flexion(protracts)

11.Getstositting

Startsinsupine–mayuseonehand/armtopushup

Usestwoarms/pullsonlegsorturnstowardsfloor.

Unable

12.Risefromfloor

NoevidenceofGower’smaneuver.

ExhibitsatleastoneoftheGower’scomponentsdescribed–inparticularrollstowardsfloor,and/orusehand(s)onlegs

(a)NEEDSexternalsupportofobjecte.g.chairOR(b)Unable

Timed

13.Standsonheels

Bothfeetatthesametime,clearlystandingonheelsonly(acceptabletomoveafewstepstokeepbalance)forcountof3

Raisesforefootonbothfeet–allmetatarsalheadsoffground–orclearlydorsiflexesonefootonly

Unable

14.Jump Bothfeetatthesametime,clearthegroundsimultaneously

Onefootaftertheother(skip)ordoesnotfullyclearbothfeetatthesametime.

Unable

15.Hoprightleg

Clearsforefootandheelofffloor

Abletobendkneeandraiseheel,nofloorclearance

Unable

16.Hopleftleg

Clearsforefootandheelofffloor

Abletobendkneeandraiseheel,nofloorclearance

Unable

17.Run(10m)

Bothfeetofftheground(nodoublestancephaseduringrunning)

‘Duchennejog’orfastwalk Walk Timed

TOTAL=/34


30

8. TimedGradedFunctionalactivities

RiseFromFloor

Placetheparticipantinasupinepositionwithhisarmsbyhissidesandhislegstogether.Noshoesorsocksshouldbeworn.

Givethefollowingverbalinstructionstotheparticipant:“WhenIsayGOyougetupasfastasyoucanandstandupstraightwithyourarmsbyyoursidelikeasoldier”.

Givethecommand“Readysteady–GO”andstartthestopwatchwhensaying“GO”.

Stopthetimerwhentheparticipantassumesanuprightpositionwithhisarmsbyhisside.

Onlyprovideachairaftertheparticipanthasattemptedtostandfromthefloorfor30secondsandfailed.

Recordthefastesttimeandthemethodtheyusedtoachievethisfastesttime.Youcanrepeatifyouthinktheycandoitfasterorthegradewasnotrepresentativeoftheirusualability.

Assignoneofthefollowinggrades:

NSAAequivalent

grade

FunctionalGrade

Description

0 1 Unabletostandfromsupine,evenwithuseofachair

DONOTRECORDTIME

0 2 Requiresfurnituretostandupfromsupinetofulluprightposture

DONOTRECORDTIME*

1 3 Rollsover,standsupwithbothhands“climbingup”thelegstoachievefulluprightposture.

TIME

1 4 Rollsover,standsupwith1handsupportonleg TIME

1 5 Rollstothesideandstandsupwithoneorbothhandsonthefloortostarttorisebutdoesnottouchlegs

TIME

2 6 Standsupwithoutrollingoverorusinghandsonlegs

TIME

*Usingfurnituretogetupmakesiteasier/fasterandiftheyhavenotusedfurniturebeforeafastertimewithfurniturewouldnotreflectprogressionaccurately


31

10-MetreRun/Walk

10mrunisgradedduringtheperformanceofthetestfortheNSAA.Youcanrepeatthetesttocheckthetimeorthegrade.

Thistestmeasureshowfastthesubjectcanrun/walk10metres.

• Askthesubjecttostanduprightwithbothfeetbehindthestartinglineandtoestouchingthestartingline.

• Theevaluatorshouldstandata12-metremark.Youstand2metrespastthe“finishline”

• Givethefollowingverbalinstructionstothesubject:WhenIsayGO,yougoasfastasyousafelycanallthewaytome

• Givethecommand“readyset–GO”andstartthestopwatchwhensaying“GO”.

• Continuouslyencouragethesubjectuntilhecrossesthefinishline.

• Stopthetimerwhenthesecondfootclearsthefinishline.

Note:Encouragethechildtokeeprunningacrossthefinishlineandnottoslowdownbeforethe10-metremark.

Scorethefastesttimeandrecordthemethodtheyusedtocompleteitinthefastesttime.

Assignoneofthefollowinggrades:

NSAAequivalentgrade

FunctionalGrade

Description

0 1 Unabletowalkindependently

0 2 Unabletowalkindependentlybutcanwalkwithknee-ankle-footorthosis(KAFO)orsupportfromaperson

0 3 Cannotincreasewalkingspeed,highlyadapted,wide-basedlordoticgait

1 4 Canpickupspeedbutcannotrun,moderatelyadaptedgait

1 5 Nearlyrunning,butrunswithadoublestancephase,i.e.,cannotachievebothfeetofftheground

2 6 Runsandgetsbothfeetofftheground(achievesflight)


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9. Timed4-Stairclimbanddescend

TheTimed4-StairClimb(or4-StepTest)quantifiesthetimerequiredforthesubjecttoascendanddescend4standardsteps(6inchesheight).Themethodusedtoascendordescendthe4stairsisalsorecordedinordertomonitoranychangeintechniqueovertime.

TestGuidelines

• Evaluatethesubjectforanysignofmusculoskeletalinjurythatmayaffectthetest.Thetestsarenottobeperformediftheinjuryaffectstheirperformance.

• StairAscend:Ensurethatthechildstopsatthetopofthestairsanddoesnotturnaroundatthetopofthestairs.

• StairDescend:Ensurethatthechildstopsatthebottomofthestairsanddoesnotcontinuewalkingawayfromthebottomstep.

• Ifitisunsafetoperformthistest,donot.Includethereasonsfornotperformingthetestinthe‘Comments’sectionoftheworksheet.

• Thepurposeofthistestistoassessthequickesttimethatasubjectcanascendanddescendthe4steps.Encouragethesubjecttoperformthetestattheir‘fastest’or‘quickest’speed.

Ifyoufeelthesubjectcouldascendthestepsmorequickly,youmayaskthemtorepeatit.

Note:Thetestshouldnotberepeatedtoimprovethequalityofthemovement.Scorethequality/methodusedwiththefastesttimefrombothascendanddescend

Conductingthe4StairClimb–Ascend

• Askthesubjecttostandcentredatthefootofthestairswithhisarmsathissides(ie,handsmaynotstartonthehandrails)andnofurtherthan20cmawayfromtheedgeofthestep

• Giveinstructionstothesubjectinawayhecanunderstandsimilarto:

• “WhenIsayGO,climbupthestairsasfastasyoucan,safely.Whenyougettothetop,stopwithyourarmsatyoursidelikeasoldier!Youcanusetherailsifyouneedthemtogofast,butnotuntilIsayGO!”

• Givethecommand,“Ready,Set,GO!”Startthestopwatchwhenyousay“GO!”

• Stopthetimerwhenthesubjectassumesanuprightpositionwithhisarmsbyhissideonthetopplatformofthestairswhenascending.

• Ifhedoesnotstopwitharmsathisside,youmayaskhimtorepeatthetest,ifyoufeelamoreaccuratetimecouldbeachieved.

• Ifyoufeelhecanperformthetestfaster,youmayaskhimtorepeatthetest,ifyoufeelamoreaccuratetimecouldbeachieved.


33

• Administerthetestagainifyoufeelhecouldgofasterbyusingadifferenttechniquebutlimitthenumberoftrialstoamaximumof3.Ifthesubjectisunabletocompletethetestinunder30seconds,itisnotnecessarytocontinuetoencouragethesubjecttocompleteit.Itisnotrequiredtorepeatthetestifthetimewas30secondsormore.

• Recordthefastesttime,totheone-tenthofasecond.

• Documentthemethodthesubjectusedtocompletetestinthefastesttime.

Methodusedtoascendstairs:

Score Method

1 Unabletoclimb4stairs

2 Abletoclimb4stairsmarkingtime*usingbotharmsonhandrail/sorlegorbody

3 Abletoclimb4stairsmarkingtime*usingonearmononehandrailoronearmonalegorbody.

4 Abletoclimb4stairsmarkingtimewithoutusinghandrailorusingarms

5 Abletoclimb4stairsalternatingfeetusinghandrail/sorlegsorbodyforsupport

6 Abletoclimb4stairsalternatingfeetwithoutusingarmsforsupport

*Markingtime=climbsonefootatatime,withbothfeetonastepbeforemovingtonextstep

Conductingthe4StairClimbDescend

• Askthesubjecttostandcentredontheplatformatthetopofthestairswithhisarmsathissides(ie,handsmaynotstartonthehandrails).

• Giveinstructionstothesubjectinawayhecanunderstandsimilarto:

• “WhenIsayGO,climbdownthestairsasfastasyoucan,safely.Whenyougettothebottom,stopwithyourarmsatyoursidelikeasoldier!Youcanusetherailsifyouneedthemtogofast,butnotuntilIsayGO!”

• Givethecommand,“Ready,Set,GO!”Startthestopwatchwhenyousay“GO!”

• Stopthetimerwhenthesubjectassumesanuprightpositionwithhisarmsbyhissideatthebottomofthestairs.

• Ifhedoesnotstopwitharmsathisside,youmayaskhimtorepeatthetest.

• Ifyoufeelhecanperformthetestfaster,youmayaskhimtorepeatthetest.


34

Administerthetestagainifyoufeelhecouldgofasterbyusingadifferenttechniquebutlimitthenumberoftrialstoamaximumof3.

Ifthesubjectisunabletocompletethetestin£30seconds,itisnotnecessarytocontinuetoencouragethesubjecttocompleteit.Itisnotrequiredtorepeatthetestifthetimewas30secondsormore.

• Recordthefastesttime,totheone-tenthofasecond.

Methodusedtodescendstairs:

Score Method

1 Unabletodescend4stairs

2 Abletodescend4stairsmarkingtime*usingbotharmsonhandrail/sorlegorbody

3 Abletodescend4stairsmarkingtime*usingonearmononehandrailoronearmonalegorbody.

4 Abletodescend4stairsmarkingtimewithoutusinghandrailorusingarms

5 Abletodescend4stairsalternatingfeetusinghandrail/sorlegsorbodyforsupport

6 Abletodescend4stairsalternatingfeetwithoutusingarmsforsupport

*Markingtime=climbsonefootatatime,withbothfeetonastepbeforemovingtonextstep


35

10. PERFORMANCEOFTHEUPPERLIMB(PUL2.0)SCALE

Introduction

ThePerformanceoftheUpperLimb(PUL)Scalehasbeendevisedtoassessmotorperformanceintheupperlimbforindividualswithdystrophinopathies.PULhasbeendevisedbythePULPhysiotherapyWorkingGroup(AnnaMayhew,MichelleEagle,ElenaMazzone,MarionMain,MariaAsh,MeredithJames,MarleneVandenhauwe,KatrijnKlingels,TinaDuong,JulaineFlorence,ValerieDecostre).Workison-goingtoenableaconversionalgorithmfromPULV1.2toPULV2.Thisversion(2.0)hasbeenupdatedtoreflecton-goingpsychometricanalysisofthescaleproperties.

EquipmentRequired

Equipment

• Selectionofmetricweights-10g,100g,200g,500gand1000g.• 6Coins(Appropriatetoyourcountry–10pence’s,quartersor50eurocentsortokens)

• Pencil(standardHBpencil)• Plasticcup(vendingcup)• PlainA4paper• Ziploccontainer(8ounce)• Numberpadandtracingdiagram(endofworksheet)• 5stackableheavycansweighing400g+/-30g• Cansare7.5to8.0cmsindiameterand10.5to11.5cmsinheight

Plastictableclothmarkedwithcircles(Appendix1)

GeneralInstructions

• Ingeneralthereisascoringsystemof0,1,2(twoitemsscore0or1only)• Ideallythesameevaluatorshouldperformevaluationsatsubsequentassessments• Itemsshouldbeperformedintheorderprovidedinthismanual• Thesubjectshouldwearlooseclothing,Tshirtetc.• Forambulantindividualssitthesubjectonaplinthiftheydon’trequirebacksupportora

chairwithabackrestbutnoarmrestsiftheymaygettiredduringthisassessment.Ensuretheirfeetaresupported/onthefloor.Useadditionalbacksupportsuchasapillowifrequired.

• Fornon-ambulantsubjectsideallyuseachairwithabackrestbutnoarmrests.Forindividualsinapoweredchairorthoseunabletotransfereasilyensuretheirchairhastheseatpositionedashorizontallyaspossible,thebackrestinanuprightpositioniftoleratedandthatthefeetaresupported,noarmrests.Itmayberequiredtodisconnectthe


36

controller.Testtheentryitemandiftheyachieve≤3,thearmrestsdonotneedtoberemoved.

• Noassistivedevicesaretobeusedforanyofthetests• Werecommendthataplinth/adjustabletableisnotusedtositonforthetestsifthe

subjectisunabletositsafelyandcomfortablywithoutabacksupport.• Anadjustableheightfirmtableshouldbeused.Heightissetindividuallyforeachsubjectat

thebeginningatacomfortableworkingposition(shouldersflexedtoapproximately30degreesflexion,forearmsrestingontablewiththeelbowsoffthetable)

Toolow TooHigh Correct

• Placethetablecloth(seeappendix1)ontheproximaledgeoftheadjustableheighttablewiththecentralcirclecentredtothesubject

• Ifstartingpositioncannotbemaintainedbecauseofcontractures,scoreis0unlessitemsstatesthatstartingpositioncanbeadaptedwhichmaythecaseinclinic


37

Conductingtheassessment

Intheworksheet,indicate:

• Preferredarmisdefinedastheoneusedtodraworwriteor,ifunable,thearmmostfrequentlyusedtoperformtasks.ItemsofthePULscaleshouldbeperformedbythepreferredarm(dominantornot)butaneffortmustbemadetousethesameonethroughoutandatsuccessivevisits.

• Makeanoteofsignificantelbowcontracturesandsupinationrangeofmotionandmarkontheworksheetifthescorewaslimitedbythese.Elbowcontractureshouldbeexpressedasitsdegreesfromfullelbowextensione.g.-10

• Ambulantisdefinedasabletowalk10mwithoutanykindofsupportandnonambulantasunabletowalk10mwithoutanykindofsupportororthoses

Score3,4,5or6onEntryitem

PerformItemAEntryItem

ContinuewithItem1toendof

test

StartatItem7onpage2

Score2orlessonitemA


38

*Iftheyscore6onItemA–adaptedBrooke–ItisnotnecessarytoperformthePULunlessyoufeelfunctioniscompromisedforadditionalreasons.

A. Entry item – Not included in total score – Adapted Brooke Scale Equipment: 200gweightandplasticcup.Coinandapencilifnecessary.

Startingposition: Supportedsitting,startwithhandsonlap,notableinfrontofthem.

Instruction:

“Raiseyourarmsouttothesideandaboveyourheadlikethis;tryandkeepyourelbowsstraight.”(Demonstrate)Forthemostableindividuals,demonstrateabductionofbotharmssimultaneouslywithelbowsinextension.Iftheyarenotable,askthem:“Canyoubringyourhandstoyourmouth?”Iftheybendtheirheadtotheirhandsinstructthemtokeeptheirheadupiftheycan.

Scoringdetails Score4:Definedas90degreesshoulderabductionsimultaneouslyScore2and3.Theyshouldnotdothisbybringingtheirmouthtotheirhands.Iftheyusesignificantheadandtrunkflexioninordertocompletethetaskmeanstheycannotscore2or3forthisitem.

Item Description 0

1 2 3 4 5 6

A.

Entryitem Nousefulfunctionofhands.

Canusehandstoholdpenorpickupacoinordriveapoweredchair

Canraise1or2handstomouthbutcannotraiseacupwitha200gweightinittomouth

Canraisestandardisedplasticcupwith200gweightinittomouthusingbothhandsifnecessary.

Canraisebotharmssimultaneously(elbowstoshoulderheightwithorwithoutcompensation.Elbowbentorinextension

Canraisebotharmssimultaneouslyaboveheadonlybyflexingtheelbowshorteningcircumferenceofthemovement/usingaccessorymuscles)

Canabductbotharmssimultaneouslyelbowsinextensioninafullcircleuntiltheytouchabovethehead.


39

HIGHLEVELSHOULDERDIMENSION

1. Shoulder abduction arms above head (scored from Entry item A) Equipment:

Startingposition:

Supportedsitting,startwithhandsonlap,notableinfrontofthem

Finalposition Botharmsextendedaboveheadthroughabduction

Instruction: “Raiseyourarmsouttothesideandaboveyourheadlikethis;tryandkeepyourelbowsstraight.”(Demonstrate)

Scoringdetails Thisitemisscoredfromtheentryitemabove.Ascoreof2istheequivalentofascore6intheentryitemandascore1isequaltoascoreof5intheentryitem.Score4orbelowintheentryitemscores0inthisitem.

Item Description 0 1 2

1

ScorefromEntryitemabove

ShoulderabductionbotharmsaboveheadRaiseyourarmsouttothesideandaboveyourheadlikethis;tryandkeepyourelbowsstraight.”(Demonstrate)

Unable Canraisebotharmssimultaneouslyaboveheadonlybyflexingtheelbow-withcompensation

Canabductbotharmssimultaneouslyelbowsinextensioninafullcircleuntiltheytouchabovethehead


40

2. Raise both arms to shoulder height – (Elbows at shoulder height) Equipment:

Startingposition Supportedsitting,startwithhandsonlap,notableinfrontofthem

Finalposition Botharmsextendedtoshoulderlevelthroughabduction

Instruction: “Raiseyourarmsouttothesidelikethis;tryandkeepyourelbowsstraight.”(Demonstrate)

Scoringdetails Score1-Thearmscanbestraightorflexedbutitistheheightofelbowswhichcountsandtheshouldershouldbeabductedto90degrees


2

Raisebotharmstoshoulderheight(elbowsatshoulderheight)“Raiseyourarmstoshoulderheightouttotheside”

Unable Canraisebotharmstoshoulderheightwith

anymethodegonearmatatimeorusingonehandtohelptheother

Canraisebothelbowstoshoulderheight

simultaneouslywithoutcompensation

e.g.simultaneouslywithelbowsstraight


41

3. Shoulder flexion to shoulder height (no weights)

4. Shoulder flexion to shoulder height with 500g Equipment: 500gWeight

Startingposition:


Positionyourselftoensurethatcompensationisvisibletoyou

Finishposition Armextendedinfront(elbowtoshoulderlevel)–throughflexionandtouchingyourhandNote:theforearmshouldbeinneutralnotpronatedorsupinated.Themovementisacombinationofalmostsimultaneousforwardflexionandelbowextension

Testingsequence Testusingnoweightfirst–Item3.

Thengivethe500gweighttotheindividualintheirlap-Item4

Instruction: Item3:“Handonlap-Reachoutandtouchmyhand”–elbowtoshoulderlevelItem4:“Handonlap–givemetheweight”Alwaystakeweightfromthemoncetheyhavereachedmaximumheight(toavoideccentricmusclework).

Scoringdetails Thepropermovementwithoutcompensationisasimultaneouscombinationofshoulderflexionandelbowextension.Absenceorasynchronyofoneofthesecomponentsoradditionalmovementisconsideredascompensation.Score2ifachievedwithoutcompensation.Score1ifachievedwithcompensationbutstillachievethecorrectendposition.


42

Item Description 0 1 2 Score

3

Shoulderflexiontoshoulderheight(noweights)“Reachoutandtouchmyhand”

Unable Ablewithcompensation

Ablewithoutcompensation

4 Shoulderflexiontoshoulderheightwith500gweightHandonlap–“givemetheweight”

Unable Abletolift500gweightwith

compensation

Abletolift500gweightwithoutcompensation


43

5. Shoulder flexion above shoulder with 500g weight

6. Shoulder flexion above shoulder with 1 kg weight Equipment: 500gand1kgWeight

Startingposition:


Positionyourselftoensurethatcompensationisvisibletoyou

Finishposition Armextendedinfront(elbowtoeyelevel)–throughflexionandplacingweightinyourhand

Note:theforearmshouldbeinneutralnotpronatedorsupinated.Themovementisacombinationofalmostsimultaneousforwardflexionandelbowextension

Testingsequence Testusingnoweightfirst.Proceedtobothadditionalitemsastheymaybeabletocompletethetaskusingcompensation.

Instruction: First“Handonlap,reachoutandtouchmyhand”–elbowtoeyelevel

Item5and6:“Handonlap–givemetheweight”

Alwaystakeweightfromthemoncetheyhavereachedmaximumheight(toavoideccentricmusclework).

Scoringdetails Thepropermovementwithoutcompensationisasimultaneouscombinationofshoulderflexionandelbowextension.Absenceorasynchronyofoneofthesecomponentsoradditionalmovementisconsideredascompensation.Theyscore2iftheydonotusecompensation.Theyscore1iftheyusecompensationbutstillachievethecorrectendposition.Iftwohandsareusedthisisunableandscores0.Otherarmmustremainintheindividualslap


44

Item Description 0 1 2 Score

5

Shoulderflexionaboveshoulderheight–500gweight“Reachoutandtouchmyhand”–elbowtoeyelevel

Unable Abletolift500gwith

compensation

Abletolift500gwithout

compensation

6 Shoulderflexionaboveshoulderheightwith1kgweightHandonlap–“givemetheweight”–elbowtoeyelevel

Unable Abletolift1kgweightwith

compensation

Abletolift1kgweightwithoutcompensation


45

MIDLEVELELBOWDIMENSION

7. Hand(s) to mouth Equipment: 200gweightandcup,adjustableheighttable

Startingposition: SupportedsittingatacomfortableworkingpositionAcupcontaining200gweightisplacedintheproximalcentralcircleofthetablecloth.

Thetaskisperformedusingthepreferredhandortwohandsifnecessary

Instruction: “Bringthecuptoyourmouthwithonehand”

Scoringdetail Toscore2theymustbringthecuptotheirmouthwithoutcompensation.Theelbowshouldnotbesupportedonthetableandthecupmustreachthemouthratherthanmouthbebroughttothecup.i.e.thereshouldnotbeexcessivetrunkorneckmovement.

Toscore1or2thehandorthecupshouldjusttouchthelipsbriefly.

Toscore1theycanuseanycompensation.Theyarescoredforfunctionratherthanquality.Twohandsisdefinedaseitherusingtwohandsonthecuporreinforcementofonehandontheotherarm.Elbowsmaybesupportedonthetableandtrunkandneckflexionareallowed.Thisitemisaboutfunctionnotperfecttechnique.


7.

Hand(s)tomouth

“Bringthecuptoyourmouthwithonehand”

Unable Abletobring200gincupWITHcompensationto

mouth

(useselbowsupport,morethanonehandand

/orbringheadtohands/orothermethod)

Abletobring200gincuptomouthwithonehandno

elbowsupport

(withoutcompensation)


46

8. Hands to table from lap Equipment: HeightadjustableTable

Startingposition: Supportedsittingwithbothhandsonlap.

Instruction: “Bringbothhandsfromlaptotableatthesametime”(Demonstrate)

Scoringdetails

Score2:Theymustmovebothhandsatthesametime.Completelyontableisdefinedastothewristcrease.Score0:Thisisunableandincludesiftheycanonlybringtheirfingertipsofoneortwohandsontothetable


8.

Hand(s)totablefromlap“Bringbothhandsfromlaptotable”

Unable Abletobringtwohandscompletely(towristcrease)ontotablebut

NOTsimultaneouslyorinoneaction

Twohandscompletelyontablesimultaneously


47

9. Move weight on table 100g

10. Move weight on table 500g

11. Move weight on table 1kg Equipment: Weights.Tableclothwithcircles

Startingposition:

Supportedsittingatacomfortableworkingposition.Ifnotpossibleduetoweakness/contractures,anadaptedpositionisallowed.

Theweightismovedfromtheoutercircleonpreferredsidetocentrecircleonthetablecloth.Startwiththe100gweight

Instruction: “Movethisweight(100g,500g,1kg)fromoutsidecircletocentrecircle”

Scoringdetails Score2:Noweightslidingorcompensationoradaptedstartingpositionisallowed.Aliftisdefinedashand,forearmandelbowclearofallsurfacesandtheweightmustbecompletelyinsidethecentralcircle.Shouldermustnotabducttoraisetheelbow.

Score1:Slidingandcompensationisallowedbuttheslidecannotbeperformedinmultiplesmallincrements(shouldbeonesmoothslide)orwithmorethanonehand.Theycanusecompensationsuchasabductedshoulderorkeepingforearmincontactwithtable.

Score0:Ifunableorusestwohandsonweight

9.

Moveweightontable100g

“Movetheweightfromoutsidecircletocentrecircle”

Unable Canmove100gweightfromoutertocentrecircleusing

compensation

(slideforearmorelbowmakecontactwithtable)

Canlift100gweightfromoutertocentrecirclewithoutcompensation

10. Moveweightontable500g

“Movetheweightfromoutsidecircletocentrecircle”

Unable Canmove500gweightfromoutertocentrecircleusing

compensation(slideforearmorelbowmake

contactwithtable)

Canlift500gweightfromoutertocentrecirclewithoutcompensation

11. Moveweightontable1kg

“Movetheweightfromoutsidecircletocentrecircle

Unable Canmove1kgweightfromoutertocentrecircleusing

compensation(slideforearmorelbowmake

contactwithtable)

Canlift1kgweightfromoutertocentrecirclewithoutcompensation


48

12. Lift heavy can diagonally Equipment: 1heavycan

PlastictableclothmarkedwithcirclesStartingposition: Supportedsittingatacomfortableworkingposition

Ifnotpossible,adaptedpositionisallowed.

Tableclothisplacedontablewiththeedgelineduptotheedgeofthetable.

Thetaskisperformedusingthepreferredhand.Theheavycanisplacedontheproximaloutercircle.

Instruction: Evaluatorwillmeasurearmlengthanddeterminecircletargetthatappropriatelyreflectsarmlength.“Liftthiscanfromthiscirclenearestyourhandtothiscirclefurthestawayandacrossyourbody”(Iftheirarmlengthisnotsufficienttoreachthefurthestcircleaslongastheyareontherighttrajectorymaybescoredas1or2.

Theyshouldnotleanonthetablewiththeirotherarm.

Scoringdetails

Score2:Compensatorymechanismsarenotallowed.Aliftisdefinedashand,forearmandelbowclearofallsurfacesandtheweightmustbeatleast85%insidethecircle.Score1:Compensationisallowed.Theweightcanbeslidorthearmmayrestonthetable.Slidingmustbeperformedinonefluidmotion.Scootingofweight(smallpushes)isnotallowed.

0 1 2

12.

Liftheavycandiagonally“Liftthiscanfromthiscirclenearestyourhandtothiscirclefurthestawayandacrossyourbody”

Unable Canmoveheavycanfromnearestcircleacrossbody

withcompensation(slideforearmorelbowmakecontactwithtable)

Canliftheavycanfromnearestcircleacrossbodywithout

compensation


49

13. Stack of three heavy cans

14. Stack of five heavy cans Equipment: Fiveheavystackablecans

Plastictableclothmarkedwithcircles

Startingposition:

SupportedsittingatacomfortableworkingpositionIfnotpossible,adaptedpositionisallowedbutonehandmustbeonthetable.Theotherontheirlap.

Tableclothisplacedontablewiththeedgelineduptotheedgeofthetable.

Thetaskisperformedusingthepreferredhand.

Thecansarearrangedontherowofcirclesclosesttothesubject.ForItem13,Thesubjectstacksthetwocansonthedominantsideontothemiddlecan.followedbythetwocansonthenon-dominantareusedtoscoreitem14.

Subjectplaceshandontableneartotheoutermostcanonthedominantside

Instruction: Item13+14-“Stackthesefivecans,oneatatimeonthemiddlecanusingonehand.”

“Tryandliftthecanlikethis”demonstrateliftingbythebodyofthecannotfromthetopofthecan.Liftingfromthetopiscompensation.

Scoringdetails

Score2–Nocompensation

Score1–compensationisallowed(E.g.liftingfromtopofcan,abductingandliftingarmexcessively).Usingtwohandsisnotallowed.

Score0–Iftheyusetwohands

Score2orlessonitemA


50

Item 0 1

2 Score

13.

Stackofthreecans“Stackthesetwocans,oneatatimeonthemiddlecanusingonehand”

Unabletostack

thirdcanevenwithcompensation

Able to stack thirdcan withcompensation

Abletostackthirdcanwithoutcompensation

14. Stackoffivecans“Stackthesetwoadditionalcans,oneatatimeontopofthiscanusingonehand”

Unabletostack

fifthcanevenwithcompensation

Able to stack fifthcan withcompensation

Abletostackfifthcanwithoutcompensation


51

15. Remove lid from container Equipment: Ziploccontainer

Startingposition:

SupportedsittingatacomfortableworkingpositionIfnotpossible,adaptedpositionisallowedbuthandsmustbeonthetable.

Containerontabledirectlyinfrontofthem

Nocoinsincontainer

Instruction: “Useyourhandsopenthiscontainer”

Scoringdetails Score1:Anymethodmaybeused.

Item Description 0 115.

Removelidfromcontainer

Unabletoopen.

Openscompletely

16. Tearing paper Equipment: SeveralsheetsofA4paperStartingposition:

SupportedsittingatacomfortableworkingpositionIfnotpossible,adaptedpositionisallowedPassthesubjectapieceofpaperthathasbeenfoldedin4.Thesheetofpapercanbefoldedin2iftheyareunabletoperformthetaskwithitfoldedinfour.Theycan’tholdthepaperagainsttheirbodyandmustusefingertipstorip

Instruction: “Tearthesheetofpaperbeginningfromhere”Thisisdefinedasthecentreofthefoldededgeandstraightacross

Scoringdetails Toscoreanitemtheymustcompletethetearacrossthewidthofthepageinnomorethan3attempts.

Item Description 0 1 216.

Tearingpaper

Unable Tearsthesheetofpaperfoldedinhalffromthefoldededge

Tears the sheet ofpaper folded in4,beginning from thefoldededge


52

DISTALWRISTANDHANDDIMENSION

17. Tracing a path Equipment: Standardpencilanddiagram

Startingposition:

Supportedsittingatacomfortableworkingposition

Thepencilisplacedinthehandofthesubjectbytheevaluator.

Writeintheusualwritingposition,tostart,papercanbepositionedbythesubjectbutmustnotbemovedthereafter

Thetaskisperformedusingthepreferredhand.Anyhandholdonthepencilcanbeused.

Instruction: “Useyourpenciltocompletethepathinonesmoothmovement”

Scoringdetails

Description 0 1 2

17.

Tracingpath Unable Abletocompletethepathwithcompensation–needstoraisepencilfrompaperorpivotarm

Abletocompletethepathwithoutstopsorraisinghandfrompaper

Thefullsizedrawingis

ontheworksheet

Forillustrativepurposeonly


53

18. Push on light Equipment: Pushbuttonlight

Startingposition:


Thebuttonlightisplacedonthetablebetweentheperson’shands.

Instruction: “Pushonthelightwiththefingersofonehand”

Scoringdetails Thelightshouldremainonthetable.Theycanonlyusefingersofonehandtoactivatethelight

Avoidshoulderabductiontouseweightofshouldertoturnonlightandstabbingthelightisnotallowed

Theelbowmustnotbehigherthanthewrist


18.

Pushonthelight

Unable Abletoturnthelightonmomentarilywithfingersofonehand

Abletoturnthelightonpermanentlywithfingersofonehand


54

19. Supination Equipment: Pushbuttonlight

Startingposition:


Placepushbuttonlightonthetableinfrontoftheindividualbetweenhishands.

Instruction: “Pickupthelightandturnyourhandover”(demonstratesupinationaction)

Scoringdetails Mustusesupinationratherthanshoulderadductiontoscore2

0 1 2

19.

Supination Unable Picksupthelightbuteitherturnshandsoverincompletelyorusescompensationtoturnitover

Picksupthelight,andturnsthehandovercompletelywithnocompensatorymovements


55

20. Pick up coins Equipment: 6Coins/TokensStartingposition:

SupportedsittingatacomfortableworkingpositionIfnotpossible,adaptedpositionisallowedbuthandsmustbeonthetable.Placecoinsonthetableinfrontoftheindividual.

Instruction: “Usingonehand,pickup6coins,oneatatime”

Donotslidethecoinstotheedgeofthetableorstackthemontopofoneanother


20.

Pickupcoins

Cannotpickuponecoin

Canpickuponecoin

Canpickupsixcoinsusingonehand


56

21. Placing finger on number diagram Equipment: Numberdiagram

Forillustration

only

1 2 3 4

* * 5*

* 6 **

7 8 910

Startingposition:


Placediagramonthetableinfrontoftheindividual.Papercanbeorientedandheldsteadyeitherbytheevaluatororthesubject.

Instruction: “Usingonefingertotoucheachnumberonthediagram”

Scoringdetails 0 1 2

21.

Placingfingeronnumberdiagram(precisionnotessential)

Cannotraisethefingerorslideitonthediagram

Abletoplacefinger(slideorlift)betweenatleasttwosquares

Abletoplacefingersuccessivelyonthenumbersofthediagram

(withorwithoutcompensation)


57

22. Pick up 10g weight finger pinch Equipment: 10gweightStartingposition: Supportedsittingatacomfortableworkingposition

Placeweightonthetableinfrontoftheindividualclosetohishand.

Instruction:“Pickupthissmallweightlikethis”

Scoringdetails Toscore1theycangripandliftthis10gweight 0 1

22.

Pickup10gweightfingerpinch

Unable

Abletogripandliftweightoffsurface


58

11. EgenKlassifikationScale1-17items

TestDescription

TheEKscalewasoriginallydevelopedtoassessfunctionalabilityafterlossofambulationinsubjectswithDMD.TheEK2scaleisanordinalscalerangingfrom0to51points,where0representsthehighestlevelofindependentfunctionand51thelowest.Thescaleconsistsof17categories(eachscoredfrom0to3)involvingvariousfunctionaldomains.TheEKscaleisadministeredbyinterviewingthesubjecttocapturehowheperformsactivitiesofdailylifeorhowheperceiveshiswell-being(asdescribedbyCategory10).Forthosetasksthatcanbeobservedintheclinic,theintervieweralsoobservesthesubjectperformingthetaskbeforeassigningascoreforthattask.

TestingGuidelines

• Thetestshouldbeconductedinthesubject’snormalwheelchairwiththeseatadjustedashorizontallyaspossible.

o Ifthesubjectusesabackorneckbrace,heshouldkeepthebraceon.

o Ifthenormalpositionoftheseatispermanentlyadjustedtotiltbackwards,thesubjectshouldbetestedinthisposition.

• Thetestsshouldbeconductedasaconversation.ThesubjectisinterviewedbytheCEabouthowhefunctions.Theonlytasksthesubjectmustdemonstratearequestions4,abilitytobalanceinthewheelchair,question5,abilitytomovethearms,andquestion8,abilitytocough.Ifthesubjectortheevaluatorisuncertainhowanactivityisperformeditispermissibletoobservetheactivity.

• Unlessotherwisestated,scoringisbasedonwhatthesubjectnormallydoesandnotwhatheiscapableofdoing.Exceptionstothisrulearetheitems“abilitytobalanceinthewheelchair”and“abilitytomovethearms”whicharedemonstrated.

• Informationmaybeacquiredbyacaregiver,ifthesubjectdoesnotknowtheanswertoaquestion.Itispreferablethatthesamecaregiverbeusedthroughoutthestudy.Thenameofthecaregiverprovidinginformationshouldberecordedateachtestingvisit.

• Ifascorefallsbetweentwocategories,theonethatrepresentsthehighestfunctionalability(lowestscore)shouldbechosen.


59

NSAAtoEK2-bridgingitem

Notintotalscorecurrentlyofthisscale–usefulrecordoftransitioningability

Questions:• Categories0-4:Howmanystepscanyoutakewithorwithoutassistance?

Scoring:• Category0:Abletotakemorethantwostepsbutwalkslessthan10metreswithout

assistance• Category1:Abletotakemorethantwostepsbutwalkslessthan10metreswithassistance• Category2:Takesoneortwostepswithoutassistance• Category3:Takeoneortwostepswithonepersonassistinginanyway• Category4:Unabletotakeanystepsbutcanbeassistedintoastandingpositionand

maintainstanding

EK1:Abilitytousewheelchair

Questions:• Categories0-3:Howdoyougetaroundindoorsandoutdoors?

• Categories0-2:Ifuncertainitisacceptabletoaskforademonstration.

Showmehow!

• Category3:Aretheresituationswhereitcanbedifficultforyoutosteerthewheelchair,forexample,whenit’scold?Thereisnoneedtoobservethis.

Scoring:• Category2:Theindividualsteersthechairwithoutdifficulty.

• Category3:Theindividualreportsthathecannotsteerthechairif,forexample,hisfingersarecold,heisdrivinguphill,iswearingalotofclothes,etc.

EK2:Abilitytotransferfromwheelchair

Questions:• Categories0-3:Howdoyoutransferfromthewheelchairtoabed?

• Categories0-1:Ifuncertainitisacceptabletoaskforademonstration.

o Showmehowyoudothis!

• Categories2-3:Areexplainedordemonstratedbythetestpersonandhelper,ifany.Thereisnoneedtoobserveapersonbeingliftedorhoisted.


60

Scoring:• Category2:Hoistwithoutheadsupportormayalsobeastandingtransfer,theperson’s

kneeagainstthehelper’sknee.

• Category3:Usesaliftslingthatreachesallthewayuparoundtheneckinordertosupporttheheadorneedstobeliftedwithsupportofheadwhentransferringfromwheelchair.

EK3:Abilitytostand

Questions:• Categories0-3:Doyousometimesstand?Howdoyoudothis?

• Categories0-2:Thereisnoneedtoobservethesubjectstanding.Howeverifyouareuncertainofthesubject’sabilityitispermissibletosayshowmeorexplaintomehowyoudoit!

Scoring:• Category3:Unabletobestood,mayormaynotbeduetocontractures.

EK4:Abilitytobalanceinthewheelchair(mustbeobserved)

Questions:• Categories0-3:Canyoubendforwardandtothesidesandreturntouprightposition?The

seatmustbeashorizontalaspossibleandnotbetilted.Anysidesupportsmustberemoved.Anelectricharnessmustnotonlybeusedtochangeposition.Abrace,ifany,iskepton.

Scoring:• Category0:Theupperbodymustreachallthewaydownandrestonthethighs,both

handsmusttouchthefootsupportandthepersonmustriseunaidedfromthisposition.Usingthehandstosupportandpropeltheupperbodyisallowed.

• Category1:Canbendtheupperbody>30degreesinatleastonedirection:eitherforwardortoonesideandreturntouprightposition.

• Category2:Canbendtheupperbodyto<30degreesinatleastonedirection:eitherforwardortoonesideandreturntouprightposition.

• Category3:Isunabletochangethepositionoftheupperbodyormoveawayfromthebackofthechair.Individualswhositinapermanentforwardleaningpositionwithsupporttothefrontofthebodyalsoscore3.

EK5:Abilitytomovethearms(mustbeobserved)

Questions:• Category0-3:Canyoumoveyourfingers,handsorarmsagainstgravity?Howmuchcan

youmovethem?

Scoring:• Category0:Canraiseatleastonearmabovethehead,compensatorymovementsallowed.


61

• Category1:Canraiseatleastoneforearmuptothemouth.Compensatorymovementsandforearmsupportareallowed.

• Category2:Canraiseatleastonehandagainstgravity.Forearmsupportisallowed.

• Category3:Unabletomovethehandsagainstgravitybutabletousethefingers.

EK6:Abilitytousethehandsandarmsforeating

Questions:• Category0-3:Canyoudescribehowyoueat?Maybeobservedifthesubjectisunclear–

showmehowyoueat.

Scoring:• Category0:Canraisecuporglassandeatusingknife,forkorspoonifrelevantwithout

needingelbowsupport

• Category1:Needselbowsupporttoeatand/ordrink

• Category2:Eatsanddrinkswithelbowsupport;withreinforcementoftheoppositehand+or–aidsand/orwithcompensatorymovementstobendthearm(forexample,bybendingtheforearmovertheedgeofthetableandusinghand-andwristmuscles).

• Category3:Hastobefed.

EK7:Abilitytoturninbed

Questions:• Category0-3:Howdoyouturninbedduringthenight?

Scoring:• Category0:Turnshimselffrombacktosidetobothsides;numberofturnsissubordinate.• Category1:Abletoturnpartiallyinbed.Mayneedsomehelporganizinglegsetc.orcan

turninonedirectionbutnotbackagain• Category2:Themostcommonnumberofturnsis0-3times.Thiscategoryincludesthose

thatcan’tturnanddon’tneedtobeturnedduringthenight.• Category3:Themostcommonnumberofturnsis4ormoretimes.Note:Sometimestheaide’sreportispreferable.Ifthereisalargedeviance,thequestionis

narroweddowntoanapproximateaverageduringtheprevious14days.

EK8:Abilitytocough

Questions:• Category0-3:Howdoyoucoughwhenyouhaveto?MayIhearhowitsounds?

Scoring:• Category0:Alwayscoughswithouthelp,powerfulcoughingsound.• Category1:Abletoclearthethroat,usuallymanageswithouthelp.• Category2:Coughswithsometypeofhelp.• Category3:Unabletocough.Needssuctionand/orhyperventilationtechniquesorIPPBin

ordertokeepairwaysclear.


62

Note:Iftheindividualreportsthathenevercoughs,evaluateaccordingtocoughsoundandthroatclearing.

EK9:Abilitytospeak

Questions:• Category0-3:Canyouspeaksopowerfullythatwhatyousaycanbeunderstoodifyousit

atthebackofalargeroom?Youmayaskthesubjecttoshout‘hello’orreciteasentenceloudlyifyouareuncertain.

Scoring:Thevoiceisevaluatedregardlessofwhethertheindividualusesassistedventilation.

• Category0:Powerfulspeech.Abletosingandspeakloudly.Isabletomaintainpowerfulspeechforalongperiodoftime.

• Category1:Speakswithasoftvoice(maybeabletoraisehisvoice,butnotforlongperiodsoftime).

• Category2:Speakswithaquietvoiceand/orneedsabreathafterjustafewwords.• Category3:Speechisdifficulttounderstandexcepttocloserelatives.

EK10:Physicalwell-being

Questions:• Category0-3:Howisyourphysicalwell-being?Howisyourappetite?Doyousleepwellat

night?Doyouhaveaheadacheinthemorning?Troublewakingupinthemorning?Howareyourmoods?Haveyoulostweight?Doyouneedtorestduringtheday?Areyouafraidtobealoneathome?

Note:Thisitemisrelatedtorespiratoryinsufficiency,nottomotordifficultiesorgastrointestinaldifficulties.

Scoring:• Category0:Nocomplaints,feelsgood.• Category1:Needstorestduringtheday.• Category2:Canalsoinvolveheadacheinthemorning,difficultygettingawake,weightloss.• Category3:Symptomsfromcategory2aresuppliedwithperspiration,fallingasleepduring

theday,stomachache,andanxiety.

EK11:DaytimeFatigue

Questions:• Category0-3:Doyouneedtorestduringtheday?Doyouneedtolimithowmuchyoudoin

aday?

Scoring:• Category0-Doesn’tgettiredduringday• Category1Needtolimitactivitytoavoidgettingtootired


63

• Category2-Needtolimitmyactivityandhavearestperiodtoavoidgettingtootired• Category3-GettiredduringdayevenifIrestandlimitactivity

EK12:HeadControl

Questions:• Category0-3:Note:Doyouuseyourheadrest(ifapplicable)whenyouareoutsideor

travellinginacar?Doyoualwaysneedtorestyourheadontheheadrest?

Scoring:• Category0:Doesnotneedheadsupport• Category1:Needsheadsupportwhengoingupanddownslope(15°standardramp)• Category2:Needsheadsupportwhendrivingwheelchair• Category3:Whensittingstillinawheelchairneedsheadsupport

EK13:Abilitytocontrolajoystick

Questions:• Category0-3:Observejoystick.Hasitbeenadaptedinanywaysoyoucanuseiteasily?

Scoring:• Category0:Usesastandardjoystickwithoutspecialadaptation• Category1:Usesanadaptedjoystickorhasadjustedwheelchairinordertousejoystick• Category2:Usesothertechniquesforsteeringthanjoysticksuchasblowingsucking

systemsorscanneddriving• Category3:Unabletooperatewheelchair.Needsanotherpersontooperateit

•

EK14:FoodTextures

Questions:• Category0-3:Areyouabletochewandswallowalltypesoffoodincludingmeat?Doyou

needitcuttingupintosmallpiecessoyoucanmanagetoeatit?

Scoring:• Category0:Eatsalltexturesoffood• Category1:Eatscutuporsmallpiecesoffoodoravoidshard/chewyfoods• Category2:Eatsminced/pureedfood• Category3:Minimaloralintakeortubefed


64

EK15:Eatingameal

Questions:• Category0-3:Doyouneedmoretimethanothersinyourfamilytoeatameal?Ifsohow

muchlongerdoyouneed?

Scoring:• Category0:Abletoconsumeawholemealinthesametimeasotherssharingthemeal• Category1:Abletoconsumeawholemealinthesametimeasothersonlywith

encouragementorneedssomeadditionaltime(<10min)• Category2:Abletoconsumeawholemealbutrequiressubstantiallymorethan10

minutesextracomparedtootherseatingthesamemealorreducesportionsize• Category3:Unabletoconsumeawholemealevenwithadditionaltime,assistance

EK16:Swallowing

Questions:• Category0-3:Doyoueverhaveproblemsswallowingorchokingonfood?Howoften?

Scoring:• Category0:Neverhasproblemswhenswallowingandneverchokesonfood/drink• Category1:Mayexperienceoccasional(lessthanonceamonth)problemsswallowing

certaintypesoffoodoroccasionallychokes• Category2:Hasregulartroubleswallowingfood/drinkorchokesonfood/drink(morethan

onceamonth)• Category3:Hastroubleswallowingsalivaorsecretions

EK17:HandFunction

Questions:• Category0-3:Canyouuseapentowriteacoupleoflines?Canyouuseakeyboardto

write?Canyouopenanewbottleofwaterorfizzydrinkbottle?Canyouwriteyournameoruseamouse?

Scoring:• Category0:Canunscrewthelidofawateroffizzydrinkbottleandbreaktheseal• Category1:Canwritetwolinesorusecomputerkeyboard• Category2:Canwritesignatureorsendtextoruseremotecontrol• Category3:Cannotusehands


65

12. RangeofMovement

GeneralGuidelines

• Endrangeofmovementshouldberecordedtothenearest5°• Useanappropriatelysizedgoniometer,longarmoneifpossible.• Formeasurementofpassiveendrangetheparticipantneedstorelaxwhilstthetherapist

rangesthejoint.Timeshouldbeallowedfortheparticipanttorelax.• Measurementsshouldbetakenwitha‘moderate’degreeofstretchappliedandthe

measurementisrecordedasthedegreeslackingfromormorethanfullnormalendrangeofmovement

SuggestedJointRangesForambulantindividualsregularmonitoringoflowerlimbsisessential.Itisimportanttomonitorupperlimbrangeseveninambulantboysandyoungmen.• Ankledorsiflexion• Hipextension• Kneeextension• ITB• Shoulderelevationthroughflexion• Elbowextension• Forearmsupination• Wristextension• Longfingerextension


66

AnkledorsiflexionPatient position

Asweareinterestedintheeffectsofgastrocnemiusshorteningonankledorsiflexion,thistestisbestundertakenwiththekneeinfullextension.Thecalcaneumisheldinneutralalignmentwhilstpressureisappliedoverthemid-sectionofthefoottodorsiflextheankleasmuchaspossible,preventinginversion.

Normal range

20ºdorsiflexionto50ºplanterflexion.

Goniometer position:

Axisofthegoniometeroverthelateralmalleolus.Stationaryarmalignedwiththefibularhead,alongthelateralaspectofthelowerleg.“Movingarm”heldparalleltothelateralaspectofthe5thmetatarsal,alignedwiththeposteriorthirdofthefoot(thisistoensurethatgastrocnemiusrangeisbeingmonitoredandnotthatoftheplanterstructuresofthefoot).Noterangeofdorsiflexionpastplantergradeasplus+andrangelackingfromplantergradeasminus-.


67

0º

HipExtensionPatient position

Supinewiththeplinthsupportingtrunkandthigh,kneeflexedoverendofplinth.Flexcontralateralhipandkneetowardschesttofixatelumbarspineandstabilisepelvis(approximately80º-NOTsomuchthatthebuttocksstarttoliftfromthebench).Thelegtobeexaminedisloweredwhilstpressureontheoppositethighismaintained.Measurementistakenwhenresistanceisfeltorthepelvisbecomesunstable.Footmustnotrestonthefloor.Tomeasurerangeofextensionbeyondthis,asubjectivemeasurecanbegainedinsidelying,orinprone

Normal range

10ºofextensionbeyondneutralto120ºofflexion.


Placeaxisofgoniometeratgreatertrochanter.Alignstationaryarmparallelwithplinth,alongthesideofthetrunk.Align“movingarm”withlateralepicondyle,parallelwithlateralfemur.Noterangeofextensionpastneutralas+xº’s,rangelackingfromneutralas-xº’s.NBexternalrotationandabductionoflegbeingexaminedmustbeprevented

Betteriflimbisdroppedoveredgeofbedtoachievefullcheckofrange


68

KneeExtensionPatient position

Supineonplinthlegstogetherandextended

Normal range

Normalrange10ºofhyperextensionto135ºflexion.Ifkneewerefixedinthepositionofthearrowthiswouldberecordedaslacking45°degreesofextension,i.e.fixedcontractureof-45.Fullyextendedis0degrees.


Placeaxisofgoniometeroveraxisofknee(lateralepicondyle).Alignstationaryarmwithgreatertrochanter,parallelwiththelateralmidlineofthefemur.Alignmovablearmwiththelateralmalleolus,parallelwiththefibula.Measurementisrecordedasdegreeslackingfromneutral.


69

ITBPatient position

Supinewithplinthsupportingtrunk.Bothhipsextendedandinneutralrotation,kneesshouldbeflexedoversideofplinth,feetshouldbeoffthefloor.ASISshouldbelevel.Noteanyasymmetryoflegs.Passivelybringthelegsintoadductionuntilthereisresistancefeltandthenletthelegsrelaxintotheirnaturalposition.

Normal range

Interpretation<20ºofabductionfrommidline–nocontracture20º-30ºofabductionfrommidline–moderatecontracture>30ºofabductionfrommidline–severecontracture


Theaxisisplacedovertheumbilicus.Stationaryarmalignedstraightdownfromumbilicus.Movingarmalignedwithlateralborderofthepatella.


70

ShoulderelevationthroughflexionPatient position

Sittingposition.Fornon-ambulantparticipants,removethearmrestofthearmbeingmeasuredwhilstensuringthattheparticipantissafe.Ensurethereisnoblocktomovementfromthewheelchair.Withthearminaneutralposition,passivelyelevatethearmthroughflexionasfaraspossible.

Normal range

From-20°to170°


Alignthestationaryarmofthegoniometerparallelwiththemidlineonthelateralsideofthethorax,theaxiswiththecentreofthehumeralhead,andthemovingarmalignedwiththemidlineofthelateralaspectofthehumerus.Measuretheangleofthelimitofpassiverangeachieved.


71

ElbowextensionPatient position

Sittingposition.Withthearminacomfortablesupinatedposition,passivelystraightentheelbow.

Normal range

From0-+10°(hyperextension)to150°


Aligntheaxisofthegoniometerwiththelateralepicondyleofthehumerus,thestationaryarmofthegoniometerwiththehumerusandthemovingarmwiththeradius.Measurethisangle


72

ForearmsupinationPatient position

Theparticipantshouldsitwiththeshoulderinneutralsothattheupperarmisclosetothesideofthebody.Flextheelbowtoabout90andsupporttheforearm.

Normal range

EasiesttodocumentinquarterrangesorasapercentageFullrange=100%Midrange=50%or½asinphoto.

Activerangeisoftenreducedifendofpassiverangeistight.Recordboth.


Notrequired

.


73

WristextensionPatient position

sittingpositioninastandardchairwithnoarms(ambulant)orwheelchairwiththerelevantarmrestremoved(non-ambulant)withtheforearmstabilizedonatableorplinthandthewristpositionedovertheedge.

Normal range

Fullrangeabout85°


Aligntheaxisofthegoniometerwiththelateralwrist(triquetrum),thestationaryarmwiththeulna,andthemovingarmwiththefifthmetacarpal.Passivelyextendthewristandmeasuretheangleoffullextension


74

LongfingerextensionPatient position

Sittingposition.

Normal range

Fullrange–CanpassivelyextendmetacarpalsandphalangeswithwristextendedMildtightness–CanpassivelyextendmetacarpalsandwristbutnotfingersModeratetightness–CanpassivelyextendwristbutnotmetacarpaljointsorphalangesSeveretightness–Cannotextendwristevenwithflexionofmetacarpalsandphalanges


Norequired


75

13. ManualMuscleTesting

Generalmethod

1. Explainordemonstratemovementtothepatient.2. Askthepatienttoperformthemovementintheantigravityposition.3. Ifunabletothencheckpatient’sunderstanding,ROMavailable,weakness.4. Based on the limiting factor(s) either instruct and demonstrate again, decide if ROM is

affectingperformanceorrepeatthetestusingantigravityposition.5. Resistanceisgiventhroughfullavailablerange.6. In the case of a contracture limiting ROM, grades are given as stated in table 1, for

availablerange.Rangelimitationshouldbenotedinthecommentssection.7. Ifuncertainastowhatgradeshouldbegiven,theloweroptionshouldbechosen.8. If general compliance is such a problem that the results do not give, in the assessors

consideredopinion,atrueindicatorofthepatient’sability,thenthisshouldbenotedandtheresultsshouldnotbeincludedinthedatabase.Ifspecificmusclegroupsareaprobleme.g.duetopain,pleasenotethisanddonotincludeincalculationoftheMRC%.

9. Equipment–aheightadjustableplinthshouldbeavailable.10. Clothing–ideallyshortsandt-shirt.Ifnotshortsthentracksuitbottoms.Jeansmayrestrict

movement–especiallyinveryweakchildren.11. Watch for compensatory movements being used to produce an action – if uncertain

palpate themuscle groupwhich should be primemovers. Be clearwith positioning andstabilization.

12. The muscle groups required for the North Star constitute the minimum data set to beassessed.Ifyouneedtoincludefurthermusclegroupstoassistinclinicaldecisionmakingfor the individual patient, please do so, but DONOT include them in the calculation ofMRC%.

Table1–NorthStar/SmartnetMMTGradingsystem

Grade Gradefor%MRC

Definition

0 0 Nothing,nomusclecontraction1 1 “Flicker”–palpablecontraction,nomovement2- 2 PartROMwithgravitycounterbalanced2 2 FullROMwithgravitycounterbalanced2+ 2 Full ROM with gravity counterbalanced and a little resistance, no

antigravitymovement3- 2 Antigravitymovementthroughpartðmostofrange3 3 Fullrangeantigravitymovement3+ 3 FullantigravityROMwithsomeresistancethroughpartofrange4 4 FullantigravityROMwithsomeresistancethroughthewholerange4+ 4 FullantigravityROMwithnormalpowerinpartofrange5 5 FullROMwithfullnormalresistancethroughoutrange


76

TheabovescaleisbaseduponthemodificationsmadetotheoriginalMedicalResearchCouncil6-point scale by staff at the Hammersmith Hospital.Most physiotherapists who regularlymusclechartarefamiliarwiththisscale(ortheexpanded16-pointversion)frompreviousworkshops.

Aminusgradedenoteslossofrange.Thisiswhy4-isnotgraded,asagradeof4is,bydefinition,fullrange.

TheMRCpercentageistobecalculatedonthe‘whole’score,ratherthanwiththe+and-grades.The‘whole’gradesforeachelementofthescalearenotedincolumn2oftheabovetable.

The test methodology which follows has been taken from a variety of sources – thecomprehensive work undertaken by the physiotherapists involved with the CIDD projects inassessmentofDMDintheStates,andfromanumberofMMTtextbooks.Referencesaregiven,andfurtherinformation/clarificationshouldbesoughtfromthemasneeded.

Choiceofmusclegroupshasbeenbaseduponpresent clinicalpractice (fromthecentreswhichcurrentlyassessmuscleactivity)andareviewoftheliteraturerelatingtoreliabilityofMMT.

UsefultextsKendallFP,McCrearyEK,ProvenancePG.1993‘Muscles:TestingandFunction’4thEdWilliams&

Wilkins*KEYTEXT*ClarksonHM.2000.‘MusculoskeletalAssessment:jointrangeofmotionandmanualmuscle

strength’2ndEd.Lippincott,William&Wilkins,PhiladelphiaDaniels&Worthingham.1980‘Muscletesting:techniquesofmanualexamination’4thEd.WB

SaundersCompany,Philadelphia


77

14. Appendix1

Tableclothdimensionsandlayout

Tablecloth*needs*to*be*75*cm*wide*and*40*cm*deep*

25*cm*from*edge*of*cloth*

10*cm*from*edge*of*cloth*and*5cm*between*rim*of*each*circle*

Edge*of*tablecloth*lined*up*with*edge*of*table*

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