CaseStudy:JohnWoodbury

CasePresentation

Johnis44yearsold.Heissittinginthechairintheexaminationroom.Ashemovesto
thetable,hestumblesonthecarpet,laughsathimselfandsaysthathappensallthetime.
Johnhasenjoyedarelativelyhealthylifewithnomajorillnesses.Heishappilymarried
withfourchildren,ages14,11,8,and5.
Youaskwhatpromptedhisvisit.Johnresponds,IwasoutsailingandnoticedIcouldnt
holdontotheropesastightlyaswasneeded.Ivenoticedapatternofgeneralweakness
legs,arm,hands,evenmyjawsneakinguponmelatelyandIthoughtIshouldcheck
itout.

Onaseparatesheetofpaper,fillinthefollowingchart,addingasmanyrowsasneeded:
WHATWEKNOW
Johnis44yearsold

WHATWETHINKWE
KNOW
Nervousand
muscularsystem
issue

WHATWEWANTTO
FINDOUT
Whatsthecauseofthe
generalmuscleweakness

Hehasapatternofgeneral
weaknesslegs,arm,
hands,jaw
Hehasarelativelyhealthy
lifewithnomajorillnesses

Whatisyourhypothesis?(Keepinmindthisissimplyastartingpointandyouwillget
moreinformationlaterthatwillmostlikelychangeyourhypothesis).
Johnseemstobesufferingfromsometypeofmuscularornervoussystemproblem.
Directedquestions:

1. Whatarethemajorsystemsofthebody?
2. WhatsystemsseemtobeaffectedinJohnscase?
3. Whatarethecelltypesspecifictothesesystems?Drawandlabelthecells
anddescribetheirfunction.
4. ListseveralpossibleconditionsJohncouldbesufferingfrom.

CaseStudy:JohnWoodbury
Part2

Johnappearsrelaxedandalert.Hefallsintheaveragerangeforweightgivenhisageand
height.Youtakehisvitalsigns:temperature,pulse,respiratoryrate,andbloodpressure
areallnormal.
YouaskJohntoswallowandyounotehehassomedifficultydoingso.Hestatesthatisa
commonissuerecently.
Youtesthisgagreflexes.Asthetonguedepressorreacheshislips,hegags.You
determinethatJohnhasanoveractivereflex.
Hedoesexhibitageneralmuscleweaknesshecanovercomealoweredresistance,but
notfullresistanceinmostcases.

Onaseparatesheetofpaper,fillinthefollowingchart,addingasmanyrowsasneeded:
WHATWEKNOW
Johnappearsrelaxedand
alert
Johnhasanoveractivegag
reflex
Heexhibitsageneral
muscleweakness

WHATWETHINKWE
KNOW
Possibleneuromuscular
junctionproblem
Possibledamagetoupper
and/orlowermotorneurons

WHATWEWANTTO
FINDOUT
Whatsthecauseofthe
generalmuscleweakness

Whatisyourhypothesis?
Johnseemstohaveapossibleneuromuscularjunctionproblemwhichmaybeaffecting
hisupperand/orlowermotorneurons.
Directedquestions:
5. Whatisaneuromuscularjunction?Drawandlabelaneuromuscularjunction.
6. Whataretheeffectsofdamagetouppermotorneurons?
7. Whataretheeffectsofdamagetolowermotorneurons?

CaseStudy:JohnWoodbury
Part3

YoutestJohnsreflexesandtheywereallnormal,withthe
exceptionoftheplantarreflex,alsoknownasBabinskis
sign.(Seepicture).Histoesspreadandextended.

YoudecidetoorderanMRIandanelectromyography.

Onaseparatesheetofpaper,fillinthefollowingchart,addingasmanyrowsasneeded:
WHATWEKNOW
Johnsreflexeswereall
normal,withtheexception

WHATWETHINKWE
KNOW
Someconditionmaybe
causinganabnormal

WHATWEWANTTO
FINDOUT
MRIandan
electromyographyresults

oftheplantarreflex,also
knownasBabinskissign.
Histoesspreadandextend

Babinskissign

Whatisyourhypothesis?
SomeconditionmaybecausinganabnormalBabinskissign.
Directedquestions:
8. WhatconditionscouldcauseanabnormalBabinskisign?
9. WhatareyoulookingforintheMRIandelectromyography?
Resources:http://endeavor.med.nyu.edu/neurosurgery/index.html

CaseStudy:JohnWoodbury
J.Woodburymyogram

Part4

TheresultsofJohnsMRIandelectromyographyarein.

NormalMRI J.WoodburyMRI

Onaseparatesheetofpaper,fillinthefollowingchart,addingasmanyrowsasneeded:
WHATWEKNOW

WHATWETHINKWE
KNOW
Possiblebraindamage

abnormalmyogram
abnormalMRI

WHATWEWANTTO
FINDOUT
Whatconditionscould
causeanabnormal
myogramandMRI

Possiblediseasednerve
cells

Whatisyourhypothesis?
Theremaybepossiblebraindamageand/ornervecelldisease.
Directedquestions:
10. Whatisanactionpotential?Whatfactorsinfluencetherateofaction
potentials?
11. Whatisasynapse?Whateventshappenatasynapse?
12. Whatconditionscouldcauseanabnormalmyogramliketheoneshown
above?
13. WhatconditionscouldcauseanabnormalMRIliketheoneshownabove?

CaseStudy:JohnWoodbury
Part5

Yourunabloodtest.Johnsserum(thisisthepartofthebloodthatdoesnotincludered
andwhitebloodcellsorplatelets)isabnormal.HehaselevatedlevelsofGlutamate.

Onaseparatesheetofpaper,fillinthefollowingchart,addingasmanyrowsasneeded:
WHATWEKNOW
Bloodserumisabnormal

WHATWETHINKWE
KNOW
PossibleCarpaltunnel

WHATWEWANTTO
FINDOUT
Whatsthecauseofthe

elevatedlevelsof
Glutamate

syndrome
PossibleAmyotrophic
LateralSclerosis

generalmuscleweakness

Whatisyourhypothesis?
PossibleCarpaltunnelsyndromeorAmyotrophicLateralSclerosis.

Directedquestions:
14. WhatconditiondoesJohnWoodburylikelyhave?
15. Whatcourseoftreatmentwouldyourecommend?

CaseStudy:JohnWoodbury
Part6

YoureferJohnWoodburytoaNeurologistforasecondopinionregardingyourdiagnosis
ofAmyotrophicLateralSclerosis.Youalsorecommendaphysicaltherapistandwrite
outadrugprescriptionforRiluzole.

Onaseparatesheetofpaper,fillinthefollowingchart,addingasmanyrowsasneeded:
WHATWEKNOW
Secondopinionisneeded
fromaNeurologist
Physicaltherapyneeded

WHATWETHINKWE
KNOW
diagnosisofAmyotrophic
LateralSclerosis

WHATWEWANTTO
FINDOUT
IsJohnsufferingfromALS

Whatisyourhypothesis?
JohnissufferingfromAmyotrophicLateralSclerosis.
Directedquestions:
16. WhatchallengeswillJohnlikelyfaceinthefuture?
17. Findthreecharitygroupsthatworkwiththiscondition.
18. WhatcanphysicaltherapydoforJohnWoodbury?
19. HowdoesRiluzoleactinthebody?
20. WouldyourecommendanyotherspecialistsforJohnandhisfamily?
21. Wouldyourecommendgeneticscreeningforhisfourchildren?
Images by:
David Holland
Neurological Disorders
UT Southwestern Medical
Center
And
The Eleanor and Lou Gehrig
MDA/ALS Research Center
The Neurological Institute of
New York Columbia University
And

TeacherResource:NervousSystem/ALS
ProblemBasedLearningUnit
ManycollegesareusingProblemBasedLearning(PBL)intheirsciencecoursesto
encouragestudentstothinkcriticallyandcreativelyaboutaparticulartopic.Thenature
ofthiseducationaltoolissuchthatstudentsareempoweredtodecidethedirectionof
theirresearch.Bygivingstudentsnecessaryinformationpiecemeal,theyhavetimeto
focusondetailswhilebeingmotivatedbythelargergoal:solvingtheproblem.This
strategyiseasilymodifiedforthehighschoolcurriculum.
ThisparticularPBLisdesignedtoteachstudentsabouttheNervousSystemand
AmyotrophicLateralSclerosis,betterknownasLouGehrigsDisease.Thesystemsof
thebodyandrepresentativecelltypesareoftentaughtthroughteacherdirectedlessons.I
thoughtitwouldbemoreengagingformystudentstolearnaboutHumanBiology
throughaninquirybasedlesson.Forexample,comparinganormalmyogramtooneof
anALSpatientandthengoingtotheirbooktoseewhatthosespikesmeancanteach
thestudenttheconceptoftheactionpotential.Inthisformat,theNervousSystemis
muchmoreexcitingfromthestudentspointofview.
Thelessonplancanchallengeanylevelofstudentbecausegeneralbiologyclassescan
keeptothebasicswhileadvancecollegeboundstudentscanexplorethemolecularand
geneticaspectsofALS.Althoughoriginallydesignedasagroupproject,thereisno
reasonanindividualcannotcompletetheexercisesbyhimorherself.
Theprojectshouldbedoneinsixparts,givingstudentstimebetweeneachsectiontofind
theanswerstotheirquestionsandproposeappropriatehypotheses.Givenenoughclass
timeandcomputersintheroom,severalsectionscouldbeaddressedinasingleclass.
Otherwise,givingasinglesectionperclassperiodisadvisablethisallowsstudentsto
workontheresearchpieceathomeanddoesnotrequirecomputersforindividualsinthe
classroom.
Oneassessmentoptionwouldbeaskitshowingdoctor/patientinteractions.Alternately,
thedirectedquestionscouldbeusedonaquizortest.
OnevariationIwouldconsiderisseparatingthedirectedquestionsfromtherestofeach
section.Thequestionsaremeanttokeepstudentsfocusedandgivethemhintsastohow
toproceed.Unfortunately,theymayalsostiflecreativity.Bygivingthemoutafteran
initialbrainstormingsession,manydifficultiescanbeavoided.

AnswerKey
1. Whatarethemajorsystemsofthebody?
Nervous,muscular,skeletal,integument,endocrine,cardiovascular,respiratory,
reproductive,digestive,lymphatic,urinary
2. WhatsystemsseemtobeaffectedinJohnscase?
Nervousandmuscular
3. Whatarethecelltypesspecifictothesesystems?Drawandlabelthecells
anddescribetheirfunction.
Nervoussystem:neurons
Muscularsystem:musclecell
Referto:http://butler.cc.tut.fi/~malmivuo/bem/bembook/02/02.htm
4. ListseveralpossibleconditionsJohncouldbesufferingfrom.
HIV,Lymedisease,MultipleSclerosis,MuscularDistrophy
5. Whatisaneuromuscularjunction?Drawandlabelaneuromuscularjunction.
Theplaceinthebodywhereaneuronmeets(orinnervates)amusclecell.

QuickTime and a
TIFF (Uncompressed) decompressor
are needed to see this picture.

(members.shaw.ca/ copingwithillness/junction.gif)

6. Whataretheeffectsofdamagetouppermotorneurons?
Thesearemotorneuronsinthebraindamageresultsinabnormal,hyperactive
reflexes
7. Whataretheeffectsofdamagetolowermotorneurons?
Thesearemotorneuronsinthespinalcorddamageresultsinmuscleweakness

8. WhatconditionscouldcauseanabnormalBabinskisign?
Refer to:
http://www.nlm.nih.gov/medlineplus/ency/article/003294.htm

Amyotrophic lateral sclerosis

Brain tumor (if it occurs in the corticospinal tract or the

cerebellum)

Familial periodic paralysis

Friedreich's ataxia

Head injury

Hepatic encephalopathy

Meningitis

Multiple sclerosis

Pernicious anemia

Poliomyelitis (some forms)

Rabies

Spinal cord injury

Spinal cord tumor

Stroke

Syringomyelia

Tuberculosis (when it affects the spine)

9. WhatareyoulookingforintheMRIandelectromyography?
electromyography(EMG): a special recording technique that
detects electrical activity in muscles. If the nerve cells diseased,
the rate of action potentials being fired may be abnormal.
magnetic resonance imaging (MRI): a magnetic field and radio
waves are used to look at soft tissue of the body. An MRI of the
brain could show damage to the brain.
10. Whatisanactionpotential?Whatfactorsinfluencetherateofaction
potentials?
Anactionpotentialisanelectricalimpulsethattravelsthelengthofanaxon,
whichtriggersachemicalsignaltobesenttothenextcell.
Thestrengthofastimulus,thedurationofastimulus,thehealthofthemyelin
sheath,etc
11. Whatisasynapse?Whateventshappenatasynapse?
Asynapseisthespacebetweenanervecellanditstargetcell(whichcouldbe
anothernervecelloramusclecell).
Atthesynapse,theactionpotentialtriggersaninfluxofCalciumions.This
triggerspresynapticvesiclestobindwiththecellmembraneandreleaseits
contentsofneurotransmitterintothesynapse.Theneurotransmitterdiffuses
acrossthesynapseandbindswithapostsynapticreceptoronthesecondcell.

Thisisachemicallygatedtransportproteinthatisnowopenandallowsionsto
flowintothesecondcell,triggeringanewactionpotentialinthesecondcell.

12. Whatconditionscouldcauseanabnormalmyogramliketheoneshown
above?
Referto:http://www.nlm.nih.gov/medlineplus/ency/article/003929.htm

Denervation (reduced nervous stimulation)

Carpal tunnel syndrome

Amyotrophic lateral sclerosis (ALS)

Myopathy (muscle degeneration, may be caused by a

number of disorders,
including muscular dystrophy)

Myasthenia gravis

Alcoholic neuropathy

Axillary nerve dysfunction

Becker's muscular dystrophy

Brachial plexopathy

Cervical spondylosis

Common peroneal nerve dysfunction

Dermatomyositis

Distal median nerve dysfunction

Duchenne muscular dystrophy

Facioscapulohumeral muscular dystrophy (LandouzyDejerine)

Familial periodic paralysis

Femoral nerve dysfunction

Friedreich's ataxia

Guillain-Barre

Lambert-Eaton Syndrome

Mononeuritis multiplex

Mononeuropathy

Peripheral neuropathy

Radial nerve dysfunction

Sciatic nerve dysfunction

Sensorimotor polyneuropathy

Shy-Drager syndrome

Thyrotoxic periodic paralysis

Tibial nerve dysfunction

Ulnar nerve dysfunction

13. WhatconditionscouldcauseanabnormalMRIliketheoneshownabove?
Answerswillvary.ManyALSpatientshavenormalMRIs
14. WhatconditiondoesJohnWoodburylikelyhave?
AmyotrophicLateralSclerosis

15. Whatcourseoftreatmentwouldyourecommend?
Riluzole

16. WhatchallengeswillJohnlikelyfaceinthefuture?
Afterdiagnosis,thelifespanis35yearsonaverage.Patientssufferagradual
weakeningofthemuscles,difficultyswallowing,lossofspeech,musclecramps
andspacticity,periodsoflaughingandcrying.Afteracertainpoint,Johnwill
needhelpbreathing(viamachines),eating(throughatube),andwillbe
bedridden.
17. Findthreecharitygroupsthatworkwiththiscondition.
ALSAssociation
CurtandShondaShillingStrikingOutALS
(Answerswillvary)
18. WhatcanphysicaltherapydoforJohnWoodbury?
PT will strengthen unaffected muscles, improve cardiovascular health, and help
patients fight fatigue and depression. (http://www.ninds.nih.gov/disorders/
amyotrophiclateralsclerosis/detail_amyotrophiclateralsclerosis.htm)
As the disease progresses, physical therapists will help John find appropriate
braces and other means to keep him mobile.
19. HowdoesRiluzoleactinthebody?
Riluzolebreaksdownglutamateinthebody.Excessglutamatehasbeenfoundto
degrademotorneurons.Bydestroyingsomeoftheglutamate,itishopedthat
nervecelldeathratewilldecrease.
20. WouldyourecommendanyotherspecialistsforJohnandhisfamily?
Iwouldprobablyrecommendapsychologisttohelpthemcopewithallofthe
emotionalaspectsofhospicecareespeciallyconsideringJohnsageandthe
agesofhiswifeandchildren.
(Answerswillvary)
21. Wouldyourecommendgeneticscreeningforhisfourchildren?
(Answerswillvary)