Neurology

233 Pages·2016·12.57 MB·English

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Neurology.org
Clinical Reasoning in Neurology:
A Case-Based Approach
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Cases from the Neurology® Resident & Fellow Section
Edited by Aaron L. Berkowitz, MD, PhD, Sashank Prasad, MD,
and Mitchell S.V. Elkind, MD, MS
Noteregardingprintappearance:BecausetheClinicalReasoningsectionappearsalmostexclusivelyonline,thereadermaynoticecolor
variationsamongtheseprintedselections.
©2016AmericanAcademyofNeurology.Allrightsreserved.AllarticleshavebeenpublishedinNeurology®.
OpinionsexpressedbytheauthorsarenotnecessarilythoseoftheAmericanAcademyofNeurology,itsaffiliates,orofthePublisher.The
AmericanAcademyofNeurology,itsaffiliates,andthePublisherdisclaimanyliabilitytoanypartyfortheaccuracy,completeness,efficacy,
oravailabilityofthematerialcontainedinthispublication(includingdrugdosages)orforanydamagesarisingoutoftheuseornon-useof
anyofthematerialcontainedinthispublication.
Clinical Reasoning in Neurology:
A Case-Based Approach
Cases from the Neurology® Resident & Fellow Section
Editors
Aaron L. Berkowitz, MD, PhD
Assistant Professor of Neurology
Harvard Medical School
Brigham and Women’s Hospital
Boston, Massachusetts
Sashank Prasad, MD
Associate Professor of Neurology
Harvard Medical School
Brigham and Women’s Hospital
Boston, Massachusetts
Mitchell S.V. Elkind, MD, MS
Professor of Neurology and Epidemiology
College of Physicians and Surgeons and Mailman School of Public Health
Columbia University
New York, New York
This publication is also available on Neurology® for the iPad® and AndroidTM devices
and features additional content.
Table of Contents
Neurology.org
THEMOSTWIDELYREADANDHIGHLYCITEDPEER-REVIEWED
NEUROLOGYJOURNAL
INTRODUCTION 41 Psychomotorregressionintheyoung
1 Clinicalreasoninginneurology:Acase-based E.M.McGovernandT.J.Counihan
April2,2013;80:e152-e155
approach:CasesfromtheNeurology®Resident&
FellowSection
45 A72-year-oldmanwithrapidcognitivedeclineand
A.L.Berkowitz,S.Prasad,andM.S.V.Elkind
unilateralmusclejerks
DISORDERSPRESENTINGWITHIMPAIREDAROUSAL M.Duncan,J.Cholfin,andL.Restrepo
June3,2014;82:e194-e197
ORCOGNITION
2 Editors’Introduction
DISORDERSPRESENTINGWITHWEAKNESS
4 A59-year-oldmanwhobecamelostinhisownhome 49 Editors’Introduction
K.Mondon,E.Beaufils,D.Perrier,A.Matysiak,and
C.Hommet 51 Awomanwithrapidlyprogressiveapraxia
April20,2010;74:e66-e68 P.Pressman,E.H.Bigio,D.Gitelman,and
C.Zadikoff
7 A57-year-oldwomanwhodevelopedacuteamnesia April9,2013;80:e162-e165
followingfeverandupperrespiratorysymptoms
B.A.McCray,D.Forst,J.Jindal,andG.V.Henderson 55 A51-year-oldwomanwithacutefootdrop
April7,2015;84:e102-e106 D.Rallis,A.Skafida,G.Alexopoulos,A.Petsanas,
A.Foteinos,S.Katsoulakou,andE.Koutra
12 A28-year-oldpregnantwomanwithencephalopathy February17,2015;84:e48-e52
Z.M.Grinspan,J.Z.Willey,M.J.Tullman,and
M.S.V.Elkind 60 A38-year-oldwomanwithchildhood-onsetweakness
October13,2009;73:e74-e79 P.S.GhoshandM.Milone
August12,2014;83:e81-e84
18 A52-year-oldmanwithspellsofalteredconsciousness
andsevereheadaches 64 A70-year-oldmanwithwalkingdifficulties
T.M.Burrus,J.D.Burns,J.HustonIII,G.Lanzino, F.M.Cox,J.J.G.M.Verschuuren,andU.A.Badrising
A.A.Rabinstein,andJ.H.Uhm November9,2010;75:e80-e84
May26,2009;72:e105-e110
69 A47-year-oldmanwithprogressivegaitdisturbance
24 A27-year-oldmanwithrapidlyprogressivecoma andstiffnessinhislegs
J.M.Wong,M.Chandra,R.VanDeBogart,B.Lu,and A.Fontes-Villalba,J.-A.Palma,M.A.Fernández-Seara,
A.H.Yee M.A.Pastor,andP.deCastro
September1,2015;85:e74-e78 May21,2013;80:e223-e227
29 Encephalopathyina10-year-oldboy 74 A79-year-oldmanwithpolyneuropathyand
L.RodanandI.Tein dysautonomia
July17,2012;79:e12-e18 C.KaramandS.N.Scelsa
May10,2011;76:e93-e97
36 A14-year-boywithspellsofsomnolenceandcognitive
changes 79 A62-year-oldmanwithrightwristdrop
C.M.deGusmao,K.P.Maski,andD.K.Urion G.Cirillo,V.Todisco,A.Tessitore,andG.Tedeschi
April22,2014;82:e142-e146 September10,2013;81:e81-e84
CONTINUED
Table of Contents
continued
84 A48-year-oldwomanwithgeneralizedweakness 124 A6-year-oldboywithuncontrollableright-sided
C.KaramandS.N.Scelsa movements
May4,2010;74:e76-e80 K.Gurcharran
January24,2012;78:e23-e26
89 A40-year-oldmanwithCIDP-likeillnessresistantto
treatment 128 A52-year-oldwomanwithsubacutehemichorea
R.Lahoria,C.Karam,A.Dispenzieri,andP.J.B.Dyck S.M.Kranick,R.S.Price,S.Prasad,andH.I.Hurtig
September3,2013;81:e65-e70 November11,2008;71:e59-e62
95 A55-year-oldmanwithweightloss,ataxia,andfoot 132 A13-year-oldboypresentingwithdystonia,myoclonus,
drop andanxiety
E.P.Flanagan,A.N.L.Hunderfund,N.Kumar, J.S.BlackburnandM.L.Cirillo
J.A.Murray,K.N.Krecke,B.S.Katz,and March13,2012;78:e72-e76
S.J.Pittock
June17,2014;82:e214-e219 137 A39-year-oldmanwithabdominalcramps
S.R.Jaiser,M.R.Baker,R.G.Whittaker,D.Birchall,and
DISORDERSPRESENTINGWITHABNORMAL P.F.Chinnery
July9,2013;81:2e5-e9
SENSATION
101 Editors’Introduction 142 Amiddle-agedmanwithepisodesofgaitimbalance
andanewlyfoundgeneticmutation
103 An85-year-oldmanwithparesthesiasandanunsteady
M.S.YugrakhandO.A.Levy
gait
October16,2012;79:e135-e139
A.L.Berkowitz,R.M.Jha,J.P.Klein,andA.A.Amato
March19,2013;80:e120-e126
DISORDERSPRESENTINGWITHVISUALDEFICITS
110 A27-year-oldmanwithhandnumbness:Exploringnew
147 Editors’Introduction
horizonsandreinventingthepast
J.Vijayan,C.Y.Chuen,A.M.Punzalan,and
149 A75-year-oldmanwith3yearsofvisualdifficulties
E.Wilder-Smith
A.L.Berkowitz,M.F.Rose,K.R.Daffner,andS.Prasad
March11,2014;82:e80-e84
October21,2014;83:e160-e165
115 A34-year-oldwomanwithrecurrentboutsofacral
155 Avideoanalysisofeyeandlimbmovement
paresthesias
abnormalitiesinaparkinsoniansyndrome
C.KaramandS.N.Scelsa
M.PoulopoulosandD.Silvers
March2,2010;74:9775-778
August4,2009;73:e20-e23
DISORDERSPRESENTINGWITHABNORMAL
159 A64-year-oldmanwithpainful,unilateralexternal
MOVEMENTS
ophthalmoplegia
119 Editors’Introduction M.T.Bhatti
August24,2010;75:e35-e39
120 An83-year-oldwomanwithprogressivehemiataxia,
tremor,andinfratentoriallesions 164 A36-year-oldmanwithverticaldiplopia
K.Aquino,I.J.Koralnik,andD.Silvers S.Prasad,N.J.Volpe,andM.A.Tamhankar
July12,2011;77:e7-e10 May12,2009;72:e93-e99
CONTINUED
Table of Contents
continued
172 OpticdiscswellinginapatientwithAIDS 198 A22-year-oldwomanwithheadacheanddiplopia
M.A.Almekhlafi,G.Williams,andF.Costello J.S.Kim
August2,2011;77:e28-e32 July7,2009;73:e1-e7
177 A75-year-oldwomanwithvisualdisturbancesand 205 Achildwithpulsatileheadacheandvomiting
unilateralataxia L.Morin,A.Smail,J.-C.Mercier,and
M.C.Eugene,D.Kitei,andD.Silvers L.Titomanlio
August17,2010;75:e29-e33 April14,2009;72:e69-e71
DISORDERSPRESENTINGWITHHEADACHE, MANAGEMENTDILEMMAS
DIZZINESS,ORSEIZURES
208 Editors’Introduction
182 Editors’Introduction
209 A42-year-oldmanwhodevelopedblurredvisionand
183 A2-day-oldbabygirlwithencephalopathyandburst droppedhisiPodwhilejogging
suppressiononEEG A.L.Berkowitz,P.E.Voinescu,and
R.DhamijaandK.J.Mack S.K.Feske
July19,2011;77:e16-e19 August19,2014;83:e89-e94
187 A55-year-oldwomanwithvertigo:Adizzying 215 A24-year-oldwomanwithprogressiveheadacheand
conundrum somnolence
D.R.GoldandS.G.Reich S.Bhattacharyya,A.L.Berkowitz,and
October23,2012;79:e146-e152 R.M.Jha
June3,2014;82:e188-e193
194 A33-year-oldwomanwithseverepostpartumoccipital
headaches 221 An87-year-oldwomanwithleft-sidednumbness
N.MaaloufandS.I.Harik S.YaghiandM.S.V.Elkind
January31,2012;78:366-369 October13,2015;85:e110-e115
Acknowledgment and Note
ACKNOWLEDGMENT Drs. Berkowitz, Prasad, and Elkind wish to thank the editorial staff of the
Resident&FellowSectionofNeurology®fortheirtirelessenthusiasmforthesection,andtheAAN
and Wolters Kluwer for their generous support of this project. This book would not have been
possiblewithouttheencouragementofPattyBaskin,ExecutiveEditor,andtheleadershipofDr.Bob
Gross, Editor-in-Chief, both of whom have always been tremendous supporters of the Resident &
Fellow Section. Finally, and in particular, we acknowledge Kathy Pieper, Managing Editor of
Neurology, for her dedication, passion, and commitment to excellence in this project, as in so
many others.
EDITOR-IN-CHIEF’SNOTE Whenthe Resident &Fellow section was started, itwould havebeen
difficulttopredicthowsuccessfulitwouldbecome.Thequalityofthecontentissuperb,submissions
areplentiful,andourstaffofyoungeditorsisenthusiasticandtalented.Thisventure,acompilation
of Clinical Reasoning cases, grew out of an idea from within the section; the hard work and ded-
icationoftheRFSteammadeitareality.Kudostoallwhowereinvolved!Thesecasediscussionsare
the stuff by which we all learned neurology, and are here collected to educate trainees across the
country.Thiseffortalsoservesasareminderoftheeducationalmissionofthesection,whichisnow
giving back to our community beyond its usual publications.
INTRODUCTION
Clinical reasoning in neurology:
A case-based approach
Cases from the Neurology® Resident & Fellow Section
AaronL.Berkowitz,MD, TheeminentneurologistC.MillerFisherwasknown report surprising and unexpected diagnoses. Others
PhD to say that neurology is learned “stroke by stroke.” describehowtoapproachcommonclinicalproblems.
SashankPrasad,MD Althoughneurologytrainingrequirestheacquisition All cases, however, emphasize the reasoning element
MitchellS.V.Elkind, of extensive “book knowledge”—neuroanatomy, that is at the core of clinical neurology. Beyond the
MD,MS neurophysiology, neuropharmacology, neuropathol- “what” of neurologic diagnosis and treatment, these
ogy, and more—the practice of clinical neurology is casesexplorethe“how”and“why.”
indeed ultimately learned case by case, patient by Over nearly 10 years, 155 cases have been pub-
patient. To see the clinical effects of precise lesions lished in the Clinical Reasoning section describing
firsthand,tohearthestoriesofpatientssufferingfrom diverse diagnoses, challenging clinical quandaries,
neurologicdisease,andtodiscussthesefindingswith and daunting management dilemmas. Most were
one’s clinical teachers at the bedside: these are the written by residents and fellows, supervised by fac-
experiencesthattransformstudentsofneurologyinto ulty,andarethusgearedtowardthoselearningclini-
clinicalneurologists. cal reasoning themselves. Many of these fascinating
Theprocessofclinicalreasoningislearnedthrough cases and the accompanying discussions, however,
practice:tryingtolocalizethelesionthatexplainsapa- arelikelytobeasinformativetoexperiencedneurol-
tient’s symptoms and signs, attempting to reconcile ogistsastotrainees.Forthisanthologywehavecom-
disparate elements of the history and examination, piledcasesthatspanthemajorcardinalpresentations
judging when to obtain and how to interpret neuro- ofneurologicdisease.Eachsectionbeginswithabrief
diagnostictests,conferringoncomplexcaseswithone’s introduction to the clinical approach for a particular
peersandmentors,andseeingtheevolutionofneuro- realmofneurology,butleavesthedetaileddiscussions
logicdiseaseandhowitmaybemodifiedbytreatment. ofdiagnosisandtreatmenttothecasesthemselves.
Yet such experiences shared between colleagues or Wehopethatourreaderswillenjoytheopportu-
between teachers and students are rarely recorded nitytolearnfromthiscollection,casebycase.
andevenmorerarelypresentedinpedagogicalform.
TheClinicalReasoningsectionoftheResident& DISCLOSURE
Fellow Section of Neurology® has provided a forum Dr. Berkowitz has received speaker honoraria from Stevens Institute
for case reports that capture the art and science of ofTechnologyandAudioDigest,andreceivespublishingroyaltiesforClin-
icalPathophysiologyMadeRidiculouslySimple,MedMaster,2007andThe
clinical neurology. Rather than encouraging case re-
ImprovisingMind,Oxford,2010.Dr.PrasadreceivesroyaltiesfortheeBook
ports that describe obscure diagnoses with heroic
Modern Neuro-Ophthalmology, independently published by Modern
leapsofdiagnosticgymnastics,theClinicalReasoning Neurology,LLC,whichheowns.Dr.Elkindreceivescompensation for
section has focused on the process of arriving at a providingconsultativeservicesforBiogenIDEC,Biotelemetry/Cardionet,
BMS-Pfizer Partnership, Boehringer-Ingelheim, Daiichi-Sankyo, Janssen
localization,diagnosis,andtreatmentplanfordiseases
Pharmaceuticals,andSanofi-RegeneronPartnership;servesontheNational,
bothmundaneandrare.EachClinicalReasoningcase Founders Affiliate,and NewYorkCitychapter boardsoftheAmerican
describes an approach to interpreting the history, Heart Association/American Stroke Association; receives royalties from
examination,anddiagnostictesting,aswellasdeter- UpToDateforchaptersrelatedtostroke;andparticipatedinlegalproceed-
ings related to Organon/Merck (NuvaRing and stroke), BMS-Sanofi
mining the localization, clinical formulation, and
Partnership(Plavixandstroke),andHi-Tech(DMAAandstroke).Goto
management plan. Some Clinical Reasoning cases Neurology.orgforfulldisclosures.
1
Disorders presenting with impaired arousal
or cognition
Moststudentsofneurologybecomeenthralledwith objectidentification)andnondeclarativememory
the subject because it encompasses disorders of (which includes procedural memory, emotional
human consciousness, comprising arousal as well memory, and priming). Declarative memory
ascomplexcognitivefunctionsincludingattention, relies upon the integrity of the Papez circuit in
memory, language, visuospatial processing, and the mesial temporal lobes and diencephalon,
emotional processing. These are the quintessential including entorhinal cortex, the hippocampus,
functions that make us human. In the context of the fornix, the mammillary bodies, the mammil-
neurologicillnessitispossibletowitnesstheextent lothalamic tract, the anterior nucleus of the thal-
towhichtheelementsofcognitioncanbecomefrac- amus, and the cingulate cortex. Diseases that
turedand separable;dysfunctioninindividual cog- affectthesestructuresproduceanterogradeamne-
nitive domains helps us to understand their sia,withimpairedabilitytorecallnewlyencoded
fundamental nature. Although cognitive processes information.
depend upon distributed networks, focal lesions (cid:129) Language networks in the brain include auditory
arecapableofdisruptingthesenetworks,producing andvisualinputstotheWernickeareainthesupe-
uniqueclinicalsyndromes.Acarefulexaminationof rior temporal lobe, the arcuate fasciculus, and the
a patient’s mental state can therefore yield enor- Brocaareaintheinferiorfrontallobe.Thisnetwork
mousinformationaboutthelocalizationanddiffer- is typically represented in the left hemisphere, but
ential diagnosis of lesions affecting the cerebral there may be bilateral or right hemispheric repre-
hemispheres.
sentationinsomeindividuals.Homologousareasin
(cid:129) Arousalreliesuponconnectionsfromtheascending the right hemisphere contribute to the generation
reticular activating system, which originates in the and processing of music as well as prosody of lan-
rostralbrainstemandprojectstoboththalamiand guage (i.e., the melody and rhythm of speech, as
diffuselythroughoutthecerebralhemispheres.Le- opposedtosyntaxandgrammar).Theevaluationof
sions in the rostral brainstem or in both hemi- language function includes an assessment of flu-
spheres can impair arousal, placing a patient on a ency,naming,repetition,comprehension,reading,
spectrumofstatesofalteredconsciousnessthatin- andwriting.Lesionsinthelanguagenetworkspro-
cludes drowsiness, somnolence, obtundation, duceaphasia,whichmaybecharacterizedasrecep-
aminimallyresponsivevegetativestate,andcoma. tive, expressive, conductive, or global based upon
(cid:129) Attention depends, to a large degree, upon the thepredominantabnormalitiesonexamination.
function of the frontal lobes. To evaluate atten- (cid:129) Visuospatialprocessingreliesupondistributednet-
tional mechanisms, one can observe the patient’s worksthatcomposethe“dorsalstream,”whichin-
ability to answer directed questions and avoid dis- cludes parietal areas specialized for processing
tractions. Working memory can be evaluated by motion and spatial relationships. Lesions that dis-
assessingdigitspan,havingthepatientspellaword rupt right parietal areas and their networks may
backwards, or having the patient continue specific produce the clinical syndrome of hemispatial
patterns. Patients with lesions affecting the dorso- neglect. Higher-order visual processing also relies
lateral prefrontal cortex demonstrate impaired ona“ventralstream,”whichincludesinferiortem-
attention and working memory. Lesions of the poralareasspecializedforprocessingvisualfeatures
medialfrontal lobescan produce akinetic mutism, ofanobject,aface,orascene.
which is a syndrome of psychomotor retardation (cid:129) Emotionalprocessingisoneofmanyfunctionsper-
resembling severe depression. Lesions of the orbi- formed by the limbic system of the brain, which
tofrontalcortexproducedisinhibitedbehaviorsthat includes the cingulate cortex, amygdala, thalamus,
maytransgressacceptedsocialnorms. and hypothalamus. These regions contribute to
(cid:129) Memory can be divided into declarative memory consciously experienced emotions but also have
(which encompasses episodic memory for auto- strong connections with functions unconsciously
biographical events and recognition memory for carried out by the autonomic nervous system.
2
Pathology of the limbic system can have complex The cases in this section illustrate principles
cognitive and behavioral manifestations that blur regarding the localization, diagnosis, and manage-
thedistinctionbetweenneurologicandpsychiatric mentofconditionsthatimpairarousalorothercog-
disease. nitivefunctions.
3