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N e u r o lo g y ® Neurology.org Clinical Reasoning in Neurology: A Case-Based Approach C lin ic a l R e a s o n in g in N e u r o lo g y : A C a s e - B a s e d A p p r o a c h 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

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management plan. Some Clinical Reasoning cases report surprising and unexpected diagnoses. Others describe how to approach common clinical problems. All cases, however .. Akins PT, Belko J, Uyeki TM, Axelrod Y, Lee KK, tional Classification of Sleep Disorders–Second Edition. (ICSD-2).
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Most books are stored in the elastic cloud where traffic is expensive. For this reason, we have a limit on daily download.