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Preserved Recognition in a Case of Developmental Amnesia

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Preserved Recognition in a Case of Developmental Amnesia Preserved Recognition in aCase of Developmental Amnesia: Implications for the Acquisition of Semantic Memory? Alan Baddeley 1,Faraneh Vargha-Khadem 2,andMortimer Mishkin 3 Abstract & We report the performance on recognition memory tests of normally associated withepisodic memory. Hisrecall of Jon, who,despite amnesia fromearly childhood, has developed previously unfamiliarnewsreel events was impaired,but gained normal levels ofperformance on tests ofintelligence, language, substantially fromrepetition over a2-day period.Our results and general knowledge. Despite impairedrecall, he performed are consistent withthe hypothesis that the recollective process withinthe normal range on each ofsix recognition tests, but he ofepisodic memory isnot necessary either forrecognition or appears to lack the recollective phenomenological experience forthe acquisition ofsemantic knowledge. & INTRODUCTION thecapacity to store and recollect experienced events; In the lastfew decades, there hasbeen extensive and semantic memory,a systemthat stores and retrieves research intothe cognitive neuropsychology of the knowledge about theworld (Tulving,1972). The term amnesic syndrome,ranging from Milner’s(1966) early episodicmemory is used generally to refer to the research onpatient HM,through the attempts to relate capacity torecollect pastexperience, either byrecalling thefunctional deficits of amnesic patientsto laboratory theevent, or inthe case of recognition, byrecollecting studiesof normal subjects(Squire, Knowlton, &Musen, information associated withthe learning experience 1993; Baddeley &Warrington, 1970), upto thepresent (Tulving,1972). Thisphenomenological aspect of epi- situationin which it is widely accepted thatdata from sodicmemory is essential to Tulving’ suse of theterm, studyof theamnesic syndromemake acrucial contribu- althoughothers use theterm somewhatmore broadly. tionto our current concepts of normal humanmemory There hasin recent years been considerable interest in (Schacter &Tulving,1994). Whilethere have been thephenomenology of recognition, withconsiderable numerousattempts to argue for different typesof evidence thatsubjects are successfullyable to categorize amnesia usuallyrelated todifferential lesions(Parkin, itemsthey have recognized on thebasis of whether they Leng, &Hunkin,1990; Huppert& Piercy,1979), such have been remembered withassociated recollective subdivisionshave proved difficult tosustain,leading toa experience, or whether subjectssimply ’ ’know’’that somewhatnegative, thoughby no means universalcon- theitem had previously been presented (Gardiner & sensusof aunitaryamnesic syndrome(Squire, 1992; Java,1993). Baddeley, 1997). Semantic memoryis assumed to reflect accumulated Thismodal view distinguishes between explicitor world knowledge suchas the meaning of the word declarative memorythat is grossly impaired in amnesia, ’’fish,’’thecolor and taste of abanana, thenumber of and arange of implicit,procedural, or nondeclarative yardsin a mile,or thenormal procedure for eating ina memorysystems that are intact (Baddeley,1997; restaurant. Whileamnesic patientsusually retain seman- Squire, 1992). These implicitmemory systems have in tic knowledge acquired before theonset of amnesia, commonthe fact thatthey operate inthe absence of theytypically have great difficultyin adding further consciousrecollection. information to theirsemantic memorysystems, for Explicitor declarative memorycan itselfbe splitinto example, amnesic patientswould generally be unable two components:episodic memory, which represents toprovide the names of current politicalfigures suchas theU.S. President, and wouldbe unaware of recent world events and of themeaning of newlycoined words 1 University ofBristol, 2 University College London and Great (Squire et al.,1993). The associationof impairedepiso- Ormond Street Hospitalfor Children, London, 3 National dic memorywith the failure to update semantic memory Institute ofMental Health,Bethesda, MD isconsistent with the view that semantic memoryre- © 2001Massachusetts Institute ofTechnology Journal ofCognitive Neuroscience13:3, pp. 357–369 flects theresidue of manyepisodes (Baddeley, 1997; witha similarprofile, whowere alsoable to absent Squire, 1992), althoughthis view is not universally held themselvesfrom workfor theseveral daysnecessary to (Mishkin,Vargha-Khadem ,&Gadian, 1998; Tulving, complete thefull testbattery, proved difficult. We were, 1972; Tulving& Markovitch,1998). however, able to locate two suchcontrol subjects. Althoughthis overall viewhas, for manyyears, been Their resultswere used,together withresults from able to account for thebulk of evidence, ithas recently the somewhatlarger butless well matched groups been challenged bythe description of whatappears to from thepublished literature, as abaseline for inter- be anew amnesic syndrome.The patientsconcerned preting Jon’sperformance. appear tohave developed amnesia to agreater orlesser degree at, or within,a few years of birth,a situation Case Description whichshould, according to themodal view, have led to grosslyimpaired semantic memory.Nevertheless, three Jon,who is now 23, was bornprematurely at 26 weeks suchcases reported byVargha-Khadem et al. (1997) ofgestation, weighing lessthan 1 kgand suffering from have demonstrated an apparently normal acquisitionof breathing problems(Gadian et al.,2000). For prolonged language, together witha remarkably well developed periodsduring hisfirst six weeks of life, he suffered knowledge of theworld. The implicationof suchcases from severe apnea, requiring intubationand positive for theconcept of semantic memoryis discussed else- pressure ventilation.He subsequentlyshowed steady where (Mishkinet al.,1998; Squire &Zola, 1998; Tulving improvementand normal development.At 3years 10 &Markovitch,1998); the purposeof the present studyis months,he hadan unconfirmed convulsiveepisode in to investigate further thenature of thememory deficit, associationwith cold and cough. Hismemory problems and inparticular, to providea more robusttest of the were firstnoted when he was about 5years old,and suggestion byVargha-Khadem et al.that this type of have since continued to be prominent.More specifi- amnesia maybe associated withpreserved recognition cally,Jon’ sparents report thathe hasdifficulty in memorydespite clear evidence of impairedrecall. Such reliablyfinding hisway, tends to forget where objects adissociationis not typical of theamnesic syndrome: and belongings are usuallykept or where theyhave just Patientsnot only fail to successfullyrecollect prior been put,and makesmany prospective memory errors, experiences, butthey also fail todistinguish between finding itdifficult to keep even regularly scheduled novelwords, pictures or events,and itemsthat have appointmentsunless reminded. Jonalso has difficulty already been presented (Squire et al.,1993). remembering detailsof everyday activitiessuch as con- We begin byrecapitulating thecase of Jon,one of the versationsor televisionprograms watched. The severity three described byVargha-Khadem et al.,illustrating the ofthedisability is such as to make itdifficult for Jonto evidence for hisamnesia, together withthe comparative cope independently,or tosucceed infinding and keep- preservation of hissemantic memory.This is then ing a job. followed bya summaryof hisneuropathology as deter- Table 1showsthe performance of Jonand thetwo minedby quantitative magnetic resonance techniques. control subjects,Andrew and Max, on intelligence, scho- We thengo on toa brief account of earlier claimsof lastic,and language performance tests,all carried out preserved recognition inpatients who have acquired between theages of 19 and 22. Jonhas above-average theiramnesic deficit as aresultof braininjury during intelligence witha full-scale IQof 114, withsubtest adulthood.We discusssome of the methodological scores ranging from scaled scores of 10 (the normal problemsfacing anyattempt to compare recall and average) oninformation,to 16 (two standard deviations recognition, before describing astudyin which we above themean) on blockdesign. His number skills as compare Jon’srecall and recognition for bothvisual measured ontheWechsler Objective Numerical Dimen- and verbal material. Inorder to studythe generality of sions(WOND) testare above average, whilehis reading our findings,we thencompare Jon’sperformance to performance iswithin the normal range for basicreading thatof normal control subjects across several other (55th percentile) and comprehension(42nd percentile) recognition paradigms.We thendiscuss the implications coupled withpoor spelling (14th percentile). Jon’s of our resultsfor thedistinction between recall and language isalso within the normal range as measured recognition memory,and for thedevelopment of se- bythe Token Test,which requires the following of mantic memory. instructionsof increasing grammatical complexity,and The testsused comprisedboth standardized mea- hissemantic judgements appear tobe normal,as mea- suresfor whichappropriate normsare available, and sured bythe Pyramids and PalmTrees Test,a testof lesswidely used experimental tests.For thelatter, the semantic access from wordsand pictures(Howard & questionarises as tothe appropriate control compar- Patterson,1992). Jon’svocabulary development isalso ison.When tested at age 19, Jonshowed a slightly withinthe normal range, withthe WAIS-R vocabulary unusualWAIS-R intelligence profile, withhis perfor- subtestproducing ascaled score of 14, whilehis com- mance IQ(120) being somewhatgreater thanhis verbal
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