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Joumnal of Neurology, Neurosurgery, and Psychiatry 1995;58:655-664 NEUROLOGICAL INVESTIGATIONS Neuropsychological assessment Lisa Cipolotti, Elizabeth K Warrington Patients with brain damage may present with impairments of memory, language, percep- tion, thought, action, and other functions. These cognitive deficits can occur both in multiple domains or as highly selective impairments. In the 19th century and early 20th century, neurologists investigated cogni- tive impairments in patients with neurological disease by clinical and descriptive methods. These methods provided new insights and allowed the isolation of distinct syndromes- for example, aphasia,'2 alexia and agraphia,3 acalculia,4 visual agnosia,5 and amnesia.6 Indeed, these discoveries formed the basis for the development of a new discipline, "neu- ropsychology", devoted to the study of the relation between the brain and cognitive functions. The clinical and descriptive meth- ods, however, provided a poor standard of description of the cognitive impairments in these syndromes. They were ". . . little more than the bald statement of the clinical opin- ion of the investigator. . .". To deal with this lack, neuropsychologists developed principled techniques for the mea- surement of cognitive functioning. In the early days, psychometric tests, originally developed for the measurement of either scholastic attainment or occupational guid- ance, were used. In particular, tests for the measurement of intellectual and memory functions became available to the clinician.8-1 Gradually, over the past four decades an increasing number of measurement tools have been specifically designed for investigating the cognitive functions of patients with sus- pected or confirmed cerebral disease. Neuropsychological assessment involves the use of a series of tests that are "reliable"-in the same circumstances they produce the same result-and "valid"-they measure what they are designed to measure. The aim of this paper is to provide an overview of the main methods for the assess- ment of cognitive function and an outline of what may prompt a neuropsychological assessment (see also Lezak,"2 Crawford et al,3 and Hodges'4). Before approaching a neu- ropsychological assessment it is necessary to have a general theoretical structure on which to base and interpret the different levels of disturbance that can arise as a result of cere- bral damage. (for a similar view see Hodges'4) In the next three sections we discuss our gen- eral theoretical schema; the methods of assessment of cognitive function; and the pur- poses of a neuropsychological examination. General theoretical schema Our approach makes the assumption that impairments in cognitive function can best be studied and understood by (a) assuming that there is a high degree of functional specialisa- tion in the cerebral cortex; (b) by undertaking a modularity approach to the analysis of com- plex cognitive skills; and (c) by assuming that brain damage can selectively disrupt some components of a cognitive system.'5 The extent to which these assumptions have a direct anatomical substrate is less established. The idea that the human brain is highly differentiated in terms of its functional organ- isation is not new. The phrenologists in the early 19th century were already speculating that the convoluted surface of the brain reflected the juxtaposition of a large number of discrete cerebral organs each subserving a particular psychological faculty.'6 Several years after these accounts, neurologists began to study and record impairments of the higher cortical functions and their accompa- nying cerebral lesions. Aphasic disorders were extensively studied and the specialised lan- guage functions of the left hemisphere were recognised.'2'7 Subsequently, after the pio- neering work of Jackson,'8 the specialised visuoperceptual functions of the right hemi- sphere were also recognised. These early workers not only localised a number of spe- cialised functions in the brain but they also discussed their findings within a theoretical framework. For example, Lichtheim'9 pro- duced a complex diagram of the various sub- components of the language system by incorporating and expanding on Wemicke's2 original scheme. In his diagram, the various subcomponents of the language functions are represented as a series of "centres" (for exam- ple, the concept centre, the centre of the motor images of the words, the centre of the auditory images of the words), each of which was thought to be located in a specific area of the brain. These different functional centres were thought to be connected with each other through sets of fibre tracts. This approach- those adopting it were termed the "diagram makers"-has some resemblance to that of Psychology Department, National Hospital for Neurology and Neurosurgery, Queen Square, London WClN 3BG, UK L Cipolotti E K Warrington Correspondence to: Professor E K Warrington. 655 on August 17, 2020 by guest. 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Page 1: Neuropsychological assessment - Journal of Neurology ... · neuropsychological assessment is to establish whetherthe subjectis still functioningat their premorbid optimal level orwhetherthere

Joumnal ofNeurology, Neurosurgery, and Psychiatry 1995;58:655-664

NEUROLOGICAL INVESTIGATIONS

Neuropsychological assessment

Lisa Cipolotti, Elizabeth K Warrington

Patients with brain damage may present withimpairments of memory, language, percep-tion, thought, action, and other functions.These cognitive deficits can occur both inmultiple domains or as highly selectiveimpairments. In the 19th century and early20th century, neurologists investigated cogni-tive impairments in patients with neurologicaldisease by clinical and descriptive methods.These methods provided new insights andallowed the isolation of distinct syndromes-for example, aphasia,'2 alexia and agraphia,3acalculia,4 visual agnosia,5 and amnesia.6Indeed, these discoveries formed the basis forthe development of a new discipline, "neu-ropsychology", devoted to the study of therelation between the brain and cognitivefunctions. The clinical and descriptive meth-ods, however, provided a poor standard ofdescription of the cognitive impairments inthese syndromes. They were ". . . little morethan the bald statement of the clinical opin-ion of the investigator. . .".To deal with this lack, neuropsychologists

developed principled techniques for the mea-surement of cognitive functioning. In theearly days, psychometric tests, originallydeveloped for the measurement of eitherscholastic attainment or occupational guid-ance, were used. In particular, tests for themeasurement of intellectual and memoryfunctions became available to the clinician.8-1Gradually, over the past four decades anincreasing number of measurement tools havebeen specifically designed for investigatingthe cognitive functions of patients with sus-pected or confirmed cerebral disease.Neuropsychological assessment involves theuse of a series of tests that are "reliable"-inthe same circumstances they produce thesame result-and "valid"-they measurewhat they are designed to measure.The aim of this paper is to provide an

overview of the main methods for the assess-ment of cognitive function and an outline ofwhat may prompt a neuropsychologicalassessment (see also Lezak,"2 Crawford et al,3and Hodges'4). Before approaching a neu-ropsychological assessment it is necessary tohave a general theoretical structure on whichto base and interpret the different levels ofdisturbance that can arise as a result of cere-bral damage. (for a similar view see Hodges'4)In the next three sections we discuss our gen-

eral theoretical schema; the methods ofassessment of cognitive function; and the pur-poses of a neuropsychological examination.

General theoretical schemaOur approach makes the assumption thatimpairments in cognitive function can best bestudied and understood by (a) assuming thatthere is a high degree of functional specialisa-tion in the cerebral cortex; (b) by undertakinga modularity approach to the analysis of com-plex cognitive skills; and (c) by assuming thatbrain damage can selectively disrupt somecomponents of a cognitive system.'5 Theextent to which these assumptions have adirect anatomical substrate is less established.The idea that the human brain is highly

differentiated in terms of its functional organ-isation is not new. The phrenologists in theearly 19th century were already speculatingthat the convoluted surface of the brainreflected the juxtaposition of a large numberof discrete cerebral organs each subserving aparticular psychological faculty.'6 Severalyears after these accounts, neurologists beganto study and record impairments of thehigher cortical functions and their accompa-nying cerebral lesions. Aphasic disorders wereextensively studied and the specialised lan-guage functions of the left hemisphere wererecognised.'2'7 Subsequently, after the pio-neering work of Jackson,'8 the specialisedvisuoperceptual functions of the right hemi-sphere were also recognised. These earlyworkers not only localised a number of spe-cialised functions in the brain but they alsodiscussed their findings within a theoreticalframework. For example, Lichtheim'9 pro-duced a complex diagram of the various sub-components of the language system byincorporating and expanding on Wemicke's2original scheme. In his diagram, the varioussubcomponents of the language functions arerepresented as a series of "centres" (for exam-ple, the concept centre, the centre of themotor images of the words, the centre of theauditory images of the words), each of whichwas thought to be located in a specific area ofthe brain. These different functional centreswere thought to be connected with each otherthrough sets of fibre tracts. This approach-those adopting it were termed the "diagrammakers"-has some resemblance to that of

PsychologyDepartment, NationalHospital forNeurology andNeurosurgery, QueenSquare, LondonWClN 3BG, UKL CipolottiE K WarringtonCorrespondence to:Professor E K Warrington.

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modem cognitive neuropsychology theorists.Despite this, the idea that cognitive skills

such as language could consist of multicom-ponents and be localised in different, highlyspecialised areas of the brain came underattack from the "global theorists".2' Of par-ticular relevance here is the development of"mass action" theories. These theories pro-posed that there was no differentiation in thecortex for specific cognitive functions; rather,that it was equipotential with respect to cog-nitive abilities.24 According to such a view,any form of neurological damage woulddeplete by a greater or lesser extent the avail-able amount of some general cognitiveresource and not specific cognitive functions.The amount of damage to the general cogni-tive resource, also termed intellect or abstractattitude, would depend on the extent of thebrain damage and not on the site of thedamage.The notion that different brain regions are

specialised for different cognitive functionsregained popularity in the 1950s and themodem revolution in imaging techniques hasmade it possible to visualise these structuresin the living human brain. The idea that thereare cognitive processing systems that involveonly specialised brain regions is now accepted". . . as one of the comerstones of modembrain science ."..".25 For about 95% of righthanders and 70% of left handers major lan-guage, literacy (reading, writing, and calcula-tion), verbal short term memory, verbal longterm memory, semantic memory, and praxisare represented in the left hemisphere. Theright hemisphere is involved in non-verbalprocessing such as the analysis of perceptualand spatial stimuli, spatial short term mem-ory, visual long term memory, spatiallydirected attention, face recognition, topo-graphical knowledge, and in some prosodiccomponents of language. For those few peo-ple who do not have normal lateralisation thispattern seems to be reversed, although a verysmall proportion of subjects may have bilat-eral organisation of some cognitive skills. Theanterior parts of both hemispheres have beenaccepted as being implicated in problem solv-ing processes that are required in a widerange of situations including practical rou-tines and social interactions as well asabstract reasoning tasks.7 The most posteriorparts of both hemispheres are involved inearly visual processing. Subcortical, as well ascortical, brain regions, are involved in atten-tion and alertness. Subcortical brain regionsare also involved in episodic memory, insome aspects of long term memory, and inthe motor control of language.12A modularity approach to the analysis of

cognitive skills implies that each complexcognitive process can be thought of as con-sisting of a series of functionally independentspecialised subprocesses.72$29 The interactionof these subprocesses results in the complexcognitive skills. The way in which the cogni-tive processes are organised is often charac-terised, similarly to the "diagram maker"approach, in terms of flow diagrams that

attempt to detail the way that the differentsubprocesses are brought together to performa specific task. Empirical support for themodularity approach can be obtained at vari-ous levels including the neurophysiological,neuroanatomical, and neuropsychological.'031For example, numeracy has been fractionatedinto several independent components: cogni-tive mechanisms for number comprehension,number production, arithmetical factretrieval, and arithmetical procedures.'2The idea that complex cognitive skills are

carried out by distinct subprocesses com-bined with the idea that there are highly spe-cialised areas in the brain, has led manycognitive neuropsychologists to assume that acerebral lesion can damage only some sub-processes within complex cognitive skills.Indeed, cognitive neuropsychologists havesucceeded in showing many dissociationsbetween the subcomponents of cognitiveskills that allow valid conclusions about thenature and functions of the impaired process-ing components to be drawn.

Methods ofassessment of cognitivefunctionsOne of the fundamental principles underlyingneuropsychological assessment is to establishwhether the subject is still functioning at theirpremorbid optimal level or whether there hasbeen a deterioration. Therefore, the methodsused for assessing cognitive functioning inneuropsychology need to be able to provide:(a) an indirect measure of the premorbidskills of a person and (b) a measure of thepresent cognitive state of that person. Oncethe two types of measures are obtained theycan be compared. This should indicate:firstly, whether the functioning has changedfrom the premorbid state; and secondlywhether this reflects organic or functionalimpairment. If the results indicate organicimpairment then an attempt will be made toestablish the extent of the change. It is notonly useful to know that a change in cognitivefunctioning has occurred; it is also useful toknow whether the change can be charac-terised as global or focal. If the cognitiveimpairment is focal, neuropsychological mea-sures can be used to specify more preciselythe cognitive impairments: whether theimpairment is indicative of lateralised dys-function or confined to the anterior or poste-rior regions of the brain. In exceptional cases,it is possible to document highly selectivecognitive impairments with a known and rela-tively precise anatomical localisation.A comprehensive neuropsychological

examination would include the assessmentof: (a) premorbid ability; (b) general intellec-tual level; (c) memory; (d) language; (e)calculation; (f) problem solving; (g) alertnessand attention; (h) visual and space percep-tion. Ideally a cognitive profile would beconstructed from performance on tests ofproved validity and comparable difficulty.A long term aim for the neuropsychologistis to achieve a level of measurement for all

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Neuropsychological assessment

Verbl IQ Speed..,.. Pe rforma nce IQ

.I\Verbamemory,/

Naming Pe

Reading _

Spelling Spatial skillsArithmetic

Percentiles<1 a1<5 a5<25 E >25<50- ¢50O

cognitive skills that permit comparison across

tasks and is sensitive to change. The methoddescribed by Newman et al in their study thatmonitored subjects at risk for Alzheimer's dis-ease exploited this methodology (figure)."

In the next section we do not attempt todescribe all the tests and techniques availableto the neuropsychologists for investigation ofall these different areas of cognition. Ratherwe focus on three main areas of cognitivefunction: intelligence, memory, and languagefunctions. These serve to illustrate the rangeof techniques and procedures available for theinvestigation of cognitive impairments."

ASSESSMENT OF PREMORBID ABILITYVarious procedures are adopted for obtainingan indirect measure of a subject's premorbidskills, which can then be compared with hiscurrent level of performance. These proce-dures can be divided into two main types:methods that use demographic data such as

age, sex, race, education, and occupation;and methods that use tests considered to berelatively resistant to neurological and psychi-atric disorders. The first type of method isbased on the known relation between a num-

ber of demographic variables and measuredIQ*.34 Not only may educational and occupa-tional records be used as a rough estimate ofa subject's optimal or premorbid level offunctioning; they may also, through the use ofvarious types of regression equations, providea more precise and objective estimate.'5 Oneof the principle limitations of this type oftechnique, however, is that educational andoccupational histories may not always bereadily available and they may be incomplete,uninformative, or anomalous.The second method involves the measure-

ment of a cognitive skill that is known to behighly correlated with intellectual factors andresistant to brain damage. This method isobviously not reliant on preexisting data.Some of the first methods of this typeinvolved the use of vocabulary.'6 These meth-ods were based on the finding that patientswith brain disease retained old, well estab-lished verbal skills, such as those implicated

in the verbal definition of words, long afterother cognitive skills were impaired. Theapplication of the same principle led to thedevelopment of various Wechsler deteriora-tion indices.'2More recently, a measure of premorbid

optimal level of functioning has been basedon the overlearned skill of reading. Nelsonand McKenna"7 first established that wordreading skill, as measured by the Schonellgraded word reading test38 was highly corre-

lated with general intelligence in a normalpopulation. Nelson and O'Connel139 thenestablished that the reading of irregularwords, such as "heir" or "chord", which can-not be pronounced correctly by applying theusual rules that map spelling on to phonol-ogy, were better indicators of premorbidintelligence (IQ) in demented subjects thanestimates based on reading regular words.Nelson subsequently developed the Nationaladult reading test (NART), which consists of50 irregular words. Indeed, the NART hasbecome one of the most commonly usedmeasures of premorbid intelligence.4' AnAmerican version of this test is also avail-able.42 One of the major limitations of theNART test is that it cannot be used withthose who have poor literacy skills or inpatients with obvious impairments of speechproduction or problems associated withdyslexia. In addition, there have been claimsthat patients with dementing disorders maynot present with preserved irregular wordreading.43 Consequently these patients pre-sent with difficulties in reading the NARTwords, and this would result in erroneous lowestimates of their premorbid IQ. In the cases

of early dementia when language skills are rel-atively unimpaired, however, it has beenshown that the NART remains stable over

time and can be used as a predictor of thepremorbid optimal level (Paque andWarrington, unpublished data).

ASSESSMENT OF GENERAL INTELLECTUAL LEVELHistorically, intelligence has been defined inmany different ways. For example,Spearman,44 although he himself avoided theterm intelligence, proposed the existence of a

central intellectual ability, which he referredto as "g". Although he never actually definedwhat g was he thought that it involved "theeduction of relations and correlates".4" In hisformulation g referred to the determinant ofshared variance among various tests of intel-lectual ability. An alternative view, associatedwith Thurstone45 and Guilford,46 involvedthe application of the term intelligence to a

large set of diverse mental abilities (or fac-tors). These included not only reasoningand problem solving on new data but also

*The term IQ was first introduced by Stern40 todescribe a method of comparing one child's score onthe Binet intelligence scale with the performance ofaverage children of the same age. It is nowadays usedto indicate intellectual level by comparing a subject'sperformance with the average scores attained by mem-bers of the same age group.

Example of a circlediagram of cognitive skills.Sectors of the circle havebeen apportioned to each ofthe cognitive skillsexamined. The concentriccircles represent the level offunctioning in terms ofpercentile score. The levelsof the subject's testperformance are indicatedby the degree of eccentricitywithin a sector. In thispatient the most prominentfeature was a globalmemory impairment morepronounced for verbalthan visual material.(Diagram courtesy ofNewman et al. 33)

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specialised knowledge derived from priorschooling or experience. In line with this,Cattell47 distinguished between fluid intelli-gence, the ability to deal with novelty and toadapt one's thinking to a new cognitive prob-lem, and crystallised intelligence, whichreflects a knowledge base and skills that havebeen previously acquired through learningand experience.The available measures of intelligence

reflect these different formulations of the abil-ities underlying intelligence. For example,Raven's test, including the coloured progres-sive matrices,"1 the standard progressivematrices,'0 and the advanced progressivematrices (sets I and II)48, are widely used forthe clinical assessment of general intelligence.The various versions of this test are believedto weigh heavily on g and measure processesthat are central to the definition of fluid intel-ligence (more recently also termed analyticintelligence49). Indeed, they require abstractreasoning, induction of relations, and educ-tion49 The test was developed as a "culturefair" measure of general intellectual abilityand because of its non-verbal format, its easeof use, and its speed (especially the colouredprogressive matrices), it has gained wide usein both clinical and research settings. Thismay be overoptimistic as educational level hassubsequently been shown to have a majoreffect on the normal subject's performance.50The Wechsler adult intelligence scale

(WAIS)5' 52 iS considered to be one of the coremeasures for evaluating general intellectualability. It involves six verbal and five non-verbal subtests that sample various skills.These subtests are thought to measure vari-ous mental abilities as would follow fromThurstone's45 and Guilford's46 views, includ-ing both the explicit knowledge base derivedfrom educational and previous experienceand the ability to deal with and solve newcognitive problems. Verbal and performanceIQs are determined from the use of theWechsler scales. Much research has focusedon discrepancies between verbal and perfor-mance IQ as a means of differentiatingbetween left and right hemisphere impair-ment53 although this has not resulted in ageneral consensus.'2 Indeed, Warringtonet al54 suggested that such scales have littlevalue as regards the localisation of a lesion or,for that matter, the identification of specificcognitive deficits. Nevertheless, the Wechsleradult intelligence scale-revised (WAIS-R), thesuccessor of the WAIS is the most often usedpsychological test of intellectual functioningand is a cornerstone for most neuropsycho-logical test batteries. It is also widely usedwith geriatric patients, and recently, norma-tive data for people who are 75 or older havebecome available.55 Numerous studies arebased on the WAIS-R.

ASSESSMENT OF MEMORYMemory is not a unitary function but rather acollection of distinct and independent com-ponents, each of which is associated with dif-ferent brain structures. A broad distinction is

generally made between short and long termmemory. Short term memory is considered tobe responsible for the immediate retention ofa limited amount of information; this infor-mation will decay in a matter of seconds if itis not refreshed. Long term memory retainslarger amounts of information for longer peri-ods-depending on the salience-which maybe for minutes, days, and years. Short andlong term memory functions can be furtherdivided into verbal and visual memoryaccording to whether they retain verbal ornon-verbal information.56 Long term memoryis also subdivided into implicit (or proce-dural) and explicit (or declarative) memory.57Implicit memory retains information thataffects behaviour but it is not available forconscious recollection (for example, motorskills, conditioned reflexes, priming). A fur-ther example is the three letter word stemcompletion task, which can be performed byguessing rather than by conscious recall.58 Inthis task patients are presented with a list ofwords and their retention is tested either bystandard recall and recognition techniques orby presenting the first three letters of thetarget item in a word completion task (forexample, "cha"-chair). Explicit memoryretains information that can be consciouslyaccessed. It is subdivided into episodic andsemantic memory.59 Episodic memory con-tains information about temporally datedepisodes or events and temporospatial rela-tions among them (for instance, this can befor both autobiographical memories andmemories of an artificial event such as a wordlist or short stories). Semantic memory con-tains our organised knowledge of conceptsand facts as well as words and their meanings(for example, encyclopaedic memories). Mostclinical assessments focus on three main typesof memory functions: short term memory,episodic memory, and semantic memory.

Assessment of short term memoryThe assessment of verbal short term memoryusually requires the repetition of a progres-sively lengthening string of digits (digit span),letters, and words. The normal range of digitsis five to nine. Spatial short term memory canbe assessed with the Corsi block tappingtest.60 This requires the subject to tap a pro-gressively lengthening sequence of blocks.

Assessment of episodic memoryMany tests and batteries are available for theassessment of episodic memory.61 These useeither a recall or recognition paradigm andtypically assess the anterograde (the ability toacquire new information) rather than the ret-rograde component (the ability to recall pre-viously learnt material). One of the oldestbatteries used is the Wechsler memory scale(and the Wechsler memory scale-revised),which requires the recall of both complex ver-bal material (for example, short stories) andvisual material (for example, reproduction ofgeometrical designs). Some of its subtests arenot dependent on memory itself but rather onattentional processes (for example, mental

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control and orientation). Unfortunately allthe subtests contribute to the final memoryquotient. A more recently developed test forthe assessment of long term verbal memory isthe adult memory and information processingbattery,62 which has many similarities to theWechsler memory scale. The Rivermeadbehavioural memory tests consist of a seriesof tests held to have ecological validity.6' Atask that is also very often used for assessingverbal anterograde recall is word list learning(for example, the auditory-verbal learningtest64). For the assessment of non-verbalanterograde recall, the two most commonlyused tests are the Rey-Osterreith complexfigure test65 and the Benton revised visualretention test.66 Both require the recall ofgeometric figures.

Warrington67 developed a test that used arecognition rather than a recall paradigm (therecognition memory test). The recognitionparadigm was chosen because it is possible tohave comparable tests of verbal and visualmemory. This test incorporates the verbaland non-verbal dichotomy by having separatesubtests with word and face stimuli. Age cor-rected percentile scores of a large standardisa-tion sample are available. Validation of thistest has shown that patients with right hemi-spheric lesions are impaired on the visual ver-sion and patients with left hemispheric lesionsare impaired on the verbal version. It has alsobeen shown that this test can detect minordegrees of memory deficit." Clegg andWarrington68 have also recently standardisedand validated four "easy" memory tests (threerecognition memory tests and a word pairedassociate learning test) for older adults (64and older) that are recommended for patientsin whom memory impairment is suspectedbut whose mental state (for example, poorattention, anxiety, or agitation) precludeslonger or more demanding tests.Most tests of retrograde verbal and visual

recall have been devised for research ratherthan clinical purposes. They normally testrecall and recognition of famous names andfamous faces. Perhaps because they soquickly become dated their standardisationand validation are problematic. A relativelynew test assessing autobiographical memoryis an exception to this rule (autobiographicalmemory interview69). This test requires therecall of personal remote facts and incidentsfrom three epochs: childhood, early adult life,and recent experience.

Assessment ofsemantic memoryPatients with a semantic memory disorderpresent a general loss of knowledge, includingobject and word meaning. This deficit canmanifest itself as an inability to comprehendwords and identify pictures and objects. Theclassic syndromes of transcortical sensoryaphasia and visual associative agnosia havebeen identified with the impairment ofsemantic memory.70 There are no standard-ised batteries for the assessment of semanticmemory because, unlike episodic memory, ithas only been studied in the past 20 years,

after the seminal paper of Tulving.59 Semanticmemory can, however, be assessed throughtests devised for other domains (mainly testsalso used for the assessment of language dis-orders). To evaluate the difficulties in worddefinition some verbal subtests of theWechsler scales, such as vocabulary andinformation, can be used. Naming tests canbe used as indirect evidence of semanticmemory impairment (see language sectionlater). The pyramids and palm tree test7' wasdeveloped specifically to evaluate impair-ments in the understanding of concepts.There is a verbal and a pictorial version ofthis task devised for assessing conceptualrelations. Limited normative data are avail-able. A further test, the British picture vocab-ulary test,72 which uses a word picturematching technique, was first developed forthe assessment of language developmentsbetween the ages 2 and 18. More recently ithas been standardised in a normal healthyelderly population (Clegg and Warrington,unpublished data).

ASSESSMENT OF LANGUAGELanguage is not a unitary fimction. The mostuseful dichotomy is to consider spoken andwritten language separately.

Spoken language can be characterised as acollection of independent components, eachof which is associated with different brainstructures. The three main central linguisticcomponents are phonology, syntax, andsemantics.7' Phonological processing analysesthe constituent sounds of words. Syntacticprocessing analyses the grammatical aspectsof language-for example, the ordering of theindividual words in the sentence. Semanticprocessing analyses the referential meaning ofwords. In addition to these three componentsthere are more specialised peripheral systemssubserving articulation and prosody.Furthermore, at least for phonology and syn-tax, receptive and expressive deficits canoccur as selective impairments.7'76Disruptions in phonological or semantic pro-cessing are found at the level of single wordswhereas disruptions in syntactic processingare found at the level of sentences.

Assessment of spoken languageThere are several traditional clinical tax-onomies of the acquired aphasias principallyinherited from the earliest scientific papers onlanguage disorder.'-3 19 These taxonomies,based on mixed functional, anatomical, andpathological terms, have inspired the develop-ment of classic aphasia batteries. The mostwidely used are the Boston diagnostic aphasiaexamination,76 the western aphasia battery,77the Porch index of communicative ability,78and the Aachen aphasia test.79 The traditionaltaxonomies that form their basis have beenquestioned in so far as they failed to capturethe multidimensional pattern of languagebreakdown, they are not useful for guidingtherapy or for the detailed analysis andunderstanding of language disorders.80 In thissection we provide a brief account of the core

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tests that could provide a framework for themore detailed assessment of a patient's lan-guage impairment. We discuss only two areasof language dysfunction: word and sentencecomprehension and word and sentenceretrieval.

Word and sentence comprehension-Wordcomprehension deficits can occur as a resultof an impairment in auditory perception or asa result of an impairment in word meaning.An auditory word perception deficit can beidentified in patients that have a deficit inword repetition that cannot be attributed to amore general articulatory deficit.8' It can beassessed through phonological discriminationtasks that are usually included in most of thetraditional aphasia batteries. Impairment ofword meaning is one component of thesemantic memory disorders (see earlier) and,as the word retrieval deficit (see later), can becategory specific. For example, selectivedeficits for abstract and concrete conceptsand within the concrete domain animate orinanimate reference and even specific wordclass effects have all been reported.82 One ofthe most direct tests of word meaning aresynonyms tests (for example, "timid" means"afraid" or "quiet"). Coughlan andWarrington83 have offered a modest standard-isation of one such test. Word meaning com-prehension can also be tested by usingword-picture matching tests such as the pyra-mids and palm tree test7l and the Britishpicture vocabulary test,72 described in thesemantic memory section. In addition, therecent psycholinguistic assessment of lan-guage processing in aphasia84 is a usefulresearch tool for assessing comprehension inthe domains of verbal and visual knowledge.One of the earliest and most commonly

used test of sentence comprehension is thetoken test devised by De Renzi and Vignolo.85This test uses tokens of different shapes,sizes, and colours and the patient is given anoral instruction in progressively more com-plex non-redundant sentences (for example,"put the red circle between the yellow squareand the green square"). There have been var-ious modifications of the test including ashortened version by De Renzi and Faglioniand a very abbreviated version by Coughlanand Warrington.8' Educationally standardisednormative data are available. Parisi andPizzamiglio86 devised a test specifically fortesting syntactic comprehension (for anEnglish version see Lesser87). Another test forgrammatical comprehension is the test forreception of grammar.88 This test was devel-oped for the assessment of language develop-ments and has been used also in the contextof acquired aphasia investigations. It shouldbe acknowledged that some normative dataare available for the sentence comprehensiontest reviewed here and are undoubtedly veryuseful for in depth assessment of a patient'saphasic deficit.

Word and sentence retrieval-Word retrievaldifficulties are exemplified by the syndromeof amnestic or nominal dysphasia and areoften present in other aphasic syndromes and

in cortical degenerative conditions. They canbe specific for particular categories such asletters, colours, body parts, proper names,and fruits and vegetables.3' To evaluate wordretrieval difficulties naming from verbaldescription (for example, "what is the nameof the large grey animal with a trunk") andpicture naming tests can be used. The gradednaming test89 was developed to identify verymild degrees of anomia. It comprises itemsof low frequency and it has been standardisedin a normal population and validated inpatients with unilateral lesions. The Bostonnaming test90 comprises line drawings ofobjects and has been widely used in aphasiastudies. Only a limited standardisation isavailable.

Spontaneous language is often elicited bycomplex picture description. The cookie jartheft picture from the Boston diagnosticaphasia examination is widely used for thispurpose. De Renzi and Ferrari9' devised thereporter test requiring the patient to act as areporter of the performance carried out bythe examiner who acts in accordance with thecommands of the token test described earlier.This test is particularly useful for the identifi-cation of impairments in grammatical sen-tence construction, although there are onlylimited normative data at present.

WRItEN LANGUAGEIn the past 30 years cognitive neuropsycholo-gists have investigated reading and writingdisorders in detail and depth. This hasresulted in the identification of new syn-dromes that take the description of readingand writing difficulties well beyond the classicsyndrome described by Dejerine3: dyslexiawith dysgraphia and dyslexia without dys-graphia. Each of these different dyslexic anddysgraphic syndromes corresponds to anidentifiable impairment in a subcomponent orsubcomponents of the reading and writingprocess. Shallice and Warrington92 have pro-posed a distinction between peripheral andcentral dyslexic syndromes and thisdichotomy applies equally well to the dys-graphia syndromes. Peripheral dyslexias anddysgraphias result from damage to processesresponsible for the categorisation of a stringof letters or phonemes as orthographic orphonological entities. Central dyslexias anddysgraphias are due to impairment in thecomprehension and production of a targetstimulus. The study of central dyslexias anddysgraphias has provided evidence thatthere are at least two parallel forms ofprocessing for reading and writing: onephonologically based and one semanticbased. Phonological processing utilises a setof rules for translating print to sound orsound to print. It is used for reading or writ-ing unfamiliar words or non-words. Semanticprocessing accesses meaningful representa-tions of the words that are in the subject'svocabulary. These two types of processingcan break down independently to producedifferent types of reading and writingimpairments.

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Assessment of written languageFollowing the seminal work of Marshall andNewcombe93 a psycholinguistic method ofassessment of written language disorders hasgained wide popularity. This method involvesthe presentation of lists of words that samplecontrasting psycholinguistic properties. It isthought that the data on the effect of the psy-cholinguistic and visual (length, script, anddisplays) variables coupled with an errorsanalysis allow conclusions to be drawn aboutthe likely origin of dysfunction within thereading system. In this section we provide adescription of some of the standardised andvalidated formal tests for the assessment ofreading and spelling disorders.Reading-Any assessment of reading skills

should include an evaluation of a patient'sability to read both single words and text. Insome peripheral dyslexias the ability to readtext can be impaired whereas the ability toread single words can be spared. (for exam-ple, attentional dyslexia94) The Neale test95for assessment of prose reading in children isuseful in this context. In addition, most of thestandard aphasia batteries include a subtestfor text reading (for example, Boston diag-nostic aphasia examination). For the formalassessment of single words, the two mostwidely used tests are the NART and theSchonell graded word reading test. Both testsare graded in difficulty and are measures ofreading skills; an estimate of premorbid opti-mal level of functioning can also be obtained(see earlier). When assessing reading skills itis important to evaluate the patient's ability toread aloud non-words. This ability can beselectively impaired despite good word read-ing as in the case of phonological dyslexia.96No formal standardised tests for non-wordreading are available; however, several listshave been devised for research purposes.

Oral and written spelling-Written and oralspelling are known to dissociate and thereforeare assessed independently. For the assess-ment of written spelling the Schonell gradedspelling test can be used. For the assessmentof oral and written spelling, Baxter andWarrington97 have recently standardised andvalidated a test that is sensitive to minordegrees of deficit in the general neurologicalpopulation. This is a graded difficulty test;thus the raw scores can be converted into per-centile scores. For patients whose poor eyesight precludes reading, spelling can provide auseful measure of premorbid abilities. Theassessment of non-word spelling is alsoimportant because patients with phonologicaldysgraphia might present with some pre-served word spelling despite impaired non-word spelling.98 Several non-word spellinglists have been developed for research pur-poses.The use of the standard word reading and

spelling tests described combined with assess-ment of the patient's ability to read and writenon-words and an analysis of the errors madeby the patient identifies more than the pres-ence of a reading or spelling disorder. It canalso provide some preliminary indication of

the status of the peripheral and central pro-cessing involved in word reading and spelling.This, rather than the description of thepresence or absence of a reading or spellingdisorder, has a clear clinical and theoreticalsignificance.

Purposes of a neuropsychologicalassessmentThere are at least three main reasons for con-ducting a neuropsychological assessment:diagnosis, treatment and management, andresearch.

DIAGNOSISA neuropsychological assessment allows thedescription and evaluation of the major cog-nitive deficits incurred in neurological patientswith possible brain disease. Furthermore, itcan indicate possible neuroanatomical corre-lates of the cognitive impairments. A neu-ropsychological assessment can, at the veryleast, provide pointers as to whether there isunilateral, bilateral, or subcortical damage.This information can be useful in diagnosis.Neuropsychological assessment has a key rolein differentiating between organic and func-tional disorders.'4 99-101 There are other neuro-logical conditions-for example, corticalatrophy, frontal and temporal lobe tumours,and undetected temporal lobe seizures-thatmay manifest themselves with symptoms thatcan be misinterpreted as functional.'02 Forexample, patients with visual disorientationdisorders due to bilateral occipital disease areoften misdiagnosed on the grounds that theirvisual handicap seems to be disproportionatein the context of normal or near normalacuity. 103On the other hand, patients with symp-

toms of pseudodementia, such as hysteria,malingering, Ganser syndrome, bipolar disor-der, and other ill defined psychiatric disordersoften present with an abrupt intellectual andmemory failure that mimics true cognitivedeficits. A neuropsychological assessment candistinguish between organic and functionaldisorders. It does this by highlighting discrep-ancies between subjective complaints andobjective performance, usually detecting anumber of inconsistencies in the patients'performance and a too obvious mismatchbetween objective performance and daily lifeactivities. Also, the body of neuropsychologi-cal knowledge on the organisation and frac-tionation of cognitive skills is nowadayshighly developed. Crucially, the way in whichthe cognitive functions can fractionate oftendiverges from the common sense opinion ofhow a cognitive function can break down.Thus the patient's pattern of performancecan be interpreted as neuropsychologicallyconvncmig or unconvincing. To consider oneexample, a neuropsychological assessment isuseful in differentiating organic and func-tional memory loss. Studies of patients withdense organic amnesia have shown that theycan still learn new associative informationprovided that they are tested using implicit

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learning tasks. 104 For example, they showsavings with repeated presentations of frag-mented or degraded stimuli (pictures orwords) in increasing degrees of completeness.Even quite severely demented patients wouldshow some learning with these tasks. Clearlya patient showing additional impairments onthese implicit learning tasks makes no neuro-psychological sense. It makes good commonsense, however, to also be impaired on thesetasks (I have poor memory, I can't rememberthings). Indeed, a poor performance on thesetests may be considered indicative of func-tional memory loss. On the contrary, a rela-tively preserved performance on these tasksconforms to an organic pattern.

Another common differential diagnosiswhere neuropsychological assessment has akey role is between early dementia, anxiety ordepressive disorders, or the normal agingprocess. The diagnosis of probable dementiais usually made by establishing whether thereis an acquired deficit of cognition withouthystopathological evidence obtained from abiopsy or necropsy. Often in the early stagesof a dementing illness, the clinical diagnosiscannot be supported by neuroimaging such asCT, MRI, or functional imaging. Patientswith depression or anxiety may complain ofintellectual or, more often, memory failuresimilar to the so called "worried well" patients.Usually depressive or anxiety pseudodementiashould be suspected when the patient com-plains of the memory problem more than thecarer.'05 In these cases recognition memorytests should be used to determine whether thefailure is due to an organic condition or to anx-iety or depression. It has been shown thatrecall tests of memory are vulnerable to theeffect of anxiety and depression, whereasrecognition memory tests are not.'06The aging process itself is associated with

cognitive and memory changes. Hence, it isoften necessary to differentiate memory fail-ure due to cognitive deterioration rather thanbenign senescent forgetfulness. In these casesperformance in recall memory tests requiringthe subjects to engage in elaborative encod-ing, as opposed to allowing them to devisetheir own encoding strategy, may discrimi-nate those with brain damage from normalelderly subjects. For example, in word listlearning the strategy of performing associa-tions between successive words improves theoverall level of recall in normal subjects.

Neuropsychological assessment can alsohave a central role in diagnosing presympto-matic cognitive impairments in familial neu-rodegenerative conditions. From the natureof the inheritance and the relatively constantages of disease onset within a family, asymp-tomatic at risk subjects below the mean age ofonset can be examined. Such studies haveshown cognitive abnormalities in apparentlyasymptomatic subjects with Huntington's andAlzheimer's disease.33 107

TREATMENT AND MANAGEMENTThe baselines of cognitive functioning pro-vided by the neuropsychological examination

allow the monitoring of certain conditions.For example, successive neuropsychologicalexaminations provide reliable indications ofwhether a pattern of cognitive deficit associ-ated with head injury or stroke is changingand if so, how rapidly and in what way. Thisinformation is useful in planning the futuremedical and social care of the patient.Similarly, repeated-neuropsychological testingof patients with degenerative disorders canprovide information about their differentrates of cognitive decline and thus help themand their family plan for their care. Theresults of a neuropsychological assessmentcan also be used in the evaluation of medicaland surgical treatments such as those associ-ated with subcortical pathology that is associ-ated with cognitive slowing (for example,Parkinson's disease and hydrocephalus). Forinstance, obtaining repeated measures of ahydrocephalic patient's performance in aseries of psychomotor tests can provide a reli-able indication of whether the underlyingneurological condition is improving or deteri-orating. Psychomotor tests are simple verbaland non-verbal tests that involve verbal andvisuomotor responses and the measurementof the patients' speed of responding. 108

Practice effects are minimal in these tests,which are at the same time sensitive to subtlechanges in cognitive efficiency. Thereforethey can be used at regular and short intervalsfor monitoring the patient's neurologicalstate. Neuropsychological assessment is alsoparticularly important in monitoring the vari-ous treatments for epilepsy.'09 110

The baselines of cognitive functioning pro-vided by the neuropsychological examinationcan be important for planning and monitoringrehabilitation programmes. In particular,when planning such programmes, neuro-psychological evaluation can provide answersto key questions such as ". . . what are realis-tic treatment goals and ... what is thepatient's capacity to benefit from availabletreatment . . .".12 Moreover, repeated neu-ropsychological testing can be used to monitorthe effects of the rehabilitation programme.Furthermore, the baselines of cognitive func-tioning provided by neuropsychological exam-ination can be used to explain to patients andtheir families their relative cognitive problemsso that they can both prepare and understandthe type of difficulties the patient may facewhen he leaves the hospital.

Neuropsychological assessment has acentral role in the medicolegal context.Neuropsychological data concerning thetype and severity of a cognitive deficit, itsprognostic value, and the implications forfuture care are central issues in the litigationover compensation awards."' In this contextneuropsychological investigation is crucial inassessing the possibility of simulated disabilitythat can sometimes occur before financialsettlement."12

RESEARCHThere are two main neuropsychologicalresearch methodologies: the single case study

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and the group study.7 For both, neuropsycho-logical assessment procedures are a crucialelement. The strength of novel findings andunexpected dissociations can only be evalu-ated by reference to performance of estab-lished tests of cognitive skills. In groupstudies that explore new hypotheses neu-ropsychological measures are necessary toobtain baseline data.

Neuropsychological research may considerapplied clinical problems or be theoreticallydriven. In applied research, batteries of neu-ropsychological tests are commonly given todescribe the cognitive profiles associated withparticular neurological diseases. For example,specific cognitive profiles have been obtainedfor diseases such as Parkinson's disease andmultiple sclerosis.113 114 Neuropsychologicalassessments are also used to identify specificpatterns of cognitive deficits associated withAlzheimer's disease and other degenerativedementias.' 15-1 18 In particular, attempts havebeen made to differentiate various subgroupsexisting within a category of dementingdisorders. The long term aim is to further theunderstanding of the disease, in particularwith regard to early diagnosis and the possi-bility of pharmacological intervention."19 120

Theoretically driven neuropsychologicalresearch has proved to be of fundamentalimportance in the study of the organisation ofcerebral functions in the brain and especiallyin the understanding of normal cognitivefunctioning. Cognitive neuropsychologicalsingle case studies have been used as a legiti-mate type of evidence to support or criticiseinformation processing models of normalcognition. Furthermore they can providevaluable findings that constrain the develop-ment of new theories. Over the years themedical literature has grown immensely. Newcognitive deficits have been identified andimportant advances have been made in theunderstanding of the relations between com-ponents of complex cognitive skills and theloci of brain lesions. The neuropsychologist'sstrategy is to harness and incorporate thefindings of research investigations to a clinicalproblem in the form of more specific andmore sensitive tests of cognitive function.These improved quantitative techniques forclinical assessment can bring to light newphenomena that in turn promote funrther the-oretical advances.

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