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

NEUROLOGICAL INVESTIGATIONS

Neuropsychological assessment
Lisa Cipolotti, Elizabeth K Warrington

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

modem cognitive neuropsychology theorists. attempt to detail the way that the different
Despite this, the idea that cognitive skills subprocesses are brought together to perform
such as language could consist of multicom- a specific task. Empirical support for the
ponents and be localised in different, highly modularity approach can be obtained at vari-
specialised areas of the brain came under ous levels including the neurophysiological,
attack from the "global theorists".2' Of par- neuroanatomical, and neuropsychological.'031
ticular relevance here is the development of For example, numeracy has been fractionated
"mass action" theories. These theories pro- into several independent components: cogni-
posed that there was no differentiation in the tive mechanisms for number comprehension,
cortex for specific cognitive functions; rather, number production, arithmetical fact
that it was equipotential with respect to cog- retrieval, and arithmetical procedures.'2
nitive abilities.24 According to such a view, The idea that complex cognitive skills are
any form of neurological damage would carried out by distinct subprocesses com-
deplete by a greater or lesser extent the avail- bined with the idea that there are highly spe-
able amount of some general cognitive cialised areas in the brain, has led many
resource and not specific cognitive functions. cognitive neuropsychologists to assume that a
The amount of damage to the general cogni- cerebral lesion can damage only some sub-
tive resource, also termed intellect or abstract processes within complex cognitive skills.
attitude, would depend on the extent of the Indeed, cognitive neuropsychologists have
brain damage and not on the site of the succeeded in showing many dissociations
damage. between the subcomponents of cognitive
The notion that different brain regions are skills that allow valid conclusions about the
specialised for different cognitive functions nature and functions of the impaired process-
regained popularity in the 1950s and the ing components to be drawn.
modem revolution in imaging techniques has
made it possible to visualise these structures
in the living human brain. The idea that there Methods of assessment of cognitive
are cognitive processing systems that involve functions
only specialised brain regions is now accepted One of the fundamental principles underlying
". . . as one of the comerstones of modem neuropsychological assessment is to establish
brain science .". .".25 For about 95% of right whether the subject is still functioning at their
handers and 70% of left handers major lan- premorbid optimal level or whether there has
guage, literacy (reading, writing, and calcula- been a deterioration. Therefore, the methods
tion), verbal short term memory, verbal long used for assessing cognitive functioning in
term memory, semantic memory, and praxis neuropsychology need to be able to provide:
are represented in the left hemisphere. The (a) an indirect measure of the premorbid
right hemisphere is involved in non-verbal skills of a person and (b) a measure of the
processing such as the analysis of perceptual present cognitive state of that person. Once
and spatial stimuli, spatial short term mem- the two types of measures are obtained they
ory, visual long term memory, spatially can be compared. This should indicate:
directed attention, face recognition, topo- firstly, whether the functioning has changed
graphical knowledge, and in some prosodic from the premorbid state; and secondly
components of language. For those few peo- whether this reflects organic or functional
ple who do not have normal lateralisation this impairment. If the results indicate organic
pattern seems to be reversed, although a very impairment then an attempt will be made to
small proportion of subjects may have bilat- establish the extent of the change. It is not
eral organisation of some cognitive skills. The only useful to know that a change in cognitive
anterior parts of both hemispheres have been functioning has occurred; it is also useful to
accepted as being implicated in problem solv- know whether the change can be charac-
ing processes that are required in a wide terised as global or focal. If the cognitive
range of situations including practical rou- impairment is focal, neuropsychological mea-
tines and social interactions as well as sures can be used to specify more precisely
abstract reasoning tasks.7 The most posterior the cognitive impairments: whether the
parts of both hemispheres are involved in impairment is indicative of lateralised dys-
early visual processing. Subcortical, as well as function or confined to the anterior or poste-
cortical, brain regions, are involved in atten- rior regions of the brain. In exceptional cases,
tion and alertness. Subcortical brain regions it is possible to document highly selective
are also involved in episodic memory, in cognitive impairments with a known and rela-
some aspects of long term memory, and in tively precise anatomical localisation.
the motor control of language.12 A comprehensive neuropsychological
A modularity approach to the analysis of examination would include the assessment
cognitive skills implies that each complex of: (a) premorbid ability; (b) general intellec-
cognitive process can be thought of as con- tual level; (c) memory; (d) language; (e)
sisting of a series of functionally independent calculation; (f) problem solving; (g) alertness
specialised subprocesses.72$29 The interaction and attention; (h) visual and space percep-
of these subprocesses results in the complex tion. Ideally a cognitive profile would be
cognitive skills. The way in which the cogni- constructed from performance on tests of
tive processes are organised is often charac- proved validity and comparable difficulty.
terised, similarly to the "diagram maker" A long term aim for the neuropsychologist
approach, in terms of flow diagrams that is to achieve a level of measurement for all
Neuropsychological assessment 657

Example of a circle Speed in the verbal definition of words, long after


diagram of cognitive skills. Verbl IQ Pe rforma nce IQ
Sectors of the circle have
..,..
other cognitive skills were impaired. The
been apportioned to each of
.I application of the same principle led to the
\Verba
the cognitive skills memory,/ development of various Wechsler deteriora-
examined. The concentric tion indices.'2
circles represent the level of
functioning in terms of More recently, a measure of premorbid
percentile score. The levels optimal level of functioning has been based
of the subject's test on the overlearned skill of reading. Nelson
performance are indicated and McKenna"7 first established that word
by the degree of eccentricity
within a sector. In this reading skill, as measured by the Schonell
patient the most prominent Naming graded word reading test38 was highly corre-
feature was a global Pe
lated with general intelligence in a normal
memory impairment more Reading _
pronounced for verbal population. Nelson and O'Connel139 then
than visual material. Spelling Spatial skills established that the reading of irregular
(Diagram courtesy of Arithmetic words, such as "heir" or "chord", which can-
Newman et al. 33)
not be pronounced correctly by applying the
Percentiles usual rules that map spelling on to phonol-
<1 a1<5 a5<25 E >25<50- ¢50O ogy, were better indicators of premorbid
intelligence (IQ) in demented subjects than
estimates based on reading regular words.
Nelson subsequently developed the National
cognitive skills that permit comparison across adult reading test (NART), which consists of
tasks and is sensitive to change. The method 50 irregular words. Indeed, the NART has
described by Newman et al in their study that become one of the most commonly used
monitored subjects at risk for Alzheimer's dis- measures of premorbid intelligence.4' An
ease exploited this methodology (figure)." American version of this test is also avail-
In the next section we do not attempt to able.42 One of the major limitations of the
describe all the tests and techniques available NART test is that it cannot be used with
to the neuropsychologists for investigation of those who have poor literacy skills or in
all these different areas of cognition. Rather patients with obvious impairments of speech
we focus on three main areas of cognitive production or problems associated with
function: intelligence, memory, and language dyslexia. In addition, there have been claims
functions. These serve to illustrate the range that patients with dementing disorders may
of techniques and procedures available for the not present with preserved irregular word
investigation of cognitive impairments." reading.43 Consequently these patients pre-
sent with difficulties in reading the NART
ASSESSMENT OF PREMORBID ABILITY words, and this would result in erroneous low
Various procedures are adopted for obtaining estimates of their premorbid IQ. In the cases
an indirect measure of a subject's premorbid of early dementia when language skills are rel-
skills, which can then be compared with his atively unimpaired, however, it has been
current level of performance. These proce- shown that the NART remains stable over
dures can be divided into two main types: time and can be used as a predictor of the
methods that use demographic data such as premorbid optimal level (Paque and
age, sex, race, education, and occupation; Warrington, unpublished data).
and methods that use tests considered to be
relatively resistant to neurological and psychi- ASSESSMENT OF GENERAL INTELLECTUAL LEVEL
atric disorders. The first type of method is Historically, intelligence has been defined in
based on the known relation between a num- many different ways. For example,
ber of demographic variables and measured Spearman,44 although he himself avoided the
IQ*.34 Not only may educational and occupa- term intelligence, proposed the existence of a
tional records be used as a rough estimate of central intellectual ability, which he referred
a subject's optimal or premorbid level of to as "g". Although he never actually defined
functioning; they may also, through the use of what g was he thought that it involved "the
various types of regression equations, provide eduction of relations and correlates".4" In his
a more precise and objective estimate.'5 One formulation g referred to the determinant of
of the principle limitations of this type of shared variance among various tests of intel-
technique, however, is that educational and lectual ability. An alternative view, associated
occupational histories may not always be with Thurstone45 and Guilford,46 involved
readily available and they may be incomplete, the application of the term intelligence to a
uninformative, or anomalous. large set of diverse mental abilities (or fac-
The second method involves the measure- tors). These included not only reasoning
ment of a cognitive skill that is known to be and problem solving on new data but also
highly correlated with intellectual factors and
resistant to brain damage. This method is
obviously not reliant on preexisting data. *The term IQ was first introduced by Stern40 to
Some of the first methods of this type describe a method of comparing one child's score on
involved the use of vocabulary.'6 These meth- the Binet intelligence scale with the performance of
ods were based on the finding that patients average children of the same age. It is nowadays used
to indicate intellectual level by comparing a subject's
with brain disease retained old, well estab- performance with the average scores attained by mem-
lished verbal skills, such as those implicated bers of the same age group.
658 Cipolotti, Wanington

specialised knowledge derived from prior generally made between short and long term
schooling or experience. In line with this, memory. Short term memory is considered to
Cattell47 distinguished between fluid intelli- be responsible for the immediate retention of
gence, the ability to deal with novelty and to a limited amount of information; this infor-
adapt one's thinking to a new cognitive prob- mation will decay in a matter of seconds if it
lem, and crystallised intelligence, which is not refreshed. Long term memory retains
reflects a knowledge base and skills that have larger amounts of information for longer peri-
been previously acquired through learning ods-depending on the salience-which may
and experience. be for minutes, days, and years. Short and
The available measures of intelligence long term memory functions can be further
reflect these different formulations of the abil- divided into verbal and visual memory
ities underlying intelligence. For example, according to whether they retain verbal or
Raven's test, including the coloured progres- non-verbal information.56 Long term memory
sive matrices,"1 the standard progressive is also subdivided into implicit (or proce-
matrices,'0 and the advanced progressive dural) and explicit (or declarative) memory.57
matrices (sets I and II)48, are widely used for Implicit memory retains information that
the clinical assessment of general intelligence. affects behaviour but it is not available for
The various versions of this test are believed conscious recollection (for example, motor
to weigh heavily on g and measure processes skills, conditioned reflexes, priming). A fur-
that are central to the definition of fluid intel- ther example is the three letter word stem
ligence (more recently also termed analytic completion task, which can be performed by
intelligence49). Indeed, they require abstract guessing rather than by conscious recall.58 In
reasoning, induction of relations, and educ- this task patients are presented with a list of
tion49 The test was developed as a "culture words and their retention is tested either by
fair" measure of general intellectual ability standard recall and recognition techniques or
and because of its non-verbal format, its ease by presenting the first three letters of the
of use, and its speed (especially the coloured target item in a word completion task (for
progressive matrices), it has gained wide use example, "cha"-chair). Explicit memory
in both clinical and research settings. This retains information that can be consciously
may be overoptimistic as educational level has accessed. It is subdivided into episodic and
subsequently been shown to have a major semantic memory.59 Episodic memory con-
effect on the normal subject's performance.50 tains information about temporally dated
The Wechsler adult intelligence scale episodes or events and temporospatial rela-
(WAIS)5' 52 iS considered to be one of the core tions among them (for instance, this can be
measures for evaluating general intellectual for both autobiographical memories and
ability. It involves six verbal and five non- memories of an artificial event such as a word
verbal subtests that sample various skills. list or short stories). Semantic memory con-
These subtests are thought to measure vari- tains our organised knowledge of concepts
ous mental abilities as would follow from and facts as well as words and their meanings
Thurstone's45 and Guilford's46 views, includ- (for example, encyclopaedic memories). Most
ing both the explicit knowledge base derived clinical assessments focus on three main types
from educational and previous experience of memory functions: short term memory,
and the ability to deal with and solve new episodic memory, and semantic memory.
cognitive problems. Verbal and performance
IQs are determined from the use of the Assessment of short term memory
Wechsler scales. Much research has focused The assessment of verbal short term memory
on discrepancies between verbal and perfor- usually requires the repetition of a progres-
mance IQ as a means of differentiating sively lengthening string of digits (digit span),
between left and right hemisphere impair- letters, and words. The normal range of digits
ment53 although this has not resulted in a is five to nine. Spatial short term memory can
general consensus.'2 Indeed, Warrington be assessed with the Corsi block tapping
et al54 suggested that such scales have little test.60 This requires the subject to tap a pro-
value as regards the localisation of a lesion or, gressively lengthening sequence of blocks.
for that matter, the identification of specific
cognitive deficits. Nevertheless, the Wechsler Assessment of episodic memory
adult intelligence scale-revised (WAIS-R), the Many tests and batteries are available for the
successor of the WAIS is the most often used assessment of episodic memory.61 These use
psychological test of intellectual functioning either a recall or recognition paradigm and
and is a cornerstone for most neuropsycho- typically assess the anterograde (the ability to
logical test batteries. It is also widely used acquire new information) rather than the ret-
with geriatric patients, and recently, norma- rograde component (the ability to recall pre-
tive data for people who are 75 or older have viously learnt material). One of the oldest
become available.55 Numerous studies are batteries used is the Wechsler memory scale
based on the WAIS-R. (and the Wechsler memory scale-revised),
which requires the recall of both complex ver-
ASSESSMENT OF MEMORY bal material (for example, short stories) and
Memory is not a unitary function but rather a visual material (for example, reproduction of
collection of distinct and independent com- geometrical designs). Some of its subtests are
ponents, each of which is associated with dif- not dependent on memory itself but rather on
ferent brain structures. A broad distinction is attentional processes (for example, mental
Neuropsychological assessment 659

control and orientation). Unfortunately all after the seminal paper of Tulving.59 Semantic
the subtests contribute to the final memory memory can, however, be assessed through
quotient. A more recently developed test for tests devised for other domains (mainly tests
the assessment of long term verbal memory is also used for the assessment of language dis-
the adult memory and information processing orders). To evaluate the difficulties in word
battery,62 which has many similarities to the definition some verbal subtests of the
Wechsler memory scale. The Rivermead Wechsler scales, such as vocabulary and
behavioural memory tests consist of a series information, can be used. Naming tests can
of tests held to have ecological validity.6' A be used as indirect evidence of semantic
task that is also very often used for assessing memory impairment (see language section
verbal anterograde recall is word list learning later). The pyramids and palm tree test7' was
(for example, the auditory-verbal learning developed specifically to evaluate impair-
test64). For the assessment of non-verbal ments in the understanding of concepts.
anterograde recall, the two most commonly There is a verbal and a pictorial version of
used tests are the Rey-Osterreith complex this task devised for assessing conceptual
figure test65 and the Benton revised visual relations. Limited normative data are avail-
retention test.66 Both require the recall of able. A further test, the British picture vocab-
geometric figures. ulary test,72 which uses a word picture
Warrington67 developed a test that used a matching technique, was first developed for
recognition rather than a recall paradigm (the the assessment of language developments
recognition memory test). The recognition between the ages 2 and 18. More recently it
paradigm was chosen because it is possible to has been standardised in a normal healthy
have comparable tests of verbal and visual elderly population (Clegg and Warrington,
memory. This test incorporates the verbal unpublished data).
and non-verbal dichotomy by having separate
subtests with word and face stimuli. Age cor- ASSESSMENT OF LANGUAGE
rected percentile scores of a large standardisa- Language is not a unitary fimction. The most
tion sample are available. Validation of this useful dichotomy is to consider spoken and
test has shown that patients with right hemi- written language separately.
spheric lesions are impaired on the visual ver- Spoken language can be characterised as a
sion and patients with left hemispheric lesions collection of independent components, each
are impaired on the verbal version. It has also of which is associated with different brain
been shown that this test can detect minor structures. The three main central linguistic
degrees of memory deficit." Clegg and components are phonology, syntax, and
Warrington68 have also recently standardised semantics.7' Phonological processing analyses
and validated four "easy" memory tests (three the constituent sounds of words. Syntactic
recognition memory tests and a word paired processing analyses the grammatical aspects
associate learning test) for older adults (64 of language-for example, the ordering of the
and older) that are recommended for patients individual words in the sentence. Semantic
in whom memory impairment is suspected processing analyses the referential meaning of
but whose mental state (for example, poor words. In addition to these three components
attention, anxiety, or agitation) precludes there are more specialised peripheral systems
longer or more demanding tests. subserving articulation and prosody.
Most tests of retrograde verbal and visual Furthermore, at least for phonology and syn-
recall have been devised for research rather tax, receptive and expressive deficits can
than clinical purposes. They normally test occur as selective impairments.7'76
recall and recognition of famous names and Disruptions in phonological or semantic pro-
famous faces. Perhaps because they so cessing are found at the level of single words
quickly become dated their standardisation whereas disruptions in syntactic processing
and validation are problematic. A relatively are found at the level of sentences.
new test assessing autobiographical memory
is an exception to this rule (autobiographical Assessment of spoken language
memory interview69). This test requires the There are several traditional clinical tax-
recall of personal remote facts and incidents onomies of the acquired aphasias principally
from three epochs: childhood, early adult life, inherited from the earliest scientific papers on
and recent experience. language disorder.'-3 19 These taxonomies,
based on mixed functional, anatomical, and
Assessment of semantic memory pathological terms, have inspired the develop-
Patients with a semantic memory disorder ment of classic aphasia batteries. The most
present a general loss of knowledge, including widely used are the Boston diagnostic aphasia
object and word meaning. This deficit can examination,76 the western aphasia battery,77
manifest itself as an inability to comprehend the Porch index of communicative ability,78
words and identify pictures and objects. The and the Aachen aphasia test.79 The traditional
classic syndromes of transcortical sensory taxonomies that form their basis have been
aphasia and visual associative agnosia have questioned in so far as they failed to capture
been identified with the impairment of the multidimensional pattern of language
semantic memory.70 There are no standard- breakdown, they are not useful for guiding
ised batteries for the assessment of semantic therapy or for the detailed analysis and
memory because, unlike episodic memory, it understanding of language disorders.80 In this
has only been studied in the past 20 years, section we provide a brief account of the core
660 Cipoloti, Wamngton

tests that could provide a framework for the in cortical degenerative conditions. They can
more detailed assessment of a patient's lan- be specific for particular categories such as
guage impairment. We discuss only two areas letters, colours, body parts, proper names,
of language dysfunction: word and sentence and fruits and vegetables.3' To evaluate word
comprehension and word and sentence retrieval difficulties naming from verbal
retrieval. description (for example, "what is the name
Word and sentence comprehension-Word of the large grey animal with a trunk") and
comprehension deficits can occur as a result picture naming tests can be used. The graded
of an impairment in auditory perception or as naming test89 was developed to identify very
a result of an impairment in word meaning. mild degrees of anomia. It comprises items
An auditory word perception deficit can be of low frequency and it has been standardised
identified in patients that have a deficit in in a normal population and validated in
word repetition that cannot be attributed to a patients with unilateral lesions. The Boston
more general articulatory deficit.8' It can be naming test90 comprises line drawings of
assessed through phonological discrimination objects and has been widely used in aphasia
tasks that are usually included in most of the studies. Only a limited standardisation is
traditional aphasia batteries. Impairment of available.
word meaning is one component of the Spontaneous language is often elicited by
semantic memory disorders (see earlier) and, complex picture description. The cookie jar
as the word retrieval deficit (see later), can be theft picture from the Boston diagnostic
category specific. For example, selective aphasia examination is widely used for this
deficits for abstract and concrete concepts purpose. De Renzi and Ferrari9' devised the
and within the concrete domain animate or reporter test requiring the patient to act as a
inanimate reference and even specific word reporter of the performance carried out by
class effects have all been reported.82 One of the examiner who acts in accordance with the
the most direct tests of word meaning are commands of the token test described earlier.
synonyms tests (for example, "timid" means This test is particularly useful for the identifi-
"afraid" or "quiet"). Coughlan and cation of impairments in grammatical sen-
Warrington83 have offered a modest standard- tence construction, although there are only
isation of one such test. Word meaning com- limited normative data at present.
prehension can also be tested by using
word-picture matching tests such as the pyra- WRItEN LANGUAGE
mids and palm tree test7l and the British In the past 30 years cognitive neuropsycholo-
picture vocabulary test,72 described in the gists have investigated reading and writing
semantic memory section. In addition, the disorders in detail and depth. This has
recent psycholinguistic assessment of lan- resulted in the identification of new syn-
guage processing in aphasia84 is a useful dromes that take the description of reading
research tool for assessing comprehension in and writing difficulties well beyond the classic
the domains of verbal and visual knowledge. syndrome described by Dejerine3: dyslexia
One of the earliest and most commonly with dysgraphia and dyslexia without dys-
used test of sentence comprehension is the graphia. Each of these different dyslexic and
token test devised by De Renzi and Vignolo.85 dysgraphic syndromes corresponds to an
This test uses tokens of different shapes, identifiable impairment in a subcomponent or
sizes, and colours and the patient is given an subcomponents of the reading and writing
oral instruction in progressively more com- process. Shallice and Warrington92 have pro-
plex non-redundant sentences (for example, posed a distinction between peripheral and
"put the red circle between the yellow square central dyslexic syndromes and this
and the green square"). There have been var- dichotomy applies equally well to the dys-
ious modifications of the test including a graphia syndromes. Peripheral dyslexias and
shortened version by De Renzi and Faglioni dysgraphias result from damage to processes
and a very abbreviated version by Coughlan responsible for the categorisation of a string
and Warrington.8' Educationally standardised of letters or phonemes as orthographic or
normative data are available. Parisi and phonological entities. Central dyslexias and
Pizzamiglio86 devised a test specifically for dysgraphias are due to impairment in the
testing syntactic comprehension (for an comprehension and production of a target
English version see Lesser87). Another test for stimulus. The study of central dyslexias and
grammatical comprehension is the test for dysgraphias has provided evidence that
reception of grammar.88 This test was devel- there are at least two parallel forms of
oped for the assessment of language develop- processing for reading and writing: one
ments and has been used also in the context phonologically based and one semantic
of acquired aphasia investigations. It should based. Phonological processing utilises a set
be acknowledged that some normative data of rules for translating print to sound or
are available for the sentence comprehension sound to print. It is used for reading or writ-
test reviewed here and are undoubtedly very ing unfamiliar words or non-words. Semantic
useful for in depth assessment of a patient's processing accesses meaningful representa-
aphasic deficit. tions of the words that are in the subject's
Word and sentence retrieval-Word retrieval vocabulary. These two types of processing
difficulties are exemplified by the syndrome can break down independently to produce
of amnestic or nominal dysphasia and are different types of reading and writing
often present in other aphasic syndromes and impairments.
Neuropsychological assessment 661

Assessment of written language the status of the peripheral and central pro-
Following the seminal work of Marshall and cessing involved in word reading and spelling.
Newcombe93 a psycholinguistic method of This, rather than the description of the
assessment of written language disorders has presence or absence of a reading or spelling
gained wide popularity. This method involves disorder, has a clear clinical and theoretical
the presentation of lists of words that sample significance.
contrasting psycholinguistic properties. It is
thought that the data on the effect of the psy-
cholinguistic and visual (length, script, and Purposes of a neuropsychological
displays) variables coupled with an errors assessment
analysis allow conclusions to be drawn about There are at least three main reasons for con-
the likely origin of dysfunction within the ducting a neuropsychological assessment:
reading system. In this section we provide a diagnosis, treatment and management, and
description of some of the standardised and research.
validated formal tests for the assessment of
reading and spelling disorders. DIAGNOSIS
Reading-Any assessment of reading skills A neuropsychological assessment allows the
should include an evaluation of a patient's description and evaluation of the major cog-
ability to read both single words and text. In nitive deficits incurred in neurological patients
some peripheral dyslexias the ability to read with possible brain disease. Furthermore, it
text can be impaired whereas the ability to can indicate possible neuroanatomical corre-
read single words can be spared. (for exam- lates of the cognitive impairments. A neu-
ple, attentional dyslexia94) The Neale test95 ropsychological assessment can, at the very
for assessment of prose reading in children is least, provide pointers as to whether there is
useful in this context. In addition, most of the unilateral, bilateral, or subcortical damage.
standard aphasia batteries include a subtest This information can be useful in diagnosis.
for text reading (for example, Boston diag- Neuropsychological assessment has a key role
nostic aphasia examination). For the formal in differentiating between organic and func-
assessment of single words, the two most tional disorders.'4 99-101 There are other neuro-
widely used tests are the NART and the logical conditions-for example, cortical
Schonell graded word reading test. Both tests atrophy, frontal and temporal lobe tumours,
are graded in difficulty and are measures of and undetected temporal lobe seizures-that
reading skills; an estimate of premorbid opti- may manifest themselves with symptoms that
mal level of functioning can also be obtained can be misinterpreted as functional.'02 For
(see earlier). When assessing reading skills it example, patients with visual disorientation
is important to evaluate the patient's ability to disorders due to bilateral occipital disease are
read aloud non-words. This ability can be often misdiagnosed on the grounds that their
selectively impaired despite good word read- visual handicap seems to be disproportionate
ing as in the case of phonological dyslexia.96 in the context of normal or near normal
No formal standardised tests for non-word acuity. 103
reading are available; however, several lists On the other hand, patients with symp-
have been devised for research purposes. toms of pseudodementia, such as hysteria,
Oral and written spelling-Written and oral malingering, Ganser syndrome, bipolar disor-
spelling are known to dissociate and therefore der, and other ill defined psychiatric disorders
are assessed independently. For the assess- often present with an abrupt intellectual and
ment of written spelling the Schonell graded memory failure that mimics true cognitive
spelling test can be used. For the assessment deficits. A neuropsychological assessment can
of oral and written spelling, Baxter and distinguish between organic and functional
Warrington97 have recently standardised and disorders. It does this by highlighting discrep-
validated a test that is sensitive to minor ancies between subjective complaints and
degrees of deficit in the general neurological objective performance, usually detecting a
population. This is a graded difficulty test; number of inconsistencies in the patients'
thus the raw scores can be converted into per- performance and a too obvious mismatch
centile scores. For patients whose poor eye between objective performance and daily life
sight precludes reading, spelling can provide a activities. Also, the body of neuropsychologi-
useful measure of premorbid abilities. The cal knowledge on the organisation and frac-
assessment of non-word spelling is also tionation of cognitive skills is nowadays
important because patients with phonological highly developed. Crucially, the way in which
dysgraphia might present with some pre- the cognitive functions can fractionate often
served word spelling despite impaired non- diverges from the common sense opinion of
word spelling.98 Several non-word spelling how a cognitive function can break down.
lists have been developed for research pur- Thus the patient's pattern of performance
poses. can be interpreted as neuropsychologically
The use of the standard word reading and convncmig or unconvincing. To consider one
spelling tests described combined with assess- example, a neuropsychological assessment is
ment of the patient's ability to read and write useful in differentiating organic and func-
non-words and an analysis of the errors made tional memory loss. Studies of patients with
by the patient identifies more than the pres- dense organic amnesia have shown that they
ence of a reading or spelling disorder. It can can still learn new associative information
also provide some preliminary indication of provided that they are tested using implicit
662 Cipolotti, Warnngton

learning tasks. 104 For example, they show allow the monitoring of certain conditions.
savings with repeated presentations of frag- For example, successive neuropsychological
mented or degraded stimuli (pictures or examinations provide reliable indications of
words) in increasing degrees of completeness. whether a pattern of cognitive deficit associ-
Even quite severely demented patients would ated with head injury or stroke is changing
show some learning with these tasks. Clearly and if so, how rapidly and in what way. This
a patient showing additional impairments on information is useful in planning the future
these implicit learning tasks makes no neuro- medical and social care of the patient.
psychological sense. It makes good common Similarly, repeated-neuropsychological testing
sense, however, to also be impaired on these of patients with degenerative disorders can
tasks (I have poor memory, I can't remember provide information about their different
things). Indeed, a poor performance on these rates of cognitive decline and thus help them
tests may be considered indicative of func- and their family plan for their care. The
tional memory loss. On the contrary, a rela- results of a neuropsychological assessment
tively preserved performance on these tasks can also be used in the evaluation of medical
conforms to an organic pattern. and surgical treatments such as those associ-
Another common differential diagnosis ated with subcortical pathology that is associ-
where neuropsychological assessment has a ated with cognitive slowing (for example,
key role is between early dementia, anxiety or Parkinson's disease and hydrocephalus). For
depressive disorders, or the normal aging instance, obtaining repeated measures of a
process. The diagnosis of probable dementia hydrocephalic patient's performance in a
is usually made by establishing whether there series of psychomotor tests can provide a reli-
is an acquired deficit of cognition without able indication of whether the underlying
hystopathological evidence obtained from a neurological condition is improving or deteri-
biopsy or necropsy. Often in the early stages orating. Psychomotor tests are simple verbal
of a dementing illness, the clinical diagnosis and non-verbal tests that involve verbal and
cannot be supported by neuroimaging such as visuomotor responses and the measurement
CT, MRI, or functional imaging. Patients of the patients' speed of responding. 108
with depression or anxiety may complain of Practice effects are minimal in these tests,
intellectual or, more often, memory failure which are at the same time sensitive to subtle
similar to the so called "worried well" patients. changes in cognitive efficiency. Therefore
Usually depressive or anxiety pseudodementia they can be used at regular and short intervals
should be suspected when the patient com- for monitoring the patient's neurological
plains of the memory problem more than the state. Neuropsychological assessment is also
carer.'05 In these cases recognition memory particularly important in monitoring the vari-
tests should be used to determine whether the ous treatments for epilepsy.'09 110
failure is due to an organic condition or to anx- The baselines of cognitive functioning pro-
iety or depression. It has been shown that vided by the neuropsychological examination
recall tests of memory are vulnerable to the can be important for planning and monitoring
effect of anxiety and depression, whereas rehabilitation programmes. In particular,
recognition memory tests are not.'06 when planning such programmes, neuro-
The aging process itself is associated with psychological evaluation can provide answers
cognitive and memory changes. Hence, it is to key questions such as ". . . what are realis-
often necessary to differentiate memory fail- tic treatment goals and ... what is the
ure due to cognitive deterioration rather than patient's capacity to benefit from available
benign senescent forgetfulness. In these cases treatment . . .".12 Moreover, repeated neu-
performance in recall memory tests requiring ropsychological testing can be used to monitor
the subjects to engage in elaborative encod- the effects of the rehabilitation programme.
ing, as opposed to allowing them to devise Furthermore, the baselines of cognitive func-
their own encoding strategy, may discrimi- tioning provided by neuropsychological exam-
nate those with brain damage from normal ination can be used to explain to patients and
elderly subjects. For example, in word list their families their relative cognitive problems
learning the strategy of performing associa- so that they can both prepare and understand
tions between successive words improves the the type of difficulties the patient may face
overall level of recall in normal subjects. when he leaves the hospital.
Neuropsychological assessment can also Neuropsychological assessment has a
have a central role in diagnosing presympto- central role in the medicolegal context.
matic cognitive impairments in familial neu- Neuropsychological data concerning the
rodegenerative conditions. From the nature type and severity of a cognitive deficit, its
of the inheritance and the relatively constant prognostic value, and the implications for
ages of disease onset within a family, asymp- future care are central issues in the litigation
tomatic at risk subjects below the mean age of over compensation awards."' In this context
onset can be examined. Such studies have neuropsychological investigation is crucial in
shown cognitive abnormalities in apparently assessing the possibility of simulated disability
asymptomatic subjects with Huntington's and that can sometimes occur before financial
Alzheimer's disease.33 107 settlement."12
TREATMENT AND MANAGEMENT RESEARCH
The baselines of cognitive functioning pro- There are two main neuropsychological
vided by the neuropsychological examination research methodologies: the single case study
Neuropsychological assessment 663

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