Neurological Testing Dr Anna Adlam

Clinical Lecturer/Clinical Psychologist University of East Anglia 5 November 2009

Overview

• Historical context • Purpose of neurological/ neuropsychological assessment • Distributed cognitive functions - disorders & tests • Localised cognitive functions - disorders & tests • Cognitive & neuropsychiatric history taking • Practise EMQs & MCQs

Historical context

Historical context

(see Benson, 1993;1996) • Behavioural neurology – Neuropsychiatry (see Martin, 2002; Kendler, 2005 for discussion) – Neuropsychology (brain-behaviour relationships, Benton, 2002) • Three contributing avenues – Clinical neuroscience • Broca, Wernicke, & Geschwind studied language disorders • First and second world war survivors with brain damage (e.g., Goldstein, 1939; Luria, 1973), Middle East (Larry Squire, today) – Educational psychology • Spearman (1904) and Binet & Simon (1908) developed tests of “intelligence”.

– Cognitive psychology • Animal studies, ‘normal’ participants, survivors of brain injury, imaging studies (MRI, PET, DTI)

Purpose of neurological/ neuropsychological assessment

Neuropsychological assessment

• Diagnosis – Psychiatric vs. neurological symptoms, localise lesions - BUT, now more sensitive neuroimaging techniques • Patient care – Management, planning • Treatment – Identifying treatment needs – Evaluation of treatment efficiency • Research • Forensic (e.g., medicolegal work)

Questions/areas that can be assessed

• Is there evidence of cognitive/intellectual impairment?

• Severity of cognitive impairments? • What is the likely diagnosis? Brain areas involved?

• Predicted level of premorbid function?

• Awareness of cognitive difficulty?

• Strengths and weaknesses?

• Changes over time: improvement or deterioration?

• Comparison with others? (e.g., age) • Malingering?

• How might this person be best supported at school/home/work/on the ward?

• How might this person respond to ‘talking therapy’? (e.g., distractibility, attention/concentration, impulsivity, perseveration, problem-solving, confabulation, planning, response to novel situations, empathy, ability to generate alternatives etc.).

Distributed vs. localised functions

Distributed vs. localised functions

• Distributed – Bilateral involvement • attention/concentration (reticular activating system - brain stem/thalamus - prefrontal, & parietal areas) • memory (limbic & frontal) • executive function & social cognition (frontal lobe) • Localised – Lateralised lesions • Left (dominant) hemisphere - e.g., spoken language, reading, writing (aphasia), calculation (acalculia), higher motor control (apraxia) • Right (non-dominant) hemisphere - e.g., spatial attention (neglect), visuo-perceptual skills (agnosia), prosodic components of language (tone, melody, intonation)

Brain-behaviour relationship

Spatial attention Working memory; Planning Emotion perception; Set-shifting; Decision making Semantic & Episodic memory; Higher order visuospatial processing

Distributed cognitive functions - Attention & arousal

Distributed functions (1) Attention & Arousal

• Attention & arousal – Arousal (wakefulness, responsivity) – Sustained attention (maintain attention) – Divided attention (respond to more than one task) – Selective attention (focus on one stimulus while suppressing awareness of competing stimuli) • e.g., driving a car while conversing with a passenger and having to overtake

Disorders of Attention & Arousal

• Impairments in attention can lead to distractibility (frontal or parietal damage) • Marked impairments can lead to disorientation in time/place • Deficits in global attention at processing is referred to as the acute confusional state (delirium) – Transient (duration rarely exceeds weeks) – If severe, consciousness is diminished due to depression of basic arousal processes (ascending reticular activating system) • Attention deficits common in Alzheimer’s disease, dementia with Lewy bodies, early stage Parkinson’s disease, traumatic brain injury, and stroke.

Neuroanatomy of Attention & Arousal

Cortical areas Prefrontal, posterior parietal, and limbic areas (top-down) Thalamus Brain-stem nuclei Intralaminar and reticular nuclei Dopaminergic, cholinergic, and serotinergic pathways Reticular formation, midline raphe, locus coeruleus, and tegmental nuclei (bottom-up)

Tests of Attention

• Orientation in time & place (also dependent on episodic memory) • Digit span esp. backwards (also dependent on working memory) • Recitation of months of the year or days of the week backwards or serial subtraction of 7s (also working memory component) • Alternation tasks, such as Trails B (also executive function) • The Stroop Test of response inhibition (also executive function) • Test of Everyday Attention (standardised assessment)

Example: Test of Everyday Attention

Distributed cognitive functions - Memory

Distributed functions (2) Memory

Memory Long-term Short-term/ working memory Explicit/ declarative Episodic Semantic Implicit/ non declarative Motor skills Classical conditioning Priming

Disorders of Memory (1)

• Duration – Short-term = a few seconds – immediate = a few minutes – long-term = > 20 minutes, days, months, years • Stages – Encoding – Storage – Retrieval (recall vs. recognition) • Short-term or working memory impairments – e.g., inability to retain verbal information over a few seconds, difficulty with dual-task performance, poor visuo-spatial short term memory • Long-term memory impairments – Anterograde vs. retrograde

Disorders of Memory (2)

• Amnesic syndrome – Preserved intellectual abilities (MQ < IQ) and semantic memory – Preserved short-term/working memory – Preserved procedural (implicit) memory – Korsakoff’s syndrome - executive function impairments, encoding < storage – Limbic damage - executive function usually intact – Frontal lobe damage - confabulation, recall < recognition – Anterograde amnesia - impaired acquisition of new episodic memory (verbal vs. nonverbal, recall vs. recognition) – Retrograde amnesia - impaired memory for past events (temporal gradient?)

Disorders of Memory (3)

• Transient amnesia – Transient global amnesia • 60-70 years, poor anterograde and patchy retrograde memory, disorientated, repetitive, no impaired consciousness, typically lasts after 4 - 6 hours - amnesic gap for duration of the attack cause unknown (migraine, distress) – Transient epileptic amnesia (TEA) • Brief episodes of confusion and disorientation, repetitive questioning, typically shorter duration than TGA (a few minutes), attacks often occur after waking, recurrent episodes, poor retrograde memory – Psychogenic fugue states • Rare, precipitated by significant life events, profound retrograde amnesia encompassing whole life including loss of personal identity but sometimes without significant anterograde memory impairment.

Neuroanatomy of Memory

Control Case 1

Tests of Memory

• Short-term/ working memory – Digit span, block span, letter-number sequencing (WAIS) • Anterograde episodic memory – Story recall and recognition (WMS), word list learning (RAVLT) – Recognition of words/faces (Warrington’s Recognition Memory Test) – Geometric figures (Rey-Osterrieth Figure, WMS-III) – Test batteries - WMS-III, RBMT, Doors & People • Retrograde episodic memory – Autobiographical Memory Interview (personal facts, events) • Semantic memory – Types of dementia, herpes simplex virus encephalitis, vascular lesions of temporal lobe – Tests of general knowledge (WAIS subtests) – Category fluency, object naming, Pyramids & Palm Trees test

Copy

Example: Rey Figure

Delayed recall (40 mins) 13 year old control Jon Kate

Distributed cognitive functions - frontal lobe function

Distributed functions (3) Frontal lobe function

• Executive function – DLPfC – Planning – Goal maintenance – Self-monitoring (e.g., monitoring errors) – Failure to inhibit response – Perseveration – Leads to poor problem solving • Social cognition – VMPfC – ‘Personality and behaviour’ – Phineas Gage – Emotion-based decision making (Damasio’s somatic marker hypothesis) – Theory of mind – Aggression and regulation of mood – Motivation

Disorders of Frontal Lobe Function

• Degenerative – Frontotemporal dementia (Pick’s disease) – Alzheimer’s diease • Vascular – Bilateral anterior cerebral artery infarction – Subarachnoid haemorrage (anterior communicating artery aneurysms) • Structural – Closed head injury – Tumours – Surgery • Deafferentation from basal ganglia – Huntingdon’s disease – Parkinson’s disease – Progressive supranuclear palsy

Tests of Frontal Lobe Function

• Verbal fluency (category and letter) • Problem-solving (e.g., Tower of Hanoi) • Trail Making Test • Set-shifting tests (WCST) • Motor sequencing (e.g., alternating hand movements, fist-palm-palm-fist, Luria three-step test, fist-edge palm) • Decision-making tests (Iowa Gambling) • Test batteries – Behavioural Assessment of the Dysexecutive Syndrome (BADS) – Hayling and Brixton Test – Delis-Kaplan Executive Function System (D-KEFS)

Example: WCST

Localised cognitive functions left hemisphere

Localised functions (1) Left hemisphere

• Disorders of language – Aphasia - loss or impairment of language function caused by brain damage • Dysarthria - impaired speech (not necessarily language impairments) • Phonology (speech sounds), semantics (meaning), syntax (structure), prosody (expression of tone, emotion) • Causes – Focal lesions, dementia, psychiatric disorders (mutism) – Dyslexia - reading impairments • Peripheral dyslexia - impaired visual decoding of written script (letter-by-letter reading, errors reading part of word) • Central dyslexia - impairment in deriving meaning (affects oral spelling), surface (loss whole word), deep (loss sound-based reading), phonological (non-word reading) – Dysgraphia - disorders of writing

Localised functions (2) Left hemisphere

• Disorders of calculation – Acalculia - inability to comprehend or write numbers – Anarithmetria - inability to perform number manipulations – Spatial dyscalculia - difficulty aligning columns of figures, or performing carrying tasks – Gerstmann’s syndrome • Angular gyrus syndrome (rare) • Agraphia (written oral spelling) • Acalculia (number reading, writing, calculations) • Right-left disorientation • Finger agnosia (naming, pointing/moving named finger, unable to recognise touch)

Localised functions (3) Left hemisphere

• Disorders of praxis – Apraxia - inability to carry out complex motor acts despite intact motor and sensory systems – Limb kinetic (basal ganglia, supplementary motor areas) finger movements for fine motor tasks, unable to copy meaningless hand movements – Ideomotor (left parietal) - unable to conduct motor acts to command, difficulty with selection, sequencing, spatial orientation, and movements – Ideational or conceptual (left temporal) - inability to conduct a complex sequence of co-ordinated movements, or mime use of objects – Orobuccal (left inferior) - difficulty performing skilled movements of face, lips, tongue, cheeks, larynx on command (e.g., pretend to blow out a match)

Localised cognitive functions right hemisphere

Localised functions (4) Right hemisphere

• Neglect – Impairments of spatially directed attention – Personal neglect - extreme cases behave as if one half of the body has ceased to exist – Motor and sensory neglect - fail to move a limb, failure in awareness of stimuli on affected side – Extrapersonal neglect - line bisection, cancellation tasks, draw or copy pictures • Dressing apraxia – Impairment in orientating body parts in relation to garments because of visuo-spatial deficits.

• Constructional disorders – Impaired copying of visually a presented model (either by drawing or building)

Localised functions (5) Right hemisphere

• Complex visuo-perceptual abilities – Recognising objects from ‘unusual views’, identify overlapping drawings, judge line orientation – Visual Object and Space Perception battery (VOSP) • Visual object agnosia – Failure to recognise objects – Apperceptive agnosia unable to recognise objects from vision but can recognise objects if held, poor copy of shapes – Associative agnosia - unable to recognise objects in all modalities (semantic loss) – Tests include object naming, object miming use, name to description, tactile naming • Prosopagnosia – Inability to recognise familiar faces but can recognise person by voice, posture, gait etc – Tests include naming photographs of familiar faces

Localised functions (6) Right hemisphere

• Disorders of colour perception – Achromatopia - inability to discriminate between colours (“everything is washed out, like black-and white TV”) – Colour agnosia - impaired reteival of colour information (e.g., “what colour is a banana?”) – Colour anomia - inability to name colours but presevred colour perception and knowledge • Balient’s syndrome – Inability to direct voluntary eye movements to visual targets – Inability to reach for, or point to, visual targets (unable to locate objects in relation to self) – Visual attentional deficits, where only one stimulus at a time is perceived (‘simultanagnosia’), unable to synthesise parts in to the whole – Functionally blind • Topographical disorientation – Unable to navigate familiar routes due to not knowing the relationship between objects and landmarks

Case examples

Frontotemporal dementia - PNFA

• Clip of person with progressive non-fluent aphasia – Left > right – Inferior frontal and insula damage

Frontotemporal dementia - SD

• Clip of person with semantic dementia – Left > right – Inferior anterior temporal lobe damage

Cognitive and Neuropsychiatric History Taking

Initial interview

• • • Need context in order to interpret test results Build rapport, assess motivation for testing, generate further hypotheses from data gathered Interview client and informant separately (with consent) – demographic data (handedness) – description of current difficulties (include mood checks/hours of sleep) – medical history (any physical impairments to consider?) – psychiatric history – educational and vocational background (good for estimating premorbid function) – birth history and early development (developmental disorder?) – family background (neurological disorders? LD?) – legal history/substance use – current situation • Selecting measures – Reliable, valid, age-appropriate normative data - refer for neuropsychological assessment by psychologist

Check list for assessment (1)

• • • • • • • Memory – Attention, concentration – Anterograde (recall new events), retrograde (previous public and personal events), semantic memory (vocabulary, names of objects, general factual knowledge) Language – Expressive - word-finding, grammar, word errors (paraphasias), writing (spelling/motor) – Receptive - comprehension, reading Numerical skills – Handling money, shopping, bills Executive function – Planning, organising, problem-solving, flexibility Visuo-spatial – Dressing, constructional abilities Neglect – Bodily neglect, extrapersonal space Visual perception – Recognising people, objects, colours

Check list for assessment (2)

• • • • • • • Route findings and landmarks – Recognition of known landmarks, learning new routes Personality and social conduct – Empathy, disinhibition, sexual behaviour, grooming, personal hygiene Eating – Appetite, food preferences, manners Mood – Depression, mania, anxiety, irritability Motivation – Drive, energy Delusions Hallucinations

Practise EMQs and MCQs See handout

Key references

• • • • • Hebben, N., & Milberg, W. (2002).

Essentials of Neuropsychological Assessment.

New Jersey, USA: John Wiley & Sons.

Hodges, J.R. (2007).

Cognitive assessment for clinicians (Second edition

). Oxford: Oxford University Press.

Lezak, M.D., Howieson, D.B., Loring, D.W. (2004).

Neuropsychological assessment (Fourth edition).

New York: Oxford University Press.

Strauss, E., Sherman, E.M.S., & Spreen, O. (2006).

edition).

New York: Oxford University Press.

A compendium of neuropsychological tests: administration, norms, and commentary (Third

Tulsky, D., Saklofske, D., Chelune, G., Heaton, R., Ivnik, R., Bornstein, R., Prifitera, A., Ledbetter, M. (2003).

Clinical interpretation of the WAIS III and WMS-III.

London: Academic Press.