Neuropsychology and differential diagnosis Julie Snowden Cerebral Function Unit Greater Manchester Neuroscience Centre Neuropsychology of Dementia Harrogate, July 2008

Cortical Subcortical Cortico- subcortical Alzheimer’s disease Frontotemporal dementia Huntington’s disease Progressive supranuclear palsy Subcortical vascular disease Dementia with Lewy bodies Corticobasal degeneration

MRI coronals SPECT coronal AD sagittal

FTD MRI coronals SPECT coronal sagittal

Alzheimer’s disease disorder of cognition Impaired memory Impaired word retrieval Spatial disorientation Social preservation Frontotemporal dementia disorder of behaviour Personality change Breakdown social conduct Impaired judgement ‘Instrumental’ skills relatively preserved

Parietal Temporal Occipital Frontal Praxis Spatial orientation (mental map) Visual perception Language Executive functions Social cognition Episodic memory (events) Alzheimer’s disease (AD) Frontotemporal dementia (FTD)

AD Cognition Memory –Repetitive in conversation –Gets lost –Mislays objects –Forgets day to day events –Loses track  Consistent impairment  Cueing limited benefit  Loss over delay  Reduced immediate span

Language Hesitant, halting Impaired word retrieval Paraphasic errors (typically infrequent) Loses train of thought/sentences unfinished Reduced span

Visual Object and Space Perception Warrington & James 1991 Perceptual errors on object naming tests “spectacles” Perception “a T”

Spatial Impairment lost in familiar surroundings driving accidents difficulty with daily activities: e.g. dressing, stairs VOSP battery Warrington & James, 1991

Constructional difficulties in Alzheimer’s disease

Frontotemporal dementia Breakdown in social, interpersonal conduct Neglect of self-care and responsibilities Emotional blunting, loss of insight Repetitive, stereotyped behaviours Hyperorality, dietary changes

Plans/Goals Organisation Attention Abstraction New plan Checking Flexibility

Planning Organisation Attention Abstraction Checking Flexibility Inertia/purposeless overactivity Disorganised behaviour Inattentive, distractible, poor persistence Concreteness of thought Poor self-monitoring, impaired insight Inflexible Frontotemporal dementia: Cognition and the frontal lobes

Wisconsin Card Sorting Test

Weigls blocks shape, colour, motif Perseverative Dismantling behaviour

Brixton spatial anticipation test Burgess & Shallice, 1997

Picture sequencing Impaired executive skills Animals “Billy the budgie and Spot the dog” F words “Frank – my brother” Verbal fluency Concrete, rule violations

Design fluency in FTD Perseveration, concreteness, rule violation

FTDAD

FTD Rapid response Can localise dots AD Slow response Poor localisation

Cognition in AD and FTD ADFTD LanguageHesitant, impaired word retrieval, paraphasic, loses track Economical, concrete echolalia, stereotypy perseveration Perceptuo- spatial/ construction Loss of spatial configuration Preserved spatial configuration Organisational failures Perseveration

Cognition in AD and FTD cont. ADFTD Memory Pervasive, consistent Cueing modest benefit Loss over delay Inefficient, inconsistent Benefits from cues No rapid forgetting Executive skills “Can’t do” Overloaded Stuck in set Perseveration Concreteness Rule violations Behaviour Appropriate, concern for accuracy Impulsivity, inattention, poor persistence, economy of effort, unconstrained by goal

“Subcortical” dementia Mental slowing Poor generation, organisation and sequencing of information Inefficient memory encoding and retrieval No aphasia, agnosia, apraxia No gross personality change

Paired-associate learning friend – train gold - sugar

Paired-associate learning uncued % correct

Paired-associate learning uncued and cued

Dementia with Lewy bodies Fluctuating mental state Formed visual hallucinations Parkinsonism AD-like cognitive change But: Fluctuations, incoherence, distractibility, intrusions, confabulation, perseveration Misperceptions (Capgras phenomena)

VOSP screening: errors ***

Prevalence of interference effects in Azheimer’s disease and dementia with Lewy bodies ** ** *

Conclusions Dementia not generalised impairment of intellect – constellation of deficits Distinct patterns reflect distribution of pathology Differentiation of forms of dementia possible on neuropsychological grounds Need to take account of qualitative features Important for treatment and management