clinical neuropsychological assessment of ftd - ftdrg. · pdf fileneuropsychological test...
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Clinical Neuropsychological Assessment of Frontotemporal
Dementia
Nora BreenRoyal Prince Alfred Hospital
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Referral : Does this patient have dementia?
NO
Neurodegenerative Disease?
YES
Alzheimer’sFTD
Behavioural FTD
Semantic Dementia
PNFA
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Progressive Non-Fluent Aphasia(PNFA)
Language FormPrimary Progressive Aphasia
FTD
Behavioural Form75% FTD Cases
Semantic Dementia
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Neuropsychological Assessment
Neuropsychologist relies equally on three components of the assessment:
1. History2. Behavioural Observations3. Neuropsychological Test Scores
3 cases – BvFTD, SD and AD
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History
Case 1 BvFTD• 59 woman, inpatient, morbidly obese• Referral: poor self-care and neglect of home
Patient history : • Denied any problems with thinking or memory • Denied personality change • Reports being self-caring, showering, shopping
and cooking meals.
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History
Case 1 BvFTD
Daughter’s history
Previously:• Mother was immaculately groomed, meticulous about
hair, nails, clothes. • Previously boundless energy, when wasn’t working she
was cleaning house or socialising.
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HistoryCase 1 BvFTDDaughter’s history continued..• 12 months ago – stopped showering/changing clothes.
No longer cared about appearance.• 30 kilo weight gain last 12 months, binge eating• Spends all day watching tv, smoking, eating• Cannot be bothered to use toilet, urinates in chair• Sits in soiled clothes until daughter forces her to
change.
• Memory is good.
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History
Case 2 SD• 62 year old woman• 3 year hx of worsening language comprehension
and speech problems
Patient described: 1. Inability to name objects “I see things and
recognise them but cannot put a name to them”
2. Unable to remember word meanings “I can hear a word but not know what you are talking about”
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History
Case 2 SD• Daughter’s additional history:
• Previously sophisticated vocabulary is diminished• She no longer understands jokes or sarcasm• Interprets spoken language very literally• Difficulty reading because can’t remember meanings
of words• *Good memory
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HistoryCase 3 AD• 74 yr old woman• Patient reported: some forgetfulness
• Husband reported:• 12-18 mth history of increasing forgetfulness regarding
everyday activities and appointments• Sometimes disoriented to day or time• Losing things• Repeating same questions – up to 6 times in one hour• Preserved personality/social skills, independent in ADL
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History Summary
BvFTD SD ADInsight Early Loss Intact IntactBehaviour Changes Yes No NoMemory Deficits No No YesLanguage Deficits No Yes No
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Behavioural observations
Case 1 BvFTD• Morbidly obese• Unkempt and dishevelled (long nails, unwashed hair,
smelled of urine)• Brusque manner • Bland affect• Confabulation• Assessed twice, 5 days apart. Remembered my name
on 2nd occasion without difficulty.
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Behavioural Observations
Case 2 SD• Immaculately groomed and dressed• Extremely pleasant and cooperative, social skills
entirely intact• Unable to follow conversation or respond to
questions unless language kept very simple• Asked if drank alcohol: “sorry, that word is
familiar but I don’t know what it is”• In spite of language difficulties, she was able to
convey reasonable account of history and current difficulties.
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Behavioural Observations
Case 3 AD• Pleasant, neatly groomed and dressed, cooperative
with testing, socially appropriate• Relied on husband to give history• Occasionally stopped half-way through task to
clarify what she was supposed to be doing.• Appropriately disappointed by her errors on
testing (insight)
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Behavioural Observations Summary
BvFTD SD ADBehaviour Impaired Normal NormalSocial Skills Impaired Normal NormalAffect Blunted Normal Normal
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Neuropsychological TestingCognitive domains assessed:• Orientation• Memory• Attention• Visuospatial and constructional skills• Language• Executive function (problem solving, flexible
thinking, planning)*Mood
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Cognitive profiles
• Specific patterns on testing are consistent with diagnosis of different types of dementia.
Bv FTD: executive impairment
SD: semantic knowledge loss, naming deficit
AD: memory impairment (rapid forgetting)
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Memory
Expected pattern:
BvFTD - learning recall ? recognition SD - hard to assess, visual memory intactAD - impaired (rapid forgetting)
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Verbal Memory
RAVLT 15 word list
5th trial Recall Recogn fpBvFTD 10 9 10 1AD 6 1 13 9
SD (not appropriate)
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Visual Memory
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Case 1 BvFTD
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Case 2 SD
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Case 3 : AD
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Executive Function
Ability to plan, problem solve, shift mental set, adapt responses according to feedback, think flexibly.
Expected pattern:BvFTD - impairedSD - intactAD - intact
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Executive Function
Wisconsin Card Sorting TestSet shifting, problem solving, mental flexibility6 possible categories
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Executive Function
WCST – ResultsCategories Perseveration
Case 1 BvFTD 0 YES *Case 3 AD 5 no
*sorted to incorrect category for entire deck!
Case 2 SD Unable to comprehend instructions
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LanguageBvFTD altered speech output (asponteneity or
rushed speech)echolaliaperseverationmutism
SD fluentloss of word meanings naming and comprehension deficit semantic errors
AD mild/moderate naming deficit
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Language - Naming
Boston Naming Test 60 Item
• BvFTD 58 /60• SD 5 /60• AD 41 /60
*SD: snail “I don’t know what that is. Is it a bird of some sort? I don’t recognise it at all.”
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Cognitive Profiles
BvFTD SD ADExecutive Function Impaired Intact IntactVisual Memory Intact Intact ImpairedVerbal Memory Intact n/a ImpairedLanguage Intact Impaired Mildly Impaired