Supporting Employees with Early Onset Dementias & other cognitive deficits Dr Miriam Prins Consultant Clinical Neuropsychologist Head of Older Adults Psychology.

Supporting Employees Supporting Employees withwith

Early Onset DementiasEarly Onset Dementias& other cognitive deficits & other cognitive deficits

Dr Miriam PrinsDr Miriam PrinsConsultant Clinical NeuropsychologistConsultant Clinical Neuropsychologist

Head of Older Adults PsychologyHead of Older Adults Psychology

Somerset Partnership NHS Foundation TrustSomerset Partnership NHS Foundation Trust

© in entire presentation M Prins 2015© in entire presentation M Prins 2015

Early onset dementiasEarly onset dementias

Vascular dementiaVascular dementia Alzheimer’s disease Alzheimer’s disease Posterior cortical atrophyPosterior cortical atrophy Frontotemporal dementiaFrontotemporal dementia Neurodegenerative disordersNeurodegenerative disorders

Vascular dementiaVascular dementiaRisk factorsRisk factors

High blood pressureHigh blood pressure DiabetesDiabetes High cholesterolHigh cholesterol TIA’s and strokeTIA’s and stroke MI, cardiovascular surgery (any surgery)MI, cardiovascular surgery (any surgery) CADASILCADASIL

CADASILCADASIL

Rare autosomal dominant familial Rare autosomal dominant familial disorderdisorder

Presents with migrainePresents with migraine Recurrent small subcortical infarcts in Recurrent small subcortical infarcts in

patients generally free of vascular risk patients generally free of vascular risk factorsfactors

TIA’s and occasionally affective disordersTIA’s and occasionally affective disorders Typically presents in 40’sTypically presents in 40’s

Alzheimer’s diseaseAlzheimer’s disease

Remember that early memory problems Remember that early memory problems may arise for a number of reasons, a may arise for a number of reasons, a number of them treatable, and mild number of them treatable, and mild cognitive impairment (MCI) may or may cognitive impairment (MCI) may or may not progress to a dementianot progress to a dementia

Alzheimer’s diseaseAlzheimer’s diseaseUsual presentationUsual presentation

Early deficits primarily in episodic memoryEarly deficits primarily in episodic memory Insidious onset and gradual progressionInsidious onset and gradual progression Typical deficits are in memory, attention Typical deficits are in memory, attention

and visuospatial perceptionand visuospatial perception Early onset likely to decline more quickly Early onset likely to decline more quickly

and more problem with naming and word and more problem with naming and word comprehensioncomprehension

Loss of confidence or skillsLoss of confidence or skills

Posterior cortical atrophyPosterior cortical atrophy

Presents with visuospatial perception Presents with visuospatial perception problems:problems:

- Misjudging where things are in space- Misjudging where things are in space - Difficulty identifying objects in poor light- Difficulty identifying objects in poor light - Over/under stepping or bumping into things- Over/under stepping or bumping into things

Memory and verbal fluency relatively well Memory and verbal fluency relatively well preservedpreserved

100% AD pathology100% AD pathology

MCIMCI

Subjective memory loss, and objective Subjective memory loss, and objective evidence of memory impairmentevidence of memory impairment

Non-memory abilities and ADLs Non-memory abilities and ADLs preservedpreserved

NOT a diagnosis but state of clinical NOT a diagnosis but state of clinical uncertainty!uncertainty!

Frontotemporal Frontotemporal DementiasDementias

Behavioural Variant FTDBehavioural Variant FTD Progressive Non-fluent Aphasia Progressive Non-fluent Aphasia Semantic Dementia Semantic Dementia

Frontotemporal dementiaFrontotemporal dementiaBehavioural variantBehavioural variant

Second most common early onset after AD, Second most common early onset after AD, usual onset in 50’s, 7.1% AD pathologyusual onset in 50’s, 7.1% AD pathology

Most predictive early symptoms: personality Most predictive early symptoms: personality change, lack of concern, inappropriate change, lack of concern, inappropriate behaviour, change in appetite or food behaviour, change in appetite or food preferencepreference

Also: increasing agitation, apathy, disinhibition, Also: increasing agitation, apathy, disinhibition, reduced insight, concrete thinking, poor reduced insight, concrete thinking, poor groominggrooming

Behavioural Variant FTDBehavioural Variant FTD

Memory loss initially usually mild, recall Memory loss initially usually mild, recall variable, recognition good, visuospatial variable, recognition good, visuospatial perception NORMALperception NORMAL

Later Dysexecutive syndrome: problems Later Dysexecutive syndrome: problems initiating, planning, sequencing, decision initiating, planning, sequencing, decision making, abstract reasoning, prospective making, abstract reasoning, prospective memorymemory

Progressive non-fluent Progressive non-fluent aphasia FTDaphasia FTD

Gradually progressive dysphasia with other cognitive Gradually progressive dysphasia with other cognitive domains reasonably well preserveddomains reasonably well preserved

Semantic knowledge usually preserved Semantic knowledge usually preserved Maintain independent living without social or Maintain independent living without social or

behavioural problems behavioural problems 44% AD pathology44% AD pathology

Semantic dementiaSemantic dementia

Speech normal but empty of substanceSpeech normal but empty of substance Loss of word meaning and comprehensionLoss of word meaning and comprehension Impaired namingImpaired naming Can read and write regular words but surface dyslexia and Can read and write regular words but surface dyslexia and

dysgraphia for irregular wordsdysgraphia for irregular words Single word repetition preservedSingle word repetition preserved Calculation preservedCalculation preserved Reversed temporal gradient in semantic memoryReversed temporal gradient in semantic memory Episodic memory relatively well preserved and orientation goodEpisodic memory relatively well preserved and orientation good Executive function normal early onExecutive function normal early on Behavioural problems develop later (but may have loss of Behavioural problems develop later (but may have loss of

empathy earlier)empathy earlier) 10% AD pathology10% AD pathology

Assessing employees for cognitive Assessing employees for cognitive problemsproblems

Observe:Observe: - Behaviour of employee with others- Behaviour of employee with others - Employee’s interaction with you- Employee’s interaction with you - Gait and Posture- Gait and Posture - Arm swing- Arm swing - Positioning- Positioning - Spontaneous conversation- Spontaneous conversation

Listen to the storyListen to the story

Start with the employee’s subjective experienceStart with the employee’s subjective experience Context of life experiences, intellectual achievement, Context of life experiences, intellectual achievement,

employment historyemployment history Listen for depth and flow of narrative and quality of the Listen for depth and flow of narrative and quality of the

languagelanguage Take them out of comfort zone!Take them out of comfort zone! With employee’s consent, obtain collateral historyWith employee’s consent, obtain collateral history Possible hidden agenda and secondary gainPossible hidden agenda and secondary gain

Keep open mind about what you are toldKeep open mind about what you are told Story may be disguised or obscuredStory may be disguised or obscured Alcohol abuse may be hiddenAlcohol abuse may be hidden Probe for unreported sleep problems, Sleep Aponea, Probe for unreported sleep problems, Sleep Aponea,

REM sleep disturbanceREM sleep disturbance Check drug history (legal and otherwise)Check drug history (legal and otherwise) Vascular risk factorsVascular risk factors Head injuries, contact sports, seizuresHead injuries, contact sports, seizures Depression and other psychiatric conditionsDepression and other psychiatric conditions

Questions to ask:Questions to ask:

What was the first thing you noticed?What was the first thing you noticed? How are things different from two months ago?How are things different from two months ago? Who is more concerned, employee or someone else?Who is more concerned, employee or someone else? Does employee turn to someone else for answers?Does employee turn to someone else for answers? Who is complaining about the problem, employee or Who is complaining about the problem, employee or

someone else?someone else? Does the employee drive? Would you be happy to ride with Does the employee drive? Would you be happy to ride with

him/her?him/her? Does the employee participate in a conversation and forget Does the employee participate in a conversation and forget

it occurred?it occurred? Does employee ask same question several times? Does employee ask same question several times?

Rehabilitation techniquesRehabilitation techniques

Expanded rehearsal (spaced retrieval)Expanded rehearsal (spaced retrieval) Prompting and cueingPrompting and cueing Visual imageryVisual imagery MnemonicsMnemonics Use of existing knowledge to help anchor and retrieve new Use of existing knowledge to help anchor and retrieve new

informationinformation Action based learning better than verbalAction based learning better than verbal External Memory aidsExternal Memory aids

- Systematic use of a notebook- Systematic use of a notebook

- Digital timer, digital watch or smart phone- Digital timer, digital watch or smart phone

- White board- White board

Referral for assessment:Referral for assessment:

Obtain the employee’s written consent to refer to their Obtain the employee’s written consent to refer to their GPGP

In the referral, explain reason for referral, your In the referral, explain reason for referral, your concerns, your observations and the story you have concerns, your observations and the story you have obtained.obtained.

Reassure the employee that you are there to support Reassure the employee that you are there to support him/her – he is likely to be frightened about his job, him/her – he is likely to be frightened about his job, what may be found and the futurewhat may be found and the future

Reassure the employee that you will work with him/her Reassure the employee that you will work with him/her to help him continue working for as long as appropriateto help him continue working for as long as appropriate

When to call it a dayWhen to call it a day

Often it is the employee who will ask to take early Often it is the employee who will ask to take early retirement because declining cognitive function will retirement because declining cognitive function will increase anxiety and lead to a loss of confidenceincrease anxiety and lead to a loss of confidence

Sometimes it will be necessary to work with employee Sometimes it will be necessary to work with employee to reduce complexity of task or modify responsibility as to reduce complexity of task or modify responsibility as ability declinesability declines

Assess risk in the context of the job employee is doing Assess risk in the context of the job employee is doing and repeat risk assessment as necessary. This may and repeat risk assessment as necessary. This may inform the process of when employee should no longer inform the process of when employee should no longer work.work.

The EndThe End