1. Supporting Employees with Early Onset Dementias & other cognitive deficits Dr Miriam Prins Consultant Clinical Neuropsychologist Head of Older Adults Psychology Somerset Partnership NHS Foundation Trust © in entire presentation M Prins 2015

2. Early onset dementias  Vascular dementia  Alzheimer’s disease  Posterior cortical atrophy  Frontotemporal dementia  Neurodegenerative disorders

3. Vascular dementia Risk factors  High blood pressure  Diabetes  High cholesterol  TIA’s and stroke  MI, cardiovascular surgery (any surgery)  CADASIL

4. CADASIL  Rare autosomal dominant familial disorder  Presents with migraine  Recurrent small subcortical infarcts in patients generally free of vascular risk factors  TIA’s and occasionally affective disorders  Typically presents in 40’s

5. Alzheimer’s disease  Remember that early memory problems may arise for a number of reasons, a number of them treatable, and mild cognitive impairment (MCI) may or may not progress to a dementia

6. Alzheimer’s disease Usual presentation  Early deficits primarily in episodic memory  Insidious onset and gradual progression  Typical deficits are in memory, attention and visuospatial perception  Early onset likely to decline more quickly and more problem with naming and word comprehension  Loss of confidence or skills

7. Posterior cortical atrophy  Presents with visuospatial perception problems:  - Misjudging where things are in space  - Difficulty identifying objects in poor light  - Over/under stepping or bumping into things Memory and verbal fluency relatively well preserved Memory and verbal fluency relatively well preserved  100% AD pathology

8. MCI  Subjective memory loss, and objective evidence of memory impairment  Non-memory abilities and ADLs preserved  NOT a diagnosis but state of clinical uncertainty!

9. Frontotemporal Dementias  Behavioural Variant FTD  Progressive Non-fluent Aphasia  Semantic Dementia

10. Frontotemporal dementia Behavioural variant  Second most common early onset after AD, usual onset in 50’s, 7.1% AD pathology  Most predictive early symptoms: personality change, lack of concern, inappropriate behaviour, change in appetite or food preference  Also: increasing agitation, apathy, disinhibition, reduced insight, concrete thinking, poor grooming

11. Behavioural Variant FTD  Memory loss initially usually mild, recall variable, recognition good, visuospatial perception NORMAL  Later Dysexecutive syndrome: problems initiating, planning, sequencing, decision making, abstract reasoning, prospective memory

12. Progressive non-fluent aphasia FTD  Gradually progressive dysphasia with other cognitive domains reasonably well preserved  Semantic knowledge usually preserved  Maintain independent living without social or behavioural problems  44% AD pathology

13. Semantic dementia  Speech normal but empty of substance  Loss of word meaning and comprehension  Impaired naming  Can read and write regular words but surface dyslexia and dysgraphia for irregular words  Single word repetition preserved  Calculation preserved  Reversed temporal gradient in semantic memory  Episodic memory relatively well preserved and orientation good  Executive function normal early on  Behavioural problems develop later (but may have loss of empathy earlier)  10% AD pathology

14. Assessing employees for cognitive problems  Observe:  - Behaviour of employee with others  - Employee’s interaction with you  - Gait and Posture  - Arm swing  - Positioning  - Spontaneous conversation

15. Listen to the story  Start with the employee’s subjective experience  Context of life experiences, intellectual achievement, employment history  Listen for depth and flow of narrative and quality of the language  Take them out of comfort zone!  With employee’s consent, obtain collateral history  Possible hidden agenda and secondary gain

16.  Keep open mind about what you are told  Story may be disguised or obscured  Alcohol abuse may be hidden  Probe for unreported sleep problems, Sleep Aponea, REM sleep disturbance  Check drug history (legal and otherwise)  Vascular risk factors  Head injuries, contact sports, seizures  Depression and other psychiatric conditions

17. Questions to ask:  What was the first thing you noticed?  How are things different from two months ago?  Who is more concerned, employee or someone else?  Does employee turn to someone else for answers?  Who is complaining about the problem, employee or someone else?  Does the employee drive? Would you be happy to ride with him/her?  Does the employee participate in a conversation and forget it occurred?  Does employee ask same question several times?

18. Rehabilitation techniques  Expanded rehearsal (spaced retrieval)  Prompting and cueing  Visual imagery  Mnemonics  Use of existing knowledge to help anchor and retrieve new information  Action based learning better than verbal  External 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

19. Referral for assessment:  Obtain the employee’s written consent to refer to their GP  In the referral, explain reason for referral, your concerns, your observations and the story you have obtained.  Reassure the employee that you are there to support him/her – he is likely to be frightened about his job, what may be found and the future  Reassure the employee that you will work with him/her to help him continue working for as long as appropriate

20. When to call it a day  Often it is the employee who will ask to take early retirement because declining cognitive function will increase anxiety and lead to a loss of confidence  Sometimes it will be necessary to work with employee to reduce complexity of task or modify responsibility as ability declines  Assess risk in the context of the job employee is doing and repeat risk assessment as necessary. This may inform the process of when employee should no longer work.

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