Civic Family Health Team September 4 2013 based on a presentation developed by Dr Michael Malek with liberal quoting from UpToDate Disclosures I have no financial or other ties to the pharmaceutical industry ID: 908693
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Slide1
DEMENTIA
Ian Arnold MD CCFP
Civic Family Health Team
September 4, 2013
(based on a presentation developed by Dr. Michael Malek)
(with liberal quoting from UpToDate)
Slide2Disclosures
I have no financial or other ties to the pharmaceutical industry
I have never taken any of the drugs mentioned in this presentation
I
do
drink coffee
I was in university in California in the '60s
(speaks for itself, as our Stephen H. would say)
No animals were harmed in the making of this presentation.
Slide3Definition
Chronic acquired decline in memory
at least one other impairment in cognitive function (personality, language, executive, visual-spatial)
Sufficient to interfere with functioning (social, occupational) and ADL’s
> 6 months since onset
Slide4Definition
DSM-IV Part 1
Evidence from the
history
and mental status examination that indicates major impairment in
learning and memory
as well as
at least one
of the following:
Impairment in handling
complex tasks
Impairment in
reasoning
ability
Impaired
spatial
ability and orientation
Impaired
language
Slide5Definition
DSM-IV Part 2
The cognitive symptoms must
significantly
interfere with the individual's work performance, usual social activities, or relationships with other people
This must represent a
significant
decline
from a previous level of functioning
The disturbances are of
insidious
onset and are
progressive
, based on evidence from the history or serial mental-status examinations
The disturbances are
not
occurring exclusively during the course of
delirium
The disturbances are
not
better accounted for by a
major psychiatric diagnosis
The disturbances are
not
better accounted for by a
systemic disease
or
another brain disease
Slide6Costs (2013)
Dementia the Most Expensive Health Condition in the U.S.
Dementia tops heart disease and cancer in terms of overall spending in the U.S.
will likely continue rising as the population ages, according to a study in the NEJM
Researchers calculated that dementia care (including out-of-pocket spending, nursing home costs, Medicare costs, and formal and informal home care) cost $41,000 to $56,000 annually per case in the U.S. In 2010.
Slide7A Caregiver's Comment
(a woman responded on-line to the above article)
9 Apr 2013
My mother's around-the- clock home care for dementia came to $145,000 for 2012. Future costs are probably being underestimated. She has no real life.
I do not understand the endless push to keep people alive as long as possible.
I am doing everything I can to be heart unhealthy to avoid living to the age of dementia.
Slide8Is the Prevalence of Dementia Changing?
In England, the prevalence fell from 8.3% to 6.5% during the past 20 years
This rigorous study is a persuasive piece of evidence that, at least in some settings, the prevalence of dementia in elders is declining and might be sensitive to societal efforts to improve education, primary prevention, and healthcare delivery.
Matthews FE et al. A two-decade comparison of prevalence of dementia in individuals aged 65 and older from three geographical areas of England: Results of the Cognitive Function and Ageing Study I and II. Lancet 2013 Jul 16
Slide9Resources
Slide10What is to be done?
V.I. Lenin 1902
'Primum non nocere'
c.1640
"given an existing problem, it may be better not to do something, or even to do nothing, than to risk causing more harm than good."
Benefits vs. Harms
Action vs. Inaction
“When in doubt, don't”
Benjamin Franklin and many others
Slide11Two words to use correctly!
SCREENING
detect early disease or risk factors for disease in large numbers of
apparently healthy
individuals.
DIAGNOSTIC TESTING
establish the presence (or absence) of disease as a basis for treatment decisions in symptomatic or screen positive individuals
Slide12What is to be done
? Part 1
V.I. Lenin 1902
Screening
2003
:The U.S. Preventive Services Task Force (USPSTF) concludes that the evidence is insufficient to recommend for or against routine screening for dementia in older adults.
2013
:Population screening to detect Alzheimer's disease or other dementia can't currently be recommended because there isn't enough evidence to show it offers practical benefits, researchers reported here at the annual Alzheimer's Association International Conference.
“the bottom line here is that there are relatively few upsides to this general population screening and potentially there are some significant downsides."
Slide13What is to be done
? Part 2
V.I. Lenin 1902
Prevention:
(from UpToDate Sept. 2013)
Most data on preventing dementia come from observational studies. Prospective studies and randomized controlled trials
have
not
shown an overall benefit
from
cholinesterase inhibitors, vitamin E, NSAIDS, or estrogen replacement
. Ongoing studies should help to clarify the role of statins.
Vitamin E supplementation
increases the risk for all-cause mortality
and there is no convincing evidence that it affects the risk of developing AD (see "Vitamin supplementation in disease prevention").
We
do not recommend NSAID use
even in patients at high risk for AD (i.e, those with very strong family histories) because of the possible increased risk of cardiovascular events associated with NSAIDs and COX-2 inhibitors.
We encourage all patients, especially those with early dementia or at higher risk for dementia, to maintain or increase
physical activity and exercise
as long as there are no contraindications. Similarly, we encourage
cognitive leisure activities and social interaction
for as long as these are feasible. However, we recognize that these lifestyle factors remain
unproven
as a means of preventing dementia.
Slide14What is to be done
? Part
3
V.I. Lenin 1902
Awareness, Diagnosis and Management:
Know what might be dementia and how to investigate
Know what treatments might be useful and when and how to prescribe
Know what resources are available to support and protect
the patient
the caregivers
the community
Slide15Risk factors: unpreventable
Age
Family history (risk doubles for each 1
st
degree relative)
Low education
Previous CVA, MI
Slide16Risk factors: preventable?
Vascular risk factors:
risk
doubles
for every vascular risk factor
Atrial fibrillation
Diabetes
Hypertension
CAD
Hyperlipidemia
Smoking
Past CVA/TIA
Slide17e.g. Diabetes
The Lancet Diabetes & Endocrinology, Early Online Publication, 20 August 2013
Risk score for prediction of 10 year dementia risk in individuals with type 2 diabetes: a cohort study
Although patients with type 2 diabetes are twice as likely to develop dementia as those without this disease, prediction of who has the highest future risk is difficult.
We therefore created and validated a practical summary risk score that can be used to provide an estimate of the 10 year dementia risk for individuals with type 2 diabetes
.
Slide18Using the Dementia Risk Calculator
Risk
doubles
for every 5 years of age
AGE:
<65
-1% ,
65
-2% ,
70
-4%,
75
-8%,
80
-16%,
85
-32%
Each additional vascular risk factor approximately
doubles
the risk
(One risk factor: risk multiplier is 2; 2 or more risk factors: risk multiplier is 4)
Positive family history
doubles
the risk.
(One family member: risk multiplier is 2; 2 or more family members: risk multiplier is 4)
Overall risk
= age risk _____% x family history
risk multiplier
___x vascular
risk multiplier
___=
___%
But why do the math? Does it help the patient?
What does the patient notice?
Often,
NOTHING
And is brought to the office by the spouse, relatives or friends who have concerns
Slide20What do caregivers notice?
from the Alzheimer Society of Canada
Memory loss that affects day-to-day function
It's normal to occasionally forget appointments, colleagues' names or a friend's phone number and remember them later.
A person with Alzheimer's disease may forget things more often and not remember them later, especially things that have happened more recently.
Difficulty performing familiar tasks
Busy people can be so distracted from time to time that they may leave the carrots on the stove and only remember to serve them at the end of a meal. A
person with Alzheimer's disease may have trouble with tasks that have been familiar to them all their lives, such as preparing a meal.
Problems with language
Everyone has trouble finding the right word sometimes, but
a person with Alzheimer's disease may forget simple words or substitute words, making her sentences difficult to understand.
What do caregivers notice? (2)
Disorientation of time and place
It's normal to forget the day of the week or your destination -- for a moment. But
a person with Alzheimer's disease can become lost on their own street, not knowing how they got there or how to get home.
Poor or decreased judgment
People may sometimes put off going to a doctor if they have an infection, but eventually seek medical attention.
A person with Alzheimer's disease may have decreased judgment, for example not recognizing a medical problem that needs attention or wearing heavy clothing on a hot day.
Problems with abstract thinking
From time to time, people may have difficulty with tasks that require abstract thinking, such as balancing a cheque book.
Someone with Alzheimer's disease may have significant difficulties with such tasks, for example not recognizing what the numbers in the cheque book mean.
Slide22What do caregivers notice? (3)
Misplacing things
Anyone can temporarily misplace a wallet or keys.
A person with Alzheimer's disease may put things in inappropriate places: an iron in the freezer or a wristwatch in the sugar bowl.
Changes in mood and behaviour
Everyone becomes sad or moody from time to time.
Someone with Alzheimer's disease can exhibit varied mood swings -- from calm to tears to anger -- for no apparent reason.
Slide23What do caregivers notice? (4)
Changes in personality
People's personalities can change somewhat with age. But
a person with Alzheimer's disease can become confused, suspicious or withdrawn
. Changes may also include apathy, fearfulness or acting out of character.
Loss of initiative
It's normal to tire of housework, business activities or social obligations, but most people regain their initiative.
A person with Alzheimer's disease may become very passive, and require cues and prompting to become involved.
Slide24What do they mean?
Apraxia
loss of the ability to execute or carry out learned purposeful movements, despite having the
desire
and the
physical ability
to perform the movements. It is a disorder of
motor planning
, but is not caused by incoordination, sensory loss, or failure to comprehend simple commands
Agnosia
loss of ability to recognize objects, persons, sounds, shapes, or smells while the specific sense is
not defective
nor is there any significant memory loss
Slide25What do they mean
? Part 2
Aphasia
a disturbance of the comprehension and formulation of language
Impaired executive function
an umbrella term for cognitive processes that regulate, control, and manage other cognitive processes, such as planning, working memory, attention, problem solving, verbal reasoning, inhibition, mental flexibility, task switching, and initiation and monitoring of actions.
Slide26How would you test?
Apraxia
imitate use of common objects, such as toothbrush, hammer
Agnosia
have patient close eyes, and ask to identify a common object placed in hand
Aphasia
name body parts or objects in room
Impaired executive function
perform series of simple tasks
3 item instruction (in MMSE)
Serial 7’s or “WORLD” backwards
Verbal word lists
Slide27Take a history!
Level of education of patient
Onset: abrupt vs gradual
Progression: steady vs step-wise
Has there been an effect on functional activities?
ADL’s
Instrumental ADL’s
Having an 'informant' is essential as the patient may not be aware of deficits
Slide28What are the other two 'D's?
Delerium
Acute onset
Fluctuating cognition; clouding of sensorium
Depression
More likely to
self-report
memory loss
'slowing' and poor effort on testing
Dementia patients try hard but get it wrong
Slide29ADLs
and IADLs
Bathing and showering (washing the body)
Bowel and bladder management (recognizing the need to relieve oneself)
Dressing
Eating (including chewing and swallowing)
Feeding (setting up food and bringing it to the mouth)
Functional mobility (moving from one place to another while performing activities)
Personal device care
Personal hygiene and grooming (including washing hair)
Toilet hygiene (completing the act of relieving oneself)
DEATH
: dressing/bathing, eating, ambulating (walking), toileting, hygiene.
Slide30ADLs and
IADLs
Part 2
Instrumental activities of daily living (IADLs) are not necessary for fundamental functioning, but they let an individual live independently in a community:
Housework
Taking medications as prescribed
Managing money
Shopping for groceries or clothing
Use of telephone or other form of communication
Using technology (as applicable)
Transportation within the community
A useful mnemonic is
SHAFT
: shopping, housekeeping, accounting, food preparation/meds, telephone/transportation.
Slide31ADLs and
IADLs Part 3
Here are samples of some commonly used assessment tools:
Katz for ADLs and
Lawton for IADLs
Slide32Do some tests!
Physical exam
MMSE
MOCA
Trails A and B
What to do if the patient doesn't speak English?
Or doesn't read English?
Slide33Order some tests
The AAN recommends screening for
B12 deficiency
and
hypothyroidism
in patients with dementia.
There are no clear data to support or refute ordering "routine" laboratory studies such as a complete blood count, electrolytes, glucose, and renal and liver function tests.
Screening for neurosyphilis is
not recommended
unless there is a high clinical suspicion.
Tailor tests to patients with a compatible history (e.g. RBC folate in a patient with ethanol dependence, or ionized serum calcium in a patient with multiple myeloma, prostate cancer, or breast cancer).
Slide34Neuroimaging
Recommendations of the 4th Canadian Consensus Conference on the Diagnosis and Treatment of Dementia (CCCDTD4) Dec. 2012
The issue of whether all patients with dementia should have structural imaging is debated at every CCCDTD conference, with the opinion that it is not required in all patients, but rather for those who have special clinical features.
The practical message is that structural imaging is not required in all (although will be indicated in most) persons with cognitive impairment. Although more costly and less available, MRI is preferable to CT.
Slide35Dementia, in all it's glory
MCI
AD
FTD
DLB
VaD
NPH
Etc.
Slide36Mild Cognitive Impairment (MCI)
(Amnestic and Non-amnestic)
Amnestic MCI (aMCI)
refers to those individuals with significantly impaired memory who do not meet criteria for dementia
(classic MCI).
Memory complaint, preferably corroborated by an informant
Objective memory impairment
(for age and education)
Preserved general cognitive function
Intact activities of daily living
Not demented
Progression to Dementia: 10%/year
(about 3x the average rate)
But 11-40% of patients with MCI
improve
, even to normal, over a one to three-year follow-up
Slide37Mild Cognitive Impairment (MCI
) -2
(Amnestic and Non-amnestic)
Single domain non-amnestic MCI (naMCI)
is similar aMCI, except that this form of MCI is characterized by a relatively isolated impairment in a single non-memory domain, such as
executive functioning,
language, or
visual spatial skills.
may progress to other syndromes, such as frontotemporal dementia (FTD), primary progressive aphasia, dementia with Lewy bodies, progressive supra-nuclear palsy, or corticobasal degeneration.
Individuals within this group appear to be
at less of a risk of conversion to dementia
, although supporting evidence is limited.
Slide38Mild Cognitive Impairment (MCI
) -3
(Amnestic and Non-amnestic)
Epidemiology
:
Findings from epidemiological studies vary significantly, partially due to the differing diagnostic criteria, measuring instruments, definitions, and use of population verses clinic-based samples.
prevalence among older adults (>70 years) to be between 14 and 18 percent
In contrast to the
impaired awareness
of deficits commonly present in patients with AD, patients with MCI are often
particularly troubled
by their symptoms.
However, over time, patients with MCI who convert to AD shift to a relatively greater preponderance of informant-reported symptoms over self-reported symptoms.
Slide39Evaluation
Mild Cognitive Impairment (MCI)
Detailed history
(from patient and family/friends)
MMSE
&/or
Montreal Cognitive Assessment (MoCA)
Physical examination
(e.g. neuro exam for possible vascular etiology)
In one community-based study
depression and other psychiatric disease (10.2 percent),
alcohol and drug related causes (6.9 percent), and delirium (1 percent).
neurologic disease: brain
tumor
, Parkinson disease, multiple sclerosis, cerebrovascular disease, and epilepsy) (25%)
Among the remaining 57.5 percent, most (31.7 percent) had circumscribed memory impairment.
Slide40Differential diagnosis of memory loss
Slide41Alzheimer's Disease (AD)
Usually presents with concerns about memory
Or memory problems can elicited at presentation.
Slide42Dementia with Lewy bodies (DLB)
Visual hallucinations (detailed, recurrent)
Pronounced fluctuation in cognition over hours/days
Parkinsonism (esp. rigidity, bradykinesia)
Executive function worse than memory
Neuroleptic sensitivity
Unexplained falls/LOC
Slide43Clinical and radiologic features of
dementia with
Lewy
bodies
Slide44Fronto-temporal Dementia (FTD)
FTD typically presents as
either
a progressive change in
personality and social behaviou
r or as a progressive form of
aphasia
; in both cases continuing ultimately to a global dementia.
The behavioural variant is more common.
Slide45Fronto
-temporal Dementia (FTD
) -2
Behavioural changes (disinhibition, apathy)
Impulsivity, poor judgement
Self-neglect
Socially inappropriate
Executive function worse than memory
Language problems
Abnormal gait
Slide46Fronto
-temporal Dementia (FTD
) -3
Personality change
This can manifest as apathy with social withdrawal, loss of spontaneity, and abulia that can be mistaken for depression. Some patients alternate between passive behaviour and disinhibited outbursts.
Lack of insight
Most patients seem unaware or incompletely aware of their deficits at presentation, and virtually all exhibit impaired insight within two years of symptom onset. On probing, this is often a
lack of concern
rather than a lack of knowledge of their impairments.
Loss of social awareness
Individuals may infringe upon social norms in a manner that is incongruent with their premorbid behaviour. Their sense of decorum appears altered; they may make offensive remarks and behave inappropriately. Personal hygiene may be affected. Patients may be incontinent — voiding urine or feces in inappropriate places without apparent concern. They may commit antisocial, even criminal acts. While inappropriate sexual comments are somewhat common in patients with FTD, hyper-sexual behaviour is not; most patients with FTD have diminished libido.
Slide47Fronto
-temporal Dementia (FTD
) -4
Stereotyped or ritual behaviours
These can include insisting on the same foods or employing a repetitive "catch-phrase." Hoarding, counting, and pacing are among the described behaviours
Change in eating patterns.
Patients with FTD frequently overeat and may binge or develop food fads. Alcohol consumption may be excessive. As the disease
progresses, there may be oral exploration of nonfood objects.
Emotional blunting and loss of empathy
.
Patients may be described as more self-centred, unconcerned about family and friends, and "cold." They may have difficulty recognizing emotional expression in others.
Mental rigidity.
Patients with FTD often appear inflexible in their adherence to routines, as well as unable to adapt to new situations and see another's point of view.
Slide48Fronto
-temporal Dementia (FTD
)-5
Deficits in modulating attention
.
This can manifest as distractibility and impersistence, or as perseverative behaviour In addition, some patients demonstrate utilization behaviours. These are stimulus-bound actions in which the individual repeatedly uses and reuses objects within their sight, despite irrelevance to situation (e.g., they might repeatedly pick up a comb in front of them and use it while participating in a conversation).
Slide49Vascular Dementia (VaD)
At present, the entity of VaD is best understood as a
heterogeneous syndrome
rather than a distinct disorder, in which the underlying cause is cerebrovascular disease in some form and its ultimate manifestation is dementia.
In patients with cognitive deficits ultimately attributable to VaD,
memory impairment appears somewhat later
, and these patients may have
significant cognitive disability
long before they meet criteria for dementia.
Slide50Vascular Dementia (
VaD
) -2
Cortical:
multiple small infarcts that are not recognized as strokes
Can appear
fluctuating or step-wise
, as some patients improve after the event
Subcortical:
both lacunar infarctions and chronic ischemia affect the deep cerebral nuclei and white matter pathways.
may be
gradual
or
stepwise
and either
slow
or
fast
in decline.
Slide51Normal Pressure Hydrocephalus (NPH)
Urinary incontinence (early in course of dementia)
Rapidly progressive dementia
Abnormal gait
Slide52Comparison
AD:
short term memory, word finding, and way finding.
VaD:
acute vascular history, focal findings, positive imaging
Mixed AD/VaD:
mixed clinical picture or positive imaging
DLB:
fluctuations in cognition, hallucinations, Parkinsonism.
FTD:
under 70, behavioural issues, social tactlessness.
NPH:
early gait apraxia, incontinence, rapid progression of dementia
Slide53You have a diagnosis. Now what?
Address vascular risk factors
Assess safety (e.g home, driving)
Assess involvement of caregivers/family
Screening for caregiver burnout
Linking with Alzheimer’s society, CCAC, Champlain LHIN or other community resources
Slide54Driving safety: quick checklist
Family expresses concerns
Severity worse than MILD
FTD or Lewy Body dementia
Visuospatial problems (e.g. clock drawing test; intersecting polygons)
Fails Trails A and/or Trails B
Poor judgement/insight
Other important medical/physical problems; poor reaction time (e.g. “drop ruler” test)
Slide55Drugs
Recommendations of the 4th Canadian Consensus Conference on the Diagnosis and Treatment of Dementia (CCCDTD4) Dec. 2012
The practical messages are that:
concurrent causes of dementia have to be managed;
CIs are recommended for AD in mild to severe stages of dementia, AD with a cerebrovascular component, Parkinson Disease dementia, but
not
for probable vascular dementia;
the combination of CIs and memantine is logical, but an additive benefit has not been conclusively demonstrated
Slide56Cholinesterase Inhibitors
Indications
Efficacy of the 3
similar
for
mild-moderate dementia
(MMSE 10-26)
Approx. 1 year’s delay in progression of Alzheimer’s
Symptomatic drugs
May help apathy, hallucinations & delusions
Slide57Cholinesterase Inhibitors
Names and dosage forms
Aricept (donezepil): 5 and 10 mg tabs
Reminyl (galantamine): 8,16,24 mg extended release caps
Exelon (rivastigmine): 1.5, 3, 4.5, 6 mg given BID; also in patch form and oral solution
Slide58Cholinesterase Inhibitors
Dosing
Titrate dose every 4 weeks to maximum dose, if tolerated
Max doses:
Aricept 10 mg qAM
Reminyl ER 24 mg q AM
Exelon 6 mg BID
Slide59Cholinesterase Inhibitors
Contraindications
Cardiac: bradycardia, SSS, heart block
Active PUD
Severe asthma or COPD
Possible interaction with other anticholinergic meds
Slide60Cholinesterase Inhibitors
Common Side Effects
GI (>10%): nausea, vomiting, diarrhea, bloating, anorexia
Muscle cramps
Sleep disturbance (insomnia, nightmares)
Fatigue
Side effects can be self-limited
Slide61Cholinesterase Inhibitors
Effective?
20%
are
“super-responders”
seen within 6 weeks
30-50 % non-responders
with continued deterioration at the previous rate of decline
The rest are
mild responders
Must individualize based on response
Caveat:
The AD2000 study, the only
non-pharmaceutical industry
sponsored trial of cholinesterase inhibitors, found
no significant benefit of donepezil compared with placebo
for the two primary endpoints: entry to institutional care and progression of disability