Download The Dementias

The Dementias
&
The Caregivers
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Have you ever heard the following?
By the way Doctor,
• I think there is something wrong
with my husband’s/wife’s memory!
• I think I’m getting Alzheimer’s!
• I can’t remember sh--!
• I am having trouble remembering names!
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What do you do?
• Do you say that’s probably normal?
• Do you begin a work-up?
• Where do you begin?
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Dementia
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Outline
• Background
• Diagnosis
• Types
• Treatment
• Prevention
• Personal experience
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Background
• Dementia
– Defined as the progressive decline in cognitive
function due to damage or disease in the
brain beyond what is expected during the
course of normal aging
– Not a disease itself, but rather a group of
symptoms that may accompany certain
diseases or conditions
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Background
• Reversible or Irreversible depending on
etiology
• Decline is broad-based and can affect
memory, attention, concentration, speech,
judgement and personality
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Background
• Dementia has always been somewhat common,
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but is becoming even more common among the
elderly in recent history
Not clear whether this increased frequency of
dementia reflects a greater awareness of
symptoms, or if people are simply living longer
and thus are more likely to develop dementia in
their older age
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Epidemiology of Dementia
• 1% of Americans 60 years old, with percentage doubling
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every 5 years to nearly 50% by 85 years old
By 2030 nearly 20% of US population will be over 65
6% will have severe dementia; 10-15% will have mild to
moderate impairment
Early dementia frequently goes undiagnosed by Primary
Care Physicians
60-70% of progressive dementia cases are due to
Alzheimer’s disease
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Delirium vs. Dementia
Delirium
Dementia
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Acute onset, waxing/waning
Almost always reversible
Arousal level fluctuates
Poor attention
Often accompanied by
hallucinations
Possible causes: systemic
infection/neoplasm,
alcohol/drugs (narcotics,
benzodiazepines), stroke,
heart disease, electrolyte
imbalance,
hyper/hypoglycemia, uremia
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Progressive onset, constant
Usually not reversible
Arousal level normal
Attention usually unaffected
Usually not accompanied by
hallucinations
Possible causes:
Alzheimer’s, multi-infarct,
Pick’s disease (or related),
alcohol, brain infection
tumor, malnutrition
(B12/thiamine deficiency)
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Role of the Family Physician
• Systematically evaluate any cognitive complaints
• Recognize cognitive impairment that exceeds normal
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aging memory lapses
Identify and screen for potentially reversible diseases
Distinguish from other more specific cortical deficits --aphasia(disorder of language), agnosia(disorder of
recognition), isolated memory deficit
Prompt identification of dementia can protect the patient
from further avoidable harm (falls, drug overdose, fires,
inadequate nutrition)
Follow patients closely who present with mild cognitive
changes
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Clinical Presentation
• Generalized and sustained progressive decline in
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intellectual functioning from a previously stable attained
level
Patients may notice that they are more “forgetful”, and
family members may notice increasing inconsistencies in
behavior and personality
As disease progresses, patients may demonstrate
increasingly impaired judgment, inability to think
abstractly or generalize, personality changes (rigidity,
irritability, confusion with minor environmental changes)
Extreme forms may present with inability to participate
in Activities of Daily Living (ADLs) and complete loss of
original personality; behavior changes including
agitation, delusions, hallucinations are possible
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History and Physical Exam
• History
– Usually involves both patient and family member or
other knowledgeable informant
– Initial questions should target common functional
lapses that occur early in dementia
• Names, important events
• Finding words to express thoughts
• Lost in familiar places
• Forgetting how to use familiar gadgets and tools
• Inability to keep up with day-to-day responsibilities
previously done without trouble
– Inquire about mood, personality, behavior changes or
disturbances
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History and Physical Exam
• Perform general physical exam including
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complete Neurological exam
Perform complete Mental Status exam
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General description
Mood
Affect
Thought
Perception
Memory and Cognition
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Mental Status Exams
• AMTS (Abbreviated Mental Test Score)
– Sensitivity 73% to 100%
– Specificity 71% to 100%
• MMSE (Mini Mental Status Examination)
– Sensitivity 71% to 92%
– Specificity 56% to 96%
• Others
– Clock drawing test
– Ask the informant (relative or other supporter) to fill out
a questionnaire about the person’s everyday cognitive
functioning. The best known questionnaire is the
Informant Questionnaire on Cognitive Decline in the
Elderly (IQCODE)
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Abbreviated Mental Test Score
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Abbreviated Mental Test Score
• Introduced by Hodkinson in 1972 to
rapidly assess elderly patients for the
possibility of dementia.
• Each question correctly answered scores
one point.
• A score of less than 6 suggests dementia
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Mini Mental Status Examination
• Systematically and thoroughly assesses mental status
• It is an 11-question measure that tests five
areas of cognitive function
• Orientation, Registration, Attention and Calculation,
Recall and Language
• Normal >30
• Mild dementia: 20-26
• Moderate dementia: 10-19
• Severe dementia: <10
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History and Physical Exam
Clock Drawing Test
• Draw a clock
Put in all the numbers
Set the hands at ten past eleven.
• Scoring system for Clock Drawing test
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There are a number of scoring systems for this test.
The Alzheimer's disease cooperative scoring system is based on a score of
five points.
1 point for the clock circle
1 point for all the numbers being in the correct order
1 point for the numbers being in the proper special order
1 point for the two hands of the clock
1 point for the correct time.
A normal score is four to 5 points.
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Laboratory Tests
• Performed to rule out treatable and reversible causes
• Tests Include:
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B12
Folic Acid
TSH
C-Reactive Protein
CBC
Electrolytes
Calcium
Renal Function
LFTs
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Ruling Out Treatable Causes
• Vitamin B12 deficiency
• Endocrine disorder (thyroid, parathyroid)
• Uremia
• Syphilis
• Brain tumor
• Normal Pressure hydrocephalus
• Thiamine deficiency (B1)
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Wernicke-Korsakoff Syndrome
• Most often due to thiamine deficiency
• Gross and microscopic findings
– Hemorrhages with hemosiderin deposits
– Discolored mamillary bodies on walls of 3rd and 4th ventricle
– Neuronal loss, gliosis, and vessel hemorrhage
• Wernicke’s Encephalopathy (reversible)
– Confusion, ataxia, nystagmus, and ophthalmoplegia
• Korsakoff’s psychosis (irreversible)
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Targets the limbic system
Confabulation
Anterograde amnesia (inability to form new memories)
Retrograde amnesia (inability to recall old memories)
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Metabolic/Nutritional Diseases
Impairing Cognitive Ability
• B12 deficiency
– Weakness and decreased vibratory sense
(worse in legs), paresthesias, hyperreflexia,
ataxia, personality change, dementia
– Pathology: subacute degeneration of the
posterior columns and lateral corticospinal
tract
– Treatment: B12 replacement
– Note: Folate deficiency has no neurologic
disease
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Wilson’s Disease
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Autosomal Recessive
Defect in copper excretion in bile
Defect in synthesis of ceruloplasmin
Leads to cirrhosis and excess free Cu in blood
Clinical Findings: signs of Parkinsonism, chorea,
and dementia
Pathology: Atrophy and cavitation of basal
ganglia, particularly the putamen
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Metabolic/Nutritional Diseases Impairing Cognitive Ability
• Hepatic encephalopathy
– hyperreflexia, asterixis, dementia, seizures,
obtundation/coma
– Pathology: Brain toxicity secondary to liver cirrhosis
causing defective urea cycle; excess ammonia causes
increase in aromatic amino acids (e.g., phenylalanine,
tyrosine, tryptophan) which are converted to false
neurotransmitters (GABA)
– Treatment: lactulose, neomycin, and protein
restriction to decrease ammonia-related toxins
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Types of Dementia-Cortical
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Alzheimer’s Dementia
Vascular Dementia (multi-infarct dementia)
Dementia with Lewy Bodies
Alcohol Induced Dementia (Thiamine Deficiency)
Frontotemporal Lobar Degeneration (Pick’s Disease)
– Frontotemporal Dementia
– Semantic Dementia
– Progressive Non-Fluent Dementia
Creutzfeldt-Jakob Disease
Dementia Pugilistica
Moyamoya Disease (rare cerebrovascular disease caused
by blocked arteries at the base of the brain in the basal
ganglia)
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Pick’s Disease
• Discovered by Dr. Arnold Pick
(Prague)1892
• Pick Body- a protein tangle that appears
as a large body in neuronal tissue
• Dr. Alois Alzheimer (1911) complete
absence of senile plaques, and
neurofibrillary tangles
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Frontotemporal Dementia(FTD)
Cortical
• Describes a clinical syndrome associated with
shrinking of the frontal and temporal lobes.
Originally known as Pick’s Disease, the name
and classification of FTD has been a topic of
discussion for over a century. The current
designation groups together Pick’s Dx, primary
progressive aphasia, and semantic dementia as
FTD. Occurs in 2% of Dementias
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Frontotemporal Dementia
Cortical
• Varying personality and behavior changes,
from apathy to hyperactivity
• Loss of empathy toward others; lack of
proper social conduct
• Memory is preserved early on
• Language difficulty
• Compulsive eating and oral fixations
• Repetitive actions
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Neurological Diseases Impairing Cognitive Ability
Cortical
• FTD (Pick’s Disease)
– Clinically resembles Alzheimer’s, more
prevalent in women, onset in 50’s
– Treatment: antidepressants, antipsychotics,
sleep aids, no benzodiazepines
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Neurological Diseases Impairing Cognitive Ability
Cortical
• Alzheimer’s disease
– Slow decline in cognitive and behavioral ability, no
focal deficits
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Alzheimer’s Dementia
• Most common cause of dementia; More common in females
(2:1)
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Presenile (<65yo)
Senile (>65yo)
Usually sporadic
10% cases have a genetic component
• It is estimated that 47% of the population over age 85 is
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afflicted. This represents a major social and public health
concern. Symptoms are those of slowly progressive global
mental deterioration.
Pathology
– There is a decrease in degradation of amyloid precursor protein
(APP). Defects in degradation of APP by secretases causes increase
in APP. This APP is then converted to amyloid beta which is
deposited into neurons. Amyloid beta is toxic to neurons.
– Increased beta-amyloid leads to increased destruction of neurons
– Increased density of neurofibrillary tangles resulting from
hyperphosphorylated tau protein
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– Increased density of beta-amyloid plaques
Alzheimer’s Dementia
Continued
• Gross and Microscopic findings
– Cerebral atrophy with dilation of the ventricles
• Due to loss of neurons in frontal, temporal, and parietal lobes
• Occipital lobe usually spar
• Clinical Findings
– NO focal neurologic deficits
– General impairment of higher intellectual function (exectuvie)
– Patients usually die of infectious process (i.e., bronchopneumonia)
• Confirmation of Alzheimer’s disease requires postmortem
examination of brain
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• Acetylcholinesterase Inhibitors
– Tacrine (Cognex)
– Donepezil (Aricept)
– Galantamine (Reminyl)
– Rivastigmine (Exelon)
• N-methyl-D-aspartate blockers (NMDA
blockers)
– Memantine (Namenda)
• Certain vascular types of dementia
• Alzheimer’s Dementia
• Can be used in conjunction with cholinesterase
inhibitors
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Types of Dementia-Subcortical
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Dementia due to Huntington’s disease
Dementia due to Hypothyroidism
Dementia due to Parkinson’s disease
Dementia due to B1 deficiency
Dementia due to B12 deficiency
Dementia due to Folate deficiency
Dementia due to Syphilis
Dementia due to Subdural Hematoma
Dementia due to Hypocalcemia
Dementia due to Hypoglycemia
Dementia due to multiple etiologies
Dementia due to other general medical conditions (CV
disease, end-stage renal failure)
• AIDS dementia complex
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• Antipsychotic Medications
– Used to treat dementia-associated psychosis
– Symptoms include paranoia, delusions, hallucinations,
screaming, and combativeness
– Examples
• Haloperidol (Haldol)
• Risperidone (Risperdal)
• Olanzapine (Zyprexa)
• Quetiapine (Seroquel)
• Antianxiety Medications
– Used to treat anxiety associated with dementia
– Benzodiazepines are often avoided
• They may increase agitation in patients with dementia
• Too sedating
– Buspirone (BuSpar) is often initial drug of choice
– Selegiline
• Used in treatment in Parkinson’s disease
• Slows progression of dementia
• Acts as an antioxidant preventing free-radical damage
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• Antidepressant Drugs
– Depression is frequently associated with
dementia
– Regulating the reuptake of serotonin,
norepinephrine, and dopamine may slow the
worsening of cognitive and behavioral
impairment often associated with depression
in demented patients
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Imaging
• CT and/or MRI commonly performed but do
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NOT have great sensitivity for diffuse metabolic
changes associated with dementia in a patient
who shows no gross neurologic deficits
May yield important information regarding
certain reversible causes of dementia such as
infarction or normal pressure hydrocephalus.
SPECT and PET imaging may be superior to
clinical exam in differentiating vascular dementia
from Alzheimer’s dementia
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Alzheimer’s Disease
-Cerebral atrophy
-Senile plaques
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Parkinsonism
• Group of disorders that alter dopaminergic
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pathways
Pathophysiology
– Degeneration of neurons in substantia nigra
– Causes deficiency of dopamine
– Neurons contain intracytoplasmic eosinophilic bodies
called Lewy Bodies
• Usually leads to disorders in voluntary muscle
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movement
May lead to dementia in some individuals
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• Parkinson’s Disease
– Extrapyramidal signs (cog-wheel rigidity,
tremor, classic shuffling gait, mask-like faces),
slow onset
– Pathology of dementia: loss of dopaminergic
neurons in substantia nigra
– Treatment: dopaminergic agents
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Neurological Diseases Impairing Cognitive Ability
• Huntington’s disease
– Chorea, muscle rigidity, dementia; onset age
30-40
– Pathology: autosomal dominant disease
(trinucleotide repeat involving chromosome
4); atrophy of striatal neurons in caudate,
globus pallidus, and putamen
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Neurological Diseases Impairing Cognitive Ability
• Normal-pressure hydrocephalus
– Gait disturbance, dementia, incontinence
– Pathology: enlarged ventricles without
atrophy
– Treatment: ventricular shunting process
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Neurological Diseases Impairing Cognitive Ability
• Multi-infarct dementia
– Focal deficits, acute, stepwise loss of function,
hypertension, old infarcts by CT or MRI
– Pathology: multiple areas of infarct usually
subcortical
– Treatment: address atherosclerotic risk
factors, identify and treat thrombus
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Multi-infarct Dementia
Neurological Diseases Impairing Cognitive Ability
• Multiple Sclerosis
– Brainstem signs, optic atrophy, long-standing
disease with exacerbations and remissions,
increased deep tendon reflexes, nystagmus,
tremor, scanning speech, paresthesias, +
Babinski sign
– Pathology: MRI showing white matter
abnormalities, specifically periventricular plaques,
multiple focal demyelination scattered in brain
and spinal cord
– Treatment: recombinant interferon,
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corticosteroids
Neurological Diseases Impairing Cognitive Ability
• Multiple Sclerosis
– Brainstem signs, optic atrophy, long-standing
disease with exacerbations and remissions,
increased deep tendon reflexes, nystagmus,
tremor, scanning speech, paresthesias, +
Babinski sign
– Pathology: MRI showing white matter
abnormalities, specifically periventricular plaques,
multiple focal demyelination scattered in brain
and spinal cord
– Treatment: recombinant interferon,
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corticosteroids
Infectious Diseases Impairing Cognitive Ability
• HIV infection
– Systemic involvement; risk factors for
acquisition; positive HIV serology
– Pathology: microglial cells fuse to form
multinucleated cells
– Treatment: treat specific infection
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Treatment ??
• Except for the treatable types, there is no
cure
• Aim of therapy is to slow the progression
of the disease
• Acetylcholinesterase inhibitors are often
used early in the course of disease
• Cognitive and behavioral interventions
may be appropriate
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Prevention of Dementia
• Lead and active life, both mentally and
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physically
Regular moderate consumption of beer and wine
may reduce risk
Hypertension medications, NSAIDS, and certain
anti-diabetic drugs may help in prevention
Some studies suggest that Mediterranean diets
such as those rich in beta-carotene may help
prevent certain cortical dementias
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Case Presentation
58 y/o White Female,G1P1, daughter, 32
week gestation
Hypothyroid x 20 + years
BCP’s until menopause then Prempro
PSH – T & A, Appy, Tubal ligation, Liver
cyst destruction (10-07)
Occupation, office manager, Dr’s office
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Clinical Presentation
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Forgetful
Lost in thought
Ritualistic (stroking hair when watching television)
Avoids complex tasks
Late in paying bills
Not as friendly (noticed by friends for about 4-6 months)
Very tired
Quiet
Loses temper easily (not to family or friends but to
phone solicitors or smokers by public entrances)
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Clinical Presentation
• Plays solitaire during every free moment in office,
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instead of doing office work, misses a lot of plays
Charts filed in wrong order
Letters misplaced on charts, ETS should have been EST
Desk a mess, not organized, has trouble finding things
Forgets a lot of things she did routinely in office
Not taking messages properly or forgetting to give
Put wrong dates and times on Appointment Cards
Not sleeping well
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Clinical Presentation
• She broke her arm changing a light bulb on the
front of the garage, a light not needed, there is
a flood light in the center of the garage. She
was on step stool. She did it when husband was
out of town. When I asked why she didn’t wait
one day for husband to change the bulb? She
stated “IT WAS BUGGING ME”. She has not
changed a light bulb in 33 years of marriage,
husband always change them promptly.
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Neuro-Psych Evaluation
• Read Report
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Imaging Studies Pardee
• Read Reports
• Show Images
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The Patient
• My wife of 35 years has developed dementia !
• But what kind ??
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REMEMBER THE CAREGIVERS
• In today’s reality, the caregivers of patients
with dementia are often the spouse or the adult
children. They are stressed, depressed,
confused, and forgotten. Until placement is an
option, the physician must facilitate home
health, respite care, home medical equipment,
and bring organization and guidance to
medications, medical maintenance, and
specialists. They must also monitor for signs of
neglect, abuse, and fatigue.
Thank You !
• Any Questions ?
• Thoughts about Long – Term Care ?
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