Transcript A Case on DEMENTIA

A Case on
DEMENTIA
Neurology
MIRANDA – MOLINA – MONZON – MORALES – MUSNI – NALLAS - NAVAL
Batch 2011 - Section C
History
A 63 y/o woman was brought by her husband for
consult because of increasing forgetfulness.
The husband reports that his wife had completed a
degree in BS Education. She has been teaching for
the past 25 yrs. Confidentially, he reports that she has
increasing difficulty remembering her class schedules
and examinations as well as conversations with
coworkers over the past year.
He likewise noted reduced interest and withdrawal
from many long-standing social activities.
Recently, she left food cooking on the stove, which
resulted in a small kitchen fire.
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History
The patient has no significant current medical
problems and takes no medications. The patient's
older brother has recently been diagnosed with the
same illness.
There were no significant PE findings.
On mental status testing, the patient was noted to be
disoriented to time and person. She had difficulty with
calculation and had impaired short-term verbal
memory. Visuospatial abilities, however, were intact.
Cranial CT scan done revealed normal findings.
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Salient Features
Pertinent Positive
• 63 year old, female
• CC: Increasing forgetfulness over the past
year
– Difficulty remembering class schedules
and exams and conversations with coworkers
– Left food cooking on the stove  small
kitchen fire
• Impaired short-term verbal memory
• Reduced interest ; withdrawal from social
activities
• Disoriented to person and time
• Difficulty with calculation
• Brother: diagnosed with same illness
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Salient Features
Pertinent Negative
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No significant medical problems
No medications taken
No significant PE findings
Visuospatial abilities intact
Normal CT
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Salient Features
• 63 year old, female
• CC: Increasing
forgetfulness over the past
year
• Impaired short-term verbal
memory
• Reduced interest;
withdrawal from social
activities
• Disoriented to person and
time
• Difficulty with calculation
• Brother: diagnosed with
same illness
Pertinent Positive
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Memory Impairment
DEMENTIA DELIRIUM
AMNESIA
Stable level of
consciousness
Impairment in
consciousness
Multiple cognitive
defects
Attention deficits
Insidious onset
Abrupt onset
Onset depends on
etiology
Behavioral
abnormality
Behavioral
abnormality
Behavioral
abnormality
Reference
Stable level of
consciousness
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Salient Features
• 63 year old, female
• CC: Increasing
forgetfulness over the past
year
• Impaired short-term verbal
memory
• Reduced interest;
withdrawal from social
activities
• Disoriented to person and
time
• Difficulty with calculation
• Brother: diagnosed with
same illness
Reference
Pertinent Positive
DEMENTIA
Stable level of
consciousness
Multiple cognitive
defects
Insidious onset
Behavioral
abnormality
Pseudodementia
is a depressionrelated cognitive
dysfunction
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Clinical Impression
Dementia
• Syndrome of cognitive decline
with variable non-cognitive
features of behavioral and
psychiatric symptoms and
disturbance in activities of daily
living
Reference
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Criteria for Dementia
DSM –IV criteria
• Multiple Cognitive Deficits
1. Memory Impairment
2. One or more
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•
Patient
• Increasing forgetfulness over the
past year
– Difficulty remembering
– class schedules
– exams
– conversations with co-workers
Aphasia
Apraxia
Agnosia
Executive
• Impaired
Social/Occupational
function
• Gradual and
progressive course
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•
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•
– Attention: Left food cooking on
the stove  small kitchen fire
Reduced interest ; withdrawal from
social activities
Disoriented to person and time
Difficulty with calculation
Impaired short-term verbal memory
Classification
Disease in which Dementia is:
• Associated with clinical and laboratory signs of
other medical diseases
• Associated with other neurological signs but not
with other obvious medical disorders
– Invariable associated with other neurologic signs
– Often associated with other neurologic signs
• Usually the only evidence of neurologic or medical
diseases
Pertinent Negatives
• No significant medical
problems
• No medications taken
• No significant PE findings
• Normal CT
Principles of Neurology , 8th Edition
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Dementia
Dementia is usually the only evidence
of neurologic or medical disease
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Alzheimers Disease
Diffuse Lewy Body Dementia
Pick Disease
Frontotemporal and frontal lobe dementias
Reference
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Dementia
• Diffuse Cerebral Atrophy
– Alzheimers Disease
– Diffuse Lewy Body Dementia
• Circumscribed Cerebral Atrophy
– Pick Disease
– Frontotemporal and frontal lobe dementias
Reference
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Dementia: Usually the only evidence of neurologic or medical diseases
Patient
Alzheimer’s
Disease
63 year old woman
≥ 60 years old;
3x higher in
women
Memory impairment
Memory
impairment
Difficulty with
calculation
Aphasia, apraxia,
agnosia, executive
dysfunction
Withdrawal from social
activities
Impaired social or
occupational
function
Disoriented to person
and time
Her brother diagnosed
with same illness
(+) Family
History
Symptoms noted
over the past year
Insidious onset
Lewy Body
Disease
Pick’s
Disease
Frontotemporal
Dementia
Early aphasia;
Dysexecutive
syndrome
Speech deterioration;
Poor judgment and
abstraction
Marked
psychiatric Sx
Prominent alteration in
personality behavior;
Early personality
changes;
Ritualistic & repetitive
behavior; Disinhibition
Fluctuating confusion
Visual Hallucinations
Paranoid delusions
Hallucinations
Neglect of personal
hygiene and grooming
(+) Family
History
Reference
2-5 years
course of
illness
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Alzheimer’s Disease
Alzheimer’s Disease
DSM IV Criteria
A. The development of multiple cognitive deficits manifested by both:
-1.Memory impairment (impaired ability to learn new information or to recall
previously learned information)
-2.One or more of the following cognitive disturbances:
(a) aphasia (language disturbance)
(b) apraxia (impaired ability to carry out motor activities depite intact motor
function)
(c) agnosia (failure to recognize or identify objects despite intact sensory
function)
(d) disturbance in executive functioning (i.e., planning, organizing, sequencing,
abstracting)
B. The cognitive deficits in criteria A1 and A2 each cause significant impairment in
social or occupational functioning and represent a significant decline from a
previous level of functioning.
C. The course is characterized by gradual onset and continuing cognitive decline.
D. The cognitive deficits in Criteria A1 and A2 are not due to any of the following:
(a) other central nervous system conditions that cause progressive deficits in
memory and cognition (e.g., cerebrovascular disease, Parkinson's disease,
Huntington's disease, subdural hematoma, normal-pressure hydrocephalus,
brain tumor)
(b) systemic conditions that are known to cause dementia (e.g., hypothyroidism,
vitamin B or folic acid deficiency, niacin deficiency, hypercalcemia,
neurosyphilis, HIV infection)
(d) substance-induced conditions
E. The deficits do not occur exclusively during the course of a delirium.
F. The disturbance is not
better accounted for by an Axis I disorder
Reference
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Alzheimer’s Disease
DSM IV Criteria
A. The development of multiple cognitive deficits manifested by both:
-1.Memory impairment (impaired ability to learn new information or to recall
previously learned information)
-2.One or more of the following cognitive disturbances:
(a) aphasia (language disturbance)
(b) apraxia (impaired ability to carry out motor activities despite intact motor
function)
(c) agnosia (failure to recognize or identify objects despite intact sensory
function)
(d) disturbance in executive functioning (i.e., planning, organizing,
sequencing, abstracting)
B. The cognitive deficits in criteria A1 and A2 each cause significant impairment in
social or occupational functioning and represent a significant decline
from a previous level of functioning.
C. The course is characterized by gradual onset and continuing cognitive
decline.
D. The cognitive deficits in Criteria A1 and A2 are not due to any of the following:
(a) other central nervous system conditions that cause progressive deficits in
memory and cognition (e.g., cerebrovascular disease, Parkinson's disease,
Huntington's disease, subdural hematoma, normal-pressure hydrocephalus,
brain tumor)
(b) systemic conditions that are known to cause dementia (e.g., hypothyroidism,
vitamin B or folic acid deficiency, niacin deficiency, hypercalcemia,
neurosyphilis, HIV infection)
(d) substance-induced conditions
E. The deficits do not occur exclusively during the course of a delirium.
F. The disturbance is not better accounted for by an Axis I disorder
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Reference
Alzheimer’s Disease
• Most common cause of
dementia
• Incidence increases with age
– 60 years and above
• 3x higher in women
• (+) Family History
– Chromosome 21 – amyloid gene – senile plaques
– Chromosome 19 – ApoE4 gene – inherited predisposition
– Chromosome 1, 14 – Presenilins 1 and 2
Reference
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Alzheimer’s Disease
Risk Factors
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Old age
Family history
Low education
Head trauma
High cholesterol
Hypothyroidism
Exposure to metals
Reference
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Alzheimer’s Disease
Risk Factors
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Old age – 63 yrs old
Family history – older brother
Low education
Head trauma
High cholesterol
Hypothyroidism
Exposure to metals
Reference
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Alzheimer’s Disease
Clinical Features
• Gradual development of forgetfulness
• Cognitive Dysfunctions
– Language: expression, comprehension,
reading, writing
– Decline in arithmetic skills
(acalculia/dyscalculia)
– Visuospatial orientation
• 4 A’s: amnesia, aphasia, apraxia, agnosia
Reference
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Alzheimer’s Disease
Clinical Features
• Gradual development of forgetfulness
• Cognitive Dysfunctions
– Language: expression, comprehension,
reading, writing
– Decline in arithmetic skills
(acalculia/dyscalculia)
– Visuospatial orientation
• 4 A’s: amnesia, aphasia, apraxia, agnosia
Reference
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Alzheimer’s Disease
Clinical Features
• Executive Dysfunction
– Planning
– Organizing
– Sequencing
– Abstract thinking
• Behavioral and personality change
• Decline in ADL
Reference
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Alzheimer’s Disease
Clinical Features
• Executive Dysfunction
– Planning
– Organizing
– Sequencing
– Abstract thinking
• Behavioral and personality change
– Withdrawal from social activities
• Decline in ADL
– Disturbance in the household and workplace
Reference
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Alzheimer’s Disease
Pathophysiology
• Generalized brain atrophy
• Loss of neurons
• Astrocytic proliferation
- inflammation
• Microscopic changes
- Neurofibrillary tangles
• Histological marker
- Amyloid deposition
• Histological marker
- Granulovacuolar degeneration
Reference
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Alzheimer’s Disease
• Diagnostic Procedures
• Cranial CT or MRI scan
- Mild AD: normal or MTL atrophy
- Advanced AD
– Generalized atrophy
• EEG
- Diffuse slowing (theta/delta range) in
late disease
• CSF analysis
- Normal, slight increase in total protein
Reference
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Alzheimer’s Disease
Diagnostic Procedures
• Neuropsychological Tests
- Poor memory, verbal skills in early
to moderate stages
• Biologic Markers
- CSF tau and β amyloid
- Inflammatory markers
– Isopostane (serum & CSF)
Reference
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Diagnostic Procedures
Diagnostic Procedures
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Mini-Mental State Examination
Blood tests
Cranial CT scan or MRI
Single-photon emission CT (SPECT)
EEG
CSF analysis
Reference
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Mini-Mental
State Examination
• assesses cognitive abilities such as
orientation to time and place, use of
language, memory, attention, and
abilities to carry out various tasks
and follow instructions
Reference
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Blood tests
• check for infections or conditions
such as vitamin deficiency, anemia,
medication levels, disorders of the
thyroid, kidneys or liver
Reference
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Cranial CT scan or MRI
• reveals reduction in the size of the
brain (atrophy), widened indentations
in the tissues, and enlargement of
the cerebral ventricles
Reference
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Single-photon emission CT
(SPECT)
• imaging detects blood flow in the
brain
• used in some medical centers to
distinguish Alzheimer’s disease from
vascular dementia
Reference
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EEG
• diffuse slowing (theta/delta range) in
late disease
Reference
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CSF analysis
• normal, slight increase in total
protein
• biologic markers: amyloid beta or
tau proteins
Reference
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Thank you!
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