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72 Cards in this Set

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Name four risk factors for Alzheimer's dementia
Risk Factors:
1. age
2. female
3. family hx of Down Syndrome
4. Thyroid disease
What are Senile Plaques?
Beta Amyloid plaques/ Neuritic Plaques
Insoluble protein core containing BETA-AMYLOID and APOLIPOPROTEIN E (APOE)
The beta amyloud and APOE are surrounded by abnormal axons and dendrites called dystrophic neuritis
What are Neurofibrillary Tangles?
Intracellular accumulations of TAU PROTEIN
prominent in the nucleus basal is, septal nuclei, and nucleus of the diagonal band where cholinergic projections arise. Also present to a lesser extent in locus ceruleus (norepinephrine) and raphe nuclei (serotonin)
The two pathologies that are the hallmark of Alzheimer's disease are:
Senile Plaques and Neurofibrillary Tangles
Senile Plaques are nonspecific, found in Down Syndrome and other forms of dementia.
Neurofibrillary Tangles are also nonspecific
The four common early sxs of Alzheimer's dementia
1. anterograde memory loss (dominant sxs)
2. anomic aphasia
3. apraxia
4. visuospatial deficits
What are Granulovacuolar Bodies and with which disease are they associated?
'bodies' found in some neurons. They are associated with DAT (especially in the hippocampus) as well as other adult onset dementias
In DAT, cell loss is most pronounced in:
a. parietal tertiary areas
b. inferior temporal cortex
c. limbic cortex
d. entorhinal cortex
D. The entorhinal cortex.
-->
The entorhinal cortex is in the anterior region of the parahippocampal gyrus and is specifically affected in Alzheimer's dementia
What chromosomes are implicated in Alzheimer's dementia?
Chromosome 1
Chromosome 12
Chromosome 19
Chromosome 21
How is Chromosome 21 associated with Alzheimer's?
* Associated with early onset AD (as early as 3rd/4th decade).
* Mutaion of Amyloid Precursor Protein (APP) gene
* Associated with Down Syndrome who have an extra chromosome 21 and develop early onset dementia.
How is Chromosome 19 associated with Alzheimer's?
* Associated with early onset AD
* Gene for APOE
-->
Multiple alleles of APOE. APOE-2 is protective, APOE4 increases risk.
The 3 stages of Alzheimer's Disease:

What are the time frame for each?
Stage 1: 1-3 years
Stage 2: 2-10 years
Stage 3: 8-12 years
Stage 1 Alzheimer's:
Which is/are not commonly seen in the first stage?

a. weak new learning
b. topographic disorientation
c. ventricular dilation on CT/MRI
d. Motor system is normal
C
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Stage 1 Alzheimer's does not include findings on MRI/CT
The following symptoms may be present in Stage 1 Alzheimer's except

a. empty speech
b. indifference
c. poor complex construction
d. fluent aphasia/poor comprehension
D
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Stage 1 Alzheimer's language functioning includes empty speech , paucity of ideas, anomia
Common symptoms associated with stage 2 Alzheimer's include all but

A. remote and recent memory impairment
B. Getting lost
C. restlessness, pacing
D. Fluent aphasia, but relatively ok repetition
E. Loss of bowl and bladder control
E
Stage 2 of Alzheimer's Dementia may include the following sxs/findings except

a. acalculia
b. ideomotor apraxia
c. CT/MRI normal or volume loss
d. echolalia or mutism
d. echolalia or mutism is a sxs of the 3rd stage of Alz

-->
3rd stage of Alzheimer's dementia includes
Motor: limbs are rigid and in flexed position
Spincter control is lost
EEG diffusely slow
What causes Creutzfeldt-Jacob's Disease?
A PRION disease (infectious protein; like virus but w/o nucleic acids)
- also described as a "slow virus"
What are the 4 prominent sxs of CJD
Dementia,
Involuntary movements (especially myoclonus)
Seizures
Vision
Name 4 prion dementias/diseases
Creutzfeldt-Jacob's Disease

Bovine Spongioform Encephalopathy

Kuru

Fatal Familial Insomnia
CJD is a rare disease affecting individuals over _____ years old and death is expected w/in ______
Affects individuals over 65 years old and death w/in 6 months.
What do the following have in common:
General Paresis (neurosyphilis),
Lyme Disease, and
African Trypanosomiasis (sleeping sickness)
These 3 conditions (may) include DEMENTIA and are caused by BACTERIA INFECTION
Lyme disease is associated with these three sxs:
meningitis, peripheral neuropathy, cognitive/memory problems.
What is Progressive Multifocal Leukoencephalopathy
Opportunistic infection associated with AIDS affecting the white matter.
What is Leukoencephalitis
Inflammation of the white matter of the brain
Postinfection encephalomyelitis,
Progresive Multifocoal Leukoencephalopathy, and
HIV Dementia are ________ caused by a __________
These conditions are Dementias caused by a VIRAL INFECTION

(postinfection encephalomyelitis refers to dementias caused after an infection with viral diseases such s measles, varicella, rubella etc)
HIV dementia is primarily a ________ dementia
subcortical
Symptoms of HIV dementia:
Cognitive sxs (4)

Behavioral/Social sxs (2)

Psychiatric sxs (2)

Motor sxs (3)
Cognitive sxs: slowness, poor concentration, problem solving, memory complaints

Behavioral/Social sxs: apaty, social withdrawal

Psychiatric sxs: visual hallucinations, delusions (or delirium)

Motor: Tremor, ataxia, imbalance
Symptoms of Dementia Pugilistica (5)
- Forgetfulness
- Unsteady wide-based gait
- Dysarthric speech
- Flat affect
- Psychomotor retardation
Possible psychiatric manifestations of Dementia Pugilistica include (3)
Euphoria, paranoia, depression
Dementia Pugilistica changes in 3 brain structures
-Ventricular Enlargement
- Thinning of CC
- Depigmentation of Substantia Nigra-
In Dementia Pugilistica, the following 2 cellular/intracellular changes are noted:
- Neurofibrillary tangles
- Astrocyte proliferation
Triad sxs of Dementia with Lewy Bodies are:
fluctuating cognition, parkinsonism, and visual hallucinations.
What is the pathology associated with Lewy Body Dementia
intraneuronal Lewy bodies in the substantia nigra (as in Parkinson’s disease) as well as in more widespread cortical and subcortical structures.
Typical age of onset of DLB is
After age 50
Men vs. women for DLB
Slightly more common in men
Genetics in DLB?
Elevated ApoE4 frequency
DLB four regions of cortical degeneration
Frontal, Anterior Cingulate, Insular, and Temporal
EEG in DLB?
EEG is often abnormal with greater temporal lobe slowing and transient slow wave activity than in Alz
What is the Pathology of DLB and how is it different from Alz and Parkinson's Disease?
Cortical neurons contain Lewy Bodies.
- Different from Alz: Neurofibrillary changes or senile plaques are not present
- Different from parkinson's: Lewy Bodies are not confined to substantial nigra
What is the neuropsychological profile of DLB - (4 items)
Poor visual-spatial perceptional and constructional abilities

Poor attention/ working memory

Poor FAS

Memory: more problems with retrieval
What is Progressive Nonfluent Aphasa
A subtype of FTD
with Left Hemisphere Perisylvian atrophy and non fluent aphasia. Comprehension is relatively spared.
What is Semantic Dementia
A subtype of FTD
Impacts temporal neocortex, may be bilateral
- Left hemisphere involvement: more impaired comprehension of words
- Right/Bilateral: visual agnosia (including for faces)
- of FTD most often sporadic, least impact of genetics.
Semantic Dementia on MRI
Anterior, Inferior region of the Temporal Lobes
Semantic Dementia on Neuropsych Exam
* diminished semantic knowledge
* diminished confrontational naming (semantic errors, hippo "dog"
* diminished category fluency
Define Vascular Dementia
Dementia from cerebrovascular disease.
May results from different causes including repeated cerebral infarction (i.e. multi-infarct dementia), a single vascular insult or chronic ischemia w/o discrete infarct.
What is the most reliable way to differentiate vascular dementia from dementia of the Alzheimer's type?
Focal neurological findings and presence of either diffused or focal cognitive findings.
Which statement is false

A. Psychomotor retardation is common in vascular dementia
B. Emotional lability is commonly seen with vascular dementia
C. Stepwise development is a reliable way to distinguish vascular dementia from Alz.
D. Vascular dementia (DSM-IV-TR) requiers at least one of the following: aphasia, apraxia, agnosia, executive dysfunction
C. Stepwise development is not a reliable way to differentiate vascular dementia from Alzheimer's dementia.
What are the common risk factors for vascular dementia (list 5)
Hypertension
Abnormal lipids
Smoking
Diabetes
Obesity
True or False
Vascular dementia onset is generally at the same age as Alzheimer's
False
Vascular dementia onset generally earlier than Altz
True or False

Vascular dementia is more common in men
True
What is Binswanger's disease
A form of subcortical dementia caused by diffused subcortical infarcts
Risk factors for Binswanger's disease?
Chronic hypertension
What areas are most often affected in Binswanger's disease?
Periventricular subcortical demyelination, temporal lobe and occipital lobe. Corpus Callosum is spared.
--->
Binswanger disease is a form of small vessel vascular dementia caused by damage to the white brain matter.[
Pick's Disease affects what brain regions?
Atrophy of frontal and anterior temporal lobes from unknown causes
What are Pick Bodies
Dense intracellular structures, approximately the size of the cell nucleus that cause mild increase in neuron size. Not associated with normal aging or AD.
It is difficult to distinguish Pick's disease from AD prior to autopsy. However, provide 4 points of difference
- Pick's may have Kluver-Bucy sxs (hyperorality)
- Pick may have confabulation
- Pick's visual-spatial better preserved
- Pick's stereotyped behaviors/speech
The following statements about Alzheimer’s disease and Pick’s disease are true, except:
In Pick's disease amyloid plaques and neurofibrillary tangles ____________
Are not present
What are the illness' feature in addition to dementia or delirium? CNS LUPUS

a. akinesia, tremor and postural reflex abnormalities
b. slow mentation and depression, but sometimes excited confusion ("madness")
c. seizures, strokes, and psychosis (the three S's)
d. lethargy and asterixis (tremor of wrist)
CNS LUPUS
In addition to dementia/delirium
c. seizures, strokes, and psychosis (the three S's)
What are the illness' feature in addition to dementia or delirium? Normal Pressure Hydrocephalus

a. Akinesia, tremor, and postural reflex abnormalities
b. Incontinence and gait apraxia
c. Nonspecific mental dullness and peripheral neuropathy
d. Slow mentation and depression, but sometimes excited confusion ("madness")
Normal-pressure Hydrocephalus
In addition to dementia or delirium:
b. Incontinence and gait apraxia
What are the illness' feature in addition to dementia or delirium? DIFFUSE LEWY BODY DISEASE

a. Akinesia, tremor, and postural reflex abnormalities
b. Incontinence and gait apraxia
c. Nonspecific mental dullness and peripheral neuropathy
d. Slow mentation and depression, but sometimes excited confusion ("madness")
DIFFUSE LEWY BODY DISEASE
In addition to dementia/ delirium

a. Akinesia, tremor, and postural reflex abnormalities
What are the illness' feature in addition to dementia or delirium? WILSON'S DISEASE

a. Akinesia, tremor, and postural reflex abnormalities
b. Incontinence and gait apraxia
c. Tremor, rigidity, and Kayser-Fleischer rings
d. Slow mentation and depression, but sometimes excited confusion ("madness")
Wilson's Disease -

c. Tremor, rigidity, and Kayser-Fleischer rings
What are the illness' feature in addition to dementia or delirium? HUNTINGTON'S DISEASE

a. Akinesia, tremor and postural reflex abnormalities
b. Amnesia with nystagmus, ocular paresis, and ataxia
c. Chorea but, in young adults, rigidity
d. Lethargy and asterixis (wrist tremor)
Huntington's Disease

c. Chorea but, in young adults, rigidity
What are the illness' feature in addition to dementia or delirium? ARSENIC POISONING


a. Akinesia, tremor, and postural reflex abnormalities
b. Incontinence and gait apraxia
c. Nonspecific mental dullness and peripheral neuropathy
d. Slow mentation and depression, but sometimes excited confusion ("madness")
Arsenic Poisoning:

c. Nonspecific mental dullness and peripheral neuropathy
What are the illness' feature in addition to dementia or delirium? Wernicke's Encephalopathy


a. Akinesia, tremor, and postural reflex abnormalities
b. Amnesia with nystagmus, ocular paresis, and ataxia
c. Nonspecific mental dullness and peripheral neuropathy
d. Slow mentation and depression, but sometimes excited confusion ("madness")
Wernicke's encephalopathy
b. Amnesia with nystagmus, ocular paresis, and ataxia
What are the illness' feature in addition to dementia or delirium? TUBEROUS SCLEROSIS

a. Akinesia, tremor, and postural reflex abnormalities
b. Amnesia with nystagmus, ocular paresis, and ataxia
c. Nonspecific mental dullness and peripheral neuropathy
d. Adenoma of face and seizures, usually beginning in childhood
TUBEROUS SCLEROSIS:

d. Adenoma of face and seizures, usually beginning in childhood
What are the illness' feature in addition to dementia or delirium? HEPATIC ENCEPHALOPATHY


a. Akinesia, tremor and postural reflex abnormalities
b. Amnesia with nystagmus, ocular paresis, and ataxia
c. Chorea but, in young adults, rigidity
d. Lethargy and asterixis (wrist tremor)
Hepatic encephalopathy

d. Lethargy and asterixis (wrist tremor)
What are the illness' feature in addition to dementia or delirium? Crutzfeldt-Jakob disease

a. Akinesia, tremor, and postural reflex abnormalities
b. Amnesia with nystagmus, ocular paresis, and ataxia
c. Myoclonus with pyramidal or extrapyramidal findings in individuals older than 65 years
d. Nonspecific mental dullness and peripheral neuropathy
Crutzfeldt-Jakib disease
c. Myoclonus with pyramidal or extrapyramidal findings in individuals older than 65 years
Which test/s is/are used to diagnose the illness? Subarachnoid Hemorrhage

a. CSF analysis
b. CT or MRI
c. EEG
d. brain biopsy
A&B
Which test is/are used to diagnose the illness? Creutzfeldt-Jakib disease


a. CSF analysis
b. CT or MRI
c. EEG
d. brain biopsy
C&D
What is the pattern of inheritance (a-d) for the following illness? Wilson's Disease

a. sex-linked recessive
b. autosomal recessive
c. autosomal dominant
d. none of the above
b. Autosomal Recessive
What is the pattern of inheritance (a-d) for the following illness? Huntington Disease

a. sex-linked recessive
b. autosomal recessive
c. autosomal dominant
d. none of the above
c. Autosomal dominant
What is the pattern of inheritance (a-d) for the following illness? Familial Creutzfeldt-Jakob

a. sex-linked recessive
b. autosomal recessive
c. autosomal dominant
d. none of the above
c. Autosomal Dominant
What is the pattern of inheritance (a-d) for the following illness? Familial Alzheimer's Disease

a. sex-linked recessive
b. autosomal recessive
c. autosomal dominant
d. none of the above
c. autosomal dominant