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Dementia Flashcards

(105 cards)

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1
Q

What are the three memory systems?

A
  1. Sensory Memory
  2. Short-term Memory
  3. Long-term Memory
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2
Q

Function of sensory memory

A

Receives information through the five senses and passes that information onto short term memory

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3
Q

Function of short term memory

A

Receives information from the sensory memory

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4
Q

How long does information stay in short term memory?

A

15-20 seconds

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5
Q

Function of long term memory

A

Recalls information gathered over time and is used to process information

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6
Q

How long does information stay in long term memory?

A

A few days —> Life

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7
Q

What are examples of dementia symptoms in newly diagnosed mild cognitive impairment patients?

A
  1. Impaired orientation to time or place
  2. Impaired recent memory
  3. Repeatedly asking questions
  4. Misplacing items repeatedly, getting lost
  5. Forgetting names
  6. Problems with conversation
  7. Impaired judgment
  8. Loss of interest in hobbies
  9. Decreased ability to perform activities of daily living
  10. Altered mood/behavior
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8
Q

What are the vascular causes of dementia?

A
  1. Multiple Infarcts
  2. Binswanger’s
  3. CADASIL
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9
Q

What is Binswanger’s Disease

A

AKA subcortical leukoencephalopathy; this is a small vessel vascular dementia caused by damage to the white brain matter. Risk factors are HTN and old age

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10
Q

What is CADASIL?

A

Cerebral Autosomal-Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) is a hereditary stroke disorder

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11
Q

What are the inflammatory causes of dementia?

A

MS and Vasculitis

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12
Q

What are infectious causes of dementia?

A

Syphilis, Lyme, HIV and other viruses, fungal diseases

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13
Q

What are physical causes of dementia?

A

Hydrocephalus, trauma

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14
Q

How many Americans are affected by Alzheimer’s?

A

4 Million

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15
Q

What percentage of patients over 85 have symptoms of Alzheimers?

A

50%

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16
Q

Ratio of people over 65 affected by Alzheimers

A

1/10

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17
Q

What are risk factors for Alzheimers?

A

Advanced age, family Hx

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18
Q

Describe the timeline progression of Alzheimers symptoms and death

A

Diagnosis - Loss of functional independence (2yrs) - Behavioral problems (3yrs) - Nursing home placement (3yrs) - Death (4-9yrs)

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19
Q

Diagnostic criteria for Alzheimers

A
  1. Memory Impairments of gradual and progressive onset
  2. Impairment in social or occupational functioning
  3. At least 1 of aphasia, apraxia, agnosia or disturbances in executive functioning
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20
Q

Neurochemical changes associated with Alzheimers

A

Decreased synthesis of Acetylcholine

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21
Q

Regions of the brain where Acetylcholine is important for memory formation

A
  1. Hippocampus
  2. Cerebral Cortex
  3. Amygdala
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22
Q

Describe the role of Nicotinic receptors in Alzheimers

A

Stimulating Nicotinic receptors improves memory

Presynaptic nicotinic receptors modulate the release of multiple neurotransmitters important for memory and mood (ACh, Glutamate, Serotonin and Noradrenaline)

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23
Q

Gross brain appearance in advanced Alzheimers

A

Cortical atrophy

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24
Q

Characteristic finding in a coronal section of the brain in a patient with Alzheimers

A

Hydrocephalus ex vacuo (ventricular dilation due to loss of cortex)

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25
What are senile plaques?
Regions of fragmented neuritis (neuronal processes) within the gray matter of the cerebral cortex
26
What cells can be involved in senile plaques?
Neurons, Astrocytes, Microglia
27
What stain is used to evaluate senile plaques?
Bielschowsky silver stain
28
What is a neurofibrillary tangle?
Neurons with intracytoplasmic proliferation of twisted filaments which appear as tangles
29
Where are neurofibrillary tangles typically found?
In the pyramidal cells of the Hippocampus and Cerebral Cortex
30
fMRI in an Alzheimers patient may show
Decreased activity in the Hippocampus
31
What is the earliest deficit in Alzheimers
Physiologic lesion in the hippocampus
32
Progression of deficits in Alzheimers
1. Memory/Language 2. Memory/Language + Visuospatial 3. Memory/Language + Visuospatial + Abstract Reasoning
33
What is the standard treatment for AD?
Cholinesterase Inhibitors
34
What drugs may be used to treat the behavioral symptoms of AD?
Antipsychotics and Antidepressants
35
What are the four AChEIs used in AD?
1. Tacrine 2. Donepezil (Aricept) 3. Rivastigmine (Exelon) 4. Galantamine (Reminyl)
36
Which AChEI's absorption are affected by food?
Tacrine (decreased) and Rivastigmine (increased b/c absorption slowed)
37
Which AChEI is available as a patch to give a more steady dose with fewer side effects?
Rivastigmine
38
Which AChEI is not metabolized in the liver?
Rivastigmine
39
What are some additional treatments for AD other than AChEIs?
1. Nemenda (Memantine)- antagonizes NMDA receptors 2. Vitamin E- Antioxidant 3. NSAIDs and HMG Co-A reductase inhibitors, red wine 4. Smoking cessation and exercise
40
Gait apraxia (magnetic gait), dementia and urinary incontinence suggests
Normal Pressue Hydrocephalus (Wet, wacky and wobbly)
41
How is the Dx of NPH made?
Clinical + imaging and diagnostic spinal tap
42
Rx for NPH?
Large volume spinal taps + shunts
43
What are the types of frontotemporal dementias?
1. Pick's Disease 2. Dementia lacking distinctive histopathological features (DLDH) 3. Progressive subcortical gliosis (PSG) 4. Primary progressive aphasia 5. ALS dementia or MND dementia 6. Disinhibition dementia- Parkinsonism-amyotrophy (DDPAC)
44
Criteria for the Dx of frontotemporal dementia
1. Early language impairment (echolalia, stereotypy and reduction of speech) 2. Early behavioral deficits 3. Early affective symptoms (depression, anxiety, somatization, spontaneity and indifference) 4. Preservation of praxis and visuospatial skills 5. Intact memory
45
What is the typical age of onset for Pick's Disease?
>60
46
Gender association with Pick's Disease
Typically female
47
Symptoms associated with Pick's Disease
1. Apathy, abulia and frontal release signs 2. Aphasia (if temporal lobe involvement) 3. Dementia sparing praxis and visuospatial fxn
48
Imaging associated with Pick's disease
Atrophy of frontal and temporal lobes with sparing of the posterior 1/3rd of Superior Temporal Gyrus
49
What are Pick's Bodies?
Pick's Bodies are argyrophilic, intracytoplasmic spherical inclusions in neurons of the cerebral cortex, basal ganglia and brainstem (usually seen w/ H&E or Silver Stain)
50
What are the 3 core features of Dementia with Lewy-Body?
1. Fluctuations in cognition (attention and alertness) 2. Visual hallucinations (well-formed and detailed) 3. Parkinsonism
51
What are supportive symptoms of dementia with Lewy Body?
Falls, syncope, LOC, neuroleptic sensitivity, delusions and hallucinations
52
What must be rules out before a dx of dementia with Lewy Body?
Strokes
53
Medication that may need to be avoided in patients with Lewy Body dementia
Antipsychotics
54
What will be the pathologic findings in a patient with Lewy Body dementia?
A pale substantia nigra and Lewy Bodies (eosinophilic, cytoplasmic inclusions)
55
What is the typical age on onset for PSP?
>40
56
Which gender is more commonly affected by PSP?
Male
57
What are the symptoms of PSP?
1. Supranuclear gaze deficits 2. Parkinsonism (bradykinesia and rigidity- lack of tremor) 3. Frequent falls 4. Pseudobulbar palsy, dysphagia and dysarthria 5. Subcortical dementia 6. Language impairment (echolalia and palilalia)
58
What will be seen on imagining in PSP?
Atrophy of the pons and midbrain
59
What is the pathology of PSP?
Neurofibrillary tangles in the midbrain neurons + atrophy of the pons and midbrain *Cortex and cerebellum are typically spared
60
What are the deficits associated with Supranuclear Palsy?
1. Downward gaze + upward palsy 2. Vertical then horizontal involvement 3. Apraxia of eyelid opening
61
What is the typical age of onset for Cortical-Basal Ganglionic Degeneration?
>70
62
What is the typical survival for a patient with CBGD?
5-10yrs
63
What are the symptoms of CBGD?
1. Asymmetrical onset 2. Extrapyramidal ridgidity (akinetic) + action/postural tremor + apraxia and "alien hand syndrome" 3. Subcortical dementia 4. Supranuclear gaze palsy 5. Involvement of corticospinal tract
64
What imaging is associated with CBGD?
MRI- asymmetric parietal lobe atrophy (CL to limb involvement) PET- decreased metabolism in thalamoparietal and medial frontal areas
65
What are the pathological findings associated with CBGD?
1. Cortical atrophy, neuronal loss and gliosis of the parietal and medial frontal lobes 2. Degeneration of the substantial nigra
66
What are the genetic findings associated with Huntington's Disease?
AD, Chromosome 4, >40 CAG repeats (>60 = childhood onset)
67
When is the typical onset of symptoms in a patient with Huntington's Disease?
35-40
68
Which gender is more commonly affected by Huntington's Disease?
Male
69
What is Huntington's Triad?
1. Personality Changes 2. Dementia 3. Psychosis
70
Describe the pathophysiology of Huntington's Disease
Degeneration of GABA and cholinergic neurons in the Striatum
71
What are the imaging findings seen in Huntington's Disease?
1. MRI/CT- large "boxcar" shaped venticles w/ caudate + cortical atrophy 2. PET- hypometabolism in the caudate and putamen 3. Atrophy of the caudate + putamen + cortex (layer 3)
72
Which region of the brain is replaced by gliosis in Huntington's Disease?
Striatum
73
What is the treatment for Huntington's Disease?
1. Haldol 2. Tetrabenzine 3. Klonopin
74
What are the genetics associated with Wilson's Disease?
AR, Chromosome 13
75
What is the typical age of onset of Wilson's Disease?
11-25
76
What are the neurologic symptoms associated with Wilson's Disease?
1. Subcortical dementia 2. Psychosis 3. Dysphagia, Dysarthria, Rigidity and Tremor
77
What are the non-neurological symptoms associated with Wilson's Disease?
1. Liver: Hepatitis/Cirrhosis | 2. Eyes: KF Rings (deposition of Copper in Descemet's Membrane of the Cornea)
78
What are the lab findings associated with Wilson's Disease?
Increased Copper, Decreased Ceruloplasmin
79
Describe the pathophysiology of Wilson's Disease
Defect in Cu-Binding P type ATPase + decreased biliary excretion of Copper
80
What are the imaging findings associated with Wilson's Disease?
CT- Atrophy and hypodensity of the basal ganglia
81
What are the pathological findings of Wilson's Disease?
1. Opalski Cells (large protoplasmic astrocytes) 2. Spongy degeneration and cavitation in putamen 3. Brick red discoloration and atrophy of BG
82
What is the treatment of Wilson's Disease?
1. D-Penicillamine and Pyridoxine (or Zinc Acetate) | 2. Liver Transplant
83
What disease is associated with atrophy of the Lenticular Nuclei?
Wilson's Disease
84
What disease is associated with Risus Sardonicus?
Wilson's Disease
85
Which virus causes progressive multifocal leukoencephalopathy?
JC Virus and SV-40
86
Which patients are more likely to develop PML?
AIDS patients, Hodgkin's and CLL patients and patients on Tysabri
87
What are the symptoms of PML?
1. Dementia 2. Visual Loss 3. Progressive Hemiparesis
88
What is the pathology of PML?
Occipital demyelination and the formation of eosinophillic intranuclear inclusion bodies in oligodendrocytes
89
What is the Tx for PML?
Vistide + Cytarabine + HAART
90
What is the triad for CJD?
Dementia + Ataxia + Myoclonus
91
What are the symptoms of Kuru?
Ataxia + Dementia
92
What are the symptoms of FFI?
Insomnia + Dysautonomia + Ataxia + Dementia
93
Which prion disease affects the thalamic and olivary nuclei via gliosis?
FFI
94
What is the EEG pattern seen with CJD?
Generalized periodic sharp wave complexes
95
What is the CSF finding of CJD?
14-3-3 protein
96
What is the MRI finding associated with Sporadic CJD?
Bilateral anterior basal ganglia high signal on Transverse FLAIR MRI
97
What is the MRI finding associated with New Variant CJD?
Bilateral High Signal of the pulvinar nuclei ("Pulvinar Sign") on Transverse FLAIR MRI
98
Disease associated with spongiform change in the brain
CJD
99
Where would you sample to test for Amyloid Angiopathy?
Rectal fat pad
100
What is the diagnostic criteria for Binswanger's Dementia?
Dementia + at lease 2 of 1. HTN or known vascular disease 2. Evidence of CVD (stroke ect) 3. Subcortical brain dysfunction (abnormal gait, muscular rigidity, neurogenic bladder)
101
What is the imaging associated with Binswanger's Dementia?
Bilateral subcortical leukoaraiosis (attenuation of white matter) on CT or MRI
102
What are the genetics associated with CADASIL?
AD, Chromosome 19, Notch 3 Gene
103
What is the typical age of onset for CADASIL?
20-40
104
What are the clinical symptoms of CADASIL?
1. Recurrent Strokes 2. Dementia 3. Migraines + Aura 4. Depression/mania
105
What are the imaging findings associated with CADASIL?
CT/MRI- Hyperintense lesions on T2 in the subcortical white matter + basal ganglia

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