Dementia Flashcards
Three memory systems:
-short term –> long term –> sensory memory
How long does information stay in short term memory? long term?
- 15-20 seconds in short term
- days to lifetime in long term
Common dementia symptoms in newly diagnosed MCI patients:
- impaired orientation
- poor short term memory
Psych symptoms assc with newly diagnosed dementia:
- anxiety
- depression
- paranoia
List the 8 primary neurodegenerative disorders:
1) AD
2) FTD
3) PD
4) DLB
5) PSP
6) HD
7) WD
8) CJD
List the three vascular dementias
1) multi infarct
2) Binswangers
3) CADASIL
Three specific infectious causes of dementia
1) syphilis
2) Lyme
3) HIV
1 Form of Dementia
AD
What MMSE score is diagnostic of mild moderate and severe A. dementia?
20-24, 11-19, less than 10
Symptoms of severe AD
apraxia
mutism
no recall
Criteria for diagnosing AD:
- GRADUAL memory impairment affecting social/occupational functioning
- Cognitive disturbance (aphasia, apraxia, etc)
Three areas of brain + 1 NT involved in pathogenesis of AD:
- hippocampus, cerebral cortex, amygdala
- Ach
Specific cholinergic changes in the brain during AD:
1) reduced choline acetyltransferase activity
2) decreased neurons in forebrain
3) loss of nicotinic receptors in hippocampus/cortex
What do presynaptic nicotinic receptors modulate?
Release of Ach, glutamate, serotonin, NE
Gross changes in AD:
cerebral atrophy
hydrocephalus ex vacuo
Senile plaques:
- location
- makeup
- stain
- cortex
- fragmented neurites, astrocytes, microglia
- silver stain
NF Tangles
- location
- makeup
- stain
- pyramidal cells of hippocampus + cortex
- intracytoplasmic twisted filaments
- H/E
Earliest AD lesion
physiologic lesion of the hippocampus
Drug treatments for AD:
- AchEi
- antipsychotics/ depressants
- estrogen replacement
What is the generic name for:
- Aricept
- Exelon
- Aricept: Donepezil
- Exelon: Rivastigmine
Benefits to use of Aricept/Donepezil:
-Long half life, high bioavailability, not affected by food
Benefits to use of Exelon/ Rivastigment?
-No hepatic metabolism/ CYP involvement
Potential preventative treatments for AD:
- red wine
- Statins
- NSAIDs
- exercise, smoking cessation
NPH Triad
1) Dementia
2) Gait Apraxia
3) Urinary Incontinence
Therapy for NPH:
1) spinal taps
2) shunts
Picks Disease:
Prominent early impairments
- language (echolalia, etc)
- behavioral changes
What is preserved in Picks Disease?
- praxia, visuospatial
- memory
Population most commonly effected by Picks:
Females over age 60
Areas of atrophy in Picks:
1) frontal
2) ANTERIOR temporal lobe
Pick Bodies Appearance + Other Path assc with Picks
- intracytoplasmic inclusions (spherical)
- ballooned neurons
Locations of pick bodies:
- cortex
- basal ganglia
- brainstem
Criteria for diagnosis with Dementia + Lewy Body
Dementia + One Hallmark
What are the core features of DLB?
1) fluctuations in cognition/alertness
2) hallucinations
3) parkinsonism
Exclusionary characteristics for DLB
strokes
other brain disorders
STEELE-RICHARDSON-OLSZEWSKI SYNDROME is aka?
Progressive Supranuclear Palsy
Who gets PSP?
Males over age forty
In addition to gaze deficits, list 4 features of PSP:
1) Parkinsonism
2) Pseudobulbar Palsy
(dysphagia)
3) Subcortical Dementia (executive)
4) Language Impairment
What is seen on imaging of PSP
atrophy of pons + midbrain
sparing of cortex + cerebellum
Pathology assc with PSP
NF tangles in midbrain
Describe Supranuclear Palsy:
Downgaze –> Upgaze Palsy
Vertical –> Horizontal Involvement
Apraxia of Eyelid Opening
Cortical Basal Ganglionic Degeneration:
Onset
M+F over age 70; unilateral onset
Survival Rate CBGD
5-10 years
Clinical Features of CBGD
1) extrapyramidal rigidity
2) tremor
3) apraxia
4) alien hand syndrome
5) subcortical dementia
6) SN gaze palsy
7) CS tract involvement
CBGD MRI findings
assymetric parietal lobe atrophy
CBGD PET findings
decreased metabolism: thalamoparietal and medial frontal lobes
CBGD Pathology:
-atrophy, gliosis in parietal + medial frontal lobes CL to involved limb
-degeneration of substantia nigra
(no bodies, plaques, tangles)
Genetic Changes assc with HD
AD
4
CAG repeat
HD symptomatic triad
1) Personality changes
2) Dementia
3) Psychosis
Pathophys cause of HD
degeneration of GABA/cholinergic neurons in the striatum
HD MRI findings
boxcar ventricles (caudate and cortical atrophy)
HD PET findings
hypometabolism in caudate and putamen
Treatments (3) for HD
Haldol
Tetrabenazine
Klonopin
Genetic Change assc with Wilsons:
AR chromosome 13 =defective Cu binding ATPase = low biliary excretion of Cu
Symptoms assc with WD
1) dementia, psychosis
2) dysphagia, dysarthria
3) rigidity, tremor
Labs assc with WD
1) High Ciu
2) Low serum ceruloplasmin
3) High LFTs
Pathologic findings assc with WD
1) opalski cells (large astrocytes)
2) degeneration, cavitation in putamen
3) red, atrophic BG
Treatment WD
Penicillamine + Pyridoxine
Zinc acetate
Liver transplant
Five important infectious causes of dementia
1) HIV
2) SSPE
3) PML
4) Neurosyphillis
5) Prion Disease
Diagnostic Criteria for AIDs Dementia
2 abnormalities for 1 month
Virus causing PML
Papova (JC, SV40)
Patients Predisposed to PML
1) Tysabri
2) AIDs
3) Hodgkins
4) CLL
Triad of PML
1) Vision Loss
2) Dementia
3) Hemeparesis
Pathology of PML
1) occipital demyelination
2) oligodendrocytes contain eos intranuclear inclusions
Treatment PML
1) Vistide
2) Cytarabine
3) HAART
LAb findings assc with CJD
EEG changes
14-3-3 protein in CSF
CJD findings on MRI
pulvinar sign (bilateral high signal in pulvinar nucleus of thalamus)
Binswanger Dementia Criteria:
1) Dementia +
2) (2/3)…systemic vascular disease, cerebral vascular disease, subcortical brain dysfunction
3) Bilateral leukoaraiosis
*Must not be multiple lesions or severe dementia
CADASIL Genetic Mutation
1) AD
2) 19
3) NOTCH 3
CADASIL clinical features
1) strokes
2) dementia
3) migraine
4) depression or mania
Onset Cadasil
20-40, death within 30 years
Which of the dementia syndromes show mainly subcortical dementia?
-PSP
-CBGD
-WD
(seen also in DLB but not predominate)
