Dementia

Question 1

Genetic mutations for Alzheimer:

Answer 1

Autosomal Dominant:
PS1, PS2, APP - chromosome 21, APOE epsilon4.
1st degree relative with AD –> 2X risk

Question 2

Genetic mutations for Frontotemporal Dementia:

Answer 2

Autosomal Dominant:
GRN
C9orf72

Question 3

Risk factors:

Answer 3

Smoking, Dyslipidemia, HTN, DM, Female

Question 4

Apraxia:

Answer 4

inability to execute motor tasks despite having the strength/control to achieve it.

Question 5

Snout:

Answer 5

Frontal Release Sign:

touching lips causes them to purse

Question 6

Suck:

Answer 6

Frontal Release Sign:

Suck anything placed in mouth

Question 7

Rooting:

Answer 7

Frontal Release Sign:

Touching cheek causes face to turn in that direction

Question 8

Grasp:

Answer 8

Frontal Release Sign:

hand will grab an object in palm

Question 9

Myerson Sign

Answer 9

Frontal Release Sign:

Repeatedly tapping the glabella causes blinking with each tap

Question 10

Palmomental

Answer 10

Frontal Release Sign:

Stroking the palm causes chin to twitch

Question 11

Amyloid Plaques

Answer 11

In Alzheimers

Extracellular, large collection of amyloid beta

Question 12

Neurofibrillary Tau Tangles

Answer 12

In Alzheimers.
Intracellar.
Misfolded and phosphorylated collection of microtubule-associated protein (tau). Flame-shaped collection in neurons.

Question 13

Notable neuroimaging atrophy in Alzheimers is:

Answer 13

Bilateral atrophy of Mesial Temporal Lobes and Parietal Lobes.
EEG may be show mild dysfunc.

Question 14

Pick Disease? Anatomic location? Variations?

Answer 14

= Frontotemporal Dementia (FTD)

Atrophy of frontal and temporal lobes.

Question 15

Behavioral varient of FTD:

Answer 15

Behavioral varient - most common: disinhibited, change in diet, loss of motivation/empathy, compulsive. Memory and visuospatial spared.
Avg. onset 50yo.
More rapid than Alzheimers - death after 5yrs instead of 10.

Question 16

Primary Progressive Dysphasia of FTD:

Answer 16

Isolated language deficiets with absence of structural lesions to explain.
Atrophy in dominant hemisphere.
Aphasia can be like: Broca’s, Anomic, or semantic processing.

Question 17

Lewy Body Dementia:

Answer 17

Lewy Bodies = alpha-synuclein containing inclusions, distributed throughout cortex.
Visuospacial - visual hallucinations and executive dysfunction, fluctuations in attentiveness, Parkinsonism
Variable time course.

Question 18

Vascular dementia

Answer 18

Ischemic small vessel disease is the most common.

Question 19

Normal Pressure Hydrocephalus:

Answer 19

Wet: urinary incontinance
Wobbly: magnetic gait - small steps with difficulty getting feet off group.
Wacky: dementia
Treat with shunt, LP first.

Question 20

Prion disease (Creutzfeldt-Jakob):

Answer 20

Rapidly progressive - weeks to months (5month survival). (there is a genetic form).
Endogenous protein PrP undergoes conformational change, altered protein can change tertiary structure of other PrP molecultes and altered proteins aggregate.

Question 21

Treatment of Alzheimer and Lewy Body:

Answer 21

Cholinesterase Inhibitors for Symptoms.

Alzheimer: Memantine (NMDA receptor Antagonist.

Question 22

Transient global amnesia

Answer 22

self-limited episode of memory loss (~24hrs). identity and remote bio info intact. stress related

Question 23

aphasia

Answer 23

language deficits. Occur with dominant hemisphere involvement - usually left. More severe and common with cortical involvement. Deep and thalamic can cause some.

Question 24

Broca Aphasia

Answer 24

Left inferior lateral frontal lobe.
Low fluency.
Impaired repetition.
Intact comprehension.

Question 25

Wernicke Aphasia

Answer 25

Temporoparietal Junction.
Impaired comprehension.
Impaired Repetition.
Fluent speech.

Question 26

Sylvian Fissure Aphasia

Answer 26

Repetition Problems

Question 27

Neglect - lesion of ___ hemisphere.

Answer 27

Non-dominant

Question 28

Lack of insight of deficit =

Answer 28

Anosognosia. (non-dominant, usually right lesion)

Question 29

Lack of recognition of side of body (usually can’t recognize left side):

Answer 29

Asomatognosia (non-dominant, right lesion)

Question 30

Self-Centered Hemisensory hemispatial neglect

Answer 30

Left side of the world gone

Question 31

Object centered hemisensory hemispatial neglect

Answer 31

left side of an object is gone

Question 32

Problems coordinating eye movement

Answer 32

Ocular apraxia

Question 33

Coordinating body movements visually

Answer 33

Optic Ataxia

Question 34

Balint Syndrome

Answer 34

Triad:
Ocular apraxia
Optic Ataxia
Simultanagnosia

Question 35

Simultanagnosia

Answer 35

Deficits with seeing the whole for the parts

Question 36

Temporal pathway of vision:

Answer 36

Ventral stream - “What” processes info about what an object is. Probs- simultanagnosia.

Question 37

Parietal pathway of vision:

Answer 37

“where” and object is in space. How to interact with it.

Question 38

Vision first enters primary visual cortex in ___ via ___. Then processing occurs in ___.

Answer 38

Enters occipital lobe via V1. Processing in visual association cortices.