Chapter 12: Dementia

Question 1

What faculties of the mind maybe impaired in Dementia?

Answer 1

Memory, orientation, visuospatial perception, language, and higher executive functions (planning, organizing, and sequencing)

Question 2

First thing to go in dementia

Answer 2

Short-term memory

Question 3

Do you use EEG in diagnosing Dementia

Answer 3

Not ordered routinely, do it if there is evidence of fluctuations in cognitive status that could represent seizures.

Question 4

Pathology of Alzheimer’s

Answer 4

Neurofibrillary tangles and senile plaques in the cerebral neocortex and hippocampus.
Brain atrophy: loss of neurons in cortex and gliosis

Question 5

Risk factors for AD

Answer 5

Age, female sex, family history, history of severe head trauma, Down’s

Question 6

Genetic risk factors for AD

Answer 6

ApoE4, amyloid precursor protein (APP), presnilin 1 and 2.

Question 7

Natural history of AD

Answer 7

Starts off with small things like getting lost, forgetting where you placed your keys, and social withdrawal…worsens to the loss of the ability to speak, understand, think , and make decisions.

Personality changes, aggressive behavior, paranoid thoughts and delusions.

Death in 5-10 years.

Question 8

Lab test for AD

Answer 8

There is none, only brain biopsy (autopsy)

Question 9

MRI studies of AD

Answer 9

Reduced hippocampus, amygdala, and thalamus.

Question 10

What are Neurofibrillary tangles

Answer 10

Intracellular accumulation of phosphorylated tau protein

Question 11

What are Senile Plaques

Answer 11

Extracellular deposits of amyloid surrounded by dystrophic axons

Question 12

Treatment

Answer 12

Treat/Prevent associated symptoms
Prevent/delay progression: ACEIs like donepezil or rivastigmine, Memantine (NMDAR ant.)
Prophylaxis: None, maybe Vit. E, NSAIDs, estrogen

Question 13

AD makes up what percent of all dementias

Answer 13

50-70% of all dementias

Question 14

Vascular dementia types

Answer 14

1. Macrovascular: large infarcts
2. Microvascular: subcortical ischemia associated with cerebral small vessel disease (lacunes or deep white matter changes on MRI).

Question 15

Vascular dementia risk factors

Answer 15

HTN, DM, age, embolism sources, extensive large artery atherosclerosis.

Question 16

Diagnosis criteria for vascular dementia

Answer 16

Dementia + 2 or more of the following: focal neurologic signs on physical exam; onset that was abrupt, stepwise, or stroke-related; or brain imaging study showing multiple strokes, lacunes, or extensive deep white matter changes.

Question 17

Who is at high risk for vascular dementia

Answer 17

HTN and Diabetics

Question 18

Vascular dementia treatment

Answer 18

Same as for preventing strokeq

Question 19

Binswanger disease

Answer 19

Associated with vascular dementia. Subcortical leukoencophalopathy.

Question 20

Dementia with Lewy Bodies prevalance

Answer 20

Now thought to be the 2nd most common cause of dementia after AD (by many)

Question 21

Dementia with Lewy Bodies clinical picture

Answer 21

Parkinsonian dementia syndrome with visual hallucinations

Question 22

Dementia with Lewy Bodies major features

Answer 22

Cognitive impairment (severe problems of visuospatial perception and visual memory), marked fluctuations of alertness, prominent visual hallucinations (up to 80%) and delusions, extrapyramidal symptoms, and extraordinary sensitivity to neuroleptics

Question 23

Lewy body is..

Answer 23

An eosinophilic intracellular inclusion of alpha synuclein.

Question 24

Dementia with Lewy Bodies: where are the Lewy Bodies

Answer 24

Found in cortical neurons, not in substantia nigra like in PD

Question 25

Dementia with Lewy Bodies pathology

Answer 25

Lewy Bodies, may see NFTs and senile plaques

Question 26

Dementia with Lewy Bodies tx

Answer 26

Low dose atypicals like Risperdal and Clozapine to treat behavior symptoms

Question 27

Dementia with Lewy Bodies prognosis

Answer 27

Death after 10-15 years

Question 28

Progressive Supranuclear Palsy

Answer 28

Subcortical dementia with prominent extrapyramidal features.

Question 29

Progressive Supranuclear Palsy major clinical findings

Answer 29

Palsy of vertical gaze, abnormal gait, frequent falls

Question 30

Progressive Supranuclear Palsy prognosis

Answer 30

Death in 6-10 years

Question 31

Progressive Supranuclear Palsy all the clinical findings

Answer 31

Supranuclear ocular palsy (failure of vertical gaze) Dysarthria; dysphagia; extrapyramidal rigidity, gait ataxia, and dementia. Falls and gait abnormalities early on, dementia early or late.

Question 32

Progressive Supranuclear Palsy Pathology

Answer 32

Atrophy of dorsal midbrain, globus pallidus, and subthalamic nucleus.

Question 33

Huntington Disease

Answer 33

Triad: Chorea, dementia, and personality and behavioral changes (OCD)

Question 34

Huntington Disease prognosis

Answer 34

Death in 10-20 years

Question 35

Huntington Disease and suicide

Answer 35

Suicide is not rare in at-risk and early onset HD patients

Question 36

Huntington Disease natural history

Answer 36

Ages 35-45

Question 37

Huntington Disease pathology

Answer 37

Caudate atrophy and putamen and loss of neurons in cerebral cortex

Question 38

Huntington Disease cause

Answer 38

Aut. dom. CAG repeats on Chr. 4 (>40 repeats)

Question 39

Huntington Disease tx

Answer 39

DA antagonists (neuroleptics). Get genetic counseling for relatives.

Question 40

Parkinson Disease dementia

Answer 40

Subcortical dementia in 30% of PD patients

Question 41

Frontotemporal Dementia

Answer 41

Anterior lobal atrophy. Patients present with cognitive and behavioral deficits other than memory loss first.

Question 42

Pick disease, primary progressive aphasia, and semantic dementia

Answer 42

All different kinds of FTD

Question 43

Pick Disease

Answer 43

Personality change, speech disturbance, inattentiveness, and sometime extrapyramidal signs.

Question 44

What are pick Bodies

Answer 44

Argyrophilic round intraneuronal incusions

Question 45

Pick dz pathology

Answer 45

Pick bodies, abnormal tau in neurons and glial cells. No senile plaques.

Question 46

Prion-related diseases

Answer 46

Cruetzfeldt-Jakob, Fatal familial insomnia, Gerstmann-Straussler-Scheinker syndrome

Question 47

Prior disease pathology

Answer 47

Spongy degeneration, neuronal loss, gliosis, and astrocytic proliferation

Question 48

CJD signs

Answer 48

Rapidly progressive dementia with pyramidal signs, myoclonus, cerebellar or extrapyramidal signs, and periodic sharp waves on EEG

Question 49

CJD on MRI

Answer 49

DWI shows evolving cortical and basal ganglionic abnormalities.

Question 50

CJD lab test

Answer 50

CSF protein 14-3-3 presence is sensitive and specific for CJD

Question 51

CJD prognosis

Answer 51

Death within a year

Question 52

HIV-associated Dementia Complex

Answer 52

Memory problems, problems concentrating, poor attention

Question 53

HIV-associated Dementia Complex MRI

Answer 53

MRI shows cortical and subcortical atrophy

Question 54

HIV-associated Dementia Complex tx

Answer 54

AZT, selegiline, memantine

Question 55

Metabolic causes of dementia

Answer 55

Vitamin B12 may cause a progressive dementing illness.

Question 56

Vitamin B12 deficiency

Answer 56

Will see spinal cord signs (subacute combined degeneration), neuropathy (paresthesias in the hands and feet, sensory ataxia, visual loss, orthostasis) and memory loss. Anemia, sore tongue

Question 57

Vitamin B12 deficiency tx

Answer 57

B12 relacement