17.7 Dementia and Degenerative Disorders

Question 1

Degeneration in what area of the brain will lead to movement problems?

Answer 1

Basal Ganglia - sometimes in the brainstem

Question 2

What does degeneration of the cortex cause?

Answer 2

Dementia

Question 3

Alzheimer’s Disease

Answer 3

Degenerative disease of the cortex

Question 4

What is the most common cause of dementia?

Answer 4

Alzheimer’s Disease

Question 5

The deposition of ______ leads to Alzheimer’s disease?

Answer 5

Aß-amyloid - cleaved form of amyloid precursor protein

Question 6

What are the clinical features of Alzheimer’s disease?

Answer 6

• Slow memory loss
• Progressive disorientation
• Loss of learned motor skills
• Personality changes
• NO early focal neurologic deficits

Question 7

Most cases of Alzheimer’s are ______

Answer 7

Sporadic

Question 8

What is a genetic risk factor of Alzheimer’s?

Answer 8

Epsilon4-ApoE

Question 9

What is a genetic decreased risk factor of Alzheimer’s?

Answer 9

Epsilon2-ApoE

Question 10

What gene mutation is early onset Alzheimer’s associated with?

Answer 10

Presenlin-1 and 2

Question 11

What is Alzheimer’s associated with?

Answer 11

Down Syndrome - amyloid precursor is found on Ch21 so there is increased Aß-amyloid

Question 12

What will be seen on the brain of an Alzheimer’s patient?

Answer 12

Diffuse cortical atrophy - with narrowing of the gyri and widening of the sulci

Question 13

Hydrocephalus Ex Vacuo

Answer 13

Consequence of brain atrophy where the ventricles of the CNS dilate

Question 14

Cerebral Amyloid Angiopathy

Answer 14

Deposition of Aß-amyloid into the blood vessels of the brain

Question 15

What protein is present in neurofibrillary tangles?

Answer 15

Tau Protein that is hyperphosphorylated

Question 16

Vascular Dementia

Answer 16

Multifocal infarction and injury that can be due to HTN, atherosclerosis or vasculitis that decreases blood flow to brain

Question 17

What is the second most common cause of dementia?

Answer 17

Vascular Dementia

Question 18

Pick Disease

Answer 18

Degenerative disease of the frontal and temporal lobe that spares the parietal and occipital lobes - leads to behavioral and language symptoms

Question 19

What is found in Pick disease?

Answer 19

Round aggregates of tau protein in neurons of the cortex

Question 20

Parkinson Disease

Answer 20

Degenerative loss of dopaminergic neurons in the substantia nigra (pars compacta)

Question 21

What is the effect of dopamine from the substantia nigra - pars compacta on the striatum?

Answer 21

• Increases stimulation with D1 receptor binding
• Decreases inhibition with D2 receptor binding

Overall increases cortical function and increases movement

Question 22

Exposure to what chemical has been known to lead to Parkinson’s?

Answer 22

MPTP

Question 23

What are the clinical features of Parkinson’s?

Answer 23

Tremor
Rigidity
Akinesia
Postural Instability

Question 24

What are the round eosinophilic bodies seen in Parkinson’s?

Answer 24

Lewy Bodies

Question 25

What are Lewy Bodies composed of?

Answer 25

Alpha synuclein

Question 26

What does early dementia in Parkinson's suggest?

Answer 26

Lewi Body Dementia - Lewy bodies in the cortex

Question 27

Huntington's Disease

Answer 27

Degeneration of the GABAergic neurons in the caudate nucleus of the basal ganglia

Question 28

What is the function of the caudate?

Answer 28

The GABA neurons of the caudate send inhibitory signals to the cortex.

Question 29

What is a main feature of Huntington's?

Answer 29

Chorea

Question 30

What is the inheritance pattern of Huntington's?

Answer 30

Autosomal Dominant

Question 31

What is the mutation seen in Huntington's?

Answer 31

CAG repeats in the huntingtin gene

Question 32

What is a common cause of death with Huntington's?

Answer 32

Suicide

Question 33

Normal Pressure Hydrocephalus

Answer 33

Increased CSF results in dilated ventricles and can cause dementia

Question 34

What are the clinical features of normal pressure hydrocephalus?

Answer 34

- Urinary incontinence - Gait instability - Dementia

Question 35

What is the treatment for normal pressure hydrocephalus?

Answer 35

Ventricle-Peritoneum Shunt

Question 36

Spongiform Encephalopathy

Answer 36

Degenerative disease due to a prion protein

Question 37

What is the pathologic protein in spongiform encephalopathy?

Answer 37

Prp c in a beta-pleated sheet configuration that is non-degradable

Question 38

What is the hallmark of spongiform encephalopathy?

Answer 38

Intracellular vacuoles in neurons and glial cells

Question 39

What are the 3 mechanisms for spongiform encephalopathy?

Answer 39

- Sporadic - Inherited - Transmitted

Question 40

Creutzfeldt–Jakob Disease (CJD)

Answer 40

Usually sporadic that can rarely arise form exposure to prion infected human tissue and presents as rapidly progressive dementia associated with ataxia and startle myoclonus - fatal within 1 year

Question 41

vCJD

Answer 41

Related to exposure to bovine spongiform encephalopathy

Question 42

Familial Fatal Insomnia

Answer 42

Inherited prion disease characterized by insomnia and an exaggerated startle response