Dementia And Degenerative Disorders

Front 1

Degeneration of the neurons of the cortex results in ?

Back 1

dementia

Front 2

Degeneration of the basal ganglia results in ?

Back 2

Movement problems

Front 3

What is the degenerative disease of the cortex that is the most common cause of dementia?

Back 3

Alzheimer Disease

Front 4

Breakdown of which protein is defective and responsible for the plaques seen in alzheimers patients? (into its beta form)

Back 4

Amyloid precursor protein --> deposition of A beta-amyloid depositing in the brain.

Front 5

Clinical features of alzheimers is due to ?

Back 5

dementia: memory loss + cognitive dysfunction without loss of consciousness

-Slow onset memory loss

-progressive disorientation

-loss of learned motor skills and language

-behavior and personality changes

-patients become mute and bedridden

-focal neurologic deficits not seen early (nothing like parkinsons or huntingtons)

Front 6

Most cases of alzheimers are _______ (sporadic or early onset)

Back 6

Sporadic

Front 7

What is the most important risk factor for sporadic AD?

Back 7

1) OLD AGE, Epsilon 4 allele of APO E (increases the conversion of APP to the beta form, resulting in extra deposition of the amyloid plaques)

Decreased risk with Epsilon 2 allele

Front 8

What are the key epidemiologic associations with AD early onset form?

Back 8

1) Presenilin 1 (familial cases) and presenillin 2

2) down syndrome (commonly occurs by 40 years of age) (APP present on chromosome 21, so since they have an extra copy, they have increased production of A beta amyloid)

Front 9

What are the few classic pathologic features of AD?

Back 9

diffuse cerebral atrophy. (Narrowing of gyri and widening of sulci are characteristic of cerebral atrophy)

because you're losing brain mass, the ventricles dilate to take up the lost space - "hydrocephalus ex vacuo"

Neuritic plaque: A beta amyloid (pink, extracellular), with entrapped neuritic processes

Cerebral amyloid angiopathy (explained on next card)

Neurofibrilary tangles: intracellular aggregates of fibers composed of hyperphosphorylated tau protein. Tau helps organize microtubules in the cytoplasm. When it becomes hyperphosphorylated, it can no longer assist with the organization of the microtubules...

Front 10

Why do Alzheimers patients have an increased risk of hemorrhage into the brain?

Back 10

because amyloid plaques can deposit on the cerebral blood vessels ( cerebral amyloid angiopathy). This weakens the wall of the blood vessel, increasing the risk for hemorrhage.

Front 11

Diagnosis of AD is ________ and made clinically after excluding other causes. How is diagnosis confirmed?

Back 11

Presumptive. Confirmed with pathology at autopsy.

Front 12

What is the main pathophysiology behind vascular dementia?

Back 12

Multifocal infarction and injury due to HTN, atherosclerosis, or vasculitis. (moderate global ischemia)

Front 13

What is the second most common cause of dementia?

Back 13

Vascular dementia (according to pathoma)

Front 14

With moderate cerebral ischemia you get ________ damage to specific areas of the cortex. What are a few of these areas?

Back 14

selective; 3,5, and 6 (pyramidal neurons); hippocampus of the temporal lobe

Front 15

Which disease is a degenerative disease of frontal and temporal cortex but spares the parietal and occipital lobes?

Back 15

Pick Disease

Front 16

In Pick Disease, you see round aggregates of ____ protein in neurons of cortex.

Back 16

Tau

Front 17

In Pick disease, which comes first, behavioral and language symptoms, or dementia?

Back 17

Behavioral and language symptoms come first, then progresses to dementia

Front 18

Parkinson Disease is a degenerative loss of ______ergic neurons in the _______ ______ __ __

Back 18

dopaminergic; substantia nigra pars compacta