Pathology I

Question 1

What is dementia?

Answer 1

A decrease in cognative ability, memory, or function with intact consciousness (p. 443)

Question 2

What proteins are altered in early onset familial Alzheimer’s disease?

Answer 2

APP (Ch. 21), Presenilin-1 (Ch. 14), Presenilin-2 (Ch. 1) (p. 443)

Question 3

What protein is altered in late onset familial Alzheimer’s disease?

Answer 3

ApoE4 (Ch. 19) (p. 443)

Question 4

What protein is thought to be protective against Alzheimer’s?

Answer 4

ApoE2 (Ch. 19) (p. 443)

Question 5

What gross findings are characteristic of Alzheimer’s disease?

Answer 5

Widespread cortical atrophy (p.443)

Question 6

What histologic findings are characteristic of Alzheimer’s disease?

Answer 6

Senile plaques (extracellular B-amyloid core; amyloid beta (p.443)

Question 7

How is amyloid beta protein synthesized?

Answer 7

By cleaving amyloid precursor protein (p.443)

Question 8

What are neurofibrilary tangles?

Answer 8

Intracellular, abnormally phosphorylated tau proteins. They contain insoluble cytoskeletal elements and tangles correlate with degree of dementia (p.443)

Question 9

What is Pick’s disease?

Answer 9

Frontotemporal dementia characterised by dementia, aphasia, Parkinsonisms, personality changes (p.443)

Question 10

What areas of the brain are typically sparred in Pick’s disease?

Answer 10

Parietal lobe, posterior 2/3 of superior temporal gyrus (p.443)

Question 11

What histologic findings are characteristic of Pick’s disease?

Answer 11

Pick bodies- spherical tau protein aggregates (p.443)

Question 12

What gross findings are characteristic of Pick’s disease?

Answer 12

Frontotemporal atrophy (p.443)

Question 13

What is Lewy body dementia?

Answer 13

Parkinsonism with dementia and hallucinations (p.443)

Question 14

What defect is associated with Lewy Body dementia?

Answer 14

a-synuclein defect (p.443)

Question 15

What is Crutzfeldt-Jakob disease (CJD)?

Answer 15

Rapidly progressive (weeks to months) dementia with myoclonus (startle myoclonus) (p.443)

Question 16

What histologic findings are characteristic of Crutzfeldt-Jakob disease?

Answer 16

Spongiform cortex with prions (proteins containing beta pleated sheets resistant to proteases) (p.443)

Question 17

Name six non-conventional causes of dementia.

Answer 17

Multi-infarct dementia (2nd most common cause in the elderly); syphilis, HIV, Vitamins B1, B3, B12 deficiency, Wilson’s disaese, Normal Pressure Hydrocephalus (p.443)

Question 18

What is multiple sclerosis?

Answer 18

An autoimmune condition characterized by inflammation and demyelination of the CNS (brain and spinal cord) (p.444)

Question 19

How do patients with MS typically present?

Answer 19

Optic neuritis (sudden loss of vision), MLF syndrome, hemiparesis, hemisensory symptoms, bladder/bowel incontinence (p.444)

Question 20

Name four classic symptoms (Charcot’s classic triad) of MS.

Answer 20

Scanning speech, Intention tremor (also incontinence, internuclear opthalmoplegia), Nystagmus (p.444)

Question 21

What lab findings are classic for MS?

Answer 21

Increased protein IgG in CSF with oligoclonal bands (p.444)

Question 22

Describe the MRI findings in a patient with MS.

Answer 22

Periventricular plaques (areas of oligodendrocyte loss and reactive gliosis) with axonal destruction (p.444)

Question 23

What is the treatment for MS?

Answer 23

B-interferon, immunosuppression, natalizumab; symptomatic treatment for neurogenic bladder, spasticity, pain (p.444)

Question 24

What is acute inflammatory demyelinating polyradiculopathy?

Answer 24

The most common variant of Guillain-Barre syndrome; an autoimmune condition that destroys Schwann cells causing inflammation and demyelination of peripheral nerves and motor fibers (p.444)