Neurodegenerative disease

Question 1

A patient has been figiting for several years and starts to experience personality changes, jerky movements affecting the entire body, short term memory loss, etc. what is this?

Answer 1

Huntingtons

Question 2

What is athetosis?

Answer 2

Writhing movements associated with chorea

Question 3

What is the pathogenesis of hunting tons?

Answer 3

CAG repeat, inherited AD

Question 4

Describe childhood hunting tons and how it is different from the adult variant

Answer 4

Called Westphal variant, more severe, looks more like Parkinson’s
Affects neostratium

Question 5

What therapies are available for hunting tons?

Answer 5

Haldol, tetrabenzine to treat chorea

Question 6

What factors are associated with increased frequency of AD?

Answer 6

age, female sex, history of severe trauma, down syndrome

Question 7

Describe normal progression of AD

Answer 7

difficulty with personal tasks progresses to losing the ability to speak, think and make decisions

Question 8

What are the pathologic features of AD?

Answer 8

brain atrophy, senile plaques,

Question 9

What therapies are used for alzheimer’s disease?

Answer 9

treating associated symptoms (depression, agitation, sleep disorders), preventing progression (therapy with acetylcholinesterase inhibitors),

Question 10

What are the criteria for diangosis of vascular dementia?

Answer 10

presence of dementia and focal neurologic signs on exam

Question 11

A patient presents with a parkinsonian picture, visual hallucinations and extrapyramidal symptoms. This is…

Answer 11

dementia with lewy bodies

Question 12

A patient presents with a failure of vertical gaze, dysarthria, dysphagia, gait ataxia and demenita. This is..

Answer 12

progressive supranuclear palsy

Question 13

A patient presents with significant alteraion in personality, social behavior and language. Think…

Answer 13

frontotemporal dementia

Question 14

What treatment is most effective for REM sleep behavior disorder?

Answer 14

clonazepam

Question 15

An eldelry lady has been abusive to her neighbors, stealing their clothes. On exam, her pupils are small and only react when she is asked to look at her nose. This is..

Answer 15

neurosyphillis - reaction to accomodation but not light

Question 16

A 58 year ol man with PMH of coronary artery bypass is having difficulty multi-tasking. This is…

Answer 16

hypoxic ischemic encephalopathy

Question 17

A 50 year old man undergoes personality change over 3 months, becoming apathetic and disinterested. He has dysarthria, an exagerrated startle response. This is..

Answer 17

CJD

Question 18

An elderly woman is afraid to be in the house alone, is hoarding and keeps getting lost on the way home. This is..

Answer 18

alzheimer’s - early signs are paranoia, etc.

Question 19

An elderly man develops cognitive impairment involving memory and impacting his occupation and social functioning. This is..

Answer 19

dementia

Question 20

What treatments are used for AD?

Answer 20

memantine - NMDA receptor antagonist;
cholinesterase inhibitors (Exelon = riverstigmine) or Donezepil = Aricept

Question 21

What therapies can be used to treat Parkinson’s?

Answer 21

Dopamine agonists, MAO Inhibitors, COMT inhibitors, anticholinergic, amantidine

Question 22

What are common side effects if dopamine agonists?

Answer 22

Nausea, vomiting, hallucinations, sleep attacks, impulse disorders

Question 23

What are common side effects of MAO inhibitors?

Answer 23

Nausea, headache, insomnia, malignant hypertension with sympathomimetics

Question 24

What are the features of multiway stem atrophy?

Answer 24

Autonomic dysfunction, more rapid progression, more symmetric than PD

Question 25

What is the classic radiologic sign if multisystem atrophy?

Answer 25

Hot cross bun sign

Question 26

What are the features of progressive supra nuclear palsy?

Answer 26

Loss of vertical saccades, dysphagia, neck dystonia,

Question 27

What is the classic radiogic features of PSP?

Answer 27

Hummingbird sign

Question 28

What are the clinical features of cortical basal degeneration?

Answer 28

Alien lib, asymmetric Parkinson’s rigidity, cortical deficits, apraxia

Question 29

A patient presents with CO poisoning. What area of the brain can be affected?

Answer 29

delayed necrosis of the globus pallidus may occur

Question 30

A 30 year ol woman develops headaches, dilated pupils that are non reactive and an inability to look up. This is..

Answer 30

perinaud’s phenomenon affecting pineal gland

Question 31

What area is typically ablated during Parkinson’s seizures?

Answer 31

subthalamic nucleus