Chronic Neuro 2

Question 1

Define dementia

Answer 1

A severe loss of memory and other cognitive abilities which leads to impaired daily function, regardless of the underlying cause

Question 2

Dementia differentials (13)

Answer 2

Alzheimer’s 
Frontotemporal dementia,
Lewy Body Dementia
Vascular Dementia
Wernicke-Korsakoff’s 
Hydrocephalus
Bilateral Subdural Haematomas
HIV, syphilis, CJD
Frontal mass, limbic encephalitis
Hypoglycaemic episodes

Question 3

5 A’s of dementia

Answer 3

Amnesia - Remembering
Anomia - Naming
Apraxia - Doing
Agnosia - Recognising
Aphasia -Speaking

Question 4

Pathophysiology of AD

Answer 4

APP breakdown by  and γ secretase → abnormal product resistant to degradation → beta Amyloid. This accumulates outside the cell to form amyloid plaques which interferes with neural communication and causes dementia. Also intracellularly causes the phosphorylation of tau causing it to dissociate from the MF and accumulate into neurofibrillary tangles which causes neuronal apoptosis and dementia

Question 5

What performs normal breakdown of APP

Answer 5

Alpha and γ secretase → normal degradation product

Question 6

RF of dementia (8)

Answer 6

Vascular RF: DM, BP, dyslipidaemia, diet
Females
Trauma
Age
Down's syndrome

Question 7

Ix for Alzheimers

Answer 7

Mainly clinical diagnosis
Can look in CSF for leaked tau protein and low amyloid due to it being in plaques
Can do brain imaging too but only really useful in advanced disease

Question 8

What are the 10 questions of the AMT

Answer 8

Age?
Time to nearest hour?
Addres for recall at the end of the test
Year?
Name of this place?
Identification of two people?
Date of birth?
Year of First World War?
Name of current monarch?
Address recall correct?

Question 9

What is vascular dementia caused by?

Answer 9

Neuronal infarcts so basically loads of mini strokes.

Question 10

What is the difference between vascular dementia and AD? (4)

Answer 10

Focal neurology
Sudden onset
Stepwise deterioration
CV RF

Question 11

What does the pathophysiology of Pick’s disease involve

Answer 11

Tau but not beta amyloid plaques

Question 12

What is a Pick body

Answer 12

Hyperphosphyorlated tau protein

Question 13

What is a hyperphosphorylated Tau protein known as

Answer 13

Pick body

Question 14

Difference in epidemiology of Picks disease and AD

Answer 14

Hyperphosphyorlated tau protein
Also typically affects people younger than in other dementias
±FHx (although most are sporadic)
Death within 5-10yrs

Question 15

Which lobes does Pick’s disease affect

Answer 15

Fronto-temporal

Question 16

Symptoms of Pick’s disease (6)

Answer 16

Personality change
Disinhibition
Overeating, preference for sweet foods
Emotional blunting
Lack of hygiene
Relative preservation of memory

Question 17

Wernicke’s encephalopathy triad

Answer 17

ACE
Ataxia
Confusion
Eye signs

Question 18

Wernicke’s encephalopathy signs (2)

Answer 18

Wernicke’s encephalopathy triad

Question 19

What eye signs are present in Wernicke’s encephalopathy (4)

Answer 19

ophthalmoplegia, nystagmus, diplopia, ptosis

Question 20

Why do alcoholics get WKS

Answer 20

generally have a poor diet. Alcohol also prevents vitamin B-1 absorption and storage.

Question 21

What is thiamine involved in (4)

Answer 21

Metabolism of carbohydrates, releasing energy.
Production of neurotransmitters including glutamic acid and GABA.
Lipid metabolism, necessary formyelin production.
Amino acid modification. Probably linked to the production of taurine, of great cardiac importance.

Question 22

What is thiamine’s role (2)

Answer 22

Thiamine acts as an essentialcoenzyme in the TCA cycle and pentose phosphate shunt.

Question 23

Ix for Wenicke’s encephalopathy (5)

Answer 23

Bloods –serum albumin and vitamin B1
ECG
CT
Neuropsychology

Question 24

Mx for Wernicke’s encephalopathy

Answer 24

IV Pabrinex immediately

Question 25

4 differences between Wernicke’s encephalopathy and Korsakoff’s psychosis

Answer 25

W:
Acute 
Confusion
Cerebellar and eye signs
Reversible

K:
Chronic
Alert
Amnesia and confabulation
Irreversible

Question 26

Motor symptoms of Huntingtons (4)

Answer 26

Chorea
Athetosis - writhing movements of hands
Ataxia
Dysphagia

Question 27

Cognitive symptoms of Huntingtons (4)

Answer 27

Lack of concentration
Depression
Dementia
Personality changes, aggression

Question 28

Prognosis of Huntington’s

Answer 28

10-15 years from onset of symptoms

Question 29

What sign will you illicit in the cranial nerve exam of a Huntington’s patient

Answer 29

Unable to protrude tongue

Question 30

Where is the pathology in Huntington’s

Answer 30

in the striatum, there is atrophy of mainly the caudate, but also putamen

Question 31

Pattern of inheritance of Huntington’s disease

Answer 31

Autosomal dominant

Question 32

Mutation in which gene and nucleotide causes Huntington’s

Answer 32

mutation of the Huntingtin gene (HTT)

Expansion of CAG nucleotide from less than 35 to over 40

Question 33

Ix for Huntingtons

Answer 33

CAG repeat testing