Dementias

Question 1

Man of 40yrs presents with difficulty concentrating, planning and organisational skills. His family mentions that he has not been himself for a while being particularly mean and uncaring. This has been getting progressively worse. His mom had something similiar but cant remember what it was called. He still knows who each of his family are and where he is. What is the likely diagnosis?

Answer 1

Huntington disease that can cause dementia due to atrophy of the brain

Differs from alzheimers that he still knows who his family are. Likely huntingtons as not fluctuating course (lewy body) and early onset

Question 2

What is the pathology to cause huntingtons disease?

Answer 2

Autosomal dominant that is due to a repeating code of CAG which is toxic to neurones

Question 3

What is the treatment for huntingtons disease?

Answer 3

No treatment but genetic councelling for family, mood stabilisers and refer to nurse specialist

Question 4

You take a histology sample of the brain showing amyloid beta deposits in the hippocampus. What is the likely diagnosis?

Answer 4

Alzheimer disease

amyloid is present in alzheimer and vascular but due to the location in the hippocampus then alzheimers as it starts there then effects the parietal lobes

Question 5

Female of 80yrs presents with difficulty getting dressed, she feels she has poor memory finding she loses her keys a lot and finds it difficult recalling peoples names. She mentions she had a previous appointment with the dentist but missed it as she forgot. You do an MRI which shows focal atrophy in the temporal and parietal lobes. What is the likely diagnosis?

Answer 5

Alzheimer disease

Issues with visuospatial like getting dressed, parking, going down stairs
Most likely as she is elderly and the classic MRI is atrophy is the temporal and parietal lobes

Question 6

What is the the treatment for alzheimers disease?

Answer 6

Address cardiovascular risk factors as good CVS improves health

Cholinesterase inhibitors ribastigmine

Question 7

What are some of the tests you have to run in order to exclude other causes of memory deficit before diagnosing dementia?

Answer 7

Bloods for B12 deficiency
TFTs
Syphilis
HIV
Calcium 
MRI

Question 8

What must a patient present with in order to classify them as having a type of dementia?

Answer 8

Deficit of cognition in one or more of the following areas; attention, exectutive function, perceptual motos, learning and memory, social cognition

Question 9

What is the firstline drug treatment for alzheimers dementia?

Answer 9

Rivastigmine

Question 10

What condition must be met in order to diagnose vascular dementia?

Answer 10

Must show cardiovascular disease on MRI and a clear relationship between dementia onset and cerebrovascular disease.

Question 11

What is the treatment for lewybody dementia?

Answer 11

Rivastigmine if mild to moderate

Levodopa if motor phenotypes

Question 12

Man of 55 years presents with not caring what he says to people so he’s hurting his family relationships, feels his memory has gotten worse and is now just binging on crisps and croissants. You do an MRI which shows atrophy in frontotemporal lobes. What is the likely diagnosis?

Answer 12

Frontotemporal dementia (picks disease)

Question 13

What are the three variants of frontotemporal dementia?

Answer 13

Behavioural, aphasia and sematic dementia

Question 14

what protienopathy would you expect in alzheimers?

Answer 14

amyloid beta protein, neurofibrillary tangles (tau protein)

Question 15

what protienopathy would you expect in parkinsons

Answer 15

alpha synuclein

Question 16

what protienopathy would you expect in CJD

Answer 16

prion

Question 17

what protienopathy would you expect in frontotemporal dementia

Answer 17

Tau