🤔 MEDIUM

TRIGGER WARNING: The following questions discuss progressive neurological conditions and death and dying. Please take care of yourself and decide if you are in the right place to do these questions. 

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Impact of Chronic Disability

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Question 1

Stem 1, question 1 of 5

34 year old Caoimhe presents to the emergency department with a sudden painful loss of  colour vision in her right eye and difficulties finding her balance in the last 48 hours. She has no fever and systemically well.

What is the most likely cause of her sudden vision loss?
A
Posterior uveitis
B
Acute angle closure glaucoma
C
Retinal haemorrhage
D
Central retinal vascular occlusion
E
Optic neuritis
Question 1 Explanation: 
Caoimhe ("Kee-va") is presenting with optic neuritis. When approaching vision loss the two key categories I find most helpful to consider are: Painful and painless vision loss. Optic neuritis often presents with painful decreased visual acuity and loss of colour vision. It is important to consider the cause of the optic neuritis, which leads us nicely onto the next question.....
Question 2

Stem 1, question 2 of 5

Caoimhe is later diagnosed with Multiple Sclerosis. Which of the following is not a possible sign or symptom of Multiple Sclerosis?
A
Opthalmoplegia
B
Transient motor dysfunction
C
Asymmetrical or single limb involvement
D
Bradykinesia
E
Balance problems
Question 2 Explanation: 
Bradykinesia and/or akinesia are hallmarks of a different neurological condition, Parkinson's disease. Due to the diffuse nature of the lesions seen in Multiple sclerosis many different neurological functions can be affected, rather than the more defined symptoms seen in Parkinson's disease.
Question 3

Stem 1, question 3 of 5

A lumbar puncture can be used aid in the diagnosis of Multiple Sclerosis, which of the following is an expected result for Multiple Sclerosis?
A
High protein
B
Presence of oligoclonal bands
C
High glucose
D
High white cell count
E
Presence of bacteria
Question 3 Explanation: 
A lumbar puncture is a highly invasive investigation so often is not performed in making the diagnosis of MS anymore, but many textbooks still describe the presence of oligoclonal bands as characteristic of MS. The other answer options may be seen in a central nervous infection like meningitis
Question 4

Stem 1, question 4 of 5

A year later Caoimhe's Grandfather is diagnosed with Parkinson's disease. What is Parkinson's disease?
A
A demyelinating central nervous conditions clinically defined by two episodes of neurological dysfunction that are separated in space and time
B
A neurodegenerative disorder characterised by progressive muscle weakness that can start in limb, axial, bulbar or respiratory muscles and then generalises causing progressive disability and death
C
An upper motor neuron lesion of cranial nerves IX, X and XII
D
A chronic progressive neurological disorder characterised by motor symptoms of resting tremor, rigidity, bradykinesia and postural instability with an insidious asymmetrical onset.
E
A lower motor neuron lesion of cranial nerves IX, X and XII
Question 4 Explanation: 
A classical definition question here. A good way to remember Parkinson's disease is TRAP: tremor, rigidity, akinesia (bradykinesia) and postural instability. The other answer options describe the following conditions respectively: Multiple sclerosis, Motor neurone disease, pseudo bulbar palsy and bulbar palsy respectively.
Question 5

Stem 1, question 5 of 5

What is the diagnostic criteria for Parkinson's disease?
A
Presence of bradykinesia with at least one of optic neuritis and balance problems
B
Presence of mask-like facies and shuffling gait
C
Presence of bradykinesia with at least one of resting tremor or rigidity
D
Presence of impaired saccadic and convergency on a background of rigidity
E
Presence of both upper and lower motor neurone symptoms that is progressive.
Question 5 Explanation: 
Parkinson's disease is a clinical diagnosis so knowing the diagnostic criteria is especially important. The presence of bradykinesia with at least one of resting tremor and rigidity is sufficient to start a trial of a dopaminergic agent to assess the response. You would be correct in thinking mask-like facies and shuffling gait are signs of Parkinson's disease but are not in the diagnostic criteria.
Question 6

Stem 2, question 1 of 5

Rob, a 44 year old man, presents to GP with a 6 month history of a right foot drop and weakness which is now progressing up his leg. Rob also reports pain and twitching in both legs causing difficulties getting out of chairs and climbing stairs and is needed the assistance of his husband.

Which of the following is not an upper motor neurone symptom?
A
Hypertonia
B
Clonus
C
Positive Babinski's reflex
D
Fasciculations
E
Spasticity
Question 6 Explanation: 
It is important to be able to differentiate between upper and lower motor neurone signs as this can often guide you as to the likely diagnosis. Upper motor neurones function to facilitate movement and the lower motor neurones are the ones that directly attach to the muscle to bring about the movement. These are two good links explaining the difference between the two groups of symptoms: https://teachmephysiology.com/nervous-system/motor-system/upper-motor-neurone/ https://teachmephysiology.com/nervous-system/motor-system/lower-motor-neurones/
Question 7

Stem 2, question 2 of 5

During the consultation Rob mentions he is a former professional rugby player and his Grandfather died in his 50's from a "progressive paralysis condition". Given Rob's family and social history in addition to his presentation, what is the most likely diagnosis?
A
Bulbar palsy
B
Amyotrophic lateral sclerosis
C
Spinal muscular atrophy
D
Polio
E
Becker's msucular dystrophy
Question 7 Explanation: 
This stem highlights two risk factors for Amyotrophic lateral sclerosis (ALS), aka motor neurone disease: Family history and professional sports playing in addition to his age of more than 40. Rob is presenting with both upper and lower motor neurone signs that are progressive and thus the only answer choice that fits this is ALS. Spinal muscular atrophy and Becker's muscular dystrophy are genetic conditions so present at brith or within first few years of life.
Question 8

Stem 2, question 3 of 5

9 months later Rob is finding is more difficult to speak and communicate his wishes, the GP discusses with Rob and his husband about advanced care planning. Which of the following is false about an advanced decision to refuse treatment?
A
The decision will only apply at a time when the individual lacks capacity to consent or refuse the specific treatment
B
It can only be a written decision and must be signed by the individual
C
The decision must state precisely what treatment is to be refused
D
The decision must set out the circumstances when the refusal should apply
E
The decision may not be applicable if there are reasonable grounds for believing that circumstances exist which the patient didn't anticipate and would have affected their decision.
Question 8 Explanation: 
An advanced decision to refuse treatment (ADRT) can be a very useful tool for individuals to use to stop them from being given treatments they do not want. Advanced decisions can be verbal, oral or written as long as all the above criteria are met. An advance refusal of life sustaining treatment has additional rules such as: it must be written, signed by person making it, witness and must specify that the treatment being refused is potentially life sustaining and that death could result from the refusal.
Question 9

Stem 2, question 4 of 5

A few weeks later, Rob decides to give his husband Lasting Power of Attorney for health and welfare decisions. Rob is aware his disease is rapidly progressing and he is entering the end of his life. Which of the following is false about Lasting Power of Attorney?
A
A lasting power of attorney decision is made while the person still has capacity
B
Only the person making the decision must be over 18 years
C
The decision must be registered with the Office of the Public Guardian
D
The person with Lasting Power of Attorney has legal authority to make decisions and must be consulted on behalf of the patient
E
The decisions made must be in the patient's best interests.
Question 9 Explanation: 
A lasting power of attorney (LPA) decision is made when a person has capacity and can related to financial affairs and/or health and welfare decisions. Both the person making the decision and the appointed person must be 18 years or over (think mental capacity act). Once complete and registered the person with LPA has legal authority and must be consulted on decisions for the patient.
Question 10

Stem 2, question 5 of 5

Rob decides he would like to die in a hospice and a short while later enters his local hospice. Rob struggles with clearing secretions, what medication can be given to help with this?
A
Levomepromazine
B
Midazolam
C
Morphine
D
Hyoscine
E
Cyclizine
Question 10 Explanation: 
This question is focussing on anticipatory medications, these are medications used in end of life to provide comfort to the individual. Levomepromazine is used for nausea and vomitting, midazlam for agitation, morphine for pain. Hyoscine is used to dry up secretions. These medications are often given subcutaneously through a syringe driver and can be given in the place the individual has chosen to spend their last moments.
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