🤔 MEDIUM

This question is the first in a two-parter, on chronic confusion and memory loss. It contains ONE patient only.  All I can say, by way of a not-too-subtle clue, is:

“ITS A TRAP!”

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chronic memory loss and dementia 1

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Question 1

Stem - note all 10 questions relate to the same patient.

A 72YOM presents with a two year history of “generally slowing down”. He explains his voice has become softer, his mood more depressed and he is no longer able to knit as he once loved. On examination, there is a notable left-sided unilateral tremor as the hand sits in the lap. He also took some time to start moving, from standing, in the waiting area.

Which is the best matched differential?
A
Alzheimer’s disease
B
Frontotemporal dementia
C
Huntington’s chorea
D
Idiopathic Parkinsonism
E
Lewy body dementia
Question 2
Of the section below, which arrow corresponds to a region most likely implicated in this disease?
A
A
B
B
C
C
D
D
E
E
Question 2 Explanation: 
A - superior colliculus ; B - medial geniculate body ; C - mesencephalic reticular formation ; D - substantia nigra (pars compacta) ; E - corticospinal (pyramidal) tract ;
Question 3
The diagnosis is known to cause, amongst other hallmarks, intracytoplasmic eosinophilic inclusions of alpha-syn. Which other condition shares these findings?
A
Alzheimer’s disease
B
Frontotemporal dementia
C
Huntington’s chorea
D
Lewy body dementia
E
Normal pressure hydrocephalus
Question 3 Explanation: 
This is the classic description of a Lewy body - namely an eosinophilic staining alpha-synuclein with fibril-corona - seen in its namesake dementia.
Question 4
What is the most characteristic symptom of basal ganglia (nuclear) dysfunction?
A
Anterograde amnesia
B
Bradykinesia
C
Cogwheel rigidity
D
Nausea
E
Resting tremor
Question 4 Explanation: 
“TRAP” is the common med student mnemonic to remember the symptoms of Parkinsonism: tremor (rest), rigidity (cogwheel), akinesia (bradykinesia really) and postural instability. NICE 2020 states you must have bradykinesia and at least one of the remaining “TRAP” symptoms to diagnose successfully.
Question 5
What is the first investigation to order?
A
MRI brain
B
No investigation
C
Olfactory testing
D
Serum ceruloplasmin
E
SPECT
Question 5 Explanation: 
A DA-challenge can be undertaken, but most Parkinsonism without atypical findings, is a clinical diagnosis.
Question 6
If the tremor were the thing most concerning the patient, what would be the appropriate first-line medication upon diagnostic confirmation?
A
Domperidone
B
Entacapone
C
Pramipexole
D
Selegiline
E
Trihexyphenidyl
Question 6 Explanation: 
This anticholinergic has better outcomes, vs LDOPA, for the early management of PD when the tremor is the symptom most affecting daily life.
Question 7
Which of the following are true statements about the prognosis of Parkinson’s disease?
A
Communication difficulties and freezing of gait are common motor complications
B
Mortality rates, matched to age controls, can be 5x higher for PD patients
C
Parkinson’s disease is characterised (NICE, 2020) as both resting tremor and one of cogwheel rigidity or bradykinesia
D
Parkinson’s disease is typically fast progressing
E
The earlier the onset of Parkinsonism, the less likely the onset of neurocognitive decline
Question 8
Given the working differential, which of the following is the best first management step?
A
At this stage, no treatment is required
B
Due to age, consider domperidone
C
Refer, untreated, to movement disorder specialist
D
Start LDOPA trial with carbidopa adjunct
E
Start LDOPA trial without carbidopa
Question 8 Explanation: 
NICE Guidelines (CKS 2020) recommend the following verbatim “People with suspected Parkinson's disease should be referred urgently, and untreated, to a specialist in movement disorders for confirmation of the diagnosis and exclusion of alternative conditions.”
Question 9
The patient is started on co-careldopa by secondary care. Should writhing movement commence several months down the line and an adjuvant be sought, what would be the most appropriate therapy to commence?
A
DBS
B
Domperidone
C
Entacapone
D
Pramipexole
E
Selegiline
Question 9 Explanation: 
COMTi, such as entacapone, are often used when LDOPA titre begins to fail and dyskinesia begins to start. They are used as adjuvant therapy to the persisting LDOPA administration.
Question 10
The patient rings his GP complaining about losing weight from chronic nausea. Which would be the most appropriate medication to prescribe to manage this?
A
Co-careldopa
B
Domperidone
C
Metoclopramide
D
PD is an absolute contraindication for anti-emesis therapy
E
Prochlorperazine
Question 10 Explanation: 
Unlike metoclopramide or prochlorperazine, domperidone are dopamine antagonists that are more peripherally selective. The former two medications are known to cause iatrogenic Parkinsonism.
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Take Home Message

Phew, a lot of content was covered here.  Basically there are lots of different kinds of PD, in this case the most common type was documented – idiopathic PD.   Here are a few tips to take away: 

  1. NICE is very particular that for a patient to have Parkinsonism you must always have:
    1. Bradykinesia and one of
      1. the rest of TRAP (see earlier) 
    2. Always refer the ?PD patient to a movement disorder specialist neurological clinic without starting the patinet on medication. 
  2. There are lots of different medications on offer for PD, but in brief:
    1. LDOPA is given first line in the older patient, alongside a DOPA decarboxidase inhibitor (carbidopa) in the standard formulation called Co-careldopa. 
    2. COMTi (eg entacapone) are preferred nowadays to MAOb-i (rasagiline) for adjuvant therapy when LDOPA starts to fail
    3. If the patient is young, they are at a better outcome for motor and non-motor symptoms.  This is important for prognosis and communication, but also because their first-line therapy is often a DA-agonist specifically eg pramipexole or ropinirole. 
    4. DA-antagonists, such as domperidone, are used for anti-nausea therapy in these patients.  Things like metoclopramide should be avoided because they will further antagonise nigrostriatal fibres.  
    5. Apomorphine is used often in the later staeges of the disease –  to help with the on-off phenomenon – either SC or PO. 
    6. If the patient presents with an affecting-daily-living resting tremor as the predominant symptom, you can be extra LDOPA-sparing and start the patient on an anticholinergic eg trihexyphenidyl

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