Monday, 17 April 2023

Tutorial 17th. April 2023

 

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62

EMQ. Headache

63

Role-play. You are called to A&E. Further details on the night.

64

Structured conversation. Topic with which you should be familiar

65

SBA.   Lynch syndrome

 

62. EMQ. Headache.

Option list.

1

abdominal migraine

2

analgesia overuse, aka medication overuse

3

bacterial meningitis

4

benign intracranial hypertension

5

BP check

6

cerebral venous sinus thrombosis

7

chest X-ray

8

cluster

9

impending eclampsia / severe PET

10

malaria

11

meningococcal meningitis

12

methyldopa

13

methysergide

14

migraine

15

MRI brain scan

16

nifedipine

17

nitrofurantoin

18

pancreatitis

19

sinusitis

20

subdural haematoma

21

subarachnoid haemorrhage

22

tension

23

ultrasound scan of the abdomen

Scenario 1. A 405-year-old para 3 is admitted at 38 weeks by ambulance with severe headache of sudden onset. She d6escribes it as “the worst I’ve ever had”. Which diagnosis needs to be excluded urgently?

Scenario 2. A 32-year-old para 1 has recently experienced headaches. They are worse on exercise, even mild exercise such as walking up stairs. She experiences photophobia with the headaches. Which is the most likely diagnosis?

Scenario 3. A woman returns from a sub-Saharan area of Africa. She develops severe headache, fever and rigors. What diagnosis should particularly be in the minds of the attending doctors?

Scenario 4. A woman at 37 weeks has s. They particularly occur at night without obvious triggers. They occur every few days.

Scenario 5. A primigravida has had s on a regular basis for many years. They occur most days, are bilateral and are worse when she is stressed. What is the most likely diagnosis?

Scenario 6. A woman complains of recent headaches at 36 weeks. The history reveals that they started soon after she began treatment with a drug prescribed by her GP. Which is the most likely of the following drugs to be the culprit: methyldopa, methysergide, nifedipine or nitrofurantoin?

Scenario 7. A woman is booked for Caesarean section and wishes regional anaesthesia. She had severe headache due to dural tap after a previous Caesarean section. She wants to take all possible steps to reduce the risk of having this again. Which of epidural / spinal anaesthesia has the lower risk of causing dural tap?

Scenario 8. A 25-year-old primigravida attends for her 20-week scan and complains of headache which started two weeks before. There is no significant history. The pain occurs behind her right eye and she describes it as severe and “stabbing” in nature. The pain is so severe that she cannot sit still and has to walk about. She has noticed that her right eye becomes reddened and “watery” during the attack and her nose is “runny”. The attacks have no obvious trigger and mostly occur a few hours after she has gone to sleep. The usually last about 20 minutes. She has no other symptoms. She smokes 20 cigarettes a day but does not take any other drugs, legal or otherwise. What is the most likely diagnosis?

Scenario 9. A woman has a 5-year history of unilateral, throbbing headache often preceded by nausea, visual disturbances, photophobia and sensitivity to loud noise. What is the most likely diagnosis?

Scenario 10. A primigravida is admitted at 38 weeks complaining of headache, abdominal pain and a sensation of flashing lights. What would be the appropriate initial investigation?

Scenario 11. A woman with BMI of 35 attends for her combined Downs syndrome screening test. She complains of pain behind her eyes. The pain is worst last thing at night before she goes to sleep or if she has to get up in the night. She has noticed she has noticed horizontal diplopia on several occasions. She has no other symptoms. Examination shows papilloedema.

Scenario 12. A grande multip of 40 years experienced sudden-onset, severe headache, vomited several times and then collapsed, all within the space of 30 minutes. She is admitted urgently in a semi-comatose state. Examination shows neck-stiffness and left hemi-paresis.

Scenario 13. What did the MMR include as “red flags” for headache in pregnancy? These are not on the option list – you need to dig them out of your head.

Scenario 14. This is not an EMQ. It relates to the incidence of various conditions in women who have migraine. Choose the appropriate arrow for each.

Option list.

A

asthma

B

developmental dysplasia of the hip in child

C

diabetes

D

Down’s syndrome in child

E

hypertension

F

ischaemic heart disease

G

PET

H

stroke

Scenario 15.

Which of the following drugs is contraindicated in the prophylaxis of migraine in pregnancy?

Option list.

A

amitriptyline

B

ß-blockers

C

ergotamine

D

low-dose aspirin

E

pizotifen

F

pregabalin

G

tricyclic antidepressants

H

verapamil

Scenario 16. Which, if any, of the following statements is true about posterior reversible encephalopathy syndrome. This is not a true EMQ as there may be > 1 true answer.

Option list.

A

‘thunderclap’ headache is typical

B

‘handclap’ headache is typical

C

classically occurs in the early puerperium and is recurrent

D

classically occurs in the early puerperium and is not recurrent

E

arterial beading is typically seen on MRI

F

arterial beating is typically seen on MRI

G

arterial bleeding is typically seen on MRI

H

venous beading is typically seen on MRI

I

venous beating is typically seen on MRI

J

venous bleeding is typically seen on MRI

K

diagnosis requires lumbar puncture and evidence of CSF pressure

L

treatment is with nimodipine

Scenario 17. Which, if any, of the following statements is true about reversible cerebral vasoconstriction syndrome. This is not a true EMQ as there may be > 1 true answer.

Option list.

A

‘thunderclap’ headache is typical

B

‘handclap’ headache is typical

C

classically occurs in the early puerperium and is recurrent

D

classically occurs in the early puerperium and is not recurrent

E

arterial beading is typically seen on MRI

F

arterial beating is typically seen on MRI

G

arterial bleeding is typically seen on MRI

H

venous beading is typically seen on MRI

I

venous beating is typically seen on MRI

J

venous bleeding is typically seen on MRI

K

diagnosis requires lumbar puncture and evidence of CSF pressure

L

treatment is with nimodipine

 

Questions from TOG article by Revell & Moorish. 2014. They are open access.

Red flag features for headaches include:

1.     headache that changes with posture                                                                            True / False

2.     associated vomiting                                                                                                         True / False

3.     occipital location                                                                                                               True / False

4.     associated visual disturbance.                                                                                        True / False

Migraine is classically,

5.     bilateral.                                                                                                                             True / False

6.     pulsating.                                                                                                                            True / False

7.     aggravated by physical exercise.                                                                                    True / False

With regard to migraine headaches in pregnancy,

8.     there is an increase in the frequency of attacks without aura.                                True / False

9.     women who suffer from this have not been shown to have an increase in the risk of pre-eclampsia.                                                                                                                                      True / False

10.   the 5HT1-receptor sumatriptan has been shown to be teratogenic.                     True / False

11.   women presenting with an aura for the first time are not at an increased risk of intracranial disease.                                                                                                                                     True / False

Posterior reversible encephalopathy syndrome,

12.   is associated with an impairment of the autoregulatory mechanism which maintains constant cerebral blood flow where there are blood pressure fluctuations.                                       True / False

13.   when it is associated with pre-eclampsia, management should follow the pathway for managing severe pre-eclampsia.                                                                                                True / False

With regard to cerebral venous thrombosis,

14.   the incidence in western countries in pregnancy ranges from 1 in 2500 deliveries to 1 in 10 000 deliveries.                                                                                                                          True / False

15.   the greatest risk in pregnancy is mainly in the last four weeks.                             True / False

16.   the most common site is the sagittal sinus.                                                                True / False

17.   a plain computed tomography is a highly sensitive investigation.                          True / False

18.   T2-weighted magnetic resonance imaging has been shown to have limited value in diagnosis.

True / False

19.   the outcome is better when it is associated with pregnancy and the puerperium compared to that occurring outside pregnancy.                                                                                            True / False

20.   when it occurs in pregnancy, it is a contraindication for future pregnancies.                          True / False

 

63. Role-play. You are called to A&E. Further details given on the night.

     

64. Structured conversation. Topic with which you should be familiar.

      The examiner will ask a series of questions.

 

65. SBA.   Lynch syndrome.

Abbreviations

CRC:              colorectal cancer.

EC:                 endometrial cancer.

IBD:               inflammatory bowel disease: Crohn’s & ulcerative colitis.

IDDM:           insulin-dependent diabetes mellitus.

Ls:                  Lynch syndrome.

MLH:             mutL-homolog family of DNA, mismatch repair genes.

MMR:           mismatch repair.

MSH:             mutS homolog family of DNA, mismatch repair genes.

Question 1.        What is Lynch syndrome?

Option List

A

auto-immune condition leading to reduced factor X levels in blood

B

hereditary condition which increases the risk of many cancers, particularly breast

C

hereditary condition which increases the risk of many cancers, particularly breast & colorectal

D

hereditary condition which increases the risk of many cancers, particularly colorectal & endometrial

E

none of the above

Question 2.        How is Lynch syndrome inherited?

Option List

A

it is an autosomal dominant condition

B

it is an autosomal recessive condition

C

it is an X-linked dominant condition

D

it is an X-linked recessive condition

E

none of the above

Question 3.        Which, if any, of the following genes can cause Lynch syndrome?

Option List

A

MLH1 + MLH2 + MOH1

B

MLH1 + MLH2 + MSH1

C

MLH1 + MLH2 + MSH6

D

MLH1 + MSH2 + MSH6

E

None of the above

Question 4.        Mutations of which 2 of the following genes cause most cases of Lynch syndrome?

Option List

A

MLH1 + MLH2

B

MLH1 + MSH1

C

MLH1 + MSH2

D

MLH2 + MSH1

E

MLH2 + MSH2

Question 5.        What is the approximate prevalence of Ls in the UK population?

Option List

A

1 in 50

B

1 in 100

C

1 in 1,000

D

3 in 1,000

E

none of the above

Question 6.        Approximately what % of individuals with Ls have had the diagnosis established?

Option List

A

< 5%

B

5 -10%

C

10-20%

D

20-30%

E

>30%

Question 7.        Which, if any, of the following conditions are associated with an risk of Ls?

Option List

A

acromegaly + Addison’s disease + coeliac disease + IBD + IDDM

B

acromegaly + disease + anosmia + coeliac disease + IBD

C

acromegaly + IBD + IDDM

D

acromegaly + IBD

E

Addison’s disease + anosmia + coeliac disease + IBD + IDDM

F

acromegaly + Addison’s disease + anosmia + coeliac disease + IBD + IDDM

G

none of the above

Question 8.        Which 2 cancers are most likely in women with Lynch syndrome?

Option List

A

breast + bowel

B

breast + pancreas

C

breast + endometrium

D

bowel + cervix

E

bowel + endometrium

F

bowel + ovary

G

bowel + pancreas

H

endometrium + ovary

Question 9.        What does NICE recommend about screening for Lynch syndrome for the population

with no personal history of colorectal cancer?

Option List

A

offer screening to those aged < 50 years with  ≥ 1 affected 1st.O relative

B

offer screening to those aged < 60 years with ≥ 1 affected 1st.O relative

C

offer screening to those with ≥ 1 affected 1st.O relative aged < 50 years at diagnosis

D

offer screening to those with ≥ 1 affected 1st.O relative aged < 60 years at diagnosis

E

none of the above

Question 10.    What does NICE recommend in relation to screening for Lynch syndrome in those with

a new diagnosis of colorectal cancer?

Option List

A

offer screening to everyone, regardless of age and family history

B

offer screening to those aged < 50 years at diagnosis

C

offer screening to those aged < 60 years at diagnosis

D

offer screening to those aged < 50 years at diagnosis with + ≥ 1 affected 1st.O relative

E

offer screening to those aged < 60 years at diagnosis with + ≥ 1 affected 1st.O relative

Question 11.    What does NICE recommend about screening for Lynch syndrome for the population

with no personal history of thyroid cancer?

Option List

A

offer screening to those aged < 50 years with  ≥ 1 affected 1st.O relative

B

offer screening to those aged < 60 years with ≥ 1 affected 1st.O relative

C

offer screening to those with ≥ 1 affected 1st.O relative aged < 50 years at diagnosis

D

offer screening to those with ≥ 1 affected 1st.O relative aged < 60 years at diagnosis

E

none of the above

Question 12.         What does NICE recommend in relation to screening for Lynch syndrome in those

with a new diagnosis of thyroid cancer?

Option List

A

offer screening to everyone, regardless of age and family history

B

offer screening to those aged < 50 years at diagnosis

C

offer screening to those aged < 60 years at diagnosis

D

offer screening to those aged < 50 years at diagnosis with + ≥ 1 affected 1st.O relative

E

none of the above

Question 13.    What does NICE recommend about screening for Lynch syndrome for the population

 with no personal history of endometrial cancer?

Option List

A

offer screening to those aged < 50 years with  ≥ 1 affected 1st.O relative

B

offer screening to those aged < 60 years with ≥ 1 affected 1st.O relative

C

offer screening to those with ≥ 1 affected 1st.O relative aged < 50 years at diagnosis

D

offer screening to those with ≥ 1 affected 1st.O relative aged < 60 years at diagnosis

E

none of the above

Question 14.    What does NICE recommend in relation to screening for Lynch syndrome in those with

a new diagnosis of endometrial cancer?

Option List

A

offer screening to those aged < 50 years with  ≥ 1 affected 1st.O relative

B

offer screening to those aged < 60 years with ≥ 1 affected 1st.O relative

C

offer screening to those with ≥ 1 affected 1st.O relative aged < 50 years at diagnosis

D

offer screening to those with ≥ 1 affected 1st.O relative aged < 60 years at diagnosis

E

none of the above

Question 15.    What does NICE recommend about screening for Lynch syndrome for the population

with no personal history of colorectal cancer?

Option List

A

offer screening to those aged < 50 years with  ≥ 1 affected 1st.O relative

B

offer screening to those aged < 60 years with ≥ 1 affected 1st.O relative

C

offer screening to those with ≥ 1 affected 1st.O relative aged < 50 years at diagnosis

D

offer screening to those with ≥ 1 affected 1st.O relative aged < 60 years at diagnosis

E

none of the above

Question 16.    What does NICE recommend in relation to screening for Lynch syndrome in those with

a new diagnosis of colorectal cancer?

Option List

A

offer screening to everyone, regardless of age and family history

B

offer screening to those aged < 50 years at diagnosis

C

offer screening to those aged < 60 years at diagnosis

D

offer screening to those aged < 50 years at diagnosis with + ≥ 1 affected 1st.O relative

E

offer screening to those aged < 60 years at diagnosis with + ≥ 1 affected 1st.O relative

Question 17.    What relationship, if any, exists between Ls and acromegaly?

Option List

A

the risk of Ls is in those with acromegaly compared with the general population

B

the risk of Ls is in those with acromegaly compared with the general population

C

the risk of Ls is unchanged in those with acromegaly compared with the general population

D

the risk of Ls in unknown in those with acromegaly

E

 

Question 18.    What is the effect of aspirin consumption on the risk of EC and CRC?

Option List

A

aspirin reduces the risk of EC and CRC

B

aspirin reduces the risk of EC but not CRC

C

aspirin reduces the risk of CRC but not EC

D

aspirin does not reduce the risk of EC or CRC

E

aspirin reduces the risk of EC and CRC, but the risks outweigh the benefits

Question 19.    A healthy woman of 35 years is diagnosed with Ls? What are the key elements of the

National Screening Programme for people with Ls?

There is no option list – just write down everything you know.

Question 20.    Which, if any, of the following were recommendations made by Monahan et al, the 30

experts who wrote to the BMJ in 2017.

Option List

A

creation of a national register of people with Ls

B

creation of a post of Consultant in Ls for each NHS Trust

C

creation of a post of Clinical Champion for Ls in each NHS Region.

D

creation of a post of Clinical Champion for Ls in the DOH.

E

none of the above

With regard to Lynch syndrome,

1.     loss of mismatch repair protein expression on immunohistochemistry of cancer is diagnostic.

True/False

2.     most carriers of the mutation associated with the syndrome know they have the condition.

True/False

3.     the first cancers associated with the syndrome are predominantly endometrial or ovarian cancers.                                                                                                                               True/False

4.     when cancers occur, they have in them an unusually high immune infiltrate.    True/False

With regard to testing for Lynch syndrome,

5.     consent must be sought before definitive germline testing for Lynch syndrome by a trained professional.                                                                                                                       True/False

6.     immunohistochemical staining of tumours for the mismatch repair proteins or microsatellite instability analysis are recognised ways of screening cancers for characteristics suggestive of the syndrome.                                                                                                                     True/False

7.     the National Institute for Health and Care Excellence endorses universal screening of colorectal cancer patients for Lynch syndrome.                                                                               True/False

8.     most gynaecological cancers found to have aberrant mismatch repair immunohistochemical staining will be in those with the syndrome.                                                  True/False

9.     the addition of MLH1 promotor hypermethylation testing in a Lynch syndrome diagnostic pathway improves specificity.                                                                               True/False

Regarding gynaecological surveillance in women with Lynch syndrome,

10.   there is strong evidence to recommend its use.                                                         True/False

11.   this should be offered to women around 25 years of age.                                       True/False

12.   counselling should include education on red flag symptoms of cancer and risk-reducing surgery.

True/False

With regard to risk-reducing strategies for women with Lynch syndrome,

13.   hysterectomy is strongly recommended for all those with the syndrome.               True/False

14.   the timing of risk-reducing surgery depends on the syndrome gene.                     True/False

15.   where possible, a laparoscopic approach is recommended.                                    True/False

16.   aspirin is not recommended as a means of reducing their overall cancer risk.              True/False

Regarding Lynch syndrome-associated gynaecological cancers,

17.   endometrial types that arise as a result of the syndrome have a poorer prognosis than sporadic types.                                                                                                                                 True/False

18.   checkpoint inhibition of the PD-1/PD-L1 pathway has been shown to be very effective in mismatch repair-deficient cancers.                                                                                       True/False

19.   vaccination against these cancers is currently the focus of research.                     True/False

20.   the Manchester International Consensus guideline is a useful reference for gynaecologists managing women with these cancers.                                                                True/False

 

 

 


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