hllj/quesmed huggingface dataset:
A 32-year-old man with HIV-associated nephropathy (HIVAN) and end-stage kidney disease (ESKD) presents to the emergency department with an oozing arteriovenous (AV) fistula following dialysis earlier that afternoon. He is referred to the vascular surgeons for ultrasound, review, and for consideration of surgical exploration.


Bloods are taken at the time with a venous blood gas, revealing the following:


||||
|--------------|:-------:|------------------|
|pH|7.32|7.35 - 7.45|
|PaO₂|14.3 kPa|11 - 15|
|PaCO₂|3.6 kPa|4.6 - 6.4|
|Bicarbonate|16 mmol/L|22 - 30|
|Base Excess|-4 mmol/L|-2 to +2|
|Lactate|1.1 mmol/L|0.6 - 1.4|


Which of the following is the most likely cause for the patient's low bicarbonate level?

Options:
explanation: Hyperventilation from anxiety would result in hypocarbia (low paCO2) but would not affect serum bicarbonate levels
name: Anxiety
label: b
answer: no
explanation: Type 2 renal tubular acidosis (RTA) is a failure of proximal tubular cells to reabsorb bicarbonate from the urine. It would result in a metabolic acidosis and reduced serum bicarbonate. Type 2 RTA is primarily a complication of genetic disorders such as Fanconi syndrome, cystinosis, and Lowe syndrome. It can be acquired in amyloidosis, multiple myeloma, and with HAART medication, but it is not linked directly to HIV or ESKD
name: Type 2 renal tubular acidosis
label: d
answer: no
explanation: This patient is experiencing metabolic acidosis (low pH and low bicarbonate) with partial respiratory compensation (hypocarbia from increased respiratory rate) as a result of chronic kidney disease
name: Acute respiratory acidosis with partial metabolic compensation
label: e
answer: no
explanation: As functioning nephrons decline in CKD, acid secretion is initially compensated by ammonium excretion (up to 40-50mL/min glomerular filtration rate). Beyond this, CKD leads to the retention of hydrogen ions which is buffered by bicarbonate in the extracellular tissue. As the patient approaches ESKD, the plasma bicarbonate stabilises between 12 and 20 mmol/L, and can be partially compensated by increased respiratory rate and hypocarbia
name: Chronic kidney disease (CKD)
label: a
answer: yes
explanation: Severe hypovolaemia can result in reduced renal perfusion and acute kidney injury, resulting in increased bicarbonate buffering of retained hydrogen ions by damaged nephrons and thus a decreased serum bicarbonate. This patient is not experiencing other signs of hypovolaemia and has not described any large volume haemorrhage from his fistula site
name: Hypovolaemia
label: c
answer: no

Correct answer: a


Learning point: In chronic kidney disease (CKD), as nephrons decline, acid retention occurs when ammonium excretion is insufficient, leading to lower plasma bicarbonate levels that stabilize between 12-20 mmol/L in end-stage kidney disease (ESKD), partially compensated by an increased respiratory rate and hypocarbia.
A 73-year-old man has presented to the GP with occasional palpitations. He has ischaemic heart disease, type 2 diabetes, and chronic kidney disease. Bloods reveal he is hypocalcaemic, with raised parathyroid hormone and phosphate and normal levels of vitamin D. ECG is unremarkable.

Which of the following is the most appropriate pharmacotherapy?

Options:
explanation: Cinacalcet is a treatment used to treat secondary hyperparathyroidism, although it is reserved for patients on dialysis. It mimics the action of calcium on the tissues (calcimimetic) and increases the sensitivity of calcium receptors on parathyroid cells to reduce PTH
name: Cinacalcet
label: d
answer: no
explanation: Calcitriol is the active form of vitamin D - 1,25-dihydroxycholecalciferol. It increases calcium uptake in the gastrointestinal tract, increases renal absorption of calcium, and stimulates osteoclastic activity. This then sequentially suppresses parathyroid hormone, helping to prevent progression from secondary hyperparathyroidism into tertiary hyperparathyroidism
name: Calcitriol
label: a
answer: yes
explanation: Calcitonin can occasionally be used in the treatment of hypercalcaemia by inhibiting osteoclast activity in bones and inhibiting renal reabsorption of calcium and phosphate. This patient is hypocalcaemic, and so this would reduce serum calcium levels further
name: Calcitonin
label: c
answer: no
explanation: Colecalciferol (vitamin D3) is the major form of vitamin D that provides nutritional supplementation. It should not be used to reduce PTH in those with normal vitamin D as an attempt to reduce the risk of progression into tertiary hyperparathyroidism. It can worsen hyperphosphataemia and lead to hypercalcaemia which can worsen cardiovascular morbidity
name: Colecalciferol
label: b
answer: no
explanation: Colecalciferol should not be used to reduce PTH in those with normal vitamin D as an attempt to reduce the risk of progression into tertiary hyperparathyroidism. It can worsen hyperphosphataemia and lead to hypercalcaemia which can worsen cardiovascular morbidity. This would be exacerbated by additional calcium carbonate supplementation and would not improve survival benefit
name: Colecalciferol with calcium carbonate
label: e
answer: no

Correct answer: a


Learning point: Calcitriol is essential in managing hypocalcaemia, particularly in chronic kidney disease, by enhancing calcium absorption and reducing parathyroid hormone levels.
A 26-year-old woman presents to the GP with paroxysmal allodynia and shock-like pains of the right side of her face. Cranial nerve examination and otoscopy are unremarkable.

Which of the following is the best next step in the management of this patient?

Options:
explanation: Lamotrigine is used as a tertiary line therapy for trigeminal neuralgia, although its evidence is limited, with a recent Cochrane review suggesting it has no efficacy in its management. Carbamazepine is the only therapy with proven efficacy in randomised controlled trials
name: Lamotrigine
label: e
answer: no
explanation: While duloxetine is used in other forms of neuropathic pain, there is no evidence for its use in trigeminal neuralgia
name: Duloxetine
label: b
answer: no
explanation: Carbamazepine is currently the first line for the treatment of trigeminal neuralgia. It acts by inhibiting voltage-gated sodium channels, thereby reducing excitability of neural membranes. It is the only therapy with proven efficacy in randomised controlled trials in treating trigeminal neuralgia
name: Carbamazepine
label: a
answer: yes
explanation: Gabapentin is used as a tertiary line therapy for trigeminal neuralgia, although there is little evidence of its use in randomised controlled trials. Carbamazepine is the only therapy with proven efficacy in randomised controlled trials
name: Gabapentin
label: d
answer: no
explanation: While amitriptyline is used in other forms of neuropathic pain, there is no evidence for its use in trigeminal neuralgia
name: Amitriptyline
label: c
answer: no

Correct answer: a


Learning point: Carbamazepine is the first-line treatment for trigeminal neuralgia, effectively reducing neural excitability by inhibiting voltage-gated sodium channels.
A 71-year-old woman presents to the GP with progressive bilateral facial weakness and diplopia for the past two months, worst in the evenings.

External examination findings are below:

[lightgallery]

Which of the following investigations is most likely to confirm the diagnosis?

Options:
explanation: A lumbar puncture can be used to help diagnose a variety of neurological conditions. It has no role in the diagnosis of myasthenia gravis and so would only be considered if previous investigations were negative and the diagnosis was unknown
name: Lumbar puncture
label: e
answer: no
explanation: MRI brain can be used to help diagnose a variety of neurological conditions. For myasthenia gravis, it can be used to exclude compressive and inflammatory lesions of the cranial nerves and ocular muscles but is less likely to be diagnostic
name: MRI brain
label: d
answer: no
explanation: Anti-VGCC antibodies can be seen in Lambert-Eaton myasthenic syndrome. Given the patient is not complaining of proximal arm or leg weakness, but predominantly facial weakness, this favours myasthenia gravis as the most likely diagnosis
name: Anti-voltage-gated calcium channels (Anti-VGCC) antibodies
label: c
answer: no
explanation: This patient is experiencing progressive facial weakness, bilateral ptosis, and diplopia - worse on progressive use, suggestive of myasthenia gravis. The first-line investigation is serum AChR antibodies (present in 85% of patients)
name: Acetylcholine receptor (AChR) antibodies
label: a
answer: yes
explanation: Thyroid function tests should be done in patients with suspected myasthenia gravis as they can often co-exist together. The best diagnostic test for myasthenia gravis, however, are AChR antibodies
name: Thyroid function tests
label: b
answer: no

Correct answer: a


Learning point: Myasthenia gravis is an autoimmune neuromuscular disorder characterized by weakness and fatigue of voluntary muscles, often associated with the presence of acetylcholine receptor (AChR) antibodies that disrupt nerve signal transmission at the neuromuscular junction.
A 21-year-old woman presents to the emergency department having tripped and fallen onto her right hand. X-ray shows a minimally-displaced clavicular fracture.

On neurological exam, she has bilateral loss of vibration sense in her hands and feet with peripheral sensory loss. She has brisk knee reflexes and upgoing plantars bilaterally.

On further questioning, she states she has been tripping and falling more frequently recently. She reveals that she has been inhaling nitrous oxide recreationally every day for the last 6 months.

Which of the following is the most likely diagnosis?

Options:
explanation: Excessive nitrous oxide inhalation oxidises the cobalt ion of vitamin B12, interfering with its bioavailability as a co-enzyme. Patients can then develop clinical symptoms of B12 deficiency, particularly subacute combined degeneration of the cord. This affects the posterior and lateral columns of the spinal cord, presenting with gradual upper and lower limb weakness, peripheral paraesthesia, and upper lower motor neurone signs, particularly with loss of vibration and touch sense
name: Subacute combined degeneration of the cord
label: a
answer: yes
explanation: Syringomyelia presents with a bilateral loss of sensation to pain and temperature, but with sparing of light touch, vibration, and proprioception. It typically affects the neck, shoulders, and arms when a syrinx is present in the cervical spine
name: Syringomyelia
label: c
answer: no
explanation: Hypoxic brain injury initially affects areas of high metabolic activity, predominantly the hippocampi, thalami, cerebral cortex, corpus striatum, and cerebellar vermi. The consequences of such can be predicted by the areas affected, such as memory impairment, ataxia, poor motor control, and difficulties with complex learning tasks. It would not present with a normal cognitive state and progressive peripheral paraesthesia
name: Hypoxic brain injury
label: b
answer: no
explanation: While oxidative stress is known to result in neuroinflammation and subsequent neurodegeneration, it not a known complication from excessive nitrous oxide use
name: Oxidative toxic neuropathy
label: e
answer: no
explanation: Guillain-Barré Syndrome (GBS) presents with progressive ascending weakness of all limbs. Reflexes are reduced or absent and, if paraesthesia is present, it is often mild
name: Guillain-Barré Syndrome
label: d
answer: no

Correct answer: a


Learning point: Excessive nitrous oxide inhalation can lead to subacute combined degeneration of the cord due to vitamin B12 deficiency.
A 52-year-old woman presents to the GP with acute right foot drop and loss of sensation on the dorsum of the foot. One month ago, she developed ulnar nerve palsy in her left hand, and six months ago, she had developed isolated 4th nerve palsy.

Which of the following conditions is associated with this disorder?

Options:
explanation: Motor neurone disease presents with gradual progression of upper and lower motor neurone signs. It would not present with the sensory deficit and would not present as rapidly as this
name: Motor neurone disease
label: d
answer: no
explanation: Multiple sclerosis typically presents with temporary visual or sensory loss with gradual progression. It can cause spastic weakness, ataxia, and tremor, but wouldn't present with sudden neurological deficit
name: Multiple sclerosis
label: b
answer: no
explanation: Syringomyelia presents with a bilateral loss of sensation to pain and temperature, but with sparing of light touch, vibration, and proprioception. It typically affects the neck, shoulders, and arms when a syrinx is present in the cervical spine. While it can present suddenly with sensory loss, it would not provide the pattern of distribution found in this patients examination findings
name: Syringomyelia
label: e
answer: no
explanation: This patient is experiencing symptoms of mononeuritis multiplex, sporadically developing peripheral neuropathies most commonly from nerve ischaemia secondary to primary or secondary vasculitis. There are multiple causes for this, broadly classifying them into ischaemic (e.g. diabetes mellitus & peripheral nerve vasculitis), inflammatory (e.g. sarcoidosis, rheumatoid arthritis, and Guillain-Barré Syndrome), infectious (e.g. Lyme disease and cytomegalovirus), drug-induced (sulfonamides and hydralazine), genetic, malignant, or primary vasculitides (e.g. giant cell arteritis, granulomatosis with polyangiitis, or polyarteritis nodosa)
name: Rheumatoid arthritis
label: a
answer: yes
explanation: B12 deficiency presents with gradually progressive peripheral paraesthesia. It would not present with such sudden onset with a seemingly random distribution of the peripheral nerves
name: B12 deficiency
label: c
answer: no

Correct answer: a


Learning point: Mononeuritis multiplex can occur in rheumatoid arthritis, presenting as multiple isolated peripheral neuropathies due to nerve ischaemia or inflammation.
A 68-year-old man has presented to accident and emergency with sudden onset vertigo, unchanged on position, with no hearing impairment or tinnitus. He has new hoarseness and dysphagia, struggling to swallow his saliva.

On examination head impulse test is negative; he has right-sided unilateral vertical nystagmus and right-sided skew deviation. He has reduced sensation on the right side of his face with right-sided ptosis and miosis.

Which of the following is the most likely diagnosis?

Options:
explanation: Vertebrobasilar insufficiency may present in this way, although symptoms are transient, similar to a posterior circulation TIA. They are often triggered by causes of transient reduced cerebral perfusion, such as orthostatic hypotension, which can accentuate previously unknown symptoms
name: Vertebrobasilar insufficiency
label: d
answer: no
explanation: Pontine infarcts can present with a range of signs from ipsilateral cranial nerve palsy (CN V, VI, VII, and VIII) with contralateral motor/sensory impairment to locked-in syndrome. A similar condition to PICA syndrome - lateral pontine syndrome - would likely produce similar symptoms with the addition of ipsilateral facial nerve palsy due to involvement of the facial nucleus
name: Pontine infarct
label: c
answer: no
explanation: Weber syndrome presents as ipsilateral oculomotor nerve palsy and contralateral weakness
name: Weber syndrome
label: b
answer: no
explanation: Lacunary infarcts present with relatively distinct symptoms based on the location within the internal capsule. 1) contralateral hemiparesis with dysarthria and dysphagia 2) ipsilateral cerebellar symptoms 3) hemiparesis with contralateral sensory impairment 4) Pure hemisensory loss. It would unlikely present with Horner's syndrome
name: Lacunar infarct
label: e
answer: no
explanation: Sudden onset vertigo, nystagmus, and ipsilateral dysphagia, reduced facial sensation, and Horner's syndrome suggests a central cause, particularly dysfunction of the lateral part of the medulla. This is commonly caused by a posterior inferior cerebellar artery or vertebral artery ischaemia. The sensation of vertigo and nystagmus arises from disruption of the lateral vestibular nucleus; dysphagia due to damage to cranial nerves IX & X; reduced facial sensation from the trigeminal nerve root; and Horner's syndrome from the hypothalamospinal fibres where sympathetic fibres run
name: Posterior inferior cerebellar artery syndrome (PICA syndrome)
label: a
answer: yes

Correct answer: a


Learning point: Posterior inferior cerebellar artery syndrome presents with vertigo, nystagmus, dysphagia, facial sensory loss, and Horner's syndrome due to medullary ischaemia.
A 28-year-old man with antiphospholipid syndrome presents with sudden painless monocular vision loss while driving. Fundoscopy shows macular oedema and multiple intra-retinal haemorrhages in each quadrant in the right eye. There is also a relative afferent pupillary defect.

Which of the following is the most likely diagnosis?

Options:
explanation: Ocular migraines can present with transient monocular blindness or flashing lights. They would not produce the fundoscopy findings shown above
name: Ocular migraine
label: b
answer: no
explanation: Optic neuritis is a gradual worsening of vision due to inflammation of the optic nerve, such as in multiple sclerosis. It would not present with sudden monocular vision loss and would not produce intra-retinal haemorrhages on fundoscopy
name: Optic neuritis
label: d
answer: no
explanation: Vertebral artery dissection presents as a severe occipital headache as well as posterior fossa ischaemic symptoms manifesting as ataxia, dysarthria, lateral medullary syndrome, and visual symptoms. It would not present with monocular vision loss or with the fundoscopy findings above
name: Vertebral artery dissection
label: e
answer: no
explanation: Usually, CRVO presents in older patients with cardiovascular history, diabetes, or hyperlipidaemia. This patient has antiphospholipid syndrome, a condition known to cause a hypercoagulable state, and so puts them at increased risk of thromboembolic disease. Sudden onset monocular vision loss with relative afferent pupillary defect (RAPD) implies a lesion of the optic nerve. As the retinal artery and veins are the sole source of blood supply to the retina, occlusion of either causes severe damage, blindness, and macular oedema
name: Central retinal vein occlusion (CRVO)
label: a
answer: yes
explanation: Retinal detachment typically presents with amaurosis fugax, floaters, and flashing lights. If the retinal detachment were so severe that it would cause full monocular blindness, then it would be visible on fundoscopy. Given the patient's history of antiphospholipid syndrome with a hypercoagulable state, thromboembolic disease is more likely such as central retinal artery/vein occlusion
name: Retinal detachment
label: c
answer: no

Correct answer: a


Learning point: Antiphospholipid syndrome is a significant risk factor for central retinal vein occlusion (CRVO) as it promotes hypercoagulability, increasing the likelihood of blood clot formation in the retinal veins.
A 60-year-old man is referred for an outpatient gastroscopy following worsening reflux symptoms over the last six months.

Gastrosopcy shows a red, velvety appearance of the oesophageal mucosa and biopsies from this region show "metaplastic columnar epithelium replacing the oesophageal squamous epithelium". There are no areas of dysplasia identified.

Which of the following is the best step in management?

Options:
explanation: High dose PPI alone should be combined with regular surveillance endoscopies to monitor for the development of dysplasia and adenocarcinoma
name: High dose PPI
label: e
answer: no
explanation: RFA is a treatment option for Barrett's with high-grade dysplasia as there is a more significant risk of progressing to cancer
name: Radiofrequency ablation (RFA)
label: c
answer: no
explanation: There is no role for oesophagectomy in Barrett's. Oesophagectomy is a treatment option for oesophageal carcinoma
name: Referral to the multi-disciplinary team (MDT) for consideration of an oesophagectomy
label: d
answer: no
explanation: The histological appearances are consistent with a diagnosis of Barrett's oesophagus. This is usually a consequence of long-standing reflux disease and describes the abnormal, reversible change of the oesophageal epithelium from stratified squamous to simple columnar. The major risk factor of Barretts's is oesophageal adenocarcinoma. Treatment is with high dose PPI and regular endoscopies to screen for the development of dysplasia and adenocarcinoma
name: High dose proton-pump inhibitor (PPI) and surveillance endoscopies
label: a
answer: yes
explanation: EMR is a treatment option for Barrett's with high-grade dysplasia as there is a more significant risk of progressing to cancer
name: Endoscopic mucosal resection (EMR)
label: b
answer: no

Correct answer: a


Learning point: Barrett's oesophagus is characterized by metaplastic changes in the oesophageal lining resulting from chronic gastro-oesophageal reflux disease, requiring PPI therapy and regular surveillance.
A 65-year-old male attends the Emergency Department complaining of progressive numbness and weakness that started in his legs and moved slowly up his body. He has recently had an episode of diarrhoea and vomiting after eating a "dodgy takeaway".

He has been transferred to the hospital's adjoining tertiary neurology centre with a presumed diagnosis of Guillain-Barré Syndrome (GBS). On arrival in the neurology ward, he appears breathless.

What is the most appropriate investigation to help predict the need for intubation?

Options:
explanation: An arterial blood may be normal in Guillain-Barré Syndrome, with hypoxia or hypercapnoea being a late sign of impending neuromuscular respiratory failure. Spirometry is recommended as a more accurate reflection of a patients requirement for respiratory support
name: Arterial blood gas (ABG)
label: d
answer: no
explanation: In the absence of signs and symptoms of pulmonary embolism, CTPA is unlikely to be of value in this case. A normal CTPA does not preclude the need for intubation in Guillain-Barré Syndrome
name: CT pulmonary angiogram (CTPA)
label: e
answer: no
explanation: Whilst a chest x-ray would rule out other causes of breathlessness, this patient is presumed to have Guillain-Barré Syndrome, and a chest x-ray is likely to be normal, and would not help to predict the need for intubation
name: Chest x-ray
label: b
answer: no
explanation: A lumbar puncture in Guillain-Barré Syndrome typically shows an elevated cerebrospinal fluid (CSF) protein with a normal cell count. LP findings do not correlate with severity of illness or need for intubation
name: Lumbar puncture (LP)
label: c
answer: no
explanation: This patient has Guillain-Barré Syndrome (GBS), characterised by the preceding gastrointestinal illness and the pattern of bilateral ascending weakness. Bedside spirometry, particularly forced vital capacity (FVC) has a significant correlation with the risk of respiratory failure and need for intubation. If the FVC < 20mL/Kg, the patient likely needs intensive care, and if <15mL/Kg, likely requires intubation
name: Bedside spirometry
label: a
answer: yes

Correct answer: a


Learning point: In Guillain-Barré Syndrome (GBS), bedside spirometry is used to assess respiratory function, detecting weakness in respiratory muscles. Reduced vital capacity and peak flow suggest the need for respiratory support or monitoring.