Passmedicine Flashcards
1
Q
A 75-year-old man has attended the falls clinic with a 1-month history of an increased frequency of falls. In the last month, he reports falling 5 times. Prior to this, he was independently mobile but now requires the assistance of a frame. He lives with his wife who says that he has brief episodes of confusion following by lucid periods. His past medical history includes hypertension, alcoholic fatty liver disease, and gout.
On examination, he has normal power and sensation in his upper limbs. He has a shuffling gait but there is generally good power in his lower limbs. His cranial nerve examination is unremarkable aside from being unable to abduct his left eye on the left lateral gaze.
What is the most likely diagnosis?
Hepatic encephalopathy Lewy body dementia Normal-pressure hydrocephalus Subdural haematoma Wernicke’s encephalopathy
A
Subdural haematoma
Fluctuating confusion/consciousness? - subdural haematoma
In the elderly population and particularly those with a background of alcohol excess, a combination of fluctuating confusion, and increased frequency of falls should raise suspicion of a subdural haematoma. The presence of a left abducens nerve palsy is suggestive of a false localising sign from a space-occupying lesion. This patient should have a CT head which would demonstrate the presence of a subdural haematoma, a lentiform-shaped collection of blood often from rupture of cortical bridging veins.
Hepatic encephalopathy is staged as minimal, mild, moderate, severe and comatose. The minimal and milder forms of the disease can be difficult to distinguish from other disease presentations. However, given the absence of further signs of decompensated liver disease including ascites and jaundice, hepatic encephalopathy seems less likely to be the underlying cause.
Lewy body dementia tends to present with fluctuating cognitive impairment. Hallucinations may also be seen as well as sleep disturbance. Patients with LBD can also experience Parkinsonian motor symptoms including rigidity, bradykinesia and a shuffling gait. However, LBD would not explain this patient’s abducens nerve palsy.
Normal-pressure hydrocephalus is the presence of excess cerebrospinal fluid in the brain without an increase in intracranial pressure. The condition typically presents as a triad of dementia, gait apraxia and urinary or faecal incontinence. It should be considered as a differential diagnosis in this patient. However, the history of fluctuating confusion is more suggestive of a subdural haematoma.
Wernicke’s encephalopathy is a reasonable differential given the history of alcohol excess. However, in Wernicke’s encephalopathy cerebellar signs tend to predominate such as gait ataxia, nystagmus and ophthalmoplegia.
2
Q
A 45-year-old woman with multiple sclerosis is commenced by your consultant on baclofen for management of muscle spams.
What is the mechanism of action of this medication?
Dopamine antagonist GABA receptor agonist Muscarinic receptor agonist NMDA receptor antagonist Serotonin–norepinephrine reuptake inhibitor (SNRI)
A
Baclofen is an agonist of GABA receptors that acts in the central nervous system
Baclofen is a GABA receptor agonist, which acts in the central nervous system. It is used to treat muscle spasticity.
Pilocarpine is an example of a muscarinic receptor agonist, used in the treatment of glaucoma.
Ketamine is an example of an NMDA receptor antagonist.
Serotonin–norepinephrine reuptake inhibitors (SNRIs) such as venlafaxine are antidepressant medications .
Dopamine antagonists include antipsychotics (such as haloperidol) and antiemetics (such as metoclopramide and domperidone).
3
Q
A 20-year-old man presents to the neurology clinic with a 6 month history of deteriorating gait.
On examination he has a wide based gait, with past pointing and high arched feet. Knee and ankle reflexes are absent, but he has an extensor plantar response bilaterally. Fundoscopy reveals a pale optic disc. There is no impairment of cognition.
What is the most likely diagnosis?
Wilson's disease Friedrich's ataxia Charcot-Marie-Tooth disease Motor neuron disease Bardet-Biedl syndrome
A
Friedrich’s ataxia
The question describes someone with cerebellar signs, mixed lower motor neuron and upper motor neuron signs, pes cavus, optic atrophy with a normal IQ.
All of which would be present in Friedrich’s ataxia. This normally presents in childhood and is autosomal recessive. Global spinal cord and cerebellar degeneration give a mixed patten of degeneration. Retinal degeneration is common, as are cardiomyopathies and diabetes.
Wilson’s disease can give ataxia due to excess copper deposition, however over neurological features may be Parkinsonian in nature e.g. resting tremor and bradykinesia. Wilson’s does not give peripheral neuropathy. A typical Kayser-Fleischer ring of copper may be seen in the iris of patients, no retinal changes are seen.
Charcot-Marie-Tooth (also known as hereditary motor and sensory neuropathy), presents with pes cavus and peripheral motor/sensory neuropathy, but would not give cerebellar or visual symptoms.
Motor neuron disease presents with mixed upper and lower motor neuron weakness - as is seen here with absent tendon reflexes and extensor plantars, however it does not give cerebellar signs or affect the retina.
Bardet-Biedl syndrome is a rare autosomal recessive condition that gives retinitis pigmentosa, but also presents with obesity, polydactyly and frequently mental retardation.
4
Q
A 28-year-old woman presents with a headache. For the four weeks, she has noticed an increased frequency of headaches, occurring nearly every day. She has a history of migraines but explains these headaches feel different. She usually takes paracetamol and sumatriptan, which she has taken every few days for the past couple of months.
Given the most likely diagnosis, what is the most appropriate management option?
Discontinue paracetamol and sumatriptan immediately
Slowly withdraw both paracetamol and sumatriptan
Withdraw paracetamol first, followed by sumatriptan
Withdraw sumatriptan first, followed by paracetamol
Withdraw sumatriptan only
A
Discontinue paracetamol and sumatriptan immediately
Medication overuse headache
simple analgesia + triptans: stop abruptly
opioid analgesia: withdraw gradually
Discontinue paracetamol and sumatriptan immediately is the correct answer. Given her history of migraines and her frequent increased use of paracetamol and triptans, the most likely cause of her headaches is medication overuse. Therefore, as she had been taken simple analgesia and triptans only, these can be stopped abruptly.
Reducing the dose/ frequently gradually is incorrect. Gradual withdrawal is only required in opioid analgesia where as in this scenario the patient is taking paracetamol and triptans only.
Increasing the dose of sumatriptan would not be helpful here. As explained, the scenario points more towards medication overuse headaches, rather than worsening migraines.
Increasing the dose of paracetamol is also incorrect for the same reason.
5
Q
A 52-year-old man is prescribed apomorphine. What type of receptors does apomorphine act on?
Opioid receptors GABA receptors Cholinergic receptors Dopamine receptors Muscarinic receptors
A
Dopamine receptors
Apomorphine - dopamine receptor agonist
Apomorphine is one of the older dopamine receptor agonists. Newer agents such as ropinirole and cabergoline have since been developed
6
Q
A 45-year-old woman who works in a children’s nursery presents due to ‘weakness’ in her right foot. This has developed over the past couple of days. She is otherwise well and reports no other symptoms.
On examination, you note a high-stepping gait on the right side. Weakness of the right foot dorsiflexors is noted. All reflexes in the lower limb are normal and no fasciculations are noted. Hip abduction strength is normal.
What is the most appropriate management?
Admit for CT head and lumbar puncture
Urgent referral to neurology
Advice to avoid leg crossing, squatting or kneeling and review in 4 weeks
Advise the patient to increase their vitamin D consumption
Arrange nerve conduction studie
A
Advice to avoid leg crossing, squatting or kneeling and review in 4 weeks
Leg crossing, squatting or kneeling may cause a foot drop secondary to a common peroneal neuropathy
This patient has foot drop secondary to a common peroneal nerve lesion. This may have been precipitated by kneeling and squatting in her job at the nursery.
In the absence of other findings, conservative management with advice is appropriate initially.
7
Q
A 66-year-old male attends a low clearance renal clinic. He was last seen 2 months ago (eGFR was 18 ml/min/1.73 m2). Over the last 6 months, he has developed an unpleasant sensation in both legs that he describes as throbbing and crawling. Whilst lying in bed, he has the uncontrollable urge to move his legs.
His past medical history includes chronic kidney disease secondary to type II diabetes, hypertension, osteoarthritis and gout. His medications include ferrous fumarate, sodium bicarbonate, glargine (Lantus), ramipril, allopurinol, sevelamer and cholecalciferol.
Given the likely diagnosis, what is the mechanism of action of the first-line drug that will be used to treat his symptoms?
Dopamine agonist Gamma-aminobutyric acid (GABA) agonist Muscarinic agonist Muscarinic antagonist Serotonin and noradrenaline neuronal reuptake inhibitor
A
Restless leg syndrome - management includes dopamine agonists such as ropinirole
Restless legs syndrome (RLS) is a syndrome of spontaneous, continuous lower limb movements that may be associated with paraesthesia. It is extremely common, affecting between 2-10% of the general population. Males and females are equally affected and a family history may be present
Clinical features
uncontrollable urge to move legs (akathisia). Symptoms initially occur at night but as condition progresses may occur during the day. Symptoms are worse at rest
paraesthesias e.g. ‘crawling’ or ‘throbbing’ sensations
movements during sleep may be noted by the partner - periodic limb movements of sleeps (PLMS)
Causes and associations there is a positive family history in 50% of patients with idiopathic RLS iron deficiency anaemia uraemia diabetes mellitus pregnancy
The diagnosis is clinical although bloods such as ferritin to exclude iron deficiency anaemia may be appropriate
Management
simple measures: walking, stretching, massaging affected limbs
treat any iron deficiency
dopamine agonists are first-line treatment (e.g. Pramipexole, ropinirole)
benzodiazepines
gabapentin
8
Q
A 34-year-old man with a history of migraine finds that paracetamol taken at the recommend dose often fails to relieve his acute attacks. He drinks 12 units of alcohol per week and smokes 15 cigarettes per day.
What factor is likely to contribute to this problem?
Bacterial overgrowth
Delayed gastric emptying
P450 enzyme induction
First pass metabolism
P450 enzyme inhibition
A
Delayed gastric emptying
Patients with migraine experience delayed gastric emptying during acute attacks. For this reason analgesics are often combined prokinetic agents such as metoclopramide. Paracetamol metabolism would not be significantly affected by changes in P450 enzyme activity (e.g. through smoking or drinking)
9
Q
A 35-year-old man is reviewed in the neurology outpatient clinic. He was referred by his general practitioner with a four month history of an insidious onset of abnormal limb movements. He has not significant past medical history and is on no regular medications.
On examination, there are sudden, brief (< 100 ms) and almost shock-like involuntary single jerks of his face and right upper limb. The movements are stimulus-sensitive and provoked by touching. The jerks are not continuous and there is some interruption. Neurological assessment is otherwise unremarkable.
A CT head is organised, which demonstrated a lesion at the left sensori-motor cortex.
This is discussed at the neuroradiology MDT and the appearances are felt to be benign and not requiring surgical intervention.
Given the likely diagnosis, what is the most appropriate medication choice to control his symptoms?
Clonazepam
Lamotrigine
Levetiracetam
Topiramate
Sodium valproate
A
Sodium valproate
Sodium valproate is the correct answer. This patient has developed myoclonic seizures likely secondary to an intracerebral lesion. Myoclonus is characterised by sudden, brief, shock-like jerks. They are often stimulus sensitive. Focal cortical myoclonus is the most common form of myoclonus and is usually caused by an underlying lesion of the sensori-motor cortex, which produces hyperexcitability (e.g. vascular, inflammatory or neoplastic). It is deemed that this lesion does not require surgical intervention and therefore pharmacological treatment is appropriate. Sodium valproate is the first-line option in treating myoclonic seizures.
10
Q
A 27-year-old woman with epilepsy manifesting as tonic-clonic seizures has been relatively well-controlled for the last 14 months on combination therapy of sodium valproate and levetiracetam. Two months after giving birth she has had a week of seizures every day and has now been started on clobazam, in addition to the anti-seizure medications listed above.
She would like to continue to breastfeed her baby and has read that some medications can harm the baby.
What medication change should occur?
No medication changes
Stop clobazam
Stop clobazam and sodium valproate
Stop levetiracetam
Stop sodium valproate
A
No medication changes
Breast feeding is acceptable with nearly all anti-epileptic drugs
11
Q
Which one of the following factors indicates a poor prognosis in patients with multiple sclerosis?
Relapsing-remitting disease
Presence of sensory symptoms
Young age of onset
Male sex
Long interval between first two relapses
A
Male sex
12
Q
A 29-year-old female presents complaining of weakness in her arms, leading to increasing difficulties at work. On examination she has a bilateral ptosis and loss of the red-reflex in both eyes. Urine testing also reveals glycosuria. What is the most likely diagnosis?
Myotonic dystrophy
Homocystinuria
Multiple sclerosis
Myasthenia gravis
HIV
A
Dystrophia myotonica DM1 distal weakness initially autosomal dominant diabetes dysarthria
These features are typical of myotonic dystrophy. The red-reflex is lost due to bilateral cataracts
13
Q
A 65-year-old gentleman is admitted to the medical take with a progressively worsening headache for 3 weeks. On closer questioning, he reveals it is worse first thing in the morning but also exacerbated by recumbency and coughing. He is also complaining of intermittent visual disturbances and on fundoscopic examination, there is papilloedema of the right disc but optic atrophy on the left.
A subsequent CT scan is performed revealing a space occupying lesion. Where is the space occupying lesion most likely situated?
Left temporal Right frontal Right temporal Left frontal Left parietal
A
Left frontal
This is an interesting case of Foster-Kennedy syndrome. This syndrome reflects a frontal lobe tumour - usually a meningioma in this age group - leading to ipsilateral optic atrophy and papilloedema of the contralateral optic nerve. The reason for the optic atrophy is as a result of direct damage from the space occupying lesion. Other causes are AVMs and juvenile nasopharyngeal angiofibroma.
14
Q
A 28-year-old lady presents with a two-day history of headaches, nausea and vomiting, and blurred vision.
She is long-sighted and has worn glasses since childhood. She has a background of severe migraines and was recently started on a prophylactic medication by her GP.
On examination, both eyes are red with mid-dilated unreactive pupils bilaterally.
Which medication is the most likely cause of this presentation?
Aspirin Propranolol Sodium valproate Sumatriptan Topiramate
Topiramate can precipitate acute angle closure glaucoma
A
Topiramate
Topiramate can precipitate acute angle closure glaucoma
Topiramate is used as a first-line agent in the prophylaxis of migraine. It is an important cause of drug-induced acute angle closure glaucoma, typically occurring within one month of treatment. Topiramate-induced acute angle closure glaucoma is usually treated with cycloplegia and topical steroids alone rather than laser peripheral iridotomy.
Propranolol can be used as a first-line prophylactic treatment for people with episodic or chronic migraine but it is not known to be associated with acute angle closure glaucoma. Adrenergic drugs, in contrast, can precipitate an attack.
Sodium valproate is licensed for migraine prophylaxis though paradoxically itself commonly causes headaches.
Sumatriptan can be used in the in the acute treatment of migraine. It can commonly cause nausea and vomiting, but not angle closure glaucoma.
High-dose aspirin can be used in the acute treatment of migraine. A single dose of 900mg can be taken as soon as the migraine symptoms develop.
15
Q
A 25-year-old female with a history of bilateral vitreous haemorrhage is referred due to progressive ataxia. What is the likely diagnosis?
Neurofibromatosis type I Neurofibromatosis type II Tuberose sclerosis Von Hippel-Lindau syndrome Sarcoidosis
A
Retinal and cerebellar haemangiomas are key features of Von Hippel-Lindau syndrome. Retinal haemangiomas are bilateral in 25% of patients and may lead to vitreous haemorr
16
Q
A 75-year old gentleman presents with a short history of neck pain, paraesthesia in his finger tips and progressive leg weakness. Following a MRI scan of his spine, he is diagnosed with degenerative cervical myelopathy due to a C4/5 disc prolapse. Which of the following is the most appropriate management?
Cervical decompressive surgery Cervical nerve root injection Analgesia and referral to physiotherapy Analgesia and review in 4 weeks time Analgesia, a hard cervical collar and review in 4 weeks
A
Cervical decompressive surgery
All patients with degenerative cervical myelopathy should be urgently referred for assessment by specialist spinal services (neurosurgery or orthopaedic spinal surgery). This is due to the importance of early treatment. The timing of surgery is important, as any existing spinal cord damage can be permanent. Early treatment (within 6 months of diagnosis) offers the best chance of a full recovery but at present, most patients are presenting too late. In one study, patients averaged over 5 appointments before diagnosis, representing >2 years [1].
Currently, decompressive surgery is the only effective treatment. It has been shown to prevent disease progression. Close observation is an option for mild stable disease, but anything progressive or more severe requires surgery to prevent further deterioration. Physiotherapy should only be initiated by specialist services, as manipulation can cause more spinal cord damage.
Prompt diagnosis and onward referral are therefore key to ensuring good outcome for your patients. There are national initiatives to raise awareness of the condition to try and improve referral times (www.myelopathy.org). All of the other listed options in this question do not control the patients primary pathology.
17
Q
65-year-old gentleman is admitted to the medical take with a progressively worsening headache for 3 weeks. On closer questioning, he reveals it is worse first thing in the morning but also exacerbated by recumbency and coughing. He is also complaining of intermittent visual disturbances and on fundoscopic examination, there is papilloedema of the right disc but optic atrophy on the left.
A subsequent CT scan is performed revealing a space occupying lesion. Where is the space occupying lesion most likely situated?
Left temporal Right frontal Right temporal Left frontal Left parietal
A
Left Frontal
This is an interesting case of Foster-Kennedy syndrome. This syndrome reflects a frontal lobe tumour - usually a meningioma in this age group - leading to ipsilateral optic atrophy and papilloedema of the contralateral optic nerve. The reason for the optic atrophy is as a result of direct damage from the space occupying lesion. Other causes are AVMs and juvenile nasopharyngeal angiofibroma
18
Q
A 21-year-old musician is brought to hospital after collapsing on stage. There was no loss of consciousness and he sustained only minor bruising to his knees. Another musician onstage at the time notes he had been laughing at the time before his legs suddenly ‘gave way’ and his mouth twitched for a few seconds.
The patient reports a similar episode occurring a few days previously. He has been extremely fatigued over the last several months, which he had attributed to touring.
ECG and blood tests are normal.
What is the most likely underlying diagnosis?
Gelastic seizures Narcolepsy Functional neurological disorder Tourette's syndrome Lennox Gaustaut syndrome
A
Narcolepsy
Laughter → fall/collapse ?cataplexy
Cataplexy describes the sudden and transient loss of muscular tone caused by strong emotion (e.g. laughter, being frightened). Around two-thirds of patients with narcolepsy have cataplexy.
Features range from buckling knees to collapse.
Gelastic seizures are not caused by laughter but the outburst of laughter is the seizure itself- this can be caused by brain neoplasms.
Tourette’s syndrome presents with verbal and physical ‘tics’.
Lennox Gastaut syndrome normally presents in childhood and can cause drop attacks but these are characterised by a single, generalized myoclonic jerk that precedes tonic contraction of axial muscles.
19
Q
A 45-year-old female with a history of epilepsy is reviewed in the neurology clinic. Which one of the following features is most likely to be attributable to sodium valproate therapy?
Clubbing Weight loss Hirsutism Renal impairment Tremor
A
Sodium valproate may cause tremor
Important for meLess important
Alopecia is much more common than hirsutism in patients treated with sodium valproate.
20
Q
Which one of the following conditions is least recognised as a cause of a seventh nerve palsy?
Acoustic neuroma Herpes zoster HIV Systemic lupus erythematosus Diabetes mellitus
A
SLE
21
Q
A 34-year-old female presents due to a number of ‘funny-dos’. She describes a sensation that her surroundings are unreal, ‘like a dream’. Following this she has been told that she starts to smack her lips, although she has no recollection of doing this. What is the most likely diagnosis?
Myoclonic seizure Focal aware seizure Focal impaired awareness seizure Focal to bilateral seizure Absence seizure
With focal aware seizures there is no disturbance of consciousness or awareness. Lip smacking is an example of an automatism - an automatic, repetitive act
A
Focal impaired awareness seizure
22
Q
A 65-year-old man with a history of Parkinson’s disease is referred to the respiratory clinic with shortness of breath. He has never smoked. Spirometry is performed:
Percentage
predicted
FEV1 71%
FVC 74%
Which one of the following drugs is most likely to be responsible?
Levodopa Entacapone Ropinirole Selegiline Pergolide
A
Pergolide
This patient has developed pulmonary fibrosis (explaining the restrictive picture on spirometry) secondary to pergolide therapy
23
Q
A 63-year-old woman with motor neuron disease is reviewed in clinic. Which one of the following interventions will have the greatest effect on survival?
Regular chest physiotherapy Total parental nutrition Riluzole Antioxidant supplementation Non-invasive ventilation
Motor neuron disease - treatment: NIV is better than riluzole
A
Motor neuron disease - treatment: NIV is better than riluzole
23
Q
A 63-year-old woman with motor neuron disease is reviewed in clinic. Which one of the following interventions will have the greatest effect on survival?
Regular chest physiotherapy Total parental nutrition Riluzole Antioxidant supplementation Non-invasive ventilation
A
Motor neuron disease - treatment: NIV is better than riluzole
24
A 35-year-old gentleman presents to general practice with neck swelling. He denies any other lumps and has no B symptoms. He has no significant past medical history and is on no medications.
On examination, you note a 5.5cm smooth and regular swelling in the anterior cervical lymph node chain. There is no other neck, groin, or axillary lymphadenopathy. There is no hepatosplenomegaly. Blood tests are all unremarkable.
He is thereafter referred for an ultrasound guided biopsy which reveals the presence of abnormal follicles with atrophic and hyalinized germinal centres surrounded by prominent mantle zones containing small lymphocytes. What is the most likely underlying condition?
```
Cat scratch fever
Myeloma
Hodgkins lymphoma
Unicentric Castleman's disease
HIV lymphadenitis
```
Unicentric Castleman's disease is a lymphoproliferative disorder associated in a subset of cases with HIV and HHV-8. Patient's with unicentric Castleman's disease tend to be asymptomatic and lymphadenopathy is constrained to one lymph node group.The most common sites of the disease being the chest (24 percent), neck (20 percent), abdomen (18 percent), and retroperitoneum (14 percent). Biopsy of the lymph node commonly shows regressed germinal centres surrounded by prominent mantle zones.
Here, there is no contact history with cats to suggest cat scratch fever. There is no history of HIV although multiple sites of lymphadenopathy could be caused by this infectious disease. Myeloma would include mention of a paraproteinaemia with plasma cells present on the biopsy with evidence of organ involvement. This biopsy result is not consistent with Hodgkin's lymphoma and there are no B symptoms.
25
Which of the following is least recognised as a cause of macroglossia?
```
Amyloidosis
Turner's syndrome
Duchenne muscular dystrophy
Acromegaly
Hurler syndrome
```
Causes of macroglossia
- hypothyroidism
- acromegaly
- amyloidosis
- Duchenne muscular dystrophy
- mucopolysaccharidosis (e.g. Hurler syndrome) - inherited condition where there is a lack of an enzyme that the body needs to digest sugar
- ?Down Syndrome
26
A 35-year-old female is admitted to hospital with hypovolaemic shock. CT abdomen reveals a haemorrhagic lesion in the right kidney. Following surgery and biopsy this is shown to be an angiomyolipoma. What is the most likely underlying diagnosis?
```
Neurofibromatosis
Budd-Chiari syndrome
Hereditary haemorrhagic telangiectasia
Von Hippel-Lindau syndrome
Tuberous sclerosis
```
Tuberous sclerosis
27
A 28-year-old woman with a history of systemic lupus erythematosus (SLE) presents with jerky, irregular movements which seem to move from one limb to another. Her symptoms are continuous and there are no other neurological features such as impairement of consciousness. Where is the neurological lesion most likely to be?
```
Cerebellum
Temporal lobe
Hippocampus
Thalamus
Caudate nucleus
```
Caudate nucleus
Chorea is caused by damage to the basal ganglia, in particular the Caudate nucleus
These symptoms are consistent with chorea, which may have a number of causes including SLE.
28
You are called to see a 62-year-old female inpatient, with a known history of epilepsy, who is having a seizure. The nurse who witnessed the seizure says it began by affecting her right hand before involving her entire right arm and then progressing to a loss of consciousness with her entire body shaking. What is the most likely diagnosis?
```
Generalised tonic-clonic
Focal impaired awareness seizure
Jacksonian march with secondary generalisation
Focal aware seizure
Myoclonic
```
Jacksonian march with secondary generalisation
A Jacksonian march is a type of focal aware seizure.
Focal highlights how it is focal epilepsy that involves abnormal electrical activity in just one part of the brain.
It characteristically starts by affecting a peripheral body part such as a toe, finger or section of the lip and then spreads quickly 'marches' over the respective foot, hand or face.
In some with Jacksonian march seizures (as in this case), the electrical disorder spreads over larger areas of the brain, causing the seizure to develop into a tonic-clonic seizure.
29
A 55-year-old woman with a background of metastatic breast cancer presents with a short history of nausea and vomiting. She has vomited five times today, despite taking regular metoclopramide. She received palliative chemotherapy three days ago.
You decide to commence treatment with ondansetron.
What is the best description of the mechanism of action of this medication?
```
Dopamine antagonist
H1 (histamine) antagonist
5-HT1 (serotonin) agonist
5-HT2 (serotonin) antagonist
5-HT3 (serotonin) receptor antagonist
```
5-HT3 (serotonin) receptor antagonist
30
Which one of the following is least recognised as a cause of autonomic neuropathy
```
Guillain-Barre syndrome
New variant CJD
Diabetes
Parkinson's
HIV
```
New variant CJD
31
Which one of the following is least recognised as a cause of autonomic neuropathy
```
Guillain-Barre syndrome
New variant CJD
Diabetes
Parkinson's
HIV
```
New variant CJD
32
A 38-year-old woman comes for review. Six months ago she fractured her left wrist whilst skiing. The fracture was treated using a cast and repeat x-rays showed that the bone had healed well. Unfortunately for the past few weeks she has been plagued with ongoing 'shooting pains' in her left hand associated with swelling. On examination the left hand is extremely tender to even light touch. Her left hand is also slightly swollen compared to the right. What is the most likely diagnosis?
```
Depression
Conversion disorder
Complex regional pain syndrome
Ulnar nerve injury
Osteomyelitis
```
Complex regional pain syndrome
Complex regional pain syndrome (CRPS) is the modern, umbrella term for a number of conditions such as reflex sympathetic dystrophy and causalgia. It describes a number of neurological and related symptoms which typically occur following surgery or a minor injury. CRPS is 3 times more common in women.
```
There are two types of CRPS:
type I (most common): there is no demonstrable lesion to a major nerve
type II: there is a lesion to a major nerve
```
Features
progressive, disproportionate symptoms to the original injury/surgery
allodynia
temperature and skin colour changes
oedema and sweating
motor dysfunction
the Budapest Diagnostic Criteria are commonly used in the UK
```
Management
early physiotherapy is important
neuropathic analgesia in-line with NICE guidelines
specialist management (e.g. Pain team) is required
```
33
A 32-year-old woman is brought into the emergency department by ambulance with following an episode in of vertigo, diplopia and dysarthria, after which she became drowsy and responsive to pain only. Her symptoms came on over ten minutes and lasted for approximately one hour. Her initial symptoms have now fully resolved, but she feels nauseous. Neurological examination is unremarkable. She has a past medical history of migraine. She takes the progesterone-only contraceptive pill, but no other medications.
What is the most likely diagnosis?
```
Focal aware seizure
Focal impaired awareness seizure
Migraine with brainstem aura
Transient ischaemic attack
Multiple sclerosis
```
Migraine with brainstem aura
Stroke and TIA are associated with sudden-onset 'negative' symptoms, migraine is more commonly associated with 'positive' symptoms
Migraine is common, with a prevalence of approximately 10% of the population. Therefore, even rare presentation of migraine may occur more commonly than common presentations of rarer conditions. This is migraine with brainstem aura (basilar-type migraine, or Bickerstaff's Syndrome). The gradual onset over ten minutes, and step-wise progression of symptoms point more towards migraine than an ischaemic event. A reminder that it is possible for migraine aura to occur without the subsequent headache, as in this case. New-onset seizures would be less likely than an atypical presentation of known migraine. Multiple sclerosis would not present like this.
34
A 67-year-old male undergoes investigations for bilateral paraesthesia in the radial aspects of both hands, over the thumbs and first fingers. He also has paraesthesia in the lateral aspects of both forearms and lower limb spasticity. Blood tests reveal a HBA1c of 46 mmol/mol. He undergoes nerve conduction studies and EMG with evidence of denervation. Which ONE of the following diagnoses is most likely?
```
Bilateral carpal tunnel syndrome
Degenerative cervical myelopathy
Multiple sclerosis
Syringomyelia
Diabetic neuropathy
```
Degenerative cervical myelopathy
Remember: LMN at the level of the lesion and UMN below the level of the lesion
35
A 25-year-old woman presents to the emergency department with a five-day history of fever, malaise, upper respiratory tract symptoms and painful skin. She then developed a dark red target pattern rash which has spread from the torso and upper limbs to involve her distal limbs, face, palms and soles.
She has recently been diagnosed with epilepsy and started on anti-convulsant medication.
On examination, there are widespread purpuric macules and confluent areas of erythema. There are scattered blisters and skin erosions including on her mucous membranes and labia. Gentle lateral pressure causes detachment of the epidermis.
Which medication is the most likely to have caused the presentation?
```
Gabapentin
Lamotrigine
Levetiracetam
Sodium valproate
Topiramate
```
Lamotrigine
36
A 58 year old gentleman presents with left sided paraesthesias affecting his thumb and first finger. He complains of grip weakness and dropping objects unintentionally. On examination, there is wasting over the thenar eminence. Which of the following signs would suggest a diagnosis other than carpal tunnel syndrome?
```
Positive Hoffmans sign
Thenar muscle wasting
Unilateral weakness of pincer grip
Positive Phalens test
Positive Tinnels test
```
Positive Hoffmans sign
A positive Hoffmans sign is a sign of upper motor neuron dysfunction and points to a disease of the central nervous system - in this case from the history degenerative cervical myelopathy [DCM] affecting the cervical spinal cord is most likely. To elicit it, the examiner should flick the patients distal phalanx (usually of the middle finger) to cause momentary flexion. A positive sign is exaggerated flexion of the thumb.
DCM is often missed initially and there is a delay in the diagnosis of this condition by >2 years in some studies [1]. This is a problem as delayed treatment limits recovery. It is most commonly misdiagnosed as carpal tunnel syndrome and in one study, 43% of patients who underwent surgery for degenerative cervical myelopathy, had been initially diagnosed with carpal tunnel syndrome [1]. DCM is therefore an important differential in patients suspected to have Carpal Tunnel Syndrome [CTS].
CTS is a disease of the peripheral nervous system, resulting from median nerve compression at the wrist inside the carpal tunnel. It therefore affects only the aspects of the hand innervated by the median nerve:
Sensation; Thumb / Index / Middle Finger. This typically manifests as intermittent pain or parasthesiae.
Motor; LOAF Muscles(lateral lumbricals, opponens pollicis, abductor pollicis brevis and flexor policis brevis). Motor signs are less commonly seen with presentations of CTS, but wasting of the thenar eminence may be present.
Tinels test and Phalens test can be positive, but not always. Both tests aim to increase the pressure within the carpal tunnel, to try to exacerbate symptoms; Tinels test via tapping on it and Phalens test by sustained full flexion of the wrist.
In focal central nervous system disorders, like DCM, examination features are known to have low sensitivity but high specificity [2]. As a disease of the cervical spinal cord, DCM can affect the sensory, motor and autonomic nervous systems from the neck downwards. Motor signs will be upper motor neuron signs such as increased toned, hyper-reflexia and pyramidal weakness. Note that the neurological signs of DCM are often subtle initially and easily missed, but as a progressive condition they are likely to get worse [3]. Therefore detecting early DCM can be challenging. A high index of suspicion, alongside a comprehensive neurological examination and monitoring for progression is require
37
A 45-year-old man is investigated for spastic leg weakness which has come on over the previous 5 days.
He undergoes a whole spine MRI which shows:
Hyperintense T2 signal extending across the spinal cord, between the levels of T9 and T12.
Which of the following is most likely to cause this clinical and radiological presentation?
```
Conus medullaris syndrome
Human immunodeficiency virus
Cauda equina syndrome
Diabetes mellitus
Brown-Sequard syndrome
```
Human immunodeficiency virus
Transverse myelitis can be caused by viral infection - such as varicella, herpes simplex, EBV and HIV
38
On examination, both eyelids display normal strength. With the left eye closed, the right eye displays a full range of movement. With the right eye closed, the left eye fails to adduct when looking towards the right. Nystagmus on the right eye is noted when the patient is asked to look to the right with both eyes. On convergence, both eyes can adduct towards the midline. The pupillary exam is normal with both pupils reacting appropriately to light.
What is the underlying pathology that has caused his diplopia?
Extrinsic compression of left oculomotor nerve
Intrinsic vascular damage to the left oculomotor nerve
Lesion of the left abducens nerve
Lesion on the left paramedian area of pons and medulla
Lesion on the left paramedian area of the midbrain and pons
Lesion on the left paramedian area of the midbrain and pons
The medial longitudinal fasciculus is located in the paramedian area of the midbrain and pons
Internuclear ophthalmoplegia is a disorder of conjugate gaze with the failure of adduction seen on the affected eye. This is caused by injury on the medial longitudinal fasciculus, located along the paramedian area of the midbrain and pons. In younger patients, lesions can be caused by multiple sclerosis but older populations can get this due to strokes. Convergence is maintained as this does not involve the medial longitudinal fasciculus. Convergence is mediated by the oculomotor nerve and the Edinger-Westphal nucleus.
38
On examination, both eyelids display normal strength. With the left eye closed, the right eye displays a full range of movement. With the right eye closed, the left eye fails to adduct when looking towards the right. Nystagmus on the right eye is noted when the patient is asked to look to the right with both eyes. On convergence, both eyes can adduct towards the midline. The pupillary exam is normal with both pupils reacting appropriately to light.
What is the underlying pathology that has caused his diplopia?
Extrinsic compression of left oculomotor nerve
Intrinsic vascular damage to the left oculomotor nerve
Lesion of the left abducens nerve
Lesion on the left paramedian area of pons and medulla
Lesion on the left paramedian area of the midbrain and pons
Lesion on the left paramedian area of the midbrain and pons
The medial longitudinal fasciculus is located in the paramedian area of the midbrain and pons
Internuclear ophthalmoplegia is a disorder of conjugate gaze with the failure of adduction seen on the affected eye. This is caused by injury on the medial longitudinal fasciculus, located along the paramedian area of the midbrain and pons. In younger patients, lesions can be caused by multiple sclerosis but older populations can get this due to strokes. Convergence is maintained as this does not involve the medial longitudinal fasciculus. Convergence is mediated by the oculomotor nerve and the Edinger-Westphal nucleus.
39
In patients with Guillain-Barre syndrome, respiratory function should be monitored with:
```
Oxygen saturations
PEFR
Flow volume loop
Arterial blood gases
Forced vital capacity
```
Forced vital capacity
40
A 44-year-old male is seen in Neurology clinic after presenting with a subacute history of a headache. His headache seems to be worse on recumbency and coughing/sneezing.
On examination, upper and lower limbs are normal with intact cranial nerves. On closer examination of the oropharynx, there is evidence of palatal myoclonus. A CT scan confirms a space occupying lesion. Where is the lesion likely located?
```
Temporal lobe
Olivary nucleus
Parietal lobe
Occipital lobe
Frontal lobe
```
Olivary nucleus
Palatal myoclonus is a specific feature of hypertrophic olivary degeneration. This is caused by a lesion in the triangle of Guillain and Mollaret (triangle linking the inferior olivary nucleus, red nucleus and the contralateral dentate nucleus). An MRI brain is the gold standard imaging for this lesion.
The other answers are not a part of the triangle of Mollaret.
41
Which one of the following is least associated with Miller-Fisher syndrome?
```
Anti-GQ1b antibodies
Areflexia
Ataxia
Postural hypotension
Ophthalmoplegia
```
Postural hypotension
Postural hypotension due to autonomic involvement is not a feature of Miller Fisher syndrome, but may be seen in Guillain-Barre syndrome
42
A 68-year-old man undergoes a lumbar puncture to investigate new confusion. His platelet count is normal, and his clotting screen is within an acceptable range. The procedure is discussed with his family as he is unable to consent and they are happy with the procedure but want to ensure that the possibility of post lumbar puncture headache is minimalised. What factor has been demonstrated to increase the likelihood of a post-LP headache?
Small needle gauge
Replacing the stylet during the procedure
Keeping the bevel of the needle parallel to the dura
Early mobilisation following procedure
Use of a Quincke (sharp) needle
Use of a Quincke (sharp) needle
Sharp needles have been found to be associated with a greater frequency of post-lumbar puncture headaches when compared to atraumatic needles inserted using an introducer. Using a small needle, replacing the stylet and maintaining the bevel parallel decreases the chance of developing a post-lumbar puncture headache, whilst early mobilisation has no impact.
Factors which may contribute to headache
- Increased needle size
- Direction of bevel
- Not replacing the stylet
- Increased number of LP attempts
Factors which do not contribute to headache
- Increased volume of CSF removed
- Bed rest following procedure
- Increased fluid intake post procedure
- Opening pressure of CSF
- Position of patient
43
A 56-year-old man presents to the Diabetes clinic for review. He has a history of poorly-controlled type 2 diabetes mellitus, which was first diagnosed fifteen years ago, and primary open angle glaucoma. He is taking latanoprost, metformin, sitagliptin and once-daily insulin glargine. His most recent HbA1c reading was 98mmol/mol.
At his last clinic review three months previously, he had complained of a burning, painful sensation in both his feet. He had been started on gabapentin, which he had uptitrated gradually to the maximum licensed dose. In spite of this, he reports no discernible improvement in the burning sensation in his feet.
What is the most appropriate management option with respect to his neuropathy?
```
Continue gabapentin, start duloxetine
Continue gabapentin, start codeine
Stop gabapentin, start amitriptyline
Stop gabapentin, start duloxetine
Refer to pain management clinic
```
Stop gabapentin, start duloxetine
Substituting gabapentin for amitriptyline would be a possible treatment strategy for some patients. However, for this patient, starting an antimuscarinic agent risks precipitating an exacerbation of symptoms from glaucoma.
44
A 68-year-old female is reviewed in the neurology clinic. She sustained an ischaemic stroke three months previously and has been left with residual disability. She has a past medical history of hypertension and hypercholesterolemia. She takes amlodipine and clopidogrel and atorvastatin.
On examination, her speech is mostly fluent. Her comprehension is normal but her repetition of phrases is poor. When asked to repeat the phrases 'bottles and rocks', she is unable to do so. There is no other residual neurological deficit.
What is the correct description of her speech disturbance?
```
Conduction dysphasia
Dysarthria
Expressive dysphasia
Global dysphasia
Receptive dysphasia
```
Conduction dysphasia
45
A 44-year-old man was admitted to the medical unit with vomiting, drowsiness and headache. On examination he was febrile, squinting to bright lights and had severe pain when extending his knee when his hip was lifted off the bed. He was started on IV cefotaxime and IV dexamethasone and underwent a lumbar puncture.
What is the most common long-term complication of this condition?
```
Encephalitis
Hydrocephalus
Sensorineural hearing loss
Seizures
Cerebral abscess
```
Sensorineural hearing loss is the most common complication following meningitis
46
A 35-year-old man with a history of schizophrenia is brought to the Emergency Department by worried friends due to drowsiness. On examination he is generally rigid. A diagnosis of neuroleptic malignant syndrome is suspected. Each one of the following is a feature of neuroleptic malignant syndrome, except:
```
Renal failure
Pyrexia
Elevated creatine kinase
Usually occurs after prolonged treatment
Tachycardia
```
Usually occurs after prolonged treatment
Neuroleptic malignant syndrome is typically seen in patients who have just commenced treatment. Renal failure may occur secondary to rhabdomyolysis
47
Which one of the following statements regarding the stopping of anti-epileptic drugs (AED) is most correct?
A. Can be considered if seizure free for > 5 years, with AEDs being stopped over 2-3 months
B. Can be considered if seizure free for > 2 years, with AEDs being stopped over 2-3 months
C. Can be considered if seizure free for > 1 year, with AEDs being stopped over 2-3 months
D. Can be considered if seizure free for > 5 years, with AEDs being stopped over 8-12 months
E. Can be considered if seizure free for > 1 year, with AEDs being stopped over 8-12 months
Can be considered if seizure free for > 2 years, with AEDs being stopped over 2-3 months
48
A 19-year-old woman presents to her General Practitioner with a bilateral sensorineural hearing loss. An MRI brain is requested and it reveals what are likely to be bilateral vestibular schwannomas.
What neurocutaneous syndrome is the patient very likely to have?
```
Sturge Weber syndrome
Von Hippel Lindau syndrome
Tuberous sclerosis
Neurofibromatosis type 1
Neurofibromatosis type 2
```
Neurofibromatosis type 2
Neurofibromatosis type 2 is a rare neurocutaneous syndrome. A classical feature includes bilateral vestibular schwannomas (previously known as acoustic neuromas), which present as a sensorineural hearing loss. Patients may have multiple schwannomas, meningiomas and ependymomas of the brain or spine. Other findings include retinal hamartomas, cataracts, Cafe Au Lait markings and peripheral nerve tumours.
Neurofibromatosis type 1, also known as von Recklinghausen disease, does not typically feature bilateral vestibular schwannomas. The typical features are Cafe au Lait spots, axillary freckling, Lisch nodules, neurofibromas and optic nerve gliomas.
Von Hippel Lindau syndrome is a condition featuring visceral cysts and benign tumours in any system, including the brain. It is not associated with bilateral vestibular schwannomas.
Tuberous sclerosis is a rare genetic disorder due to mutation of either the TSC1 or TSC2 genes. Its features are multiple central nervous system hamartomas (tubers), subependymal giant cell astrocytomas of the brain, kidneys angiomyolipomas, cardiac rhabdomyomas, facial angiofibromas, Shagreen patches and retinal astrocytic hamartomas.
Sturge Weber syndrome characterised by a 'port-wine stain' of the forehead, learning disabilities, seizures and glaucoma.
49
A 29-year-old female with progressive dementia and myoclonus is seen in the memory clinic. On examination, the patient has marked myoclonus with impairment of the concentration and memory aspects of the Addenbrooke's test. An MRI reveals a 'hockey stick sign'. A few weeks later the patient develops akinetic mutism and paresis of vertical upgaze. What is the likely diagnosis given the clinical and radiological findings?
```
Progressive supranuclear palsy
Variant Creutzfeldt-Jakob disease
Lewy body dementia
Wilson's disease
Huntington's disease
```
Variant Creutzfeldt-Jakob disease
This is a case of variant Creutzfeldt-Jakob disease (vCJD). The typical presentation is that of a younger patient with progressive dementia (less rapid the sporadic CJD) with myoclonus and, in the later stages, mutism and vertical upgaze palsy (found in 50%). An MRI brain reveals a characteristic 'hockey stick sign' where the pulvinar region and dorsomedial thalamus are hyperintense on T2-weighted imaging (or pulvinar sign where the pulvinar region is hyperintense only). CSF protein for 14-3-3 and periodic sharp wave complexes on the EEG are more commonly seen in sporadic CJD.
Wilson's disease often has extrapyramidal signs with other features such as liver disease and Kayser-Fleischer rings. Progressive supranuclear palsy would present with a downgaze vertical gaze palsy more commonly and Lewy body dementia would have more Parkinsonian features. There is no obvious family history of Huntington's here and there would be caudate atrophy on the MRI.
50
Which of the following is least associated with Parkinsonism?
```
Chlorpromazine
Progressive supranuclear palsy
Dementia pugilistica
Lead poisoning
Wilson's disease
```
Lead poisoning
51
Which one of the following side-effects is not recognised in patients taking sodium valproate?
```
Alopecia
Weight gain
Hepatitis
Induction P450 system
Teratogenicity
```
Induction P450 system
52
A 65-year-old female is returned to the surgical ward after an uneventful vaginal hysterectomy. She has no significant past medical history and is not taking any regular medications. She is provided with regular anti-emetics and pain relief as per routine surgical and anaesthetic postoperative instructions.
Three hours after returning to the surgical ward, she complains of palpitations and worsening dyspnoea.
On examination, she appeared agitated with some accessory muscle use.
An updated set of observations are taken:
Heart rate 156 beats per minute
Blood pressure 102/65 mmHg
Oxygen saturations 98% on 3L/min nasal specs
Respiratory rate 24/min
A bedside ECG is performed which reveals polymorphic ventricular tachycardia.
Which of the following postoperative medications is the likely cause of her deterioration?
```
Morphine
Ondansetron
Metoclopramide
Diclofenac
Cyclizine
```
Ondansetron
5-HT3 antagonists such as ondansetron can predispose to prolonged QT interval and increased risk of polymorphic VT
53
A 34-year-old female presents with vomiting preceded by an occipital headache of acute onset. On examination she was conscious and alert with photophobia but no neck stiffness. CT brain is reported as normal. What is the most appropriate further management?
```
CT brain with contrast
Repeat CT brain in 24h
CSF examination
Cerebral angiography
MRI brain
```
CSF examination
If subarachnoid haemorrhage is suspected but the CT head is normal, a lumbar puncture is required to confirm or exclude this diagnosis
If the CSF examination revealed xanthochromia, or there was still a high level of clinical suspicion, then cerebral angiography would be the next step.
53
A 34-year-old female presents with vomiting preceded by an occipital headache of acute onset. On examination she was conscious and alert with photophobia but no neck stiffness. CT brain is reported as normal. What is the most appropriate further management?
```
CT brain with contrast
Repeat CT brain in 24h
CSF examination
Cerebral angiography
MRI brain
```
CSF examination
If subarachnoid haemorrhage is suspected but the CT head is normal, a lumbar puncture is required to confirm or exclude this diagnosis
If the CSF examination revealed xanthochromia, or there was still a high level of clinical suspicion, then cerebral angiography would be the next step.
54
A 42-year-old woman with relapse-remitting multiple sclerosis (MS) has been suffering from increasingly painful spasms and cramping in her lower limbs for the past 18 months.
After discussion with her neurologist, it is agreed that this symptom is due to her chronic spasticity in her legs.
She has previously not tried any medication besides simple analgesia to manage this symptom but has now been offered an oral medication to specifically manage the spasticity.
What is the mechanism of action of this drug?
```
Acetylcholinesterase inhibitor
Dopamine agonist
Gamma-aminobutyric acid (GABA) agonist
Glutamate receptor antagonist
Non-selective monoamine reuptake inhibitor
```
Gamma-aminobutyric acid (GABA) agonist
Baclofen is an agonist of GABA receptors that acts in the central nervous system
This woman is likely to have been offered baclofen, which is licensed to treat chronic spasticity arising from disorders such as multiple sclerosis.
Baclofen stimulates GABA receptors in the central nervous system, thereby inhibiting the release of excitatory neurotransmitters. This results in the blocking of mono- and polysynaptic reflex transmission. Clinically, this leads to reduced muscle spasticity, which can improve pain and mobility in patients with MS.
Acetylcholinesterase inhibitors have no role in treating muscle spasticity. They are used in other neurological conditions such as Alzheimer's disease (e.g. rivastigmine) and myasthenia gravis (e.g. pyridostigmine).
Dopamine agonists such as ropinirole have a role in treating symptoms of restless legs syndrome (RLS) but not spasticity pain.
Glutamate receptor antagonists (such as amantadine) are used in Parkinson's disease. They have no role in treating muscle spasticity.
Non-selective monoamine reuptake inhibitors (also known as tricyclic antidepressants) are licensed to treat emotional lability in patients with MS but do not improve muscle spasticity symptoms.
55
A 76 year-old man is brought in to the Emergency Department with confusion, a temperature of 39.9 degrees Celsius and rigidity. From a recent clinic letter you see he lives alone at home and depends on help from his daughter, who has recently had a prolonged stay in hospital for appendicitis. Upon her discharge she went to see him, found him unwell and called 999. She had called him and he had been well the day before. He has a background of hypertension and Parkinson's disease for which he takes ramipril, amlodipine and 'a Parkinson's tablet' which he does not have with him. His chest x-ray and urine dip are clear and he is unable to give a history. A CT brain shows no acute abnormality.
What is the most appropriate intervention?
```
Broad spectrum antibiotics
Neurosurgical referral
Bromocriptine
Intravenous aciclovir
Cyproheptadine
```
Bromocriptine
The presentation is characteristic of neuroleptic malignant syndrome, but without a history of neuroleptic drug use. In a patient with Parkinson's disease, this raises the question of Parkinsonian malignant syndrome - a similar clinical picture triggered by abrupt withdrawal from antiparkinsonian medication. In this case, the patient is dependent on his daughter bringing in his prescriptions and has run out during her stay in hospital - hence why he does not have the anti-Parkinsonian medication with him. Although the exact pathogenesis of malignant syndrome is not understood, reinitiation of Parkinson's therapy is curative.
Piperacillin and tazobactam therapy would cover a possible sepsis, but is not a typical treatment for a potential central nervous system infection and therefore would be the incorrect choice even if sepsis was the underlying cause of this patient's symptoms. Neurosurgical referral is clearly inappropriate without any clear operative treatment. Intravenous aciclovir is the treatment for herpes simplex encephalitis, which is a differential in this case. It is made less likely only by a lack of any prodromal illness. Cyproheptadine would be a treatment option in serotonin syndrome, but this man is not on any serotonergic drugs and has no reason to have taken any, making this less likely.
56
A 51-year-old man presented to the Emergency Department with painless loss of vision in his right eye. This had been preceded by episodes of floaters.
His past medical history included well-controlled type 2 diabetes mellitus, for which he was not on any medications, and a period of poorly-controlled hypertension five years ago, which had culminated in him requiring surgery.
He recalls that his father had died from kidney cancer in his sixties, but is unable to provide any further past medical history.
What is the most likely unifying diagnosis that explains this presentation?
```
Hereditary haemorrhagic telangiectasia
Multiple endocrine neoplasia type 2 (MEN2)
Neurofibromatosis type 1
Neuromyelitis optica
Von Hippel-Lindau syndrome
```
Von Hippel-Lindau syndrome
Patients with von Hippel-Lindau syndrome may develop retinal haemangiomas which present as a vitreous haemorrhage
Remember with NMO you have optic neuritis which is PAINFUL loss of vision
57
A 56-year-old-male on haemodialysis via a right internal jugular tunnelled line presented with a 2-week history of fevers, night sweats and weight loss.
Blood and line cultures grew Staphylococcus aureus and a transesophageal echocardiogram confirmed a vegetation. His tunnelled line was removed and he dialysed via a temporary femoral line.
After 2 weeks of IV flucloxacillin, he began to get night sweats, fevers and a persistent headache. He became confused and hypoactive, needing full nursing care now.
What is the most likely cause for his symptoms?
```
Myocardial abscess
Meningitis
Brain abscess
Stroke
Temporary line sepsis
```
Brain abscess
Headache, fever and focal neurology and suggestive of a brain abscess
This is a case of a brain abscess formation as a complication of infective endocarditis secondary to tunnelled line sepsis. Patients who dialyse via a tunnelled line are at significantly increased risk of infections vs patients who dialyse via a fistula. In this case, the tunnelled line was the likely medium by which the mitral valve became infected.
Patents need to be watched closely for signs of an embolic phenomenon when diagnosed with endocarditis. The common embolic phenomenon is renal involvement (blood + protein on a urine dip and an acute kidney injury), hepatic or splenic infarcts (may show up as pain in the right or left upper quadrant or deranged liver function tests), ocular involvement (reduced vision causing retinal deposits or ocular artery occlusions) or central nervous system involvement (septic emboli to the brain causing stroke-like symptoms).
If the septic deposits seed and aren't fully treated, they can go on to develop a brain abscess. This will typically show up as headaches, fevers and some focal neurology on examination. It will occur in the context of infection getting worse while on appropriate initial antibiotics.
Looking at the other options:
Myocardial abscess formation should be closely looked out for. It will often manifest as a heart block on an ECG due to its proximity to the AV node. The PR interval should be watched closely as a new 1st-degree heart block could be the first sign of this.
Meningitis is a distinct possibility here. If there were signs of meningism or the CT was negative for an abscess, then a lumbar puncture should be attempted to look for CSF involvement.
Low molecular weight heparin is a contraindication in infective endocarditis. This is because of the significant risk of haemorrhagic transformations of septic emboli to the brain. Strokes in this context could either be septic emboli getting stuck in vessels, or a hemorrhagic transformation of previous emboli.
In a patient with fevers and any plastic line in situ, line sepsis should always be considered. Femoral lines are notorious for higher risk of infection. The line site should be checked daily for erythema or exudate.
58
A 10-year-old is referred to neurology due to episodes her GP feels are epileptiform. Her mother reports that she appears to just 'stop', sometimes even in mid conversation, for several seconds at random times during the day. During these episodes she can be unresponsive to questioning and has no recollection of them.
Which of these drugs is contraindicated in this condition?
```
Ethosuximide
Sodium valproate
Lamotrigine
Carbamazepine
Clonazepam
```
Carbamazepine is contraindicated in absence seizures
59
A 45-year-old male with a history of alcoholic liver disease presents with increasing confusion. On assessment, he is noted to be obtunded with a Glasgow coma scale of 14 out of 15, has a wide-based gait and nystagmus. There is no history or signs of recent trauma.
What is the most specific finding on brain MRI for this patient's condition?
```
Left-sided subdural haematoma
Generalised cerebral atrophy
Hydrocephalus
Enhancement of the mamillary bodies
Right-sided cerebellar infarction
```
Enhancement of the mamillary bodies
This patient is suffering from Wernicke's encephalopathy as indicated by the history of alcoholic liver disease with confusion, ataxia and ophthalmoplegia. The MRI finding of enhancement of the mamillary bodies due to petechial haemorrhages is specific for Wernicke's encephalopathy, although sensitivity is only 50%.
60
A 67-year-old woman comes for review with her husband. Her husband complains that she is constantly getting up from bed at night and pacing around the bedroom. She complains of 'antsy' legs and a 'horrible, creeping sensation'. Her symptoms generally come on in the evening and are only relieved by moving round. Given the likely diagnosis, what is the most appropriate treatment?
```
Ropinirole
Carbamazepine
Amitriptyline
Citalopram
Quinine
```
Restless leg syndrome - management includes dopamine agonists such as ropinirole
61
A 67-year-old female with stable rheumatoid arthritis attends a clinic for her annual review. She reports that she has noticed a loss of sensation in both her hands over the last 4 months and that it has been getting worse. In addition to arthritis, she also has hypertension and diabetes. She is currently taking infliximab, metformin, glipizide, and amlodipine. Her last HbA1c was 53 mmol/mol.
On examination, you note bilateral loss of temperature and pain sensation on the medial aspect of both hands and elbows. Proprioception and vibration sensation are preserved.
What is the most likely diagnosis?
```
Amyotrophy
Diabetic neuropathy
Mononeuritis multiplex
Syringomyelia
B12 deficiency
```
Syringomyelia classically presents with cape-like loss of pain and temperature sensation due to compression of the spinothalamic tract fibres decussating in the anterior white commissure of the spine
62
Which one of the following haematological disorders is most associated with gingival hyperplasia?
```
Chronic lymphocytic leukaemia
Myelofibrosis
Polycythaemia rubra vera
Haemophilia A
Acute myeloid leukaemia
```
Acute myeloid leukaemia
Gingival hyperplasia: phenytoin, ciclosporin, calcium channel blockers and AML
63
A 44-year-old woman presents to her neurologist with complaints of stiffness and involuntary spasms of her muscles. She has a background of relapsing-remitting multiple sclerosis. She takes natalizumab. She does not smoke or drink alcohol. She lives with her husband and two children.
On examination, there is evidence of increased tone in the upper limbs in a flexor distribution.
From the list below, what is the appropriate initial choice of medication to relieve her symptoms?
```
Dantrolene
Diazepam
Gabapentin
Ropinirole
Tizanidine
```
Gabapentin
Baclofen and gabapentin are first-line for spasticity in multiple sclerosis
Important for meLess important
Gabapentin is the correct answer. This patient has symptoms and signs of spasticity secondary multiple sclerosis as evidenced by increased tone and rigidity in her upper limbs. The first-line treatment for spasticity in MS is either gabapentin or baclofen.
64
A 72-year-old man becomes confused at the end of a charity walk for Alzheimer's. He is brought to the hospital by paramedics because he repeatedly asked other attendees on the walk why he was there and how he had got there. There is no past medical history of note and he takes no regular medication. He is orientated in time and person and knows he has been brought to the hospital. His blood pressure is 123/82 mmHg, his pulse is 70 beats per minute and regular. The neurological exam is unremarkable, routine blood tests and CT head are normal. He gradually recovers over the course of 3hrs.
What is the most appropriate intervention?
```
Aspirin
Clopidogrel
Reassurance
Rivaroxaban
Warfarin
```
The most likely diagnosis here, with acute onset of retrograde amnesia with preserved orientation and consciousness, is transient global amnesia, (TGA). The exact cause of TGA is unclear, although it may be similar in aetiology to migraines. There is no evidence in prospective cohort studies to suggest an association between TGA and increased risk of stroke.
Aspirin and clopidogrel, both antiplatelet agents, are not indicated here because TGA does not mark out the patient as being at increased risk of ischaemic stroke. Rivaroxaban and warfarin are both indicated for anticoagulation in patients with atrial fibrillation, which hasn't been identified here.
65
A 69-year-old man is diagnosed as having Parkinson's disease. Which one of the following psychiatric problems is most likely to occur in this patient?
```
Tics
Psychosis
Mania
Dementia
Depression
```
Parkinson's disease - most common psychiatric problem is depression
66
Each one of the following is associated with Friedreich's ataxia, except:
```
Increased risk of deep vein thrombosis
Optic atrophy
Cardiomyopathy
Nystagmus
High-arched palate
```
Increased risk of deep vein thrombosis
67
Which one of the following is least likely to produce a lymphocytosis in the cerebrospinal fluid?
```
Systemic lupus erythematous
Guillain-Barre syndrome
Viral encephalitis
Partially treated bacterial meningitis
Behcet's syndrome
```
Guillain-Barre syndrome
```
The following conditions are associated with raised lymphocytes
viral meningitis/encephalitis
TB meningitis
partially treated bacterial meningitis
Lyme disease
Behcet's, SLE
lymphoma, leukaemia
```
68
5-year-old girl has been referred to a paediatric neurologist with frequent episodes which her parents describe as 'daydreaming'. They report that she would suddenly stare blankly into space during a conversation or activity and would be unresponsive for approximately 10-15 seconds. These episodes occur several times a day and she has no recollection of the incidents.
An electroencephalogram (EEG) has been arranged which shows generalised spike-wave activity. Results of blood tests and neuroimaging are unremarkable.
Her parents are concerned about the long-term outlook of her condition.
Based on the seizure type, what is the most likely prognosis?
A. <10% probability of being seizure free in adolescence
B. 25-30% probability of being seizure free in adolescence
C. 45-50% probability of being seizure free in adolescence
D. 70-75% probability of being seizure free in adolescence
E. 90-95% probability of being seizure free in adolescence
Absence seizures - good prognosis: 90-95% become seizure free in adolescence
69
A 40-year-old man undergoes a temporal lobectomy after the discovery of a brain tumour. Which one of the following consequences would be least likely to develop?
```
Prosopagnosia
Astereognosis
Wernicke's aphasia
Superior homonymous quadrantanopia
Auditory agnosia
```
Astereognosis occurs in parietal lesions
| Tactile agnosia - inability to recognize common objects, such as coins, keys, and small blocks, by touch
70
An 18-year-old girl with a known Chiari 1 malformation presents to her General Practitioner with loss of sensation in both of her arms and forearms, and on the back of her neck. On testing, she specifically can't feel pain and temperature but can detect fine touch, proprioception and vibration.
Which of the following abnormalities has this patient most likely have?
```
Syringomyelia
Hydrocephalus
Brain stem compression
Cerebellar compression
Peripheral neuropathy
```
Syringomyelia
Chiari malformations are often associated with syringomyelia due to disturbed cerebrospinal fluid flow at the foramen magnum
Chiari 1 malformation is a condition characterised by herniation of the cerebellar tonsils through the foramen magnum. It causes symptoms by compressing the brainstem, cerebellum and by disturbing the flow of cerebrospinal fluid (CSF). Disturbed CSF flow either causes hydrocephalus (uncommon) or syringomyelia (common; ~50%).
Syringomyelia is a dilatation of a CSF space within the spinal cord. It occurs within the cervical and thoracic segments and causes compression of the spinothalamic tracts decussating in the anterior white commissure. This results in dissociative loss of sensation of pain, temperature and non-discriminative touch. There is classically a 'cape-like' distribution of this sensory loss.
71
A 23-year-old man presents with a 4 day history of an itchy and sore right ear. He has recently returned from holiday in Spain. On examination the right ear canal is inflamed but no debris is seen. The tympanic membrane is clearly visible and is unremarkable. What is the most appropriate management?
```
Topical corticosteroid + aminoglycoside
Topical corticosteroid
Refer to ENT
Topical corticosteroid + clotrimazole
Oral flucloxacillin
```
Topical corticosteroid + aminoglycoside
This patient has otitis externa, which commonly develops after swimming on holiday. The first line management is either a topical antibiotic or a combined topical antibiotic and steroid.
72
A 54-year-old man presents concerned about leg weakness. On examination he is noted to have increased tone in both legs, brisk reflexes and weakness in both lower limbs. Examination of his upper limbs is normal. Which one of the following is least likely to produce these symptoms?
```
HIV
Amyloidosis
Hereditary spastic paraplegia
Multiple sclerosis
Parasagittal meningioma
```
Amyloidosis is the least likely of the above options to result in a spastic paraparesis
73
You are a CT1 in Acute Medicine covering a general medical ward at night. You are asked to see a 60-year-old patient with known myasthenia gravis who is complaining of increased breathlessness and fatigue. Which of the following antibiotics is most likely to be responsible for a myasthenic crisis?
```
Metronidazole
Doxycycline
Gentamicin
Clarithromycin
Amoxicillin
```
Gentamicin
The most common exacerbating factor is exertion resulting in fatigability, which is the hallmark feature of myasthenia gravis . Symptoms become more marked during the day
The following drugs may exacerbate myasthenia:
penicillamine
quinidine, procainamide
beta-blockers
lithium
phenytoin
antibiotics: gentamicin, macrolides, quinolones, tetracyclines
This question asks which antibiotic is the most likely cause of an exacerbation. Although macrolides, tetracyclines, and metronidazole are occasionally associated with exacerbation in myasthenic patients, they are usually well tolerated in this population.
Aminoglycosides, including gentamicin, are relatively contra-indicated in myasthenia as they are more commonly associated with exacerbations. The mechanism is thought to be competitive inhibition of the release of acetylcholine at the presynaptic membrane of the neuromuscular junction.
Amoxicillin is generally considered to be safe in myasthenia gravis.
74
You are a CT1 in Acute Medicine covering a general medical ward at night. You are asked to see a 60-year-old patient with known myasthenia gravis who is complaining of increased breathlessness and fatigue. Which of the following antibiotics is most likely to be responsible for a myasthenic crisis?
```
Metronidazole
Doxycycline
Gentamicin
Clarithromycin
Amoxicillin
```
Gentamicin
The most common exacerbating factor is exertion resulting in fatigability, which is the hallmark feature of myasthenia gravis . Symptoms become more marked during the day
The following drugs may exacerbate myasthenia:
penicillamine
quinidine, procainamide
beta-blockers
lithium
phenytoin
antibiotics: gentamicin, macrolides, quinolones, tetracyclines
This question asks which antibiotic is the most likely cause of an exacerbation. Although macrolides, tetracyclines, and metronidazole are occasionally associated with exacerbation in myasthenic patients, they are usually well tolerated in this population.
Aminoglycosides, including gentamicin, are relatively contra-indicated in myasthenia as they are more commonly associated with exacerbations. The mechanism is thought to be competitive inhibition of the release of acetylcholine at the presynaptic membrane of the neuromuscular junction.
Amoxicillin is generally considered to be safe in myasthenia gravis.
75
A 33-year-old male is admitted with a subacute headache, confusion, and nausea. On closer questioning, you note that this gentleman appears irritable, confused, and is hearing voices. Prior to this, his wife reports he has had a recent viral upper respiratory tract infection. He is otherwise fit and well and takes no regular medications.
On examination, you note that he is neglecting to use his right arm. He is otherwise, neurologically intact. A subsequent CT head reveals no space occupying lesion, infarct or bleed. Later, an MRI is performed which reveals bilateral asymmetric and poorly marginated hyperintense T2-weighted and FLAIR lesions within the subcortical white matter. A lumbar puncture reveals no oligoclonal bands in the CSF.
What is the likely underlying diagnosis?
```
Alzheimer's disease
Multiple sclerosis
Meningitis
Herpes simplex encephalitis
Acute disseminated encephalomyelitis
```
Acute disseminated encephalomyelitis
The answer is acute disseminated encephalomyelitis which is a neurological condition which occurs typically a few weeks following a viral illness or vaccination. It can present with motor weakness, encephalopathy, seizures and coma. It is found on T2-weighted MRI imaging which reveals poorly-defined hyperintensities in the subcortical white matter. These lesions can develop throughout the course of the illness and hence serial MRIs may be required.
Multiple sclerosis is a differential diagnosis but is not necessarily definitively linked with a preceding illness. Alzheimer's disease and herpes encephalitis would not present with these MRI findings. The absence of prominent meningism features makes meningitis less likely.
76
A 55-year-old man is seen in the neurology clinic with sensory loss.
On examination he has loss of fine touch, joint position sense and temperature sensation bilaterally in the lower limbs, extending from his knees down, as well as in his fingertips.
His motor function is 5/5 in all four limbs.
His medications include: calcichew, alfacalcidol, sodium bicarbonate and ramipril
Which of the following is most likely to be the cause of these clinical findings?
```
Guillain-Barre syndrome
Lead poisoning
Polio
Chronic kidney disease
Amyotrophic lateral sclerosis
```
Uraemic polyneuropathy is predominantly sensory
Of the above, CKD (leading to uraemia) is the only option to cause a predominantly sensory polyneuropathy.
Guillain-Barre syndrome and lead poisoning cause a polyneuropathy which is predominantly motor.
Polio and amyotrophic lateral sclerosis affect the anterior horn cells of the motor tract, within the spinal cord. As such, they cause a motor deficit.
77
Which of the following drugs is least likely to cause peripheral neuropathy?
```
Amiodarone
Vincristine
Trimethoprim
Isoniazid
Nitrofurantoin
```
Trimethoprim
78
A 36-year-old woman presents with a progressively worse tingling sensation in the legs and poor balance. She has no significant past medical history.
On examination, proprioception and vibration sense are both heavily reduced in the lower limbs. Distal paraesthesia is evident. Knee reflexes are brisk.
A blood film is taken which demonstrates macrocytic anaemia and hypersegmented neutrophils.
Given the likely diagnosis, which parts of the spinal cord are affected?
Anterior corticospinal tract and lateral corticospinal tract
Dorsal column and lateral corticospinal tract
Spinocerebellar tract and dorsal column
Spinothalamic tract and dorsal column
Spinothalamic tract and lateral corticospinal tract
Dorsal column and lateral corticospinal tract
In subacute combined degeneration of the spinal cord, the dorsal columns and lateral corticospinal tracts are affected
79
42-year-old woman presents as she has noticed a 'droop' in the right side of her face since she woke up this morning. There is no associated limb weakness, dysphagia or visual disturbance. On examination you notice right-sided upper and lower facial paralysis. Which one of the following features would be most consistent with a diagnosis of Bell's palsy?
```
Vesicular rash around the ear
Hyperacusis
Sensory loss over the distribution of the facial nerve
Pins and needles in the right arm
Rhinorrhoea
```
Hyperacusis
80
A 5-year-old boy is referred by his GP to the neurology clinic with abnormal movements. His mother noticed that for the last year, the boy is starting to fall over more and more frequently. He has also been having increasingly slurred speech. These have been getting progressively worse. He has had recurrent chest infections in his childhood.
What is the most likely diagnosis?
```
Friedreich's ataxia
Infantile-onset spinocerebellar ataxia
Di-George syndrome
Cerebral palsy
Ataxic telangiectasia
```
Ataxic telangiectasia is characterised by cerebellar ataxia and telangiectasia, onset is in childhood
81
A 31-year-old female with progressive leg weakness has nerve conduction studies for suspected Guillain-Barre syndrome. Which one of the following findings would be most consistent with this diagnosis?
Reduced conduction velocity
Extended series of repetitive discharges lasting up to 30 seconds
Increased conduction velocity
Diminished response to repetitive stimulation
Reduced wave amplitude
Slowing of the nerve conduction velocity usually indicates there is damage to the myelin sheath, as in Guillain-Barre syndrome
Reduced conduction velocity
82
A 27-year-old female suffered a crush injury at work where an air vent fell and trapped her arm. This led to fixed focal dystonia resulting in flexion contracture of the right wrist and digits.
On examination, there is evidence of intrinsic hand muscle wasting. The right forearm is supinated, the wrist hyperextended and the fingers flexed with a decrease in sensation along the medial aspect of the hand and arm. A reduction in handgrip strength was noted.
Which nerve roots are affected in this case?
```
C5
C5/C6
C7
C8
C8/T1
```
C8/T1
82
A 27-year-old female suffered a crush injury at work where an air vent fell and trapped her arm. This led to fixed focal dystonia resulting in flexion contracture of the right wrist and digits.
On examination, there is evidence of intrinsic hand muscle wasting. The right forearm is supinated, the wrist hyperextended and the fingers flexed with a decrease in sensation along the medial aspect of the hand and arm. A reduction in handgrip strength was noted.
Which nerve roots are affected in this case?
```
C5
C5/C6
C7
C8
C8/T1
```
C8/T1
83
A 61 year-old man presents to the respiratory clinic with a 2-month history of progressive weakness and shortness of breath. He finds it difficult to stand from sitting, and struggles climbing stairs. He is an ex-smoker with chronic obstructive pulmonary disease (COPD). He had a recent exacerbation one month ago for which he was treated by the GP with a course of oral prednisolone, during which time his weakness transiently improved. On examination you note a left-sided monophonic wheeze and reduced breath sounds at the left lung base. Blood tests and a chest x-ray are requested.
```
Hb 145 g/L
WCC 10.9 109/l
Na+ 138 mmol/L
K+ 4.3 mmol/L
Urea 6.8 mmol/L
Creatinine 93 mmol/L
Calcium 2.62 mmol/L
Phosphate 1.44 mmol/L
```
Chest x-ray Hyperexpanded lungs, left lower lobe collapse, bulky left hilum
What is the most likely cause of this patient's weakness?
```
Myasthenia gravis
Steroid-induced myopathy
Lambert-Eaton myasthenic syndrome
Hypercalcaemia
Motor neurone disease
```
Lambert-Eaton myasthenic syndrome
This man has a small-cell lung cancer (SCLC) and associated Lambert-Eaton myasthenic syndrome - a well-recognised paraneoplastic manifestation of SCLC. This classically effects the proximal muscles, especially in the legs, causing difficulty in standing from a seated position and climbing stairs. In contrast to myasthenia gravis, eye involvement is uncommon. Treatment with steroids is often helpful, which explains his transient symptomatic improvement during treatment for his COPD exacerbation.
84
A 29-year-old female has just given birth a baby boy who weighs 3.1kg. The baby unfortunately develops bleeding via the umbilicus, mucous membranes, gastrointestinal tract. The mother has a past medical history of epilepsy.
What anti-epileptic is most likely implicated?
```
Sodium valproate
Carbamazepine
Lamotrigine
Levetiracetam
Phenytoin
```
Phenytoin induces vitamin K metabolism, which can cause a relative vitamin K deficiency, creating the potential for heamorrhagic disease of the newborn
85
A 62-year-old man is admitted to the Emergency Department with a left hemiplegia. His symptoms started around 5 hours but he initially thought he had slept in an awkward position. He has no past medical history of note but on examination is found to have and irregular pulse of 150 / min. The ECG confirms atrial fibrillation. A CT head is immediately arranged and reported as normal. What is the most appropriate initial management?
```
Aspirin
Aspirin + dipyridamole
Alteplase
Warfarin
Aspirin + warfarin
```
Aspirin
Rate control should also be initiated. He is outside the thrombolysis window so alteplase is not an option. The 2004 RCP guidelines recommend that anticoagulation should be commenced 14 days after an ischaemic stroke. Earlier anticoagulation may exacerbate any secondary haemorrhage.
Dipyridamole should not be used in the acute phase.
86
A 27-year-old man presents to the Emergency Department with 2 day history of severe headache and pyrexia (38.2ºC). A CT scan is reported as follows:
CT: Brain
Petechial haemorrhages in the temporal and inferior frontal lobes. No mass effect. Brain parenchyma otherwise normal
What is the most likely diagnosis?
```
Brain abscess
Meningococcal meningitis
Cerebral malaria
Herpes simplex encephalitis
New variant CJD
```
Herpes simplex encephalitis
CT head showing temporal lobe changes - think herpes simplex encephalitis
CT: medial temporal and inferior frontal changes (e.g. petechial haemorrhages) - normal in one-third of patients
87
A 24-year-old lady presents with a two-week history of frontal headache associated with blurred vision and nausea. She has a history of asthma and acne. She uses a salbutamol inhaler as needed, a topical retinoid and is halfway through a three-month course of doxycycline. On examination, she has no neurological deficits but is noted to have bilateral gross papilloedema. Her body mass index is 26kg/m². A CT scan shows no hydrocephalus or mass lesions.
Which of the following is the most likely cause of her symptoms?
```
Migraine
Retinoid use
Venous sinus thrombosis
Subarachnoid haemorrhage
Tetracycline use
```
This patient has symptoms and signs consistent with idiopathic intracranial hypertension. This condition is known to be associated with tetracycline use.
88
Which one of the following is least associated with the development of chorea?
```
Ataxic telangiectasia
SLE
Wilson's disease
Pregnancy
Infective endocarditis
```
Chorea can be a very rare manifestation of infective endocarditis, following embolisation to the basal ganglia. It is however the least likely of the above five
89
A 45-year-old male presents to the acute medical take with a three day history of progressive bilateral leg weakness. Which of the following constellation of signs on examination is the most consistent with a diagnosis of Guillain-Barre Syndrome?
Bradycardia with lower limb hyporeflexia and flaccid paralysis
Tachycardia with lower limb hyporeflexia and spastic paralysis
Bradycardia with lower limb hyperreflexia and spastic paralysis
Tachycardia with lower limb hyporeflexia and flaccid paralysis
Tachycardia with lower limb hyperreflexia and flaccid paralysis
Tachycardia with lower limb hyporeflexia and flaccid paralysis
Absent or depressed deep tendon reflexes are classical findings in Guillain-Barre Syndrome (GBS). Hyperreflexia is seen in a GBS variant known as Bickerstaff's encephalitis.
The paralysis in GBS is flaccid.
Autonomic symptoms are common in GBS. The most frequently encountered are tachycardia and urinary retention. Although autonomic dysfunction may manifest as hypertension, hypotension, bradycardia, or ileus, these are not as commonly seen.
90
A 45-year-old lady presents with a 2-month history of left-hand weakness. She has no past medical history. On examination, there is a mild weakness of the left upper and lower limbs with a right sided facial weakness which spares the forehead. Where is the lesion?
```
Right cerebrum
Left cerebrum
Right pons
Left pons
Cervical spinal cord
```
Right pons
91
Which type of motor neuron disease carries the worst prognosis?
```
Relapsing-remitting
Progressive bulbar palsy
Progressive muscular atrophy
Spinocerebellar ataxia
Amyotrophic lateral sclerosis
```
Progressive bulbar palsy
92
A 68-year-old man presents to the emergency department with sudden-onset difficulty in speaking and comprehending language. He also has significant weakness of the right arm. He has a past medical history of diabetes and hypertension.
After examination, a CT head scan shows left-sided cerebral hypoattenuation, indicating ischaemia.
Given the likely diagnosis, he is referred to the appropriate team and spends a considerable amount of time in hospital. After discharge, the community team follows him up and wishes to measure his level of disability and dependence with regards to daily living.
Which of the following is best for this purpose?
```
Barthel scale
FAST test
MUST
ROSIER scale
Waterlow score
```
The Barthel index is a scale that measures disability or dependence in activities of daily living in stroke patients
93
Which one of the following dopamine receptor agonists used in the management of Parkinson's disease is least associated with pulmonary, retroperitoneal and pericardial fibrosis?
```
Pergolide
Lisuride
Bromocriptine
Cabergoline
Ropinirole
```
Ropinirole
94
A 28-year-old woman is brought by Emergency ambulance to the hospital. She is accompanied by her husband who tells you that his wife has suffered a progressive flu like illness over the past few days. Over the past 24hrs she has begun to develop short term memory problems, been behaving oddly, and has now developed worsening drowsiness and disorientation. On examination her Glasgow Coma Scale is 13. You confirm confused, rambling speech. She has neck stiffness, fundoscopy is unremarkable.
Investigations
```
Hb 130 g/l Na+ 134 mmol/l
Platelets 182 * 109/l K+ 4.7 mmol/l
WBC 9.2 * 109/l Urea 5.9 mmol/l
Neuts 2.8 * 109/l Creatinine 92 µmol/l
Lymphs 5.8 * 109/l CRP 61 mg/l
Eosin 0.4 * 109/l
```
Which of the following is the most likely underlying diagnosis?
```
Herpes encephalitis
Meningococcal meningitis
Severe influenza
Measles encephalitis
Tuberculous meningitis
```
Herpes encephalitis
The progressive confusion seen here with memory loss, lymphocytosis and a moderate elevation of CRP, following a flu like illness, fits well with a diagnosis of herpes encephalitis. MRI brain is the investigation of choice initially, which should demonstrate temporal lobe changes, although often CT only is available out of hours. CSF lymphocytosis is seen, there is a mild elevation in protein and glucose is either normal or slightly decreased. IV acyclovir is the intervention of choice.
Meningococcal meningitis isn't suggested because of the peripheral blood lymphocytosis, and the lack of features of meningococcal bacteraemia such as a skin rash. Measles encephalitis is associated with a subacute presentation of gradual memory loss, and tuberculous meningitis seems less likely given we are provided with no history of respiratory symptoms, or possible TB exposure. Severe influenza with encephalitis is a possible differential, although a predilection for memory loss would be unusual.
95
A 51-year-old man with a history of schizophrenia is reviewed. He has developed parkinsonism secondary to his antipsychotic medication. Which one of the following drugs is most useful in the management of tremor?
```
Apomorphine
Cabergoline
Selegiline
Amantadine
Benzhexol
```
Benzhexol (antimuscarinic) is now more commonly referred to as trihexyphenidyl. It is now mainly used for drug-induced parkinsonism rather than idiopathic Parkinson's disease
96
A 50-year-old man presents to the emergency department with a one-hour history of right-sided weakness and facial droop, which has now entirely resolved. He has a past medical history of migraines with aura, which started around 8 years ago, and low mood. He had a similarly brief episode of sudden dizziness and slurred speech six months ago but did not seek medical attention. His father had similar experiences in his 50s but died in a road traffic accident aged 60.
The patient's CT head is normal. He has an MRI head which shows a number of hyperintense lesions. What is the most likely cause for his symptoms?
```
Early onset Alzheimer's disease
CADASIL
Huntington's disease
Variant Creutzfeldt-Jacob disease
Atypical migraine
```
CADASIL is a rare cause of multiple cerebral infarctions
The correct answer is CADASIL - cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy. This is a genetic condition due to a mutation in the NOCTH3 gene. It usually presents with the onset of migraines in middle age, followed by recurrent TIAs and strokes, and resulting in neuro-cognitive decline, psychiatric problems and dementia. MRI often shows multiple widespread hyper-intense lesions in the white matter, basal ganglia, thalamus and pons
97
You are reviewing a 22-year-old man who has developed headaches. Which one of the following features is most typical of migraines?
```
Pain on neck flexion
Phonophobia
Epiphora - excessive watering of eye
Recent viral illness
Bilateral, 'tight-band' like pain
```
Phonophobia
98
A 34-year-old male rugby player presents to the GP surgery with a 5-day history of sudden onset severe pain in the right shoulder and upper arm, which has now subsided but followed by a 1-day history of weakness in the shoulder. On examination, there is reduced power in the deltoid muscle. There is a full range of passive movement of the shoulder joint. The rest of the neurological examination is normal.
What is the most likely diagnosis?
```
Shoulder joint dislocation
Osteoarthritis
Adhesive capsulitis
Brachial neuritis
Cervical artery dissection
```
Brachial neuritis is characterized by acute onset unilateral severe pain followed by shoulder and scapular weakness several days later
Important for meLess important
Brachial neuritis is characterized by acute onset of unilateral severe pain followed by shoulder and scapular weakness several days later.
The clinical information rules out dislocation.
The patient is too young to have significant osteoarthritis and the presentation is too acute to be osteoarthritis. Osteoarthritis would also present with joint pain on passive movement which is not the case here.
Cervical artery dissection is a cause of stroke. It would present with upper motor neurone signs and pain is not such a prominent component.
Brachial neuritis
Brachial neuritis is characterized by acute onset unilateral severe pain followed by shoulder and scapular weakness several days later
Important for meLess important
Brachial neuritis is characterized by acute onset of unilateral severe pain followed by shoulder and scapular weakness several days later.
The clinical information rules out dislocation.
The patient is too young to have significant osteoarthritis and the presentation is too acute to be osteoarthritis. Osteoarthritis would also present with joint pain on passive movement which is not the case here.
Cervical artery dissection is a cause of stroke. It would present with upper motor neurone signs and pain is not such a prominent component.
99
Which one of the following features is most likely to be seen following facial nerve paralysis?
```
Hyperacusis
Hyperlacrimation
Hyperesthesia
Hyperalgesia
Hypersalivation
```
Hyperacusis results from paralysis of the stapedius muscle. Overflow of tears may occur but hyperlacrimation does not.
100
A 47-year-old man presents to the Emergency Department with a three day history of severe headache associated with vomiting. There is no past medical history of note. On examination blood pressure is 98/62 mmHg, pulse is 108 bpm and temperature is 37.0ºC. There is mild neck stiffness and a partial third nerve palsy of the left eye. Blood rests reveal:
Hb 14.8 g/dl
Plt 373 * 109/l
WBC 13.6 * 109/l
Na+ 132 mmol/l
K+ 5.2 mmol/l
Urea 4.2 mmol/l
Creatinine 99 µmol/l
Free T4 9 pmol/l
(range 10-22)
What is the most likely diagnosis?
```
Subarachnoid haemorrhage
Cavernous sinus thrombosis
Meningitis
Pituitary apoplexy
Lateral sinus thrombosis
```
Pituitary apoplexy
The hypotension, electrolytes and low free T4 point towards hypopituitarism. Clinically, pituitary apoplexy can mimic a subarachnoid haemorrhage
101
You see a 23-year-old man who complains of sudden unavoidable urges to sleep. When these occur, he can collapse on the spot and immediately lose consciousness. This can be brought on by strong emotions such as fear or laughter.
Which of the following neuropeptides is low in this condition?
```
GABA
Orexin
Cryptochrome
Melatonin
Leptin
```
Narcolepsy is associated with low orexin (hypocretin) levels
102
A 53-year-old woman presents to the emergency department with a sudden-onset, severe headache, describing it as the worst of her life. She had been sitting at her desk when the headache came on. She has associated nausea and vomiting.
On examination, she has some neck stiffness and photophobia and appears drowsy. A CT scan shows hyperdense across the basal cisterns and sulci.
Which of the following is indicated in managing the complications of this condition?
```
Amlodipine
Diltiazem
Felodipine
Nifedipine
Nimodipine
```
Nimodipine is used to prevent vasospasm in aneurysmal subarachnoid haemorrhages
103
A 36-year-old man with difficult to control epilepsy is reviewed in clinic. He is currently taking phenytoin but presents due to fatigue. A full blood count is performed:
Hb 10.1 g/dl
MCV 121 fl
Plt 234 * 109/l
WCC 4.6 *109/l
What is the most likely cause for his tiredness?
```
Iron deficiency
Vitamin B12 deficiency
Liver dysfunction secondary to phenytoin
Haemolytic anaemia secondary to phenytoin
Folate deficiency
```
Folate deficiency
104
A 40-year-old woman has lost her mobility over the last three years.
On examination of the lower limbs, there is Medical Research Council (MRC) grade 3 power throughout both legs. Tone is increased and reflexes are brisk. On examination of the upper limbs, there is MRC 5 on the left and 4+ on the right.
Which of the following is most likely to have lead to her leg weakness?
```
Multiple sclerosis
Guillain-Barré syndrome (GBS)
Central pontine myelinolysis
Charcot–Marie–Tooth disease
Miller Fisher syndrome
```
Multiple sclerosis is a cause of spastic paraparesis
GBS is a polyneuropathy and therefore causes lower motor neurone signs.
Central pontine myelinolysis is a disease of the brainstem and therefore typically causes quadraparesis (in this case the arms are mostly spared). The weakness would be expected to come on more rapidly.
Charcot-Marie-Tooth is a hereditary neuropathy. Onset is typically much earlier than this case.
Miller Fisher syndrome is a variant of GBS that typically affects the cranial nerves.
105
A 48-year-old man presents to his GP with a number of symptoms that have come on over the past few days. His vision is blurred and his right eye is painful, as well as having a generalised headache.
On examination, the right eye has deviated inferiorly and laterally. There is visible ptosis of the upper right lid, and the pupil is dilated. It does not respond to light. This eye does not follow movements well, but the left eye appears unaffected and is normal upon testing.
Where is the most likely location of the lesion?
```
Anterior cerebral artery
Internal carotid artery
Medulla
Oculomotor nucleus
Posterior communicating artery
```
Painful third nerve palsy = posterior communicating artery aneurysm
Posterior communicating artery
Painful third nerve palsy = posterior communicating artery aneurysm
The correct answer is posterior communicating artery. The cluster of symptoms described here points towards a third (oculomotor) nerve palsy, with the eye classically deviated 'down and out' and the presence of ptosis. The presence of pupil dilation and pain points towards a compressive lesion. This is most commonly a posterior communicating artery aneurysm and requires urgent intervention.
The anterior cerebral artery is incorrect. This does not have a direct physical relationship to the oculomotor nerve and would be very unlikely to present with these signs and symptoms.
The internal carotid artery runs through the cavernous sinus alongside the oculomotor nerve. An aneurysm of this can compress the nerve; however, aneurysms of the posterior communicating artery are more likely to do so.
The medulla is not relevant to this presentation. Several cranial nerves arise from the medulla - the oculomotor nerve does not.
The oculomotor nerve arises from the oculomotor nucleus of the midbrain. A lesion here would be unlikely to cause a painful palsy.
106
A 44-year-old woman presents with a three month history of worsening involuntary movements of the head. These are worse when she is stressed and improved by alcohol. They are not present when she is sleep. There are no other neurological symptoms of note and neurological examination is unremarkable other than spotaneous movements of the head which are worse when she looks to either side. Her father had a similar complaint but never sought medical attention. What is the most likely diagnosis?
```
Parkinson's disease
Cerebellar tremor
Huntington's disease
Multiple sclerosis
Essential tremor
```
Essential tremor is the most common cause of titubation (head tremor). Whilst the majority of patients will complain of hand tremor titubation may occur in isolation. The tremor is characteristic as it is worse on movement and during stress and relieved by alcohol and sleep. The family history is also a pointer.
107
A 14-year-old male is noted to have optic atrophy on fundoscopy. Neurological exam reveals dysarthric speech and nystagmus. Knee and ankle jerks are absent but there is an extensor plantar response. What is the likely diagnosis?
```
Leber's optic atrophy
Ataxic telangiectasia
Friedreich's ataxia
Subacute combined degeneration of the cord
Multiple sclerosis
```
Friedreich's ataxia
108
Which one of the following side-effects is least associated with the use of levodopa?
```
Psychosis
'On-off' effect
Postural hypotension
Cardiac arrhythmias
Galactorrhoea
```
Galactorrhoea
109
Which one of the following causes of peripheral neuropathy is most associated with an axonal, rather than demyelinating, pathology?
```
Paraprotein neuropathy
Guillain-Barre syndrome
Hereditary sensorimotor neuropathies (HSMN) type I
Amiodarone
Vasculitis
```
Vasculitis
110
Which one of the following is a contraindication to the use of a triptan in the management of migraine?
```
Concurrent pizotifen use
Patients older than 55 years
A history of epilepsy
Previous intracranial tumour
A history of ischaemic heart disease
```
Cardiovascular disease is a contraindication to triptan use
111
A 72-year-old gentleman with a history of hypertension and stroke presents to the emergency department accompanied by his wife. His wife reported him waking at 7am confused. Repetitive questioning was a prominent feature. He was observed to be rubbing his hands together during the event. He was able to recognise his wife and correctly reported his address and date of birth to paramedics. The event lasted approximately 10 minutes. On arrival to the emergency department he a Glasgow Coma Scale score of 13 (E3 M6 V4). A full neurological examination was otherwise normal. What is the most likely diagnosis?
```
Epilepsy
Transient psychogenic amnesia
Transient global amnesia
Brain tumour
Transient ischaemic attack
```
Epilepsy
The gentleman in this scenario presents with an episode of transient amnesia. The key to this question is distinguishing between transient global amnesia and transient epileptic amnesia.
Transient global amnesia is a clinical syndrome of uncertain aetiology, characterised by a discrete episode lasting for a few hours (always less than 24 hours) of anterograde amnesia, retrograde amnesia, repetitive questioning with an absence of other cognitive or neurological impairments.
Diagnostic criteria (in addition to the above features) are as follows; reliable witness to episode, an absence of head trauma or loss of consciousness at the onset, preserved personal identity and absence of epileptic features.
Epilepsy can present with discreet episodes of amnesia. This syndrome is called transient epileptic amnesia. Features that suggest epilepsy are; shorter duration (should be less than 1 hour), multiple attacks, onset on waking from sleep and accompanying epileptic features - e.g. motor automatism, stereotyped behaviours, limb shaking.
The correct answer is epilepsy rather than transient global amnesia due to the presence of motor automatism (rubbing hands together) and the short duration of the event. The previous stroke may be the underlying cause of his seizure. The mildly reduced score on the Glasgow Coma Scale may indicate a post-ictal state.
112
A 67-year-old man is reviewed in the falls clinic. Over the past few months he has sustained a number of falls. His daughter reports that he is starting to 'shuffle around the house' and has particular problems going up and down stairs. She also notes that he appears to be confused at times and often forgets his grandchildren's names.
On examination he appears to move and follow commands slowly. There is a resting tremor in the left hand more so than the right. Some rigidity is also noted when examining his arms. Examination of the cranial nerves is unremarkable other than a problem following movement in the vertical plane.
What is the most likely diagnosis?
```
Parkinson's disease
Motor neuron disease
Multiple system atrophy
Dementia with Lewy bodies
Progressive supranuclear palsy
```
Progressive supranuclear palsy
Progressive supranuclear palsy: postural instability, impairment of vertical gaze, parkinsonism, frontal lobe dysfunction
113
Which one of the following is least recognised as causing idiopathic intracranial hypertension?
```
Oral contraceptive pill
Tetracycline
Ciclosporin
Prednisolone
Vitamin A
```
Ciclosporin
114
32-year-old woman is brought in to the emergency department having a seizure. She has a history of epilepsy and takes carbamazepine. However, her friend tells you she was at a party drinking alcohol last night and it is unclear if she has taken her medication today.
She has been having a tonic-clonic seizure despite receiving buccal midazolam at home and 10mg rectal diazepam in the ambulance. Once IV access is established IV lorazepam is started in the department. Unfortunately, she continues to seize at 35 minutes and is in status epilepticus.
What would be the most appropriate next step in management?
```
IV Carbamazepine
IV Phenytoin
IV Propofol
IV Sodium valproate
IV Thiamine
```
In status epilepticus, a phenytoin infusion should be given if not responding to benzodiazepines
115
A 40-year-old female presents with progressively increasing headaches associated with tinnitus and occasional visual issues. The patient reports the headaches are exacerbated by leaning forward and can take several hours lying in bed to resolve. She has a past medical history of type 2 diabetes and obesity.
Funduscopy reveals swelling of the optic nerves and a raised opening pressure is measured on lumbar puncture.
Which of the following eye defects is most commonly associated with the patient’s condition?
```
Eye deviation 'down and out
Loss of red reflex
Miosis
Proptosis
Relative afferent pupillary defect
```
Eye deviation 'down and out
Raised ICP can cause a third nerve palsy due to herniation
This patient is most likely suffering from raised intracranial pressure (ICP) secondary to idiopathic intracranial hypertension (previously known as benign intracranial hypertension). Common symptoms of raised ICP including headaches, nausea, tinnitus and eye issues. Ocular nerve palsies, such as a third nerve/oculomotor palsy as described by a ‘down and out’ deviation, can occur as ICP increases. This is due to brain herniation and can result in significant diplopia if left untreated.
116
An obese 24-year-old female presents with headaches and blurred vision. Examination reveals bilateral blurring of the optic discs but is otherwise unremarkable with no other neurological signs. Blood pressure is 130/74 and she is apyrexial. What is the most likely underlying diagnosis?
```
Multiple sclerosis
Meningococcal meningitis
Brain abscess
Normal pressure hydrocephalus
Idiopathic intracranial hypertension
```
Idiopathic intracranial hypertension
Obese, young female with headaches / blurred vision think idiopathic intracranial hypertension
The combination of a young, obese female with papilloedema but otherwise normal neurology makes idiopathic intracranial hypertension the most likely diagnosis
117
What is the mechanism of action of sumatriptan?
```
5-HT3 agonist
5-HT2 antagonist
5-HT2 agonist
5-HT1 antagonist
5What is the mechanism of action of sumatriptan?
```
```
5-HT3 agonist
5-HT2 antagonist
5-HT2 agonist
5-HT1 antagonist
5-HT1 agonist
```
Triptans are serotonin 5-HT1B and 5-HT1D receptor agonists
118
You review a 65-year-old man who is currently taking antipsychotic medication. His carers have noticed that his movements have been very slow over the past few weeks. Which one of the following would suggest a diagnosis of Parkinson's disease rather than drug-induced parkinsonism?
```
Rigidity
Masked face
Bilateral symptoms
Flexed posture
Restlessness of arms and legs
```
Rigidity
Rigidity and rest tremor are uncommon in drug-induced parkinsonism. Masked face and flexed posture can be seen in both conditions. Bilateral symptoms are more common in drug-induced parkinsonism. Restlessness of arms and legs (akathisia) is a common side-effect of antipsychotics.
119
A 22-year-old man was seen in the Emergency Department after his mother reported three separate seizure episodes. He was unable to remember what happened and was unaware anything was happening at the time. His mother describes the episodes lasting 30 seconds each and no obvious trigger. She reported that he lost consciousness and began chewing and licking his top lip, but did not have any limb movements or incontinence. She also said that after each episode he struggled to 'find his words' for at least a minute.
What type of seizure and localising lobe does the above description correspond most to?
```
Focal awareness impaired, parietal lobe
Focal aware, frontal lobe
Focal awareness impaired, frontal lobe
Focal awareness impaired, temporal lobe
Focal aware, temporal lobe
```
Focal awareness impaired, temporal lobe
Lip smacking + post-ictal dysphasia are localising features of a temporal lobe seizure
119
A 22-year-old man was seen in the Emergency Department after his mother reported three separate seizure episodes. He was unable to remember what happened and was unaware anything was happening at the time. His mother describes the episodes lasting 30 seconds each and no obvious trigger. She reported that he lost consciousness and began chewing and licking his top lip, but did not have any limb movements or incontinence. She also said that after each episode he struggled to 'find his words' for at least a minute.
What type of seizure and localising lobe does the above description correspond most to?
```
Focal awareness impaired, parietal lobe
Focal aware, frontal lobe
Focal awareness impaired, frontal lobe
Focal awareness impaired, temporal lobe
Focal aware, temporal lobe
```
Focal awareness impaired, temporal lobe
Lip smacking + post-ictal dysphasia are localising features of a temporal lobe seizure
120
A patient presenting to the emergency department undergoes a CT head scan. The report describes a hypodense collection around the convexity of the brain that is not limited to suture lines.
What is the most likely radiological diagnosis?
```
Subarachnoid haemorrhage
Extradural haematoma
Acute subdural haematoma
Chronic subdural haematoma
Intracerebral haematoma
```
Chronic subdural haematoma
n CT imaging, a chronic subdural haematoma will appear as a hypodense (dark), crescentic collection around the convexity of the brain
Important for meLess important
On CT imaging, acute haematomas appear bright (hyperdense) whereas chronic haematomas appear dark (hypodense). Extradural haematomas are limited by suture lines whereas subdural haematomas are not. Intraparenchymal haematomas arise within the brain substance. Subarachnoid haemorrhage are typically seen as hyperdensity within the basal cisterns and sulci of the subarachnoid space.
121
A 24-year-old woman with Charcot-Marie-Tooth disease (type 1) asks how likely it is that any future children will have the disease. What is the most accurate answer?
```
Three times as likely as background population
25%
Between 5 - 10%
Same as background population
50%
```
Charcot-Marie-Tooth disease (hereditary sensorimotor neuropathy type I) is an autosomal dominant condition and therefore 50% of children will be affected
121
A 24-year-old woman with Charcot-Marie-Tooth disease (type 1) asks how likely it is that any future children will have the disease. What is the most accurate answer?
```
Three times as likely as background population
25%
Between 5 - 10%
Same as background population
50%
```
Charcot-Marie-Tooth disease (hereditary sensorimotor neuropathy type I) is an autosomal dominant condition and therefore 50% of children will be affected
122
A 20-year-old man presents with recent episodes of severe, stabbing pain in the right eye. These episodes typically occur once a day and last around 30 minutes. His wife reports him pacing around and shouting with the pain. She also reports that his right eye appears red and that he has clear nasal discharge during the episodes.
Given the likely diagnosis, which one of the following should you advise the patient to avoid to help prevent further episodes?
```
Opioid medications including codeine
Stress
Alcohol
Sunlight
Excessive exercise
```
Alcohol is a common trigger for cluster headaches
123
Which of the following features is least likely to be found in a patient with tuberous sclerosis?
```
Adenoma sebaceum
Cafe-au-lait spots
Retinal hamartomas
'Ash-leaf' spots
Lisch nodules
```
Lisch nodules are seen in neurofibromatosis
124
A 61-year-old woman presents with bilateral tinnitus. She reports no change in her hearing or other ear-related symptoms. Ear and cranial nerve examination is unremarkable. Which medication is she most likely to have recently started?
```
Ciprofloxacin
Nifedipine
Repaglinide
Quinine
Bendroflumethiazide
```
Quinine
125
A 20-year-old male patient presents to the GP surgery with a 2-year history of upper back pain, shoulder and arm weakness. On inspection, you notice that he has an asymmetric smile. On further examination, there is marked weakness and wasting of the right facial muscles, right trapezius, deltoid and biceps muscles. There is also winging of the right scapula. There is some weakness and wasting of the same muscle groups on the left side but to a much lesser extent. A type of muscular dystrophy is suspected.
What is the inheritance pattern of this condition?
```
Autosomal dominant
Autosomal recessive
X-linked recessive
X-linked dominant
Idiopathic
```
Autosomal dominant
Facioscapulohumeral muscular dystrophy is an austosomal dominent disorder
Important for meLess important
This patient presents with the classic appearance of facioscapulohumeral muscular dystrophy, which is an autosomal dominant disorder. There is typically an asymmetric pattern.
Facioscapulohumeral muscular dystrophy (FSHMD) is an autosomal dominant form of muscular dystrophy. As the name suggests it typically affects the face, scapula and upper arms first. Symptoms typically present by the age of 20 years.
Features
facial muscles are involved first - difficulty closing eyes, smiling, blowing etc
weakness of the shoulder and upper arm muscles
abnormal prominence of the borders of the shoulder blades - 'winging'
lower limb: hip girdle weakness, foot drop
126
Which one of the following is not a recognised causes of miosis?
```
Old age
Pontine haemorrhage
Holmes-Adie pupil
Argyll-Robertson pupil
Horner's syndrome
```
Holmes-Adie pupil is a benign condition most commonly seen in women. It is one of the differentials of a dilated pupil
127
A 60-year-old male with insulin-controlled diabetes presents with leg and arm weakness which started several months ago with muscle cramps and spasms following a short hospital admission for pneumonia. The patient now reports difficulty climbing stairs and getting up from sitting due to weakness.
On examination, there is muscle wasting with 3/5 power in the right lower limb and 4/5 power in the remaining limbs. Sensation and coordination, as well as cranial nerves, are all intact.
What is the most likely diagnosis?
```
Amyotrophic lateral sclerosis
Becker muscular dystrophy
Guillain-Barré syndrome
Myasthenia gravis
Peripheral neuropathy
```
Amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis is associated with mixed UMN and LMN signs (usually no sensory deficits)
128
A 62-year-old man with a history of hypertension and epilepsy is noted to have gingival hyperplasia on examination in the cardiology clinic. Which one of the following drugs is most likely to be responsible?
```
Sodium valproate
Lisinopril
Atorvastatin
Nifedipine
Carbamazepine
```
Nifedipine
129
A 45-year-old man presents with dizziness and right-sided hearing loss. Which one of the following tests would most likely indicate an acoustic neuroma?
```
Jerky nystagmus
Left homonymous hemianopia
Tongue deviated to the left
Fasciculation of the tongue
Absent corneal reflex
```
Loss of corneal reflex - think acoustic neuroma
130
A 60-year-old woman presents with a tremor. Which one of the following features would suggest a diagnosis of essential tremor rather than Parkinson's disease?
```
Difficulty in initiating movement
Tremor is worse following alcohol
Postural instability
Unilateral symptoms
Tremor is worse when the arms are outstretched
```
Tremor is worse when the arms are outstretched
Difficulty in initiating movement (bradykinesia), postural instability and unilateral symptoms (initially) are typical of Parkinson's. Essential tremor symptoms are usually eased by alcohol.
131
A 31-year-old man attends his appointment with an oncologist after being referred by his family physician. The man reported feeling nauseous and having a persistent headache for the past month. The pain was not relieved by the regular over-the-counter painkiller. The patient confirms that he has a significant family history of cancer and this is why he has been referred to see the oncologist.
After chromosome analysis which revealed an abnormality related to the chromosome 3p, the patient is explained that his condition will put him at higher risk of developing several tumors, which could be both benign and malignant. The oncologist tells the patient that this condition can be passed on to future generations and that the patient's children have a fifty-percent chance of developing the condition, given his spouse does not carry the mutation as well. The patient is happy to know that he will not need a screening flexible sigmoidoscopy.
Which of the following diseases is the patient at the highest risk of developing due to his underlying condition?
```
Lung carcinoma
Clear-cell renal carcinoma
Prostate carcinoma
Breast carcinoma
Osteogenic sarcoma
```
Clear-cell renal carcinoma
This patient presented with a persistent headache and feeling nauseous, in the setting of a positive family history of cancer. This has prompted the family physician to make a referral to an oncologist for further investigations. An abnormality related to the chromosome 3p, which carries the VHL gene, indicates that this man might be suffering from Von Hippel-Lindau (VHL) syndrome. This is an autosomal dominant condition which increases the risk of developing several cancers. Clear-cell renal carcinoma is one of the tumors which patient with VHL syndrome are at an increased risk of suffering from, although the most common tumors are hemangiomas. None of the other cancers mentioned below are associated with VHL although they may occur in a person with VHL.
132
Which one of the following is most associated with a good prognosis in Guillain-Barre syndrome?
```
Age > 40 years
Female sex
No history of a diarrhoeal illness
High anti-GM1 antibody titre
Low peak expiratory flow rate
```
Preceding gastrointestinal infections are associated with a poor prognosis in Guillain-Barre syndrome. The sex of the patient has not been shown to correlate with outcome
