Passmedicine Flashcards
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
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.
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)
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).
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
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.
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
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.
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
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
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
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.
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
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
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
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)
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
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.
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
No medication changes
Breast feeding is acceptable with nearly all anti-epileptic drugs
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
Male sex
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
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
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
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.
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
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.
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
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
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
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.
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
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
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
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.
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
Sodium valproate may cause tremor
Important for meLess important
Alopecia is much more common than hirsutism in patients treated with sodium valproate.
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
SLE
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
Focal impaired awareness seizure
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
Pergolide
This patient has developed pulmonary fibrosis (explaining the restrictive picture on spirometry) secondary to pergolide therapy
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
Motor neuron disease - treatment: NIV is better than riluzole
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 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.
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
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
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.
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.
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
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
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
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
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.
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
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
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
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
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.
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.
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
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.
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
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
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.
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
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
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
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
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.
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.
Which of the following is least associated with Parkinsonism?
Chlorpromazine Progressive supranuclear palsy Dementia pugilistica Lead poisoning Wilson's disease
Lead poisoning
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
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
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.
