The most important features of Lewy body dementia that differentiate it from the other forms of dementia are the:

• Visual hallucinations

• Fluctuating course with lucid intervals

• Signs of mild Parkinsonism

Typical presentation of Lewy body dementia :

• Dementia is usually the presenting feature, with memory loss, decline in problem-solving ability, and spatial awareness difficulties.

• Characteristically there are fluctuating levels of awareness and attention.

• Signs of mild Parkinsonism (tremor, rigidity, poverty of facial expression, festinating gait). Falls frequently occur.

• Visual hallucinations and illusions. 

This patient has hyponatremia leading to cerebral oedema. Cerebral oedema is defined as an excess in the accumulation of fluid in the intracellular or extracellular spaces of the brain there are various causes for this phenomenon, but there are typically four types that can be seen in clinical practice.

The important type to concentrate on for this question is the osmotic type. When plasma is diluted, such as in hyponatremia states, it creates an abnormal pressure gradient between the brain’s internal environment and the serum. This causes the movement of water into the brain. This patient has clear hyponatremia as evidenced by her lab values.

An important cause of hyponatremia is high blood sugar and iatrogenic administration of a hypotonic solution. This patient has been receiving a hypotonic solution and 5% of intravenous dextrose for an extended period. This would naturally increase her blood glucose levels and dilute her intracellular volume leading to hyponatremia. For this reason, sodium chloride 0.9% is usually used instead of hypotonic solutions.

Osmotic shrinkage of the brain describes the result of osmotherapy whereby the volume of intracranial fluid is reduced by shifting fluid out of the brain and into the serum. An example of this type of therapy is administering intravenous mannitol to reduce intracranial pressure. This answer is incorrect as it is the opposite of what the question asks for.

Cerebral demyelination describes any condition that causes the myelin sheath of neurons to become damaged. Although a presenting symptom of demyelination is weakness, this answer is not correct since this patient does not present with the host of signs and symptoms that can be seen in demyelinating diseases which can be anything from diplopia to speech problems.

A common iatrogenic cause of cerebral demyelination is central pontine myelinolysis which is caused by rapid correction of low blood sodium levels. Cytotoxicity is incorrect since the stem gives no clue as to any cytotoxic drug that this patient might have taken. An important cytotoxic drug to remember for the exam is isoniazid, which can lead to cerebral oedema.

Reye’s syndrome describes a rapidly progressive encephalopathy that usually presents with liver toxicity, personality changes, confusion, and even seizures. The cause is unknown, but viral infections are thought to play a role. This answer is incorrect since no clue in the stem alludes to the patient having had a viral infection nor are her liver function test results included .

Remember to focus on the causes of unilateral vision loss. Know how to differentiate all the above options. The bruit in the neck is the examiner’s way of telling you that this man has atherosclerosis which is a risk factor for amaurosis fugax.

For amaurosis fugax, this is a classic case stem: sudden, painless, unilateral vision loss and description of a “black curtain coming down”.

Retinal vein thrombosis and retinal artery occlusion present as painless unilateral vision loss but their duration would be much longer if not permanent.

Optic neuritis would have clues in the stem of multiple sclerosis. Acute angle glaucoma would produce a painful vision loss and keywords such as “haloes” would be seen.

Amaurosis Fugax :

• Painless transient monocular visual loss (i.e. loss of vision in one eye that is not permanent)

• It is indicative of retinal ischaemia, usually associated with emboli or stenosis of the ipsilateral carotid artery

Presentation:

• Sudden, unilateral vision loss; “black curtain coming down”

• Duration: 5-15 minutes; resolves within < 24 hours

• Associated with stroke or transient ischaemic attack (TIA) and its risk factors (i.e. hypertension, atherosclerosis)

• Has an association with giant cell arteritis.

This is a diagnosis of Lambert-Eaton syndrome.

A key difference from myasthenia gravis is that on examination the patient has increased strength on repetition of power. It is also a paraneoplastic disorder closely associated with small-cell lung cancer.

Because Lambert-Eaton and myasthenia gravis present similarly, the case stem usually would provide you with an investigation or examination clue, including autoantibodies and EMG results.

Note that thyrotoxicosis is associated with myasthenia gravis, not Lambert-Eaton.

Lambert-Eaton syndrome :

- Young, female patients; mostly autoimmune

- Proximal weakness at the pelvic girdle/shoulder girdle

- Weakness improves with exercise as well as reflexes

- Cranial nerve involvement: dysphagia, dysarthria, ptosis, diplopia 

Diagnosis:

- EMG: decreased amplitude in CMAP after a single supramaximal stimulus but increases after exercise

- Edrophonium test: may be positive but not as prominent as in myasthenia gravis

- Look for tumor: CT/MRI of chest, abdomen, pelvis + tumor markers

Treatment:

- Treat tumor – first line

- Consider methylprednisolone and IV immunoglobulin.

This man is suffering from Wernicke’s encephalopathy.

Wernicke's encephalopathy is a neuropsychiatric disorder caused by thiamine deficiency which is most commonly seen in alcoholics.

A classic triad of confusion, ophthalmoplegia, and ataxia may occur. Treatment is with urgent replacement of thiamine If not treated Korsakoff's syndrome may develop as well.

This is termed Wernicke-Korsakoff syndrome and is characterized by the addition of Antero- and retrograde amnesia and confabulation in addition to the above classic triad.

Wernicke’s Encephalopathy: 

1. Confusion

2. Ataxia

3. Ophthalmoplegia

A brainstem lesion makes the most sense here as the oculomotor nerve arises from the anterior aspect of the mesencephalon (midbrain) while the trigeminal nerve arises from the pons. The involvement of the oculomotor nerve could explain the double vision and the involvement of the trigeminal nerve explains the facial numbness.

This young lady is likely having a recurrence of her migraine. It is not stated in the stem whether the headache is of a similar type to her previous migraines but one can assume given there are no neurological signs and no mention of neck stiffness or photophobia.

Since this is likely a migraine, no further investigations are required. If there were signs of neck stiffness or photophobia or if the headache was described as the worst headache of her life, obtaining a CT scan of the head would be the choice to rule out a subarachnoid haemorrhage.

This is a classic case of Meniere’s disease.

All four clues are present: dizziness, tinnitus, deafness, and increased feeling of pressure in the ear.

Know how to differentiate between Meniere’s with options A to C. These topics frequently appear on the neurology component of the exam.

Note that option B is ruled out due to the normal MRI.

In option C, the case would need to give a history of bacterial or viral infection.

In option D, there would be other manifestations, such as weakness or paresthesia of the limbs.

Meniere’s disease :

• Dizziness, tinnitus, deafness, increased feeling of pressure in the ear. Note: Vertigo is usually the prominent symptom

• Episodes last minutes to hours

• MRI is normal

• Usually a female >> male ; 20-60 years old

• Typically symptoms are unilateral but bilateral symptoms may develop after a number of years

Treatment:

• Acute attacks: Buccal or intramuscular prochlorperazine. Admission is sometimes required.

Alzheimer’s disease is a frequent neurology topic for exam. The main clues usually have an elderly patient undergoing a memory change with some inability to perform day-to-day tasks.

Depending on the severity of the patient, they may have language deficits, executive dysfunction, and cortical atrophy in CT brain.

Acetylcholinesterase inhibitors donepezil, galantamine, and rivastigmine are the first line. Memantine is the second line for severe cases.

This patient is not suffering from hallucinations; therefore, options C and D are ruled out.

While the patient does have depression and would be placed on antidepressants, amitriptyline is contraindicated as it has anticholinergic side effects.

This is a classic case of Meniere’s disease. All four clues are present: dizziness, tinnitus, deafness, and increased feeling of pressure in the ear. It is important to remember that in Meniere’s disease, the attacks can last for several hours at a time, which differentiates it from benign paroxysmal positional vertigo.

This is a diagnosis of obstructive sleep apnoea syndrome. This topic would overlap between neurology and respiratory medicine.

The typical clues: a middle-aged to elderly male who works at the office, obese, with a history of hypertension and/or diabetes, and “snoring loudly at night”.

The initial test is pulse oximetry but the definitive investigation is polysomnography (gold standard).

Multiple sleep latency test is the definitive investigation for narcolepsy.

Obstructive sleep apnoea syndrome :

- Middle-aged to elderly male

- Increased daytime sleepiness, fatigue, sleep disruption

- Snoring loudly at night

- Associated with hypertension, diabetes, obesity

Diagnosis:

- Pulse oximetry, overnight study of breathing pattern (initial investigation)

- Polysomnography (gold standard)

Treatment:

- Conservative: weight loss and reduced alcohol consumption

- Continuous positive airway pressure (CPAP).

The likely cause of his confusion and worsening headache is a subdural haemorrhage. A CT scan is diagnostic.

Subdural haematoma may be acute or chronic. In chronic subdural haematoma, symptoms may not be apparent for several days or weeks. Symptoms of subdural haematomas are fluctuating levels of consciousness, ± insidious physical or intellectual slowing, sleepiness, headache, personality change, and unsteadiness.

Chronic subdural haematoma occurs in very old or severe alcoholics. A shrunken brain is rattled around the head by minor trauma, tearing venous sinuses. Over several days or weeks, mental function deteriorates as haematoma forms.

CT scan is diagnostic, and surgical evacuation provides a dramatic cure. Remember, chronic subdural haematoma usually presents as an elderly, on anticoagulation, or an alcoholic who may have a history of falls. Slow onset of symptoms compared to epidural haematoma.

DIFFERENTIATING TYPES OF TRAUMATIC BRAIN INJURY :

Extradural haematoma:

• Often associated with skull fracture and middle meningeal artery injury

• Lucid intervals (Patient goes back to doing what he was doing before falling unconscious again)

Subdural haematoma:

Chronic Subdural haematoma :

• Usually elderly, on anticoagulation or alcoholic

• History of minor fall with minor head injury

• Slow onset of symptoms compared to epidural haematoma

Acute Subdural haematoma :

• The probable reason is that it is difficult to differentiate from epidural as both have lucid intervals

• Think bigger trauma. Sicker patient

• Patient is barely awake during the lucid intervals In reality, we do not rely on clinical features to differentiate epidural haematoma from acute subdural haematoma because both of them will get a CT scan!

Subarachnoid haemorrhage:

• Aneurysm formation is the most common aetiology

• Usually spontaneously in the context of a ruptured cerebral aneurysm but may be seen in association with other injuries when a patient has sustained a traumatic brain injury

• Association with polycystic kidney disease, Ehlers-Danlos syndrome, and other connective tissue disease

• Hallmark – THUNDERCLAP HEADACHE

• Meningeal irritation (neck stiffness, photophobia).

This is a classic presentation: Gait disturbance, sphincter compromise, and dementia.

NPH has a much slower progressive memory loss than Alzheimer’s.

Normal pressure hydrocephalus :

• TRIAD: gait disturbance, urinary incontinence, cognitive dysfunction

• Most common in elderly

• CT / MRI brain: enlarged lateral and third ventricles

Diagnosis:

• Clinical diagnosis with CT/MRI brain signs of enlarged lateral and third ventricles

Treatment:

• Cerebrospinal fluid (CSF) shunting: ventriculoperitoneal, ventriculopleural, or ventriculoatrial.

The answer here is aspirin 300 mg. The history and examination are convincing for a stroke. Before any treatment can happen we need to exclude a haemorrhagic stroke with the help of imaging. This was done in this question which showed an ischaemic stroke.

The other options are less likely to be the appropriate next course of action.

The window period to administer alteplase is 4.5 hours from the onset of stroke symptoms. If we cannot be certain of this period, we cannot proceed to give alteplase. Firstly, there was no time given in the stem. Secondly, the patient woke up to the symptoms of a stroke. The ischaemic event could have happened anytime during the night.

Warfarin should not be started until brain imaging has excluded haemorrhage, and usually not until 14 days have passed from the onset of an ischaemic stroke. Note that alteplase is preferred over streptokinase.

So streptokinase is rarely the right answer.

Statins need to be offered but this is not the most appropriate next course of action. There is no urgency in giving statins.

Note for the exam, the keywords for subarachnoid haemorrhage (SAH) are “worst headache of my life”, “headache of severe intensity”, “thunderclap headache”, “sudden onset”, occipital”. The other main clue in this stem is the patient was found drunk. Excess alcohol consumption is one main risk factors leading to SAH.

SUBARACHNOID HEMORRHAGE :

Usually the result of bleeding from a berry aneurysm in the Circle of Willis 

• Sudden and severe occipital headache described as the “worst headache of my life” or “thunderclap headache”

• Neck stiffness or pain

• Vomiting, collapse, seizures

Associations :

• Hypertension

• Polycystic kidney disease. Berry aneurysms are found in 10% of patients with autosomal dominant adult polycystic kidney disease

• Ehlers Danlos syndrome

Diagnosis :

• CT brain

• Lumbar puncture : The CSF of a lumbar puncture bloody then xanthochromic (bilirubin).

Lucid intervals followed by unconsciousness are typical for an epidural haemorrhage in which the middle meningeal artery is involved.

Extradural (epidural) haematoma often associated with skull fracture and middle meningeal artery injury. Involves arterial blood.

Features :

• Features of raised intracranial pressure

• Patients may exhibit a lucid interval

Management : Surgical procedure: Burr hole followed by craniotomy and evacuation of the haematoma.

Signs of Alzheimer’s include:

• Memory lapses

• Forgetting the names of people and places

• Inability to remember recent events

• Problems with planning and decision-making

• Confusion

• Wandering, disorientation

Schizophrenia is characterized by false beliefs, confusion, and auditory hallucinations.

Bell’s palsy is the most likely diagnosis here although it is very important in clinical practice to exclude the remaining disorders. Bell’s palsy may present with pain around the ear during the early days.

Ramsay-Hunt syndrome may present similarly to this but usually with an association with an ear rash.

Antivirals would be appropriate for such a presentation.

Parotid tumours can also compress the facial nerve causing facial palsy however this stem did not include any mass or swelling around the jaw.

Multiple sclerosis is an upper motor neuron disease which would mean that she would be able to raise her eyebrow.

Treatment of Bell's Palsy :

- Within 72 hours on set, give prednisolone (also in pregnancy)

- Acyclovir if suspecting

Which side of the face is affected by Bell’s palsy?

Answer: The patient’s right face (i.e. right facial nerve) is affected. He is trying to smile and only his left facial muscles are working.

When people with amyotrophic lateral sclerosis can no longer get enough nourishment from eating, it is advisable to insert a feeding tube into the stomach. This method is called percutaneous endoscopic gastrostomy. Since this patient is having difficulty eating and since this is a chronic degenerative condition in which long-term feeding is required, this is the single best method to provide adequate nutrition to him.

The use of a feeding tube also reduces the risk of choking and aspiration pneumonia. The tube is not painful and does not prevent people from eating food orally if they wish.

Dietitians advise preparing numerous small meals throughout the day that provide enough calories, fiber, and fluid for patients with ALS.

Sudden onset severe occipital headache and vomiting with stiffness make the likely diagnosis to be subarachnoid haemorrhage. The neck stiffness is due to meningeal irritation. Usually, patients would be complaining of photophobia as well. Patients often describe the headaches as “the worst headache of my life”.

Given her symptoms of dysphagia, tongue fasciculations, nasal regurgitations, and dysarthria, the likely diagnosis is bulbar palsy.

The phrase "A bovine cough" is used to describe the non-explosive cough of someone unable to close their glottis. It is seen in vagus nerve lesions and may be associated with dysphonia.

Bulbar relates to the medulla. Bulbar palsy is the result of diseases affecting the lower cranial nerves (VII-XII).

Presentation :

• Tongue - weak and wasted and sits in the mouth with fasciculations.

• Drooling - as saliva collects in the mouth and the patient is unable to swallow (dysphagia).

• Dysphonia - a rasping tone due to vocal cord paralysis; a nasal tone if bilateral palatal paralysis.

• Articulation - difficulty pronouncing “r”; unable to pronounce consonants as dysarthria progresses.

The case stem for epidural haemorrhage usually has a young athletic patient that had a head injury during a sports match. They will experience a “lucid interval” before having a decrease in mental function. The stem may or may not give you a CT brain result: “bi-convex” mass.

This picture is of amyotrophic lateral sclerosis with bulbar onset. In MND, motor nerves become damaged and eventually stop working.

Therefore, the muscles that the damaged nerves supply gradually lose their strength. 

The main types of MND are:

Amyotrophic lateral sclerosis (ALS) : This is the classical MND and the most common type.  Symptoms tend to start in the hands and feet. The muscles tend to become stiff as well as weak at first.

Progressive bulbar palsy (PBP) : The muscles first affected are those used for talking, chewing, and swallowing (the bulbar muscles). This is an uncommon form of MND. The small muscles of the hands and feet are usually first affected but the muscles are not stiff.

Primary lateral sclerosis (PLS) : This is a rare type of MND. It mainly causes weakness in the leg muscles. Some people with this type may also develop clumsiness in their hands or develop speech problems.

The only subtype that has the potential to be asked is Amyotrophic lateral sclerosis (ALS)

Inattention or neglect is a feature of parietal lobe lesions. Seizures may occur in space-occupying lesions.

The most likely explanation for this answer is that the cerebral tumour has caused the right parietal lobe of the brain to be affected which can lead to neglect for the left side of the visual field, causing a patient with neglect to behave as if the left side of sensory space is nonexistent (although they can still turn left).

The most important features of Lewy body dementia that differentiate it from the other forms of dementia are the:

• Visual hallucinations

• Fluctuating course with lucid intervals

• Signs of mild Parkinsonism

Two of which are found in this stem: signs of parkinsonism and visual hallucinations.

She is an elderly lady on anticoagulation. A trivial fall may have gone unnoticed. Warfarin and a high INR are risk factors for a subdural haemorrhage. Progressive confusion over days to weeks supports this diagnosis.

This is most likely a diagnosis of Diabetes Insipidus.

The diencephalon consists of the thalamus, the hypothalamus, the epithalamus, and the subthalamus. The most likely location for an intracranial tumour causing diabetes insipidus would be the diencephalon.

In contrast, the most likely location for a brain tumour causing SIADH (Syndrome of Inappropriate Antidiuretic Hormone) would be the cerebrum or cerebellum. Both DI and SIADH are common in cancer patients or those with brain tumours so it is important to know the most likely tumour locations causing these diseases. 

DIABETES INSIPIDUS TYPE AND FEATURES :

Cranial DI :

• Decreased secretion of ADH reduces the ability to concentrate urine and so causes polyuria and polydipsia.

Nephrogenic DI :

• Decreased ability to concentrate urine because of resistance to ADH in the kidney.

Tuberculous meningitis would show lymphocytosis and decreased glucose.

Characteristic CSF findings in meningitis:

Bacterial meningitis:

Glucose- Low Protein, High White cells - mainly neutrophils

Viral meningitis:

Glucose-Normal, Protein-Normal to high, White cells-Increased lymphocytes

Tuberculous meningitis:

Glucose- low Protein, high White cells-Neutrophils in early disease, lymphocytes later

The questions are usually very straightforward. They would usually only ask for diagnosis, investigation, and management. However occasionally, there would be a few odd questions where they would ask for the mechanism behind certain pathology.

In this case stem, the patient has a history of prostate carcinoma; therefore, the symptoms presented here would most likely indicate metastasis/tumour that is compressing on the cauda equina.

Option A would produce a degree of voiding dysfunction but not in the initiation of voiding and there would not be sensory changes.

Option C although possible will not produce severe back pain or perineal numbness.

Cauda Equina syndrome :

- Severe back pain - “saddle distribution sensory loss”

- Difficulty to initiate voiding

- Urinary or faecal incontinence

- Distal motor weakness

Diagnosis:

- MRI spine

Treatment:

- This is a neurosurgical emergency and needs a neurosurgery referral.

The imaging features are consistent with cerebral metastases which likely originated from the breast by haematogenous spread. The key treatment components of patients with cerebral metastasis revolve around the control of peritumoral oedema and control of high intracranial pressure of which corticosteroids do the trick.

In most subdural haemorrhage case stems for exam, most will present as elderly and male (chronic subdural haemorrhage). Pay attention to history details such as history of falls or other trauma.

Subdural haemorrhage :

- Fluctuating levels of consciousness

- Physical or intellectual slowing

- Sleepiness, headache, personality changes

- Unsteadiness - Seizures

Diagnosis:

- CT brain / MRI brain : Clot +/- midline shift & “crescent-shaped” haematoma

Treatment: Evacuation via burr hole craniotomy.

The exam clues for vascular dementia: > 50 years with a history of smoking, hypertension, and past stroke or transient ischaemic attack.

Cardiovascular risk factors would ALWAYS be present in the stem. Often there would be a “step-wise” deterioration of symptoms. 

The ataxia, intentional tremors, and dysarthria point towards a cerebellar disorder.

Aetiology includes stroke or transient ischaemic attack (TIA)

Presentation : As the cerebellum is associated with motor control, lesions produce a range of movement disorders (ataxias). Lesions of the midline vermis of the cerebellum cause truncal ataxia, while lesions of the cerebellar hemispheres cause limb ataxia of the ipsilateral side.

Post-management of Transient ischaemic attack (TIA) Clopidogrel (75 mg daily) is the preferred long-term antiplatelet (off-label use in TIA). Current guidelines recommend treatment with high-intensity statin following a TIA for all patients unless contraindicated.

For exam, stroke questions come in various forms ranging from the anatomical locations of the lesion, to investigations and management. Pay special attention to the anatomy of the lesions especially the vascular distribution and the functions of the different parts of the brain. i.e. anterior cerebral artery

– frontal and medial cerebrum : middle cerebral artery

– lateral part of the hemisphere : posterior cerebral artery

– occipital lobe : basilar artery

Post-management of Transient Ischaemic Attack (TIA) : Aspirin is given for 2 weeks followed by clopidogrel (75 mg daily) as clopidogrel is the preferred long-term antiplatelet for secondary prevention.

If clopidogrel is contraindicated or not tolerated, give a combination of modified-release dipyridamole (200 mg twice daily) and low-dose aspirin. A statin should also be offered as soon as possible after a TIA.

This patient is presenting with upper motor neuron signs and optic neuritis. The diagnosis is relapsing-remitting multiple sclerosis. The patient is eligible for interferon-beta management; however, the question asks for the most appropriate initial management, which in this case would be methylprednisolone.

The keys, in this case, stem: the patient has been struggling with word choice (temporal lobe) and disinhibition / urinating on the sofa (frontal lobe).

Alzheimer’s disease usually presents with memory loss first before personality changes.

Vascular dementia would have a “step-wise progression” and they would have had some sort of previous cardiac/vascular event in the stem.

Lewy body disease would require a clue into having some Parkinsonian symptoms.

This is termed Wernicke-Korsakoff syndrome and is characterized by the addition of Antero- and retrograde amnesia and confabulation in addition to the classic triad of confusion, ataxia, and ophthalmoplegia found in Wernicke's encephalopathy.

Those with Korsakoff syndrome may "confabulate," or make up, information they can not remember. They are not "lying" but may believe their invented explanations. They create events to fill the gaps in day-to-day memory.

For example, a person who has been in hospital for several weeks may talk convincingly about having just visited their aunt earlier that day. This is more common in the early stages of the condition. Individuals may seem able to carry on a coherent conversation, but moments later be unable to recall that the conversation took place or to whom they spoke.

They can often answer questions promptly with inaccurate and sometimes bizarre answers.

An elderly man with a history of falls and on anticoagulation with progressive confusion over the last couple of days points towards the diagnosis of chronic subdural haemorrhage. Chronic subdural haematoma occurs in very old or severe alcoholics.

A shrunken brain is rattled around the head by minor trauma, tearing venous sinuses. Over several days or weeks, mental function deteriorates as haematoma forms.

CT scan is diagnostic, and surgical evacuation provides a dramatic cure. Remember, Chronic subdural haematoma usually presents as an elderly, on anticoag or an alcoholic who may have a history of falls. Slow onset of symptoms compared to epidural haematoma.

Shy-drager syndrome : A rare neurodegenerative disorder, caused by cell loss in certain areas of the brain and the spinal cord, leading to a variety of symptoms characterized by Parkinsonian features, cerebellar ataxia, and autonomic dysfunction (particularly urogenital).

Presentation:

• Urinary dysfunction is the most common presentation

• Erectile dysfunction

• Postural hypotension

• Cerebellar ataxia

• Parkinsonian symptoms with poor response to levodopa.

In the exam, if you get a stem with characteristics of parkinsonism (rigidity, pill-rolling tremor of the hands) plus autonomic dysfunction like urinary incontinence, erectile dysfunction, consider Shy-drager syndrome as the answer.

Absence seizures would usually be a child with either the parent or teacher noticing that the patient is “daydreaming” often and when they resume their studies they are not able to perform well.

Absence seizure :

- Child usually <10 years old

- Loss of awareness (“daydreaming”), staring blankly into space, will not respond to their surroundings

- May be accompanied by upturning of eyes, other repetitive movements

- Will return to normal activities after a seizure but may not perform well and feel tired

- No photosensitivity

- Maybe triggered by hyperventilation

Diagnosis:

- EEG

- Check FBC, glucose levels, ECG, and MRI for other underlying causes

Treatment: - If recurring, sodium valproate or ethosuximide.

Cervical spondylosis is chronic cervical disc degeneration with herniation of disc material, calcification, and osteophytic outgrowths.

Headache in the back of the head and pain in flexing the neck is an early feature of cervical spondylosis which gradually progresses to later symptoms like radiculopathies due to root compression in arms and hands.

It is quite unclear what sort of speech difficulties this patient has. However with a combination with nystagmus, one can assume that this patient is having cerebellar dysarthria (also known as ataxic dysarthria).

Cerebellar dysarthria is an acquired neurological and sensorimotor speech deficit caused by damage to the superior cerebellum and the superior cerebellar peduncle.

If, for example, there is damage to the left cerebellar hemisphere in a right-handed person, ataxic dysarthria could develop. Of all the individual dysarthria types, it most clearly reflects a breakdown in timing and coordination.

This is a classic presentation of acute migraine. First-line therapy is triptans according to NICE guidelines.

Be aware that opioids are never given during an acute onset of migraine.

Migraine :

• History of unilateral, throbbing headache

• Associated with nausea / vomiting, photophobia

• Associated with aura/triggers

• May be described as: “need to limit activities” or “prefer to stay in a dark room”

Diagnosis:

• Clinical diagnosis

• May require CT / MRI head to rule out other pathology

Treatment:

• Sumatriptan - first line

• Monotherapy: Triptan, NSAIDs, Aspirin, Paracetamol

• Combination therapy: Triptan + NSAID; Triptan + Paracetamol

• Transcutaneous stimulation of the vagus nerve

• Avoid triggers.

Drop attacks are sudden spontaneous falls while standing or walking, with complete recovery in seconds or minutes. There is usually no loss of consciousness, and the event is remembered.

The remaining options given usually have a prodrome or identifiable symptoms before the episodes like dizziness or pallor.

Drop attacks refer to unexplained falls with no prodrome, no loss of consciousness, and rapid recovery.

The proportion of falls due to ‘drop attack’ increases with age.

Causes :

• Vertebrobasilar insufficiency

• Weak legs (eg cauda equina syndrome)

Note that the usual cause is sudden weakness of the legs that causes the patient, usually an older woman, to fall to the ground.

There is no warning, no loss of consciousness, and no confusion afterward.

The condition is usually benign, resolving spontaneously after several attacks.

The hallmark of Pick’s disease is behavioral changes, inappropriate behavior, and disinhibition. In the early stages of Pick’s disease memory, visuospatial skills, and calculation are relatively intact and this differs from Alzheimer’s where usually memory would be affected first.

Myofascial pain syndrome is a chronic pain disorder characterized by regional muscle pain associated with restricted painful regions or trigger points. The pain is often aching and deep in nature. Myofascial pain syndrome typically occurs after a muscle has been contracted repetitively.

In this case, the long car ride journey has caused muscles to contract repetitively. In the options given, only myofascial pain/muscle sprain relieves on change of position.

The trapezius, levator scapula, infraspinatus, and scales are commonly affected areas but in this question with the mid-thoracic vertebra, it is likely the trapezius that is affected the most. The remaining options are wrong.

Osteoporotic fracture of the vertebrae would cause severe pain which will not improve on bending or any movement.

Change to carbamazepine is the best option given the options provided. If there was an option that said, to change to carbamazepine and add folic acid 5mg, that would be an even better answer. One would not stop antiepileptic medication especially if the patient has only been seizure-free for one year.

The patient needs to be seizure-free for more than 2 years before it would be considered reasonable to stop antiepileptic medication before pregnancy.

If a woman is taking antiepileptic drugs and planning a pregnancy, the general advice to give to her is about the increased risks of fetal malformations, neurodevelopmental delay, and possible increased seizure frequency in pregnancy. Since there is no agreement as to which antiepileptic drug is most or least teratogenic, the antiepileptic drug that stops seizures in a given patient is the one that should be used.

However, there is one exception to the rule, SODIUM VALPROATE! The risk of teratogenicity with Valproate is greater than the risk of teratogenicity with other antiepileptic drugs. Hence, sodium valproate should always be changed to another antiepileptic drug before pregnancy.

The lesions of multiple sclerosis most commonly affect the white matter in the optic nerve, brainstem, basal ganglia, and spinal cord, or white matter tracts close to the lateral ventricles.

The brainstem provides the main motor and sensory innervation to the face and neck via the cranial nerves.

It includes the midbrain, the medulla oblongata, and the pons. Since this patient is experiencing deficits in her trigeminal nerve (CN V) and her vestibulocochlear nerve (CN VIII), this answer makes the most sense.

The history of falls and broad-based clumsy gait (balance and gait disturbance), strange behavior and disorientation to time and place (due to dementia), and episodes of enuresis (urinary incontinence) point towards normal pressure hydrocephalus.

Normal pressure hydrocephalus will present with prominent gait abnormalities early in the course of the disease that usually precede the onset of cognitive impairment. There will also be associated urinary incontinence. Normal pressure hydrocephalus is a reversible cause of dementia seen in elderly patients.

It is thought to be secondary to reduced CSF absorption at the arachnoid villi. Has a classical triad of:

• Urinary incontinence

• Dementia

• Gait abnormality (may be similar to Parkinson's disease)

“the wet, wobbly and wacky grandpa”

Wernicke's encephalopathy is a neuropsychiatric disorder caused by thiamine deficiency which is most commonly seen in alcoholics. A classic triad of confusion, ophthalmoplegia, and ataxia may occur.

Treatment is with urgent replacement of thiamine (vitamin B1).

If not treated Korsakoff's syndrome may develop as well. This is termed Wernicke-Korsakoff syndrome and is characterized by the addition of Antero- and retrograde amnesia and confabulation in addition to the above classic triad.

When a toddler cries after a minor injury, stops breathing, and loses consciousness for a few seconds followed by rapid recovery, you should be thinking of two conditions:

1. Blue breath-holding spells

2. Reflex anoxic seizures are paroxysmal, spontaneously reversing brief episodes of asystole triggered by pain, fear, or anxiety. They are non-epileptic events caused by a reflex asystole due to increased vagal responsiveness.

They begin when a toddler typically aged from 6 months to 2 years has a sudden surprising pain or an unexpected fright, stops breathing then goes pale, rigid, and may have upward eye deviation with clonic movements.

These episodes last less than a minute and it is followed by rapid recovery. Management is just reassurance and advises parents to place the child in a recovery position during episodes.

Remember: Reflex anoxic seizures do not cause tongue biting – An important point when differentiating from epilepsy.

This is a typical case stem for migraine in exam. The patient will present with or without aura (sensory or motor) followed by the headache itself.

THIS IS EXTREMELY IMPORTANT.

The key to differentiating between a migraine or a headache of a more serious origin (e.g. subarachnoid hemorrhage) is the onset of a unilateral, throbbing headache AFTER an aura.

Anaesthetic review would be the next most appropriate step of action. The history and examination are convincing for a stroke but the patient has rapidly dropped her consciousness levels, and before any investigations and treatment can happen this needs to be addressed.

An anaesthetist would rather know at this stage rather than when a peri-arrest call is put out as her GCS reaches 4 or 5. The airway can be stabilized so that a CT scan can be performed safely.

The other options are less likely to be the appropriate next course of action. Thrombolysis or aspirin are the treatment options for ischaemic strokes but cannot be started until a CT scan excludes a haemorrhage.

Seizures lasting longer than 5 minutes should be treated urgently with intravenous lorazepam (repeated once after 10 minutes if seizures recur or fail to respond).

Intravenous diazepam is effective but it carries a high risk of thrombophlebitis (reduced by using an emulsion formulation). Absorption of diazepam from suppositories is far too slow for the treatment of status epilepticus.

The fact that she is pregnant does not change the management of a convulsive status epilepticus.

STATUS EPILEPTICUS MANAGEMENT:

• ABC protocol

• Give high-concentration oxygen

• Check blood glucose levels

• Secure intravenous access in a large vein

• Administer intravenous lorazepam as first-line treatment in hospital for ongoing generalised tonic-clonic seizures 

• Administer intravenous diazepam if intravenous lorazepam is unavailable, or buccalmidazolam if unable to secure immediate intravenous access. Administer a maximumof two doses of the first-line treatment (including pre-hospital treatment)

• If seizures continue, administer intravenous phenobarbital or phenytoin as a second-line treatment in hospital

• If seizure continues despite the above (>30 minutes), referral to ICU.

The patient presents with symptoms of acute alcohol withdrawal. This patient would require benzodiazepines as part of his management and would need to be referred to the medical team from A&E. IV Thiamine would also be given in the hospital. This is to help prevent Wernicke’s encephalopathy.

The clinical symptoms match those of a chronic subdural haematoma. This would be caused by a rupture of a bridging vein. Among the options given, the cerebral vein would be the most likely. Chronic subdural haematoma occurs in very old or severe alcoholics.

A shrunken brain is rattled around the head by minor trauma, tearing venous sinuses. Over several days or weeks, mental function deteriorates as haematoma forms. CT scan is diagnostic, and surgical evacuation provides a dramatic cure.

This is a classic case of alcohol withdrawal, which develops about 12 to 24 hours after the drinking abruptly stops. The symptoms can last for days afterward. The fact that the stem gives you that this patient has lost his job, is unkept and smells of stale sweat sounds much like there is a background of alcoholism in this picture.

Benzodiazepines would be the most appropriate initial management as they help with alcohol detoxification.

Video electroencephalogram : This investigation will help rule out any form of epilepsy.

Sudden onset of severe headache, vomiting then collapse are more suggestive of subarachnoid haemorrhage.

This is also supported by the medical history of Ehlers-Danlos syndrome.

Occasionally the stem would include signs of meningeal irritation. This includes neck stiffness and photophobia. Patients often describe the headaches as “the worst headache of my life”.

The history and examination are convincing for a stroke. Before any treatment can happen we need to exclude a haemorrhagic stroke with the help of imaging. This was done in this question which showed an ischaemic stroke. The next step is to start thrombolytics.

The other options are less likely to be the appropriate next course of action.

Anticoagulants should not be started until brain imaging has excluded haemorrhage, and usually not until 14 days have passed from the onset of an ischaemic stroke.

Thrombolysis or aspirin are the treatment options for ischaemic strokes but cannot be started until a CT scan excludes a haemorrhage.

All of the above options are popular topics in exam.

Meniere’s disease usually involves tinnitus, deafness, and increased ear pressure in the case stem.

Vestibular neuronitis, while similar to Meniere’s does not have hearing loss.

Acoustic neuroma as it grows into the cerebellopontine angle would involve cranial nerves V, VII, and VIII; therefore, you would have a variation of symptoms involving these three nerves.

Between options A and B, the symptoms in the case stem would not spontaneously resolve for option A.

Please see Q-39

For the exam, it is important to know the difference between dementia and delirium.

The onset of delirium is more acute with fluctuating levels of consciousness. There will be disorientation, mood changes, paranoia, and some memory impairment. Urinary tract infections are a common cause of delirium in the elderly.

Delirium Presentation:

• Acute onset

• Fluctuating levels of consciousness

• Often in the elderly

• Disorientation, mood changes, paranoia

• Memory impairment

• Abnormalities of perception e.g. hallucinations or illusions

• Withdrawn or heightened arousal

Common precipitating factors

• Metabolic derangement

• Hypoxia

• Infection

• Dehydration

• Constipation

• Medications e.g. benzodiazepines

Treatment:

• Treat underlying cause

• Consider anti-psychotics.

Please see Q-13

The patient in this scenario is exhibiting signs of disinhibition. This feature is most characteristically seen in those with frontotemporal dementia. They may also have features of insisting on ingesting sweet things as well as angry and violent outbursts.

Alzheimer’s disease is not correct as in Alzheimer’s disease, patients classically show signs of reduced function such as the reduced ability to speak with eventual aphasia.

To help remember, think of the three A’s: apraxia (inability to carry out purposeful movements), aphasia (loss of ability to speak), and agnosia (loss of ability to recognize what objects are and do as well as people).

Huntington’s disease is not correct as this is primarily a neurological disorder that is characterized by movement

Lewy Body dementia is not correct as the characteristic features here include increased risk of falls as well as visual hallucinations.

Cerebrovascular accident is not the correct answer as this is more typically characterized by motor/sensory loss of function. 

Electric shock-like, sharp, shooting, and stabbing are features usually used to describe trigeminal neuralgia.

Trigeminal neuralgia :

• Unilateral, shooting, or stabbing electric shock-like facial pain

• Pain exacerbated with movement or touch especially in the jaw (CN V, 2nd and 3rd branch distribution)

• Abrupt in onset and termination

Diagnosis :

• Clinical 

• MRI is routinely done to rule out other pathology (i.e. schwannoma, meningioma)

Treatment :

• Medication then surgery

• Carbamazepine > Lamotrigine / phenytoin / gabapentin

• Surgical: Microvascular decompression

The corneal reflex in this stem is intact which makes the the choice of answer clearer.

Post management of Transient ischaemic attack (TIA), Stroke :

Clopidogrel (75 mg daily) is the preferred long-term antiplatelet. If clopidogrel is contraindicated or not tolerated, give a combination of modified-release dipyridamole twice daily and low-dose aspirin.

Ensure a statin has been offered as soon as possible after a TIA Stroke.

Aspirin 300 mg daily for 2 weeks is given immediately after an ischaemic stroke is confirmed by brain imaging.

This boy has just had a complex partial seizure. The term “partial” (or focal) seizure means that the electrical activity is limited to a part of one cerebral hemisphere. The term “complex” seizure means there was the loss of awareness. The seizure is likely caused by the lesion seen on the CT scan that is occupying space.

Epilepsy - is incorrect as the termed epilepsy would require at least two unprovoked seizures occurring more than 24 hours apart for the definition to fall into place.

Dementia  - is incorrect as dementia is a brain disorder causing memory loss and decline in cognition. It occurs in the elderly and does not cause seizures.

Huntington’s chorea - is also incorrect. Firstly, symptoms of Huntington’s disease become noticeable between the ages of 35 and 44 years old. This boy is too young to develop symptoms of Huntington’s disease. Secondly, Huntington’s chorea is characterized by jerky, random, and uncontrolled movements.

These are classic features of Bell’s palsy. Bell’s palsy can strike anyone at any age although less common than 15.

Facial weakness presentations at a young age like this stem usually warrant an urgent referral to pediatrics as Bell’s palsy is less likely to be a cause and should be investigated for other potential reasons. This question however clearly points towards the diagnosis of Bell’s palsy.

For the exam, it is important to know how to differentiate the different types of headaches (e.g. intracerebral haemorrhage, migraine, cluster headache, tension headache)

Features of a cluster headache in the exam usually include a male undergoing some sort of activity with a rapid onset of a severe headache with eye involvement.

The eye pain is usually periorbital. As the name implies, the frequency of these headaches occurs in “clusters”.

Look at the time frame in the stem: there is usually a clue to having frequent past episodes with/without a period of remission.

CLUSTER HEADACHE :

- Excruciating headache: Pain is often worse compared to tension-type headaches or even migraines.

- Unilateral orbital, supraorbital, temporal - Intermittent for days to years

- Pain description can vary (can be sharp, burning, or throbbing)

- Triggers include stress, alcohol, exercise

- Accompanied by redness, lacrimation, lid swelling, nasal stuffiness

- Intense pain around one eye (recurrent attacks 'always' affect the same side). Pain is so severe that patients are often restless or agitated.

Management:

- Acute: sumatriptan subcutaneous + 100% oxygen

- Prophylaxis: Calcium channel blockers (verapamil)

Stroke questions come in various forms ranging from the anatomical locations of the lesion, to investigations and management. Pay special attention to the anatomy of the lesions especially the vascular distribution and the functions of the different parts of the brain. In this case stem, the vascular system affected is the vertebrobasilar circulation.

Therefore, symptoms will arise from one or all of the following areas: cerebellum, brainstem, and occipital lobes.

Knowing the contents of the ABCD2 and the CHA2DS2-VASc scoring system is necessary for the exam and knowing when to apply each scoring system is vital.

The ABCD2 score is to assess the risk of future stroke in the next 7 days in patients who have already suffered from a transient ischaemic attack (TIA).

After a TIA, early recurrent stroke is common. Around 10-15% have a second TIA/CVA in the first week often within the first 48 hours. The ABCD2 score helps the physician decide on how to manage the patient.

The CHA2DS2-VASc score is to assess the risk of future stroke in patients who have atrial fibrillation. This score is then used to determine whether or not treatment is required with anticoagulation therapy.

Remember:

ABCD2 = Risk of future stroke following TIA because of how soon to refer to a specialist.

CHA2DS2-VASc = Risk of future stroke in patients with atrial fibrillation because of the necessity of anticoagulation therapy (Warfarin or Aspirin)

The case stems from subarachnoid haemorrhage usually middle-aged patients with sudden, severe onset headaches, which may be described as “the worst headache of their lives” or “thunderclap headache”. This is also important to note since there are middle cerebral artery [MCA] occlusions (due to ischaemia) and

MCA berry aneurysms (causing subarachnoid haemorrhage). Ischaemic occlusions would usually have more stroke symptoms provided in the stem such as motor and sensory loss, rarely presenting with headache. 

The topic to be discussed here is Diabetes Insipidus which accounts for her urinary frequency. In this question, cranial diabetes insipidus is described. It is due to a deficiency of circulating arginine vasopressin (antidiuretic hormone). The pathophysiology behind this is pituitary infiltration by metastases originating from the breast.

A little anatomy knowledge is needed in this question. Among all of the answers, the diencephalon would fit as it gives rise to the posterior forebrain structures including the thalamus, hypothalamus, posterior portion of the pituitary gland, and pineal gland.

This is a classic example of cauda equina syndrome. It is a serious neurologic condition in which damage to the cauda equina causes loss of function of the nerve roots of the spinal canal below the termination (conus medullaris) of the spinal cord.

Any lesion which compresses or disturbs the function of the cauda equina may disable the nerves although the most common is a central disc prolapse. Spinal cord compression or cauda equina syndrome are neurological emergencies that require immediate referral and intervention.

The management of true cauda equina syndrome frequently involves surgical decompression. Pain due to a herniated lumbosacral disc may settle within six weeks. If it does not, or there are red flag signs such as the possibility of cauda equina syndrome like in this case, referral to an orthopaedic surgeon or a neurosurgeon should be considered.

Red flags that suggest cauda equina syndrome include:

• Severe or progressive bilateral neurological deficit of the legs, such as major motor weakness with knee extension, ankle eversion, or foot dorsiflexion

• Recent-onset urinary retention or urinary incontinence 

• Recent-onset faecal incontinence 

• Saddle anaesthesia or paraesthesia

These are features of acute alcohol withdrawal. Chlordiazepoxide would help reduce his symptoms.

The reason combined hormonal contraception is not used in patients with migraine with aura is that they have a 2-4 time added risk of cerebrovascular accident. It is quite important to remember the differences between migraines with and without auras.

Auras can be described as warning signs before the headache begins.

Aura characteristics:

• Occurs before the onset of headache

• Lasts 5 to 60 minutes

• Visual symptoms (homonymous hemianopia, fortified spectra, scotoma), unilateral paraesthesia or weakness, dysphasia.

• Headache either begins before the end of the aura or within an hour of the end

Migraine with aura is an absolute contraindication to using the combined oral contraceptive pill due to an increased risk of cerebrovascular accidents. Mirena IUD is adviced in patients who suffer from migraine with aura.

Benzodiazepines are the recommended drugs for alcohol detoxification. They have a slower onset of action and therefore are less likely to lead to abuse. A reduced dose of chlordiazepoxide is commonly used. Diazepam is an alternative. In this particular case, he has hallucinations which are a sign that he is having not just an acute alcohol withdrawal but moving on to delirium tremens.

Patients with marked agitation or hallucinations and those at risk of delirium tremens (characterized by delirium, hallucinations, coarse tremor, and disorientation) may be prescribed antipsychotic drugs, such as haloperidol or olanzapine, as adjunctive therapy to benzodiazepines.

This patient has an upper motor neuron lesion as signified by a pathological extensor plantar response and hyperreflexia. Every option that was given could cause the symptoms of stroke. However, it is important to know which is the most common artery to be occluded in stroke. This would be the middle cerebral arteries.

You should suspect Guillain-Barre syndrome in ascending symptoms of paresthesia in line with the recent gastroenteritis. The pathophysiology is complicated but you would need to remember that it is an acute inflammatory demyelinating neuropathy.

GUILLAIN-BARRÉ SYNDROME :

Guillain-Barre syndrome Is a disorder causing demyelination characterized by weakness, paresthesia, and hyporeflexia. Around two-thirds precedes an infection, usually the respiratory or gastrointestinal tract.

This association with the preceding infection suggests that antibodies to the infectious organism also attack antigens in peripheral nerve tissue. In the stem, there would usually be a history of gastrointestinal or respiratory infection from anywhere between a few days to 3 weeks before the onset of weakness.

Presentation:

• Weakness - Presents with an ascending pattern of progressive symmetrical weakness, starting in the lower extremities. Reaches a level of maximum severity two weeks after the initial onset of symptoms. Facial weakness, dysphasia, or dysarthria. In severe cases, muscle weakness may lead to respiratory failure.

• Pain - Neuropathic pain, particularly in the legs

• Reflexes - Reduced or absent

• Sensory - Paresthesia and sensory loss, starting in the lower extremities.

Chemotherapy drugs such as vincristine are known for the side effects of peripheral neuropathy due to neurotoxicity.

Patients with this side effect would present with peripheral paraesthesia which is seen as numbness or tingling. Chemotherapy-induced peripheral neuropathy is typically characterized by a glove-and-stocking distribution in the hands and feet with sensory loss or hypersensitivity and in some cases motor and autonomic dysfunction.

If you were to see burn marks on your fingertips in any given question, think of a loss of sensation of temperature and pain in the hands. This is commonly seen in syringomyelia and syringobulbia. The wasted and weak hands with diminished reflexes and weak spastic legs with dissociated sensory loss are features suggestive of syringomyelia.

Syringomyelia and Syringobulbia : Syringomyelia is a rare condition in which there is a fluid-filled tubular cyst (syrinx) within the central, usually cervical, spinal cord. This leads to various neurological symptoms and signs, including pain, paralysis, stiffness, and weakness in the back, shoulders, and extremities.

Syringomyelia may also cause loss of extreme temperature sensation, particularly in the hands, and a cape-like loss of pain and temperature sensation along the back and arms. Syringobulbia occurs when the syrinx extends into the brainstem. This may affect one or more cranial nerves, resulting in facial palsies. Sensory and motor nerve pathways may be affected by interruption and/or compression of nerves

. Note: Progression of symptoms and deterioration occur over many years for syringomyelia and syringobulbia Symptoms of both Syringomyelia and Syringobulbia

• Damage to the spinal cord often leads to progressive weakness in the arms and legs, stiffness in the back, shoulders, arms, or legs, and chronic, severe pain. These can be presented at the presentation.

• Other symptoms may include headaches, a loss of the ability to feel extremes of hot or cold (especially in the hands), and loss of bladder and other functions.

• Pain and temperature sensation are lost due to spinothalamic tract damage

. • Classically, the sensation loss is experienced in a shawl-like distribution over the arms, shoulders, and upper body.

• Dysaesthesia (pain experienced when the skin is touched) is common.

• Light touch, vibration, and position senses in the feet are affected as the syrinx enlarges into the dorsal columns.

• Muscle wasting and weakness begin in the hands and then affect the forearms and shoulders.

• Tendon reflexes are lost.

This is a diagnosis of multiple sclerosis. The definitive diagnostic test would be an MRI brain and spinal cord.

This patient is presenting with upper motor neuron signs and optic neuritis. Multiple Sclerosis Presentation: A variety of symptoms involve motor and sensory mainly of the brainstem and cerebellum. It may be easier to divide them into groups to remember.

1. Transverse myelitis:

• Weakness, sensory symptoms

• Urinary urgency and retention

• Flexor spasms

• Spastic quadriparesis or paraparesis

• 2. Brainstem:

• Ataxia

• Diplopia

• Dysarthria

• Facial numbness

• Ophthalmoplegia

• Gaze palsy

• 3. Cerebellum

• Ataxia • Dysarthria

• Nystagmus 

Question usually has some clue to optic neuritis. Optic neuritis is an acute, sometimes painful, reduction or loss of vision in one eye and is a relatively common presenting symptom of MS. Colour vision may be impaired

• Also depression is common The pattern is usually: symptoms evolve over days, plateau, then resolve over days/weeks

Diagnosis:

• Mostly a clinical diagnosis

• MRI (definitive diagnostic test): demyelination and/or lesions disseminated in time and place

• Oligoclonal bands in CSF

Treatment:

• Acute: IV or oral methylprednisolone

• Interferon-beta or glatiramer acetate (first line).

Cranial nerve palsies for CN III, VI, and VII are very commonly asked. In addition to the clinical findings on examination, another exam clue pointing to this diagnosis is the “pregnancy in the third trimester”. Bell’s palsy is three times more common in pregnancy. Another scenario that may be given would be a patient who is diabetic.

Look closely at the age: If the examiners would want you to pick stroke, they would have given a much older patient and they would provide some findings of upper/lower limb deficit.

There is no travel history or going to the jungle; therefore, this option is unlikely. Option B would have a prior infection in the case stem and would most likely present with a rash. Bell’s palsy is a diagnosis of exclusion.

So be sure to exclude all other possibilities in practice before you label someone with Bell’s palsy. One special take-home point is that if the patient can close their eyes and raise their eyebrow on the affected side, then it is NOT Bell’s palsy, but an upper motor neuron lesion. Bell’s palsy is a lower motor neuron facial nerve palsy.

The likely diagnosis here is organic brain damage as he is unable to remember the accident. One can easily exclude post-traumatic stress disorder with that history as patients with PTSD are quite the opposite and would remember the experience and have flashbacks and nightmares about it. It is normal to have an amount of sadness after a major accident that involves brain injury.

This differs from severe depression, as symptoms of severe depression interfere with normal function. If the severity of sadness persisted and there was a degree of functional impairment and disability associated with low mood, then we would be able to say that this is severe depression. Symptoms of an organic brain injury may include confusion, impairment of memory, and agitation which can be seen in this stem.

Key features for case stem: young female with ascending polyneuropathy usually after a trigger (i.e. infection).

There are absent reflexes therefore multiple sclerosis is ruled out since it involves upper motor neurons. Myasthenia gravis usually would have more emphasis on fatigue especially in the proximal muscles and cranial nerves would be affected.

There is no clue in the stem that the patient is diabetic and even if he is, the onset is acute while option D would require a more gradual progressive process.

While the patient did have an infection, there was no history of travel and for option E to occur it would most likely be a diagnosis of Lyme disease.

A chronic alcoholic with a history of falls with progressive confusion over the last couple of days points towards the diagnosis of chronic subdural haemorrhage. Subdural haematoma may be acute or chronic. In chronic subdural haematoma, symptoms may not be apparent for several days or weeks.

Symptoms of subdural haematomas are: fluctuating level of consciousness, ± insidious physical or intellectual slowing, sleepiness, headache, personality change, and unsteadiness.

A chronic subdural haematoma occurs in very old or severe alcoholics. A shrunken brain is rattled around the head by minor trauma, tearing venous sinuses. Over several days or weeks, mental function deteriorates as haematoma forms. CT scan is diagnostic, and surgical evacuation provides a dramatic cure.

Remember, chronic subdural haematoma usually presents as an elderly, on anticoagulation, or an alcoholic who may have a history of falls. Slow onset of symptoms compared to epidural haematoma.

The diagnosis here is status epilepticus. The patient continues to have a seizure despite 2 doses of diazepam. Therefore, the next appropriate management would be phenytoin infusion. If the patient was not given two doses of rectal diazepam in the community, then we would administer IV lorazepam as first-line treatment in the hospital. However, because two doses of diazepam were given (most likely in the community), we would move to second-line which would be IV phenytoin.

Preference goes to IV phenytoin before using IV phenobarbital.

If this also fails then the next step would be referral to ICU and then consider intubation. For exam, know the initial management of all acute neurological emergencies.

Note that buccal midazolam is usually the first-line treatment in children, young people, and adults with prolonged or repeated seizures in the community. However, if buccal midazolam is unavailable, rectal diazepam is used. Hence, the probable reason why rectal diazepam was used in this stem.

Note that the management for ongoing generalized tonic-clonic seizures (convulsive status epilepticus) in the hospital is different from the community. Below we will only discuss management in the hospital.

This patient has lucid intervals. It is important to note that acute subdural haematoma also has lucid intervals. And thus when a patient presents with lucid intervals it can be both an extradural haematoma or an acute subdural haematoma. In general, for extradural haematoma the trauma is usually trivial and the patient goes back to doing what he was doing before falling unconscious again.

Whereas acute subdural haematoma is usually a sicker patient who barely awakes during lucid intervals.

But again in clinical practice, we do not lie on clinical features to differentiate epidural haematoma from acute subdural haematoma because both of them will get a CT scan Also, note the difference between acute subdural haematoma and chronic subdural haematoma.

In chronic subdural haematoma, it is usually elderly, on anticoag, or an alcoholic. Symptoms for chronic subdural haematoma are slow onset compared to epidural haematoma. As this question did not specify whether subdural haematoma is acute or chronic, it would be more correct to choose extradural (epidural) haematoma as the answer.

Extradural (epidural) haematoma Often associated with skull fracture and middle meningeal artery injury. Involves arterial blood.

Features :

• Features of raised intracranial pressure

• Patients may exhibit a lucid interval.  

Management :

Surgical procedure: burr hole over pterion (to ensure that further haemorrhage escapes instead of expanding the clot further) followed by craniotomy and evacuation of the haematoma.

Know the difference between generalized and partial seizures. Generalized seizures usually have patients with a loss of consciousness (i.e. “collapsed”) while partial seizures may or may not have a loss of consciousness depending if they are simple or complex. When both halves of the body (right and left) are involved, it is classified as generalized.

In this case, this patient’s seizure activity is due to alcohol abuse and therefore should follow guidelines on management related to seizures secondary to alcoholism.

Stiffness is the tonic phase followed by twitching which is the clonic phase. Myoclonic seizures are irregular jerks of the trunk or limbs that occur while the patient is conscious and last for less than a second.

Most of us have this experience when we are falling asleep and suddenly jerk as if we fell from a building. That is considered a normal phenomenon. Myoclonic seizures in an awake state are not normal and require specialist assessment.

Restless legs (previously called Willis-Ekbom syndrome) are common and can be very disturbing. The typical features include the urge to move (usually legs). It is often accompanied by the abnormal sensation of aching or tingling and in some, pain is a feature.

The symptoms tend to appear in the evening. They are also more commonly seen in pregnant women and often worsen as pregnancy progresses. The symptoms are relieved by moving. Useful advice includes avoiding alcohol, smoking, and caffeine.

Exercise and good sleep hygiene have been shown to benefit symptoms. It is common to measure serum ferritin levels in people suffering from restless legs syndrome as restless legs syndrome is associated with iron deficiency hence the reason the values were given in this stem. If ferritin is low, start iron supplementation, despite normal haemoglobin levels.

Since in this question, ferritin levels are within normal limits, she would not require iron supplementation. Only around 20% of sufferers would need a drug treatment.

Common medications used for the treatment of restless leg syndrome include:

• Dopamine agonist

• Gabapentin

• Pregabalin

There are two close answers here: syringobulbia and syringomyelia. Syringomyelia does not have any cranial nerve involvement but syringobulbia does and thus causing the facial palsy in the above question.

This man is suffering from Wernicke’s encephalopathy. Eye-movement abnormalities seen in Wernicke’s encephalopathy include gaze-evoked nystagmus, spontaneous upbeat nystagmus, and horizontal or vertical ophthalmoplegia. Wernicke's encephalopathy is a neuropsychiatric disorder caused by thiamine deficiency which is most commonly seen in alcoholics.

A classic triad of confusion, ophthalmoplegia, and ataxia may occur. Treatment is with urgent replacement of thiamine If not treated Korsakoff's syndrome may develop as well.

This is termed Wernicke-Korsakoff syndrome and is characterized by the addition of Antero- and retrograde amnesia and confabulation in addition to the above classic triad.

This is a typical case stem for migraine. The patient will present with or without aura (sensory or motor) followed by the headache itself. To differentiate from more serious headaches (i.e. intracranial haemorrhages), the clues usually would be “throbbing headache”, unilateral, and/or photophobia.

Note that topiramate is teratogenic/causes embryopathy and is a preventative measure for migraine. Also, pay attention to what question asks: If the woman is on COCP and has migraine with aura, advise cessation of COCP as it increases the risk of ischemic stroke.

Absence seizures would usually be a child (<10 years) with either the parent or teacher noticing that the patient is “daydreaming” often and when they resume their studies they are not able to perform well.

In this scenario, however, the diagnosis was already given but they want you to know what is the history that could give you the diagnosis. Staring blankly into space upturning of eyes for a few seconds and resuming activity almost immediately is indicative of absence seizures.

For exam, make sure you can differentiate the different types of headaches (tension, cluster, migraine). Cluster headache case stems usually have a young male with a headache localized to one side along with some autonomic features. Also, there should be a history of episodes over a couple of weeks. Another clue is people with cluster headaches usually move around while in migraines they tend to be lying down in a dark room.

Have a basic idea of the major causes of syncope. The most common amongst young women would be vasovagal syncope likely due to hypotension and stimulated by one of the following triggers: pain, fear, excitement, and standing for a prolonged period. Vasovagal syncope

Presentation:

• Young adults more common than women

• Stimulated by either emotional stress or orthostatic stress

• Sweating, pallor, nausea Diagnosis:

• Clinical history under recommended diagnostic criteria (ESC guidelines)

• ECG to rule out other pathology

Treatment:

• Reassurance and counseling of avoidance of triggers

• Educate how to recognize prodromal symptoms and perform manoeuvres to stop the syncope

• Tilt table training

This man is suffering from mechanical lower back pain. He should continue to mobilize and take regular analgesia (not just when required).

Of note, he may have been doing some heavy lifting while moving to his new accommodation which has exacerbated the condition. He does not have any red flags. 

Red flags for lower back pain are :

• Age over 50 years or under 18 years old

• Unexplained weight loss > 10 kg within 6 months

• Night pain or pain at rest

• Pain persists for more than 6 weeks

• Failure to improve with therapy

• History of cancer

• Urinary incontinence or retention

• Faecal incontinence

• Saddle anaesthesia

• Progressive neurological deficit

• Vertebral tenderness

Bed rest is not recommended. The current guidance is to continue mobilizing and consider an exercise program. Spinal manipulation therapies can be offered as part of a treatment package including exercise.

NICE guidelines on lower back pain do not recommend acupuncture for lower back pain. Paracetamol alone is not effective for the treatment of lower back pain.

While this is more of a dermatology / connective tissue topic than a neurology topic, neurofibromatosis type I (NF1) does present with neurological deficits.

Know how to differentiate between type I and type II as well as the basic management.

Neurofibromatosis type I :

• Cafe au lait spots, axillary freckling and neurofibromas

• Lisch nodules (hamartomas on the iris)

• Optic gliomas

• Scoliosis, bone dysplasias

• Epilepsy / seizures

• Cognitive impairment

Treatment:

• Conservative

• Routine monitoring and counselling

• Neurofibromas - if irritation, removal by laser or surgical

• Malignant peripheral nerve sheath tumors - radiation and surgical excision.

Benzhexol is an anticholinergic agent and it is the drug of choice to treat Parkinson’s disease-induced tremors. Anticholinergic agents have a limited role and should only be prescribed in young patients with severe tremors and dystonia. Orphenadrine is also another anticholinergic agent that is commonly used.

We should be suspecting subarachnoid haemorrhage in these cohorts of patients who present this way. It is important to note that a diagnosis of subarachnoid haemorrhage cannot be made on clinical grounds alone. The following are investigative steps to take if you suspect someone has had a subarachnoid haemorrhage.

1. CT scan: CT scanning without contrast is the first line of investigation.

2. Lumbar puncture: If the CT scan is negative but the history is suggestive, a lumbar puncture should be undertaken.

3. MRI: Can be more sensitive than CT, but only after several days have passed. After a subarachnoid haemorrhage is confirmed, its origin needs to be determined. The choice between cerebral angiography and CT angiography to identify aneurysms.

Know the difference between psychogenic tremors and essential tremors. The given stem has the clinical features of an essential tremor.

In psychogenic tremors, there is often an abrupt onset and spontaneous remission, and the characteristic of the tremor changes when the patient is distracted.

Parkinsonian tremors usually would have the classical sign of ‘pill-rolling’ in combination with rigidity and bradykinesia which is absent in this stem. Cerebellar disease could present with intentional tremors which is not seen in this stem.

Intentional tremors occur during voluntary active movements of the upper body. The tremor would worsen as goal-directed movement approaches its intended target. The stem would usually include overshooting or undershooting their target, a condition known as dysmetria.

It is a form of ataxia. For example, a patient would find difficulty due to his tremor when asked to touch his nose and then the examiner’s fingers. Essential tremor symptoms

• Distal symmetrical postural tremor of the upper limbs

• Initially transient. May progress to become persistent.

• Amplitude of tremor may depend on physiological or emotional state

• May be seen to improve following alcohol ingestion

• Not seen at rest

Management :

• Propranolol is the first line.

These questions do sometimes appear on the exam and therefore would be useful to know.

Usually, it would be the major dermatomes of the upper and lower limbs In the exam, questions may appear with a diabetic neuropathy stem or a trauma with sensory loss. Alternatively, it may also appear as just a straightforward statement asking for the dermatome of a specific part of the body with no case stem. 

She has features suggestive of multiple sclerosis. The investigation of choice is an MRI scan.

Multiple Sclerosis : A variety of symptoms involve motor and sensory mainly of the brainstem and cerebellum. It may be easier to divide them into groups to remember. 

1. Transverse myelitis:  Weakness, sensory symptoms - Urinary urgency and retention. Flexor spasms, Spastic quadriparesis or paraparesis.

2. Brainstem:  Ataxia, Diplopia, Dysarthria, Facial numbness, Ophthalmoplegia, Gaze palsy.

3. Cerebellum - Ataxia, Dysarthria, Nystagmus   

Optic neuritis is an acute, sometimes painful, reduction or loss of vision in one eye and is a relatively common presenting symptom of MS. Colour vision may be impaired.

• Also depression is common. The pattern is usually: symptoms evolve over days, plateau, then resolve over days / weeks.

Diagnosis:

• Mostly a clinical diagnosis

• MRI (definitive diagnostic test): Demyelination and/or lesions disseminated in time and place

• Oligoclonal bands in CSF

Treatment :

• Acute: IV or oral methylprednisolone

• Interferon-beta (first line).

She is suffering from Migraine. The hints provided here are that it is a unilateral headache associated with photophobia and nausea. The other options given here are much less likely.

Subarachnoid haemorrhage - Usually presents with the worst headache ever. What we call a “thunderclap headache”.

Chronic Subdural haematoma - Questions usually give a history of an elderly, on anticoagulation, or an alcoholic.

Intracranial neoplasm - Possible but less likely compared to migraine. The history of headaches would usually be longer. It is a space-occupying lesion, so one can expect a raised intracranial pressure (ICP). Headache, which is typically worse in the mornings, nausea and vomiting, seizures, and papilloedema.

Cluster headache - Usually presents with intense pain around one eye accompanied by redness, lacrimation, lid swelling, and also nasal stuffiness.

Amyotrophic lateral sclerosis (ALS) is the most common form of motor neuron disease in addition to multiple sclerosis (MS). Know the distinction between both for exam as they will commonly appear as either a question or an option.

MS usually presents in episodes intermittently and affects different anatomic locations.

Myasthenia gravis would present with weakness and fatigue along with a positive autoimmune panel.

Guillain-Barre syndrome would usually give a clue to a prior infection.

Polymyositis would affect the lower limbs first with fatigue.

This man is suffering from Wernicke’s encephalopathy. Vitamin B1, also called thiamine, is needed to treat Wernicke’s encephalopathy.

Wernicke's encephalopathy is a neuropsychiatric disorder caused by thiamine deficiency which is most commonly seen in alcoholics. A classic triad of confusion, ophthalmoplegia, and ataxia may occur.

Treatment is with urgent replacement of thiamine. If not treated Korsakoff's syndrome may develop as well. This is termed Wernicke-Korsakoff syndrome and is characterized by the addition of Antero- and retrograde amnesia and confabulation in addition to the above classic triad.

It is often difficult to tell the difference between an epileptic seizure and a non-epileptic attack disorder (NEAD) in clinical practice. Non-epileptic attacks may look like epileptic seizures but they are not caused by an electrical disturbance in the brain. From the history given, this sounds more like a non-epileptic attack disorder.

The key phrases here that point towards the diagnosis of NEAD are the pelvic movements, episodes where eyes are closed, no drooling of saliva which means the mouth is closed during the attacks, and lastly no incontinence.

Video electroencephalogram (EEG) is an investigation especially useful to diagnose NEAD or if the diagnosis is uncertain. This involves hooking up EEG equipment and also having a continuous video of the patient to capture the activity of the brain and physical movements and sounds during seizures.

Know that lesions to the posterior circulation would affect functions of the brainstem, cerebellum, and occipital lobe. This would be consistent with the vertigo, right hemianopia, nystagmus, and ataxia seen in this patient.

Stroke in the posterior circulation presentation :

• Dizziness / vertigo, right hemianopia, nystagmus, ataxia, dysdiadochokinesia

• Usually sudden onset with possible further progression that takes place over hours

• May have underlying ischaemic heart disease, hypertension, carotid bruits, atrial fibrillation

Diagnosis :

• Non-contrast CT brain - initial investigation

• MRI brain - more sensitivity

Treatment:

• Ischaemic stroke - Thrombolysis (alteplase) & aspirin

• Maintain oxygen saturation, glucose control, blood pressure control

• Maintain nutrition and hydration

• Early mobilization

The patient had an ischaemic stroke and the standard treatment would be Aspirin for 14 days, and then Clopidogrel long-term.

The question is asking for the long-term treatment and therefore this would be Clopidogrel.

Alteplase - for patients who are appropriate for thrombolysis therapy who present within 4.5 hours of the onset of stroke symptoms, and intracranial haemorrhage has been excluded by imaging techniques.

Aspirin is started as a standard therapy for confirmed ischaemic stroke and is continued until 2 weeks, at which time definitive long-term antithrombotic treatment should be initiated.

Aspirin is only recommended long-term for patients intolerant to clopidogrel.

An elderly man with a history of falls and on anticoagulation with progressive confusion over the last couple of days points towards the diagnosis of chronic subdural haemorrhage.

Chronic subdural haematoma occurs in very old or severe alcoholics. A shrunken brain is rattled around the head by minor trauma, tearing venous sinuses. Over several days or weeks, mental function deteriorates as haematoma forms.

CT scan is diagnostic, and surgical evacuation provides a dramatic cure.

Remember, Chronic subdural haematoma usually presents as an elderly, on anticoag or an alcoholic who may have a history of falls. Slow onset of symptoms compared to epidural haematoma.

Clopidogrel is now recommended by NICE ahead of the combination use of aspirin plus dipyridamole in people who have had an ischaemic stroke.

The history and examination are convincing for a stroke. Before any treatment can happen we need to exclude a haemorrhagic stroke with the help of imaging. This was done in this question which showed an ischaemic stroke. The next step is to start thrombolytics.

The other options are less likely to be the appropriate next course of action.

Anticoagulation should not be started until brain imaging has excluded haemorrhage, and usually not until 14 days have passed from the onset of an ischaemic stroke. 

It is recommended to prefer alteplase over streptokinase.

Question clues towards Alzheimer’s disease: memory changes first before personality. As night progresses they are unable to sleep. They would also have difficulty in daily tasks and be confused.

Vascular dementia would have a “stepwise progression” and they would have had some sort of previous cardiovascular event in the stem.

Lewy body dementia would require a clue into having Parkinsonian symptoms and hallucinations. 

Normal pressure hydrocephalus presents with gait disturbance, urinary incontinence, and confusion.

This gentleman likely had some cognitive impairment when his wife was alive. She likely helped him with his day-to-day activities. Therefore the problems likely did not surface.

Now that he has no support, his impairments have likely worsened and he has not been able to manage alone. Orientation to time is usually lost first typically in dementia, followed by an orientation to place, and finally to person when it is most severe.

This is a classic example of cauda equina syndrome.

It is a serious neurologic condition in which damage to the cauda equina causes loss of function of the nerve roots of the spinal canal below the termination (conus medullaris) of the spinal cord. Any lesion which compresses or disturbs the function of the cauda equina may disable the nerves although the most common is a central disc prolapse. Investigation of choice is an MRI.

Broca’s Aphasia - Broca’s area is a region in the inferior frontal lobe of the dominant hemisphere responsible for speech production. Lesions in this area produce a non-fluent or expressive aphasia. Patients will typically exhibit slow and halting speech. However, their comprehension is not affected.

Unlike Wernicke’s aphasia, patients with Broca’s aphasia are aware of their language difficulties.

For example, a person with Broca's aphasia may say slowly something like, "Bathe. Dog" meaning to say, "I bathe the dog today". You can see that the content of the information is correct, but the grammar and fluidity of the sentence are incorrect. They know what to say, they just cannot get it out.

The symptoms here are classic for Guillain-Barre syndrome. The weakness progresses quickly and always begins in the lower limbs. There is usually a history of a respiratory or gastrointestinal infection preceding these symptoms.

Viral infection may cause facial nerve palsy.

Facial Nerve Palsy Presentation :

Weakness of the muscles of facial expression and eye closure. The face sags and is drawn across to the opposite side while smiling. Voluntary eye closure may not be possible and can produce damage to the conjunctiva and cornea. In severe cases, dysarthria and difficulty with eating may occur.

The clinical examination above is classic of Parkinson’s Disease :

Bradykinesia, rigidity, resting tremor, and postural instability. While the beginning of the case stem may point to possible malignancy, the examination results point to option D.

Parkinson’s disease: > 65 years.

Classic presentation on examination: Bradykinesia, rigidity, resting tremor, and postural instability - +/- Extra-neurological features

Diagnosis: Clinical diagnosis

Treatment: Referral to a neurologist before starting pharmacological management - Levodopa (gold standard).

Although folate deficiency is more common in people who regularly misuse alcohol this is not the answer in this stem. A haemoglobin of 128 g/L is barely enough to cause any symptoms of anaemia. This man is suffering from Wernicke’s encephalopathy.

Eye-movement abnormalities seen in Wernicke’s encephalopathy include gaze-evoked nystagmus, spontaneous upbeat nystagmus, and horizontal or vertical ophthalmoplegia.

Wernicke's encephalopathy is a neuropsychiatric disorder caused by thiamine deficiency which is most commonly seen in alcoholics.

A classic triad of confusion, ophthalmoplegia, and ataxia may occur.

Treatment is with urgent replacement of thiamine (vitamin B1). If not treated Korsakoff's syndrome may develop as well.

This is termed Wernicke-Korsakoff syndrome and is characterized by the addition of Antero- and retrograde amnesia and confabulation in addition to the above classic triad.

You may have difficulty in choosing either ABCD2 or CHA2DS2-VASc as the answer but remember that in this case stem, they are asking for a risk of stroke in the next few days. ABCD2 score would be more appropriate.

This case stem is rare to appear in exam, but knowing the presentation of how the question will appear differentiates it from options B, C, and E which occur more frequently in the exam. Cavernous sinus thrombosis can appear as an option to those questions.

Cavernous sinus thrombosis :

- Headache

- can be severe intensity

- Chemosis, oedematous eyelids, proptosis, painful ophthalmoplegia

- Fever

- Usually preceded by sinusitis

Diagnosis: CT/MRI venography

Treatment: 

- Referral to neurosurgery

- Antibiotics

- Anticoagulation : heparin

- +/- Corticosteroids and surgical drainage.

Ataxia and slurred speech are features of the cerebellar lesion. Posterior fossa lesions are best imaged using magnetic resonance imaging.

A CT head would be the correct answer if asked about the best initial investigation to ensure there is no haemorrhage. Brain PET has limited clinical use.

Please see Q-68

Know the types of meningitis since it overlaps with quite a few topics on the exam. Pay particular attention to the lumbar puncture results to differentiate each. Kernig’s sign does not need to be present for it to be a diagnosis of meningitis.

If you forget the lab results, look at the history: viral meningitis has less severe symptoms than bacterial meningitis which can progress rapidly and produce signs of septic shock.

TB meningitis is more gradual with fever, weight loss, and headache with progression to focal deficit & altered consciousness.

Presentation :

• Classic: headache, fever, photophobia, neck stiffness

• Cranial palsies: CN III, IV, VI, VII

Diagnosis:

Lumbar puncture :

• Normal or high CSF pressure

• Increased lymphocytes

• Elevated protein

• Glucose normal

Treatment :

• Regardless of whether meningitis is bacterial, viral, or TB as a cause - Give antibiotics before performing CT brain and lumbar puncture.

• If no rash: IV cefotaxime or IV ceftriaxone + IV vancomycin + IV ampicillin

• If raised intracranial pressure: Give mannitol.

The woman has presented with features of myasthenia gravis. Almost all patients with myasthenia gravis will have ocular manifestations at some point during their disease. The most important feature is that muscles fatigue more readily after exercise.

Antibodies to acetylcholine receptors are seen in 85-90% of cases hence the answer is serum skeletal muscle nicotinic acetylcholine receptor antibody. Tensilon test is not routinely done any longer.

MYASTHENIA GRAVIS :

• Female (20-30 years); males (50-60 years)

• Painless muscle weakness that increases with exercise. This feature of fatigue often gives the diagnosis in the exam.

- It is even seen in the voice. For example, getting the patient to count up to 50. As the patient nears 50 their voice becomes less audible as they are fatiguing.

• Ocular muscles involved, drooping eyelids

• Dysphagia, dysarthria, dysphonia

• Normal reflexes

• Associated with hyperthyroidism

A useful mnemonic to remember for clinical features of myasthenia gravis is “D”.

• Diplopia

• Drooping eyelids

• Dysphagia

• Dysarthria

• Dysphonia

The best answer in the choices that fits in with this patient’s signs and symptoms is diencephalon.

The diencephalon consists of the thalamus, the hypothalamus including the posterior pituitary, the epithalamus, and the subthalamus. A lesion near or at the posterior pituitary would give rise to this patient’s symptoms of diabetes insipidus. The signs and symptoms of sodium imbalance are primarily neurological.

Delirium describes an acute confusional state and is very common in the elderly. It involves altered perception and thought and is of acute onset. The other answers are less likely.

Dementia is incorrect as a person with dementia would have a chronic set of symptoms and a slow decline in mental functioning over a long period. Mania describes an elevated mood.

This can present as an abnormally high level of arousal or energy.

This patient does not present with any of those symptoms. Schizophrenia is less likely as a majority of schizophrenics develop their symptoms around adolescence or early adulthood. This patient is 61 years old. Bipolar disorder presents with alternating episodes of hypomania and depression.

The diagnosis here is migraine.

This question is a helpful reminder that the mean age of onset of migraines is 19 years old. It is usually a young to middle-aged person who presents with migraines. It is highly unlikely that they would ask you to choose between an oral triptan, NSAID, aspirin, or paracetamol for the acute treatment of migraines. 

Stroke questions come in various forms ranging from the anatomical locations of the lesion to investigations and management. Pay special attention to the anatomy of the lesions especially the vascular distribution and the functions of the different parts of the brain.

Key clues to cerebellum lesions: dysdiadochokinesia, ataxia, nystagmus, intention tremor, slurred speech, hypotonia, past pointing.

In regards to his diagnosis of chronic alcoholic abuse, add thiamine to the mix to prevent Wernicke’s encephalopathy even though he is not showing any signs of Wernicke’s encephalopathy.

Parenteral thiamine should be prescribed for treatment of suspected or confirmed Wernicke’s encephalopathy, and for prophylaxis in alcohol-dependent patients attending the hospital for acute treatment.

Subarachnoid haemorrhage (SAH) is spontaneous in the context of a ruptured cerebral aneurysm but may be seen in association with other injuries when a patient has sustained a traumatic brain injury. Aneurysm formation is the most common aetiology.

There is also an association with polycystic kidney disease, Ehlers-Danlos syndrome, and another connective tissue disease. Head trauma is rare as a cause of SAH.

Daytime sleepiness and obesity point to the diagnosis of obstructive sleep apnoea syndrome. Choking episodes during sleep are also a suggestive feature of obstructive sleep apnoea syndrome.

Obstructive sleep apnoea syndrome :

- Middle-aged to elderly male

- Increased daytime sleepiness, fatigue, sleep disruption

- Snoring loudly at night

- Associated with hypertension, diabetes, obesity

Diagnosis:

- Pulse oximetry, overnight study of breathing pattern (initial investigation)

- Polysomnography (gold standard)

Treatment:

- Conservative: weight loss and reduced alcohol consumption

- Continuous positive airway pressure (CPAP).

This is a 2 step question. First figure out the diagnosis and through that find out the answer to the question. Some questions will require you to do this.

In this case, the diagnosis is subarachnoid hemorrhage (SAH) and it is also implied in the case stem that the patient has possible polycystic kidney disease (hypertension and recurrent kidney stones).

One of the major complications of SAH is hyponatremia.

Hyponatremia commonly occurs in patients with aneurysmal subarachnoid haemorrhage. The mechanism that has been proposed as a cause is the syndrome of inappropriate anti-diuretic hormone.

Cauda Equina Syndrome would fit the best. Although lumbosacral disc herniation and degenerative process of the spine are causes of cauda equina syndrome it would be more accurate to put Cauda Equina Syndrome as the answer due to the perineal sensory loss.

Cauda Equina Syndrome is a serious neurologic condition in which damage to the cauda equina causes loss of function of the nerve roots of the spinal canal below the termination (conus medullaris) of the spinal cord.

This male has most likely suffered a midbrain infarct. This specific presentation is called Weber’s syndrome which is a result of a midbrain infarct. It is a form of stroke characterized by the presence of:

1. Contralateral hemiparesis 

2. An ipsilateral oculomotor nerve palsy

CEREBRAL ABSCESS : Cerebral abscesses may show many of the same features of brain tumors as they both are space-occupying lesions. The major difference is that cerebral abscess has features of a fever and an obvious source of infection nearby such as otitis media or mastoiditis.

Presentation :

• Headache is the most common symptom and it is often on the same side as the abscess

• Fever

• Changes in mental state (drowsiness, confusion)

• Focal neurological deficits

• Grand mal seizures

• Nausea and vomiting

• Neck stiffness

• Papilloedema, this is due to cerebral oedema

Investigations :

• CT scanning is the investigation of choice. Cerebral abscesses appear as a radiolucent space-occupying lesion. 

The symptoms here are classic for Myasthenia gravis. It is important to remember that there is a predisposition for autoimmune diseases to run in families hence the history of thyroid disease in the family in the stem.

She is suffering from Wernicke’s encephalopathy. One of the eye abnormalities seen in Wernicke’s encephalopathy is horizontal or vertical ophthalmoplegia.

Wernicke’s encephalopathy is a neuropsychiatric disorder caused by thiamine deficiency which is most commonly seen in alcoholics. A classic triad of confusion, ophthalmoplegia, and ataxia may occur.

It is also seen in pregnancy where there is severe persistent hyperemesis gravidarum.

For this reason, it is important to replace thiamine in patients with hyperemesis gravidarum before they develop Wernicke’s encephalopathy.

Treatment in this case is with urgent replacement of thiamine (vitamin B1).

Wernicke’s Encephalopathy triad of:

1. Confusion

2. Ataxia

3. Ophthalmoplegia

Among the given answers, subdural haematoma fits the best. The multiple bruises on her head suggest head injury which can lead to subdural haematoma. Headache, confusion, and impaired vision for 4 days could occur in chronic subdural haematoma. She is an elderly lady thus a trivial fall may have gone unnoticed.

This is a classic presentation of a transient ischaemic attack. Under the ABCD2 score, this patient has a score of 4. The most appropriate step according to guidelines is for carotid doppler scanning to check for carotid artery stenosis to assess the need for carotid endarterectomy.

MRI would eventually be done but it would not be the “next step” as the patient no longer has residual neurological findings and his symptoms have completely resolved; therefore MRI at this moment would not be clinically beneficial.

Plaques can develop anywhere in the central nervous system in patients with multiple sclerosis, but when they develop in the brain stem, several functions can be impaired and may result in an evolving vestibular syndrome. Vertigo is often seen with diplopia due to the demyelination of cranial nerves III, IV, and VI as they leave the brainstem in patients suffering from multiple sclerosis.

PSEUDODEMENTIA: Severe depression that presents with poor memory and concentration and impaired functional capacity. Seen in elderly patients whereby they exhibit symptoms consistent with dementia but the cause is depression.

Features- Onset relatively abrupt, Poor memory, Often with a history of depression. If asked questions, would reply “I don’t know, I can’t be bothered, it’s too difficult” (This is because they are often upset but their attention and concentration are often intact)

Response to antidepressant medication.

The patient has Guillain-Barre syndrome. Clues that allude to this diagnosis include his symptoms of ascending limb weakness accompanied by hyporeflexia as well as his history of a recent illness.

Guilllain-Barre syndrome is a disorder whereby antibodies created by the body to attack infectious organisms (usually of the gastrointestinal or respiratory tract) also attack antigens in the peripheral nerve tissue.

The major complaint in most patients is weakness or neuropathic pain.

This is a diagnosis of extradural haemorrhage. While this is a frequent neurology topic, the presentation here has left out the usual clue: “lucid interval”. However, the stem provides another clue to the diagnosis: the CT findings which are indicative of an extradural haemorrhage.

In this case, this patient not only has raised intracranial pressure but he is also showing signs of neurological deficit (unequal pupils) in addition to the low GCS.

Therefore, he is not suitable for conservative management and requires immediate craniotomy for evacuation of the haematoma.

This man is suffering from Horner’s syndrome secondary to the Pancoast tumour. Horner’s syndrome describes an ipsilateral deficiency of sympathetic activity. There are many causes of Horner’s syndrome, but the one that is tested the most in exams is a Pancoast tumour.

A significant differential when a patient presents to the Emergency Department with acute vertigo accompanied by neurological symptoms is brain stem and cerebellar strokes. Brain stem strokes have complex symptoms and are difficult to diagnose.

The hallmarks of brain stem strokes are symptoms of vertigo, ataxia, and diplopia.

Other symptoms include slurred speech, numbness of the face, and decreased level of consciousness. The distractor is cerebellar strokes.

Cerebellar infarcts also present with acute vertigo. If the inferior cerebellum is affected, in addition to vertigo, patients could have nystagmus and postural instability however a pure cerebellar stroke would not usually have limb paraesthesia or diplopia.

NICE has suggested short-term olanzapine or haloperidol (usually for a week or less) be used in patients with delirium who are in distress once non-pharmacological de-escalation techniques have failed. 

The name of the condition is “Lateral medullary syndrome”.

Lateral medullary syndrome is a neurological disorder causing a range of symptoms due to ischemia in the lateral part of the medulla oblongata in the brainstem. The ischemia is a result of a blockage most commonly in the vertebral artery or the posterior inferior cerebellar artery.

This syndrome is characterized by sensory deficits that affect the trunk and extremities contralaterally and sensory deficits of the face and cranial nerves ipsilaterally.

Specifically, a loss of pain and temperature sensation if the lateral spinothalamic tract is involved. The cross-body finding is the chief symptom from which a diagnosis can be made (in short ipsilateral Horner syndrome and contralateral loss of pain and temperature sense).

The patient may have ataxia (cerebellar involvement), dysphagia (nucleus ambigus that involves vagus and glossopharyngeal nerve dysfunction), and slurred speech. Vestibular nuclei dysfunction may cause vomiting, vertigo, and nystagmus.

Herpes zoster ophthalmicus occurs when the varicella-zoster virus is reactivated in the ophthalmic division of the trigeminal nerve. Herpes zoster is a common infection caused by the human herpesvirus 3, the same virus that causes varicella (i.e., chickenpox).

3rd nerve damage can cause fixed dilated pupil.

Diagnosis is meningococcal meningitis. All contacts need rifampicin prophylaxis.

Here arm hanging down compressing the radial nerve at the spiral groove is the cause of the given scenario.

Diagnosis is TIA. The probable cause is carotid narrowing from atherosclerosis.

Compression of ulnar nerve at the elbow, known as cubital tunnel syndrome, causes numbness in the 5th finger, along the half (lengthwise) of the 4th (ring) finger closest to the 5th finger, and the back half of the hand over the 5th finger.

Diplopia on seeing to the right indicates right lateral rectus palsy which is supplied by the right abducent nerve.

Diencephalon is the caudal (posterior) part of the forebrain, containing the epithalamus, thalamus, hypothalamus, ventral thalamus, and the third ventricle. The hypothalamus produces ADH and hens lesion of the diencephalon (hypothalamus) may produce cranial diabetes insipidus.

The Obturator nerve is responsible for the sensory innervation of the skin of the medial aspect of the thigh.

In the elderly head injury usually leads to subdural hematoma even if a head injury is minor or trivial and extradural hematoma in the elderly is extremely uncommon even in more severe head injury. Warfarin is also a risk factor for subdural hematoma from a minor trauma.

Management:

1st line: Evacuation by barr hole craniostomy.

2nd line: Craniotomy if the clot is organized. Up to 3 weeks burrhole may be possible but longer than this clot is mostly organized and Flap craniotomy is usually required.

This is a case of Herpes zoster oticus and if the facial nerve is also involved then it is called Ramsay Hunt syndrome.

In Brown-Sequard syndrome paralysis and loss of proprioception on the same (or ipsilateral) side as the injury or lesion, and loss of pain and temperature sensation on the opposite (or contralateral) side as the lesion.

Trigeminal neuralgia.

Electric pains in her face that start with the jaw and move upwards this description indicates neurological pain of the trigeminal nerve. Patients with classical trigeminal neuralgia have a normal neurologic examination.

Sensory abnormalities in the trigeminal area, loss of corneal reflex, or evidence of any weakness in the facial muscles should prompt the physician to consider symptomatic trigeminal neuralgia or another cause of the patient's symptoms.

Carotid artery divides into internal and external carotid of which the internal continues as middle cerebral ultimately.

But just before it becomes middle cerebral internal carotid gives rise to ophthalmic branch. So middle cerebral occlusion may give partial visual loss but not complete mono-ocular blindness.

For complete mono-ocular blindness occlusion should be proximal to the ophthalmic artery i.e. either in the internal carotid or more proximally to the carotid artery.

Empiric therapy should begin as soon as bacterial meningitis is thought likely. In patients with meningitis caused by penicillin- or cephalosporin-resistant strains, vancomycin may be used in combination with a third-generation cephalosporin but should not be used alone.

Though meropenem can be an alternative to cefotaxime and ceftriaxone due to its seizure activity it is not preferred as 1st line. Fluoroquinolones are also not used as 1st line.

This is migraine with aura. Criteria of migraine without aura:

5 headaches + nausea/vomiting or photophobia + any 2 of:

i) unilateral

ii) pulsating

iii) worsen by routine activity

Lateral peroneal nerve is another name for superficial peroneal nerve.

Radial nerve

Diagnosis is multiple sclerosis for which MRI is the investigation of choice.

The diagnosis is Weber syndrome which is characterized by presence of ipsilateral oculomotor nerve palsy and contralateral hemiparesis or hemiplegia.

Proctalgia fugax is a functional anorectal disorder characterized by severe, intermittent episodes of rectal pain that are self-limited. The diagnosis of proctalgia fugax requires the exclusion of other causes of rectal or anal pain. Normal rectal examination and flexible sigmoidoscopy exclude other options.

IV benzylpenicillin.

Likely diagnosis is meningococcal meningitis.

Aspirin may cause Reye’s syndrome in young girls. This is a case of pediatric migraine (pediatric migraine up to 17yrs).

In pediatric migraine paracetamol is 1st line treatment (also ibuprofen). In pediatric migraine, the GP cannot prescribe triptan. 

Age 33 considerable head trauma, and lucid interval are the points in favour of extradural hemorrhage.

Normal pressure hydrocephalus (NPH) is an abnormal buildup of cerebrospinal fluid (CSF) in the brain's ventricles or cavities.

It occurs if the normal flow of CSF throughout the brain and spinal cord is blocked in some way. This causes the ventricles to enlarge, putting pressure on the brain.

Age (usually occurs in the 60s or 70s), loss of bladder control (enuresis), waddling gait and behavior change, and dementia are all features of normal pressure hydrocephalus.

Myoclonus can be described as brief jerks of the body; it can involve any part of the body, but it is mostly seen in limbs or facial muscles. The jerks are usually involuntary and can lead to falls. EEG is used to read brain wave activity.

When myoclonic jerks are occasionally associated with abnormal brain wave activity, they can be categorized as myoclonic seizures. If the abnormal brain wave activity is persistent and results from ongoing seizures, then a diagnosis of myoclonic epilepsy may be considered.

Alzheimer's disease, is a chronic neurodegenerative disease that usually starts slowly and gradually worsens over time. It is the cause of 60–70% of cases of dementia. The most common early symptom is difficulty in remembering recent events.

As the disease advances, symptoms can include problems with language, disorientation (including easily getting lost), mood swings, loss of motivation, not managing self-care, and behavioural issues. As a person's condition declines, they often withdraw from family and society. Gradually, bodily functions are lost, ultimately leading to death.

Balance difficulties, vomiting and nausea suggests cerebellar lesion. In posterior fossa lesion MRI is preferred.