Neurology

Question 1

Define Bell’s palsy?

Answer 1

Bell’s palsy is an idiopathic syndrome that causes damage to the facial nerve leading to a lower motor neuron facial palsy.

Question 2

What causes Bell’s palsy?

Answer 2

The aetiology of Bell’s palsy remains unknown. Viral infections, particularly reactivation of herpes simplex virus type 1 (HSV-1), have been implicated as a possible cause.
Other viral pathogens, such as Epstein-Barr virus (EBV) and varicella-zoster virus (VZV), have also been suggested as potential triggers.
However, the exact mechanisms by which these viruses lead to facial nerve dysfunction are not fully understood.

Question 3

Clinical features of Bell’s palsy?

Answer 3

Acute (but not sudden) onset of unilateral lower motor neuron facial weakness (classically affecting the forehead as this area has bilateral nervous supply from both sides of the brain)
Mild to moderate postauricular otalgia, which may precede the paralysis.
Hyperacusis
Nervus intermedius symptoms, such as altered taste and dry eyes/mouth.
Patients may subjectively describe “numbness” or “heaviness” without objective facial somatosensory disturbances.

Question 4

What are the investigations for Bell’s palsy?

Answer 4

The diagnosis of Bell’s palsy is primarily clinical, based on the characteristic signs and symptoms.
However, in some cases, additional investigations may be considered to exclude other potential causes or to assess the severity of nerve dysfunction. These investigations may include:
Otoscopy to assess if any vesicular lesions are present in the external auditory meatus
Full blood count (FBC)
Erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP)
Viral serology (e.g. HSV-1, EBV, VZV)
Lyme serology (if suggestive symptoms or exposure)
Electromyography (EMG)
Imaging studies (e.g. CT or MRI Head)

Question 5

What is the short term management for Bell’s palsy?

Answer 5

The management of Bell’s palsy includes:
Prompt administration of oral steroids: 50mg of oral prednisolone or prednisone once daily for 10 days, followed by a taper.
Supportive treatments:
Artificial tears and ocular lubricants to manage dry eyes.
Eye patch/tape to prevent corneal exposure and injury.

Question 6

What may be considered in treatment for Bell’s palsy?

Answer 6

Although the pathophysiology of Bell’s palsy has not been definitively linked to active herpes virus infection, empirical treatment with aciclovir may be considered, particularly in cases where the clinical distinction between Bell’s palsy and Ramsay-Hunt syndrome is difficult.

Question 7

What is the long term management of Bell’s palsy?

Answer 7

Pain management with analgesics or anticonvulsants may be necessary in cases of severe otalgia or neuropathic pain.
Physical therapy, including facial exercises and massage, may be recommended to maintain muscle tone and prevent contractures during the recovery phase.
Psychological support and counseling may be beneficial for patients experiencing emotional distress or body image concerns due to facial weakness.

Question 8

What percentage of patients with Bell’s palsy have a complete recovery?

Answer 8

Complete recovery: Approximately 70-80% of patients with Bell’s palsy achieve complete recovery of facial function without residual deficits.
Most improvement occurs within the first 3 months, with the greatest recovery seen within the first 6 weeks. However, some patients may continue to show gradual improvement even beyond this timeframe.

Question 9

What two factors can decrease the likelihood of recovery for Bell’s palsy?

Answer 9

Age and severity: Older age and more severe initial facial weakness are associated with a higher risk of incomplete recovery.

Question 10

Define a brain abscess?

Answer 10

A brain abscess is a collection of pus within the brain parenchyma. It is a very serious condition which can lead to significant morbidity and mortality, especially if left untreated.

Question 11

What causes brain abscesses?

Answer 11

Brain abscesses are usually result from direct extension of an infection. This may be sinusitis, dental or cranial procedures or injury. Sometimes, the bacteria may spread through the bloodstream, for example a septic embolus from endocarditis. The most commonly isolated bacteria is Streptococcus, though other pathogens such as oral bacteria, Staphylococci, gram-negatives, TB, fungi and parasites can also cause brain abscesses, particularly in susceptible people

Question 12

What are the signs and symptoms of brain abscesses?

Answer 12

Signs of infection: fever, nausea & vomiting, meningism
Raised intracranial pressure: headache, third nerve palsy, papilloedema, seizures
Focal neurology depending on location of abscess

Question 13

What are the investigations for brain abscesses?

Answer 13

Bloods - including FBC, CRP, clotting, VBG, cultures
Cross-sectional imaging:
MRI is most sensitive for brain abscess, and will show an enhancing lesion.
If stroke is suspected, an urgent CT head will be required as per local pathways.
Neurosurgical aspiration/excision & cultures

Question 14

What is the management for brain abscesses?

Answer 14

It is important to remember that when a patient is acutely unwell, start with an A-E assessment and consider initiating the sepsis six if the patient is showing signs. Call for help early - the exact team will depend on local protocols & clinical context. If the the patient is displaying acute neurological symptoms, initiate the stroke pathway as per local guidelines.
Exact management will be guided by seniors in neurology/neurosurgery & microbiology, but may include:
Neurosurgical intervention: aspiration or excision
Medical: antibiotics - initially a 3rd-generation cephalosporin + metronidazole. This may differ based on culture results and immune status of the patient. Typically, antibiotics continue for several weeks.
Symptom management - for example anticonvulsants for seizures, dexamethasone for severely raised intracranial pressure

Question 15

What are the complications of brain abscesses?

Answer 15

Sepsis
Raised intracranial pressure & herniation
Permanent neurological deficits
Death

Question 16

What are risk factors for brain abscesses?

Answer 16

Risk factors for brain abscess include:
Sinusitis
Dental, head & neck procedures
Cardiac defects & endocarditis
Poor immune response including HIV, immunocompromise

Question 17

What would a CT reveal in a brain abscess?

Answer 17

ring enhancing lesion

Question 18

Define brain metastases?

Answer 18

Brain metastases are secondary brain tumours that occur following spread from a tumour away from the central nervous system.

Question 19

What causes brain metastases?

Answer 19

Malignancies usually spread to the brain haematogeneously. Cells seed into the brain’s small blood vessels and are often protected from anti-cancer therapies by the blood-brain barrier.

Question 20

What are the most common primary malignancies that metastasise to the brain?

Answer 20

Lung cancer
Breast cancer
Colorectal cancer
Melanomas
Renal cell carcinoma

Question 21

Signs and symptoms of brain metastases:

Answer 21

Brain metastases may occur in people with a known cancer, or may be their first presentation of malignancy. Symptoms can be due to local interruption of the nerve pathways or due to raised intracranial pressure. Key clinical features include:
Headaches
Seizures
Altered mental status, including confusion
Cerebellar signs, including ataxia
Nausea and vomiting
Visual disturbance
Focal neurology
Patients may also have signs of the primary malignancy, for example breathlessness and haemoptysis for lung cancer.

Question 22

What are the investigations for brain metastases?

Answer 22

In a patient presenting with acute neurological features, it is important to rule out acute intracranial events, for example stroke. Therefore, a head CT is often employed first-line.
According to NICE, MRI is the best and initial imaging modality for diagnosing brain metastases. Further investigations may be necessary to identify the primary malignancy, for example chest imaging and biopsy.

Question 23

What is the management for brain metastases?

Answer 23

Management of brain metastases requires a multidisciplinary approach. There are many factors to consider including the nature of the metastases, the patient’s age, performance status and primary tumour.
Systemic anti-cancer therapy is advised for people who have brain metastases likely to respond effectively, for example, germ cell tumours or small-cell lung cancer
Symptomatic management involves corticosteroids (usually dexamethasone) to reduce oedema, anti-seizure medications and antiemetics. Specific treatment for raised intracranial pressure may be necessary as well. Therapeutic options include radiotherapy (targeted, and sometimes whole-brain), chemotherapy and surgical resection.
Following treatment, patients will often have serial imaging follow up to assess any ongoing symptoms/potential progression

Question 24

What is chronic fatigue syndrome also referred to as?

Answer 24

Myalgic encephalomyelitis

Question 25

Define chronic fatigue syndrome?

Answer 25

CFS is a chronic illness characterised by persistent or recurrent fatigue that is not relieved by rest and is associated with significant functional impairment. It is a chronic multisystemic condition, with common features being post-exertional malaise, cognitive difficulties, unrefreshing sleep, autonomic dysfunction and pain. Symptoms should not be explained by any other cause.

The diagnosis should be suspected if fatigue has been present for at least 6 weeks with associated functional impairment, and symptoms are not secondary to another illness

Question 26

Risk factors for chronic fatigue syndrome?

Answer 26

Risk factors include:
Family history
Epstein-Barr infection in adolescence
Covid-19 infection

Question 27

How is chronic fatigue syndrome classified?

Answer 27

CFS can be classified by symptom severity
This is a way of approximating the impact of symptoms on daily life
However these categories are not clear-cut as symptoms may fluctuate in intensity and some symptoms may be more severe than others
There are additional management considerations for patients with severe or very severe CFS (see Management section for details)

Question 28

What are the symptoms of mild chronic fatigue syndrome?

Answer 28

Patients are self-caring
May need some support with domestic tasks
Mobility may be affected
Often still in education or work, may need reduced hours and days off
Leisure and social activities usually curtailed

Question 29

What are the symptoms of moderate chronic fatigue syndrome?

Answer 29

Restricted in all activities of daily living (ADLs)
Reduced mobility
Usually have stopped education or work
Sleep is usually poor quality
Rest periods usually needed

Question 30

What are the symptoms of severe chronic fatigue syndrome?

Answer 30

No or minimal self-care (e.g. washing fash)
Mobility affected e.g. may require a wheelchair, often unable to leave the house
May spend most of the time in bed
Severe cognitive difficulties
Often hypersensitive to light and sound

Question 31

What are the symptoms of very severe chronic fatigue?

Answer 31

Patients are bedbound
Dependent for all self-care including personal hygiene and eating
May require nutritional support (e.g. PEG feeding)

Question 32

What are the signs and symptoms of chronic fatigue syndrome?

Answer 32

Patients should have all of the following symptoms, which have persistent over at least 3 months

Disabling fatigue
Worse on activity
Not significantly relieved by rest
Not due to excessive physical or mental exertion
Post-exertional malaise
Disproportionate to the activity
Prolonged e.g. may last days/weeks/longer
May be delayed in onset by hours/days
Sleeping difficulties
Sleep may be disturbed and/or unrefreshing
Patients may feel exhausted and stiff on waking
May have altered sleep pattern or hypersomnia
Cognitive difficulties
May describe “brain fog”
Poor concentration
Difficulties speaking or word-finding
Slow responses
Short-term memory impairment

Question 33

What are the other symptoms of chronic fatigue syndrome?

Answer 33

Pain
May have generalised myalgia
May have headaches or eye/abdominal/joint pain
No signs of arthritis (e.g. erythema or swelling)
Being touched may be painful
Sensory sensitivities
e.g. to light/sound/taste/touch smell
May have temperature hypersensitivity (e.g. sweating/chills/hot flushes/feeling cold)
May be intolerant to alcohol/foods/certain chemicals
Autonomic dysfunction including orthostatic intolerance
Dizziness
Palpitations
Fainting
Nausea
Neuromuscular symptoms
Twitching or myoclonic jerks may be seen
Flu-like symptoms
Sore throat
Tender lymph nodes

Patients should be examined to look for signs of differential diagnoses
A mental state examination should be done to assess mood and cognition
Postural orthostatic tachycardia syndrome (POTS) may be seen (when the heart rate rises significantly when standing from lying down)
Postural hypotension may also be present

Question 34

What investigations should we do for chronic fatigue syndrome?

Answer 34

There are no investigations that can diagnose CFS and the diagnosis is clinical.
However, all patients presenting with persistent fatigue should have the following blood tests to screen for an underlying physical cause:
Full blood count looking for anaemia, polycythaemia, inflammatory markers and haematological malignancy
U&Es for renal impairment or electrolyte imbalance
Liver function tests for liver disease
Bone profile to check calcium and phosphate
Myeloma screen especially in older patients or those with hypercalcaemia
Thyroid function tests for hypo or hyperthyroidism
HbA1c for diabetes
Ferritin which may be raised in inflammation or low in iron deficiency
ESR which is raised in inflammation or infection as well as some malignancies
Creatine kinase for muscle damage e.g. muscular dystrophy
IgA tissue transglutaminase for coeliac disease, which may present without gastrointestinal symptoms

Question 35

What might urinalysis show in chronic fatigue syndrome?

Answer 35

Urinalysis should also be done, which may show glycosuria in diabetes, haematuria in renal tract inflammation, infection or malignancy, or proteinuria in inflammation or chronic kidney disease

Question 36

Name some other random investigations based on clinical presentation for chronic fatigue syndrome?

Answer 36

Vitamin D in patients at risk of deficiency
Vitamin B12 and folate e.g. in anaemic patients or those with a restricted diet
Early morning cortisol to screen for adrenal insufficiency
HIV test in at risk patients (or all patients living in a high-prevalence area)
Hepatitis serology in at risk patients (e.g. those from an endemic area)
Monospot test for glandular fever in patients under the age of 40
Chest X-ray and sputum samples for tuberculosis in patients at risk (e.g. travel to an area where TB is endemic)
ELISA for Lyme disease if there is a history of tick bite
CT or MRI head if there are focal neurological signs or symptoms to rule out an intracranial cause for fatigue

Question 37

Describe the management for chronic fatigue syndrome:

Answer 37

Management of CFS should be person-centred and tailored to individual needs
All patients should be referred to a specialist multidisciplinary team for confirmation of diagnosis and development of a care and support plan
Patients with severe/very severe CFS may require home visits for assessment and support
Advice should include:
Education on CFS and its management
Signposting to national and local resources and support
How to manage daily activity and not to “push through” symptoms of fatigue
Resting as much as needed (which may involve alterations to their daily activities)
Incorporating relaxation techniques into rest periods
Managing flare-ups and relapses
Maintaining a balanced diet and fluid intake
Developing good sleep habits
How to access social care support and assessment e.g. for disability benefits, adaptations to the home such as a stairlift
For moderate/severe/very severe CFS, consider the need for mobility aids (e.g. wheelchairs)
Support patients in work or education and ensure reasonable adjustments are put in place
Energy management is an important component of CFS care
This is a self-management strategy supported by the multidisciplinary team
Patients are encouraged to recognise their personal energy limits
Activities and rest periods should be planned accordingly
All types of activities including social, cognitive and physical should be considered
Plans should be regularly reviewed
Physiotherapy and occupational therapy input may be helpful for support with mobility and physical activity
Exercise programmes should be supported by specialist services for patients who would like to incorporate this into their CFS management
Cognitive behavioural therapy should be discussed
Patients are supported to manage symptoms, improve functioning and relieve distress
It is structured and time-limited
It focuses on the specific challenges faced by the patient
Comorbid conditions such as depression should be identified and managed
Associated symptoms such as orthostatic intolerance may require specialist input - this is usually managed with lifestyle changes; medications such as beta-blockers may be used
Additional investigations and analgesia may be required for pain management
Dietetic assessment is required for patients with weight loss or gain, those at risk of malnutrition e.g. due to a restrictive diet, or those with severe/very severe CFS
Vitamin D supplements should be considered for housebound patients

Question 38

What are the complications for chronic fatigue syndrome?

Answer 38

Negative impacts on work, education, relationships and quality of life
Depression and/or anxiety
Malnutrition due to restrictive diet, poor appetite, food intolerances or difficulty with chewing/swallowing
Falls due to weakness and deconditioning
Pressure ulcers due to immobility
Deep vein thrombosis due to immobility
Contractures due to prolonged immobility
Vitamin D deficiency if leaving the house if difficult
Loss of earnings - 50% of patients with CFS have to stop work due to their symptoms

Question 39

What is the prognosis for chronic fatigue syndrome?

Answer 39

Negative impacts on work, education, relationships and quality of life
Depression and/or anxiety
Malnutrition due to restrictive diet, poor appetite, food intolerances or difficulty with chewing/swallowing
Falls due to weakness and deconditioning
Pressure ulcers due to immobility
Deep vein thrombosis due to immobility
Contractures due to prolonged immobility
Vitamin D deficiency if leaving the house if difficult
Loss of earnings - 50% of patients with CFS have to stop work due to their symptoms

Question 40

Define encephalitis:

Answer 40

Encephalitis is a pathological condition characterised by inflammation of the brain parenchyma.

Question 41

What causes encephalitis?

Answer 41

The aetiology of encephalitis is predominantly viral, with the most common pathogen being the herpes simplex virus type 1 (HSV-1). Other viral causes can include HSV-2, enterovirus, cytomegalovirus, Epstein-Barr virus, varicella-zoster virus, HIV, and flaviviruses, including the West Nile virus.
Additionally, bacterial pathogens like those that cause meningitis, Lyme disease and Mycoplasma can cause meningoencephalitis. Parasitic infections with malaria or toxoplasmosis should be considered, especially with recent travel or immunocompromise. Autoimmune encephalitis, such as NMDA-receptor-antibody-associated encephalitis, should also be considered in the differential diagnosis.

Question 42

Signs and symptoms of encephalitis?

Answer 42

Clinical features of encephalitis primarily include altered mental status, more so than in meningitis.
Other suggestive features include:
Fever
Flu-like prodromal illness
Seizures
Focal neurological deficits
Headaches
Behavioural changes
Meningismus

Question 43

What is meningismus?

Answer 43

clinical syndrome of headache, neck stiffness, and photophobia, often with nausea and vomiting

Question 44

What are the investigations for encephalitis?

Answer 44

Encephalitis should be suspected in any patient presenting with sudden onset behavioural changes, new seizures, and unexplained acute headache accompanied by meningism.
The standard work-up for encephalitis parallels that for meningitis and typically includes:
A routine panel of blood tests including FBC, U+E, LFTs (as part of a septic screen)
Blood cultures and viral PCR
Cerebrospinal fluid (CSF) analysis with viral PCR
Consideration for malaria blood films in cases of exposure
Central Nervous System (CNS) imaging through CT and MRI can provide valuable insight. For instance, HSV typically affects the temporal lobes and bilateral multifocal haemorrhage is typical.
Other tests may include sputum cultures, throat swab, chest X-ray and EEG (particularly if presenting with seizures)

Question 45

What is the management for encephalitis?

Answer 45

Management of suspected encephalitis is empirical and involves broad-spectrum antimicrobial cover with 2g IV ceftriaxone BD and 10 mg/kg aciclovir TDS. Once the causative organism is identified, targeted treatment may begin.
Supportive management of complications is also crucial and can include the termination of seizure activity using anticonvulsants.
Acute encephalitis is a notifiable disease and should be reported to the local health protection team.
Ampicillin can also be added if Listeria monocytogenes is suspected

Question 46

Define epilepsy?

Answer 46

Epilepsy is a neurological disorder characterised by an enduring predisposition to generate epileptic seizures and the neurobiological, cognitive, psychological, and social consequences of this condition.
Seizures are transient occurrences of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain.

Question 47

What causes epilepsy?

Answer 47

• Primary epilepsy is idiopathic
• Secondary causes:
  
  • Tumour
  • Meningitis
  • Vasculitis
  • Alcohol withdrawal
  • Haemorrhage
  • Metabolic

Question 48

What conditions are associated with epilepsy?

Answer 48

• Cerebral palsy
• Tuberous sclerosis
• Mitochondrial disease

Question 49

What type of seizures are there?

Answer 49

Focal seizure
Generalised Seizure
Status epilepticus

Question 50

What is a focal seizure?

Answer 50

• Seizure localised to specific cortical regions
• May be complex (consciousness affected) or non-complex (consciousness not affected)

Question 51

What is a generalised seizure?

Answer 51

Affects the whole brain and consciousness is lost immediately
- Absence
- Tonic
- Atonic
- Tonic-clonic
- Myoclonic

Question 52

What is Status epilepticus and how do we treat?

Answer 52

seizure lasting longer than 5 minutes or ≥2 seizures within a 5-minute period without the person returning to normal between them
IV lorazepam or PR diazepam

Question 53

Features of a frontal lobe focal seizure?

Answer 53

• Motor convulsions
• May show post-ictal flaccid weakness (post-ictal meaning just after seizure ends)
• Jacksonian march (clonic movements starting in 1 extremity and moving proximally through body) → usually starts as twitching/tingling of area like little toe or finger

Question 54

Features of a temporal lobe focal seizure?

Answer 54

Most common type of partial seizure

• Aura- involving?
  
  • Weird smells
  • Involuntary movements
  • Deja vu
  • Abdo pain
• Lip smacking/plucking/grabbing (automatisms)
• Post-ictal dysphasia
• Hallucinations

Question 55

Features of an occipital lobe seizure?

Answer 55

Visual disturbances (flashers and floaters)

Question 56

Features of a parietal lobe seizure?

Answer 56

Sensory issues (paraesthesia- tingling, numbness)

Question 57

Features of a tonic-clonic seizure?

Answer 57

• Vague symptoms before attack e.g. irritability
• Tonic phase (generalised muscle spasm- goes stiff and falls to floor)
• Clonic phase (repetitive synchronous jerks- jerking limbs or loss of bladder control)
• Urinary incontinence
• Tongue biting
• Post-ictal phase- including? (3)
  
  • Impaired consciousness
  • Lethargy
  • Confusion

Question 58

Features and treatment for an absence seizure?

Answer 58

• Onset in childhood
• Loss of consciousness but maintained posture (don’t fall down)
• No post-ictal phase
• Often begins abruptly without warning and ends abruptly
• Patient has no recollection of episode
• Stares blankly into space
  3 Hz spike and wave
  Treat with Ethosuximide

Question 59

Features of myoclonic seizure?

Answer 59

• convulsions without the muscle tensing (tonic phase)
• Sudden jerking of limb, trunk or face with preserved consciousness

Question 60

Features of atonic seizures?

Answer 60

• Sudden muscle relaxation causing patients to fall to the ground and then may motionless
• Can also result in incontinence
• Can result in post-ictal confusion

Question 61

Features of a tonic seizure?

Answer 61

muscle tensing without convulsions (clonic phase)

Question 62

General features of focal seizures?

Answer 62

With impaired consciousness (‘complex’): patients lose consciousness, usually post an aura or at seizure onset. Commonly originate from the temporal lobe, and post-ictal symptoms such as confusion are common.
Without impaired consciousness (‘simple’): patients retain consciousness, experiencing only focal symptoms. Post-ictal symptoms are absent.
Evolving to a bilateral, convulsive seizure (‘secondary generalised’): patients first experience a focal seizure that evolves into a generalized seizure, typically tonic-clonic

Question 63

What is a Jacksonian march seizure?

Answer 63

A type of focal motor seizure that progressively ‘marches’ through adjacent areas of the brain
Typically starts in the hand or face then gradually spreads to other muscle groups following the smatotopic organisation of the motor cortex (starting from hand and spreading to arm, shoulder and face)
The seizure may progress into a generalised tonic clonic seizure
Often associated with structural brain lesions

Question 64

What is Todd’s paresis?

Answer 64

Refers to temporary postictal weakness or paralysis following a seizure
Usually lasts minutes to hours but can last up to 48 hours
Usually unilateral but can be bilateral
It is transient so the patient will reover following resolution of the postictal state

Question 65

What are the investigations for epilepsy?

Answer 65

Detailed history and neurological examination
Collateral history and, if present, reviewing any videos of previous episodes (with the patient’s consent)
Imaging such as CT or MRI
Electroencephalogram (EEG)
Video-EEG telemetry can also be considered in select cases
Other investigations can be considered to investigate contributory causes which include blood tests, lumbar puncture and more advanced imaging investigations.

Question 66

What are the principles of epilepsy AGM management?

Answer 66

Aim for optimum quality of life through seizure control, balanced against potential side effects, particularly teratogenesis in women of childbearing age.
Initiation of medication may not always be appropriate after a “provoked” first seizure; discuss such cases with a specialist.
The choice of antiepileptic drugs involves complexity due to the lack of head-to-head trials comparing different medications.
Interactions with other medications, particularly with phenytoin and carbamazepine, should be considered.
Issues regarding teratogenicity, particularly with valproate, which carries a high risk of neural tube defects, should be considered. Lamotrigine is a better choice for women of childbearing age.

Question 67

What two drugs should be considered as first-line for focal seizures?

Answer 67

Lamotrigine or Levetiracetam

Question 68

What can be offered as first line for myoclonic seizures in males?

Answer 68

Sodium valproate

Question 69

What is the drug choice for absence seizures?

Answer 69

ethosuximide

Question 70

What drug may worsen myoclonic seizures

Answer 70

carbamazepine

Question 71

What is the guidance for patients after the “first fit”

Answer 71

Usual protocols will advise to refer to outpatient neurology following a patient’s first seizure
Most neurologists would start an ASM after a second seizure, unless there is a specific structural cause or a second fit would be unacceptable to the patient.
Following a discussion regarding the risks and benefits of starting an ASM, patients are given information on reducing risks e.g. driving advice or taking showers rather than baths.
Patient education via leaflets or online websites are utilised heavily as part of the initial discussions and further follow up or advice is usually relayed via a dedicated epilepsy nurse

Question 72

Name some complications of epilepsy?

Answer 72

Status epilepticus: Seizures lasting more than 5 minutes, necessitating immediate medical intervention OR more than 2 seizures within 5 minutes without returning to normal between each seizure.
Psychiatric complications: Increased risk of depression and suicide.
Sudden unexpected death in epilepsy (SUDEP): Thought to occur due to excessive electrical activity inducing a cardiac arrhythmia and subsequent death.

Question 73

What are the side effects of topiramate?

Answer 73

Abdominal pain, cognitive impairment, confusion, mood changes, muscle spasm, nausea and vomiting, nephrolithiasis, tremor, weight loss.

Question 74

What are the side effects of lamotrigine?

Answer 74

Blurred vision, arthralgia, ataxia, diarrhoea, dizziness, headache, insomnia, rash, tremor.
Steven johnson syndrome - Toxic Epidermal Necrolysis or Hypersensitivity syndrome in 1:4000 patients. It is more common in children that adults, more common with co-administration of Valproate, higher doses and rapid titration

Question 75

What are the side effects of carbamazepine?

Answer 75

Ataxia, blood disorders, blurring of vision, fatigue, hyponatraemia, skin problems

Question 76

What are the side effects of sodium valproate?

Answer 76

Ataxia, Anaemia, confusion, gastric irritation, haemorrhage, hyponatraemia, tremor, weight gain.

Question 77

What are the side effects of phenytoin?

Answer 77

Acne, anorexia, constipation, dizziness, gingival hypertrophy, hirsutism, insomnia, rash, tremor.

Question 78

What is the DVLA guidance for epilepsy?

Answer 78

For car/motorbike licence: reapply after 6 months for a one-off seizure, or after 1 year for more than one seizure. After a seizure following a change in anti-epileptic medications, reapply to drive if the seizure was more than 6 months ago or you’ve been back on the previous medication for 6 months.
For bus/coach/lorry licence: reapply after 5 years for a one-off seizure if no anti-epileptic medications have been taken during this period. For more than one seizure, reapply after 10 seizure-free years during which no anti-epileptic medication has been taken.

Question 79

Explain the difference between non-epileptic seizures and epileptic seizures?

Answer 79

Non-epileptic seizures can present with features such as pelvic thrusting, head movements during the attack, eyes closed, and prolonged duration (greater than 3 minutes), contrasting with the postictal symptoms of confusion, tongue biting, incontinence, and raised prolactin levels typically observed in epileptic seizures.

Question 80

What do we give for status epilepticus in hospital vs community

Answer 80

• IV lorazepam (hospital)
  
  • PR diazepam (community)

Question 81

Which medications are most commonly associated with reducing the seizure threshold?

Answer 81

Ciprofloxacin and other quinolones can precipitate seizures in patients with or without an underlying seizure disorder. They should be prescribed with extreme caution in patients with epilepsy.

Question 82

Describe juvenile myoclonic seizures?

Answer 82

Juvenile myoclonic epilepsy is usually seen in adolescence. It is commonly seen 1-2 hours after waking up and they can be triggered by a lack of sleep. Myoclonic seizures are often described as shock-like irregular movements of a limb. It will likely need treatment with anti-epileptics but the condition has a high response rate to medications.

Question 83

When is phenytoin given?

Answer 83

There is evidence for the use of phenytoin within the first 7 d after a traumatic brain injury to prevent early seizures; however, there is insufficient evidence to show a benefit beyond this

Question 84

Patients with temporal lobe epilepsy may experience hippocampal damage leading to deficits in x?

Answer 84

Long term memory

Question 85

What is an essential tremor?

Answer 85

Essential tremor (ET) is a chronic neurologic condition that typically manifests as an involuntary shaking or trembling usually on changing posture or performing an action, most commonly in the hands and arms, but can also affect other body parts such as the head, voice, lower limbs, tongue, face, and the trunk.

Question 86

What causes an essential tremor?

Answer 86

The etiology of ET is complex and not completely understood, but it is believed to be multifactorial, involving both genetic and environmental factors.
In approximately 50% of the cases, ET is inherited as an autosomal dominant trait, suggesting a significant genetic component.

Question 87

What is the main clinical feature of an essential tremor?

Answer 87

The main clinical feature of ET is a postural or kinetic tremor, which predominantly affects the upper limbs distally.

Question 88

What are the additional symptoms of essential tremor?

Answer 88

Involvement of the head, lower limbs, voice, tongue, face, and the trunk, although less common.
Increased tremor amplitude over time, causing difficulty with tasks such as writing, eating, holding objects, dressing, and speaking.
Exacerbation of tremor during situations of anxiety, stress, and social interaction.
Potential development of severe psychosocial disability, including depression, particularly in patients with head and voice tremors.

Question 89

Classically what can improve an essential tremor?

Answer 89

Classically, ingestion of alcohol can temporarily improve the tremor.

Question 90

What are the investigations for essential tremor?

Answer 90

The diagnosis of ET is mainly clinical.
Once suspected, a thorough evaluation of functional and psychosocial disabilities should be performed using objective scales to determine the need for pharmacotherapy.
Further investigations, such as blood tests or neuroimaging, may be warranted to rule out other potential causes of tremor.

Question 91

What is the management for essential tremor?

Answer 91

Management of ET is primarily symptomatic and may include:
Pharmacological therapy, using agents such as propranolol (first line), primidone, topiramate, gabapentin, clonazepam.
Surgical intervention for severe cases, including deep brain stimulation, focused ultrasound thalamotomy, and radiosurgical (Gamma Knife) thalamotomy.

Question 92

If a patient has an essential tremor but suffers from asthma what is the treatment?

Answer 92

Propranolol is contradicted. Other first line pharmacological treatments include topiramate and primidone

Question 93

Define malaria:

Answer 93

Malaria is a disease caused by protozoan parasites of the Plasmodium genus

Question 94

Name the different malaria species:

Answer 94

The different malaria species are:
P. falciparum (the most pathogenic species)
P. vivax
P. ovale
P. malariae
P. knowlesi

Question 95

What is the aetiology for malaria?

Answer 95

The parasitic life cycle of malaria involves a cyclical infection of two hosts: humans and female Anopheles mosquitoes. Infection in humans occurs in two stages: the liver stage and the blood stage. The clinical signs and symptoms of the disease are due to blood-stage parasites and tend to occur at different times depending on the species. Plasmodium falciparum has an incubation period of 7-30 days.
Following the bite of an infected female Anopheles mosquito, parasites, known as sporozoites, are inoculated into the human host, where they transit in the bloodstream to the liver. Sporozoites initially establish infection in hepatocytes, where they replicate and mature into hepatic schizonts. These subsequently rupture and release a form of parasite known as a merozoite into the bloodstream.
Merozoites infect erythrocytes, forming trophozoites that reproduce asexually and mature into blood-stage schizonts, which also rupture, causing the further release of merozoites. Some trophozoites differentiate into male or female gametocytes, which may be ingested by their second host, the Anopheles mosquito, in subsequent blood meals. Gametocytes reproduce within the stomach of the mosquito, where they form oocysts which grow and release sporozoites, which travel to the mosquito’s salivary glands to further perpetuate the cycle of further inoculation into a new human host.
The clinical manifestations of the disease are due to blood-stage parasites, which cause the accumulation of toxic substances and waste during their development in erythrocytes. Lysis of red blood cells results in the releasing these factors and potent inflammatory immune responses. The incubation period until symptoms appear tends to vary between 7 to 30 days.

Question 96

What are the risk factors for severe disease in malaria?

Answer 96

Pregnancy
Children
Elderly
Complex comorbidities
Immunosuppression

Question 97

What are the initial symptoms of malaria?

Answer 97

Fevers which may be cyclical, sweats and chills
Headache, malaise, aches & pains
Abdominal pain, nausea & vomiting, diarrhoea
Patients may have splenomegaly, jaundice, hypotension, pallor and reduced urine output

Question 98

What are the features of severe malaria?

Answer 98

Abnormal behaviour, seizures or altered consciousness suggest cerebral malaria
Parasitaemia >2%, severe anaemia or haemoglobinuria
Hypoglycaemia, metabolic acidosis, hypovolaemia
Temperature >39C
Acute respiratory distress syndrome, disseminated intravascular coagulation or shock
Renal impairment
Hb < 70 g/L
Hypoglycaemia (<2.2 mmol/L)

Question 99

What are the investigations for malaria?

Answer 99

Thick and thin blood films are the gold-standard method of diagnosing malaria. The thick film identifies the presence of parasites, and the thin film determines the species. If negative, films should be repeated.
Rapid diagnostic tests can also be used but do not replace the need for blood films.
Other important investigations include:
FBC & film, clotting, U&E’s, LFT’s, glucose measurement
Urine dip ± culture
ABG if severe symptoms
Other investigations to consider include blood cultures, stool culture, chest X-ray and lumbar puncture.

Question 100

What is the first line treatment for uncomplicated falciparum in malaria?

Answer 100

artemisinin-based combination (Artemether with lumefantrine) therapy

Patients will also require supportive care and antipyretics. In severe cases, ICU admission may be indicated.

Question 101

What is the first line treatment in uncomplicated non-falciparum in malaria?

Answer 101

artemisinin-combination therapy or chloroquine

Patients will also require supportive care and antipyretics. In severe cases, ICU admission may be indicated.

Question 102

What is the first line treatment in severe falciparum in malaria?

Answer 102

IV artesunate

Patients will also require supportive care and antipyretics. In severe cases, ICU admission may be indicated.

Question 103

As malaria is a notifiable disease what does one have to do?

Answer 103

Malaria is a notifiable disease and all cases should be reported to the local health protection team in the first instance.

Question 104

What is malaria prevention advice?

Answer 104

Avoidance of travel - particularly those who are at higher risk of severe infection
Measures to avoid mosquito bites - avoiding exposure at dusk & down, mosquito nets & screens, repellant, loose clothing
Chemoprophylaxis - drugs available include chloroquine, mefloquine and doxycycline, all of which have pro’s and cons and, as such, should be an individualised decision
Education about malaria, its symptoms and seeking medical attention early. Standby emergency medication may be prescribed if the person is far from medical care.

Question 105

In uncomplicated non-falciparum malaria what treatment do we give to prevent relapses after chloroquine?

Answer 105

Primaquine is the only drug effective against the hypnozoites (dormant liver-stage parasites) of Plasmodium ovale. After treatment of the acute blood-stage infection with chloroquine, starting primaquine is essential to eradicate the liver-stage hypnozoites. Without this treatment, the patient is at risk of future relapses, as P. ovale can remain dormant in the liver and reactivate later.

Question 106

which malaria prophylaxis agents should be avoided in a patient with epilepsy?

Answer 106

Mefloquine (Lariam) and chloroquine increase the risk of seizures and are therefore contraindicated in those with epilepsy.

Question 107

Define meningitis:

Answer 107

Meningitis is an inflammation of the meninges, which are composed of three layers: the dura mater, arachnoid mater, and pia mater. This inflammation may arise from both infective and non-infective aetiologies.

Question 108

What is the epidemiology of meningitis?

Answer 108

Bacterial meningitis, while not the most common form of meningitis, is particularly significant due to its high morbidity and mortality rates.
Viral meningitis, predominantly caused by enteroviruses, is more common but typically less severe. Fungal and parasitic causes are relatively rare, except in immunosuppressed individuals.

Question 109

Name the infective causes of meningitis:

Answer 109

Bacterial: Streptococcus pneumoniae (most common bacterial cause), Neisseria meningitidis, Haemophilus influenzae, Listeria monocytogenes, among others.
Viral: Enteroviruses are overall most common (Echoviruses, Coxsackie viruses A and B, poliovirus), herpes viruses (HSV2, HSV1), Paramyxovirus, measles and rubella viruses, Varicella Zoster Virus, Arboviruses, Rabies virus.
Fungal: Particularly Cryptococcus neoformans, mainly affecting the immunosuppressed population.
Parasitic: Amoeba (Acanthamoeba), Toxoplasma gondii.

Question 110

Name the non-infective causes of meningitis:

Answer 110

Non-infective causes of meningitis encompass:
Malignancies such as leukaemia, lymphoma, and other tumours
Chemical meningitis
Certain drugs, including NSAIDs and trimethoprim
Systemic inflammatory diseases such as sarcoidosis, systemic lupus erythematosus, Behcet’s disease.

Question 111

What are the cardinal features of meningitis?

Answer 111

Headache
Fever
Neck stiffness
Photophobia
Nausea and vomiting
Focal neurology
Seizures
Reduced conscious level
Features of overwhelming sepsis, such as non-blanching petechial rash indicative of impending Disseminated Intravascular Coagulation (DIC).

Question 112

What is the kernig sign?

Answer 112

Kernig’s sign is a test performed to evaluate the presence of meningeal irritation and stiffness in the hamstrings and lower back. To perform this test, the patient is positioned lying on their back with the hip and knee flexed at 90 degrees. The examiner then attempts to extend the patient’s knee. If the patient experiences pain and resistance to knee extension, especially when attempting to straighten the leg, it is considered a positive Kernig’s sign. This sign suggests meningeal irritation or inflammation.

Question 113

What is the Brudzinski’s sign?

Answer 113

Brudzinski’s sign is another manoeuvre used to assess for meningeal irritation. This test involves passive neck flexion, where the examiner gently flexes the patient’s neck forward toward the chest while the patient is lying on their back. If the patient involuntarily flexes their hips and knees in response to neck flexion, it is considered a positive Brudzinski’s sign. This involuntary movement indicates irritation of the meninges.

Question 114

What are the diagnostic investigations for meningitis?

Answer 114

Blood tests: Full Blood Count, Urea and Electrolytes, Clotting, Glucose, PCT
Arterial Blood Gas
Blood cultures
Bacterial throat swab for meningococcus
PCR for meningococcus & pneumococcus
HIV test
Imaging: CT Head if there are signs of raised intracranial pressure (ICP)
Lumbar puncture for Cerebrospinal Fluid (CSF) analysis, once confirmed there are no signs of raised ICP.

Question 115

What are the CSF findings in meningitis?

Answer 115

Question 116

What is the findings in cryptococcal meningitis?

Answer 116

may give any of the results, so should be considered as a differential in any HIV or immunocompromised patient. Classically the opening pressure is very high, and this is a poor prognostic sign. If suspected, request cryptococcal antigen or India Ink staining.

Question 117

What is the management for meningitis?

Answer 117

Empirical antibiotic therapy for suspected bacterial meningitis typically includes 2g of IV ceftriaxone twice daily to ensure CNS penetration, with IV amoxicillin added in patients at age extremes for listeria coverage.

In primary care, IM benzylpenicillin or ceftriaxone should be given while awaiting urgent transfer to hospital, especially if meningococcal disease is suspected.

Dexamethasone should be given if bacterial meningitis is strongly suspected in the absence of a rash
This has been shown to reduce neurological sequelae in bacterial meningitis but not meningococcal meningitis

In cases of suspected viral encephalitis, IV aciclovir should also be administered. For patients allergic to penicillin, alternatives such as chloramphenicol may be used.
It’s important to note that any empirical antibiotic regimen should be adjusted based on culture results when available.

Question 118

What should close contacts get when a case of meningitis is confirmed?

Answer 118

Close contacts of the patient should receive prophylactic antibiotics. This will be guided by specialists but may be a single dose of oral ciprofloxacin, or rifampicin.
Bacterial meningitis is a notifiable disease and any suspected cases should be reported to the local health protection team.

Question 119

What are the complications of meningitis?

Answer 119

Septic shock
Disseminated Intravascular Coagulation
Coma
Subdural effusions
Syndrome of inappropriate antidiuretic hormone secretion
Seizures
Delayed complications: Hearing loss, cranial nerve dysfunction, hydrocephalus, intellectual deficits, ataxia, blindness
Death

Question 120

What are the findings in Cryptococcal meningoencephalitis?

Answer 120

Immunocompromised individuals, particularly those with HIV and low CD4 counts, are at increased risk for cryptococcal meningoencephalitis, characterised by fever, headaches, photophobia, neck stiffness, cranial nerve palsies, and nonspecific neurological signs, with normal or bland CT head scans and LP findings showing raised opening pressure, slightly elevated protein, and lymphocyte predominance.

Question 121

A lumbar puncture is performed and the findings are consistent with bacterial meningitis.
Blood cultures grow gram-positive rods.
Which of the following is most likely to be the causative organism?

Answer 121

Listeria monocytogenes is a gram-positive rod bacteria that can cause meningitis. It can be contracted from certain foods, such as soft cheeses. Complications during pregnancy include chorioamnionitis and premature labour, and it can also be transmitted through the placenta causing fetal infection. Treatment is with ampicillin +/- an aminoglycoside.

Question 122

Define migraine?

Answer 122

Migraine is a primary headache disorder characterised by intense episodes of debilitating headaches, usually unilateral and pulsating in nature. Symptoms may be preceded by an ‘aura’ which manifests as visual disturbances or sensory changes. The pain usually lasts from 4-72 hours and can be accompanied by nausea, vomiting, photophobia, and phonophobia.

Question 123

What causes migraines?

Answer 123

The exact cause of migraines is not fully understood, but it is likely a combination of genetic and environmental factors.
The triggering factors are variable and can include certain foods, changes in weather, stress, hormonal changes, and certain medications such as oral contraceptives.

Question 124

What are the signs and symptoms of migraines?

Answer 124

Aura (usually visual or sensory symptoms preceding the headache)
Unilateral throbbing headache
Photophobia and phonophobia
Nausea and/or vomiting

Question 125

What is the International Headache Society criteria for migraine without aura:

Answer 125

Question 126

What are the investigations for migraines?

Answer 126

Diagnosis is primarily clinical, based on the history and examination.
A headache diary is important to help identify triggers and response to treatment.
If secondary causes of headaches are suspected, further investigations may be warranted, such as neuroimaging (MRI or CT) or blood tests (ESR, CRP for giant cell arteritis).

Question 127

What is the acute management for migraines?

Answer 127

Avoidance of triggers:
Identify and avoid potential triggers like certain foods, stress, and poor sleep.

Medications for acute attacks:
Triptans (e.g., Sumatriptan) – avoid in patients with ischaemic heart disease as it vasoconstricts
Paracetamol or an NSAID (e.g., Ibuprofen) can be used in combination with triptans.
Anti-emetics (e.g., Metoclopramide)

Special considerations:
Female patients with migraine with aura should avoid the combined oral contraceptive pill due to increased risk of ischaemic stroke.

Question 128

Prophylaxis for migraines?

Answer 128

Medications:
Propranolol (contraindicated in asthma) - but first line
Topiramate (teratogenic)
Amitriptyline.
Candesartan.

Injections:
Greater Occipital Nerve Block
Botulinum Toxin Injection

Newer treatments:
Rimegepant (per NICE guidance, May 2023):
Used for preventing episodic migraine.
Suitable when at least 3 preventive treatments have failed.
Indicated for adults with 4-15 migraine attacks per month.

Regular use of acute migraine medications (e.g., triptans, NSAIDs) more than 10-15 days per month can lead to medication overuse headache (MOH).
Patients should be counseled on limiting the use of acute treatments to prevent MOH.

If a patient has 2 or more attacks a month resulting in disability for 3 days or more, standard analgesia and triptans are contraindicated or ineffective, or an uncommon form of migraine, prevention should be considered.

Question 129

How to differentiate between migraines and idiopathic intracranial hypertension?

Answer 129

Idiopathic Intracranial Hypertension can present with headaches that appear similar to migraines but usually have some atypical features, e.g., becoming worse on lying down or with vagal manoeuvres such as coughing or sneezing.

Question 130

How should sumatriptan be taken for migraines?

Answer 130

Sumatriptan should be taken once the headache starts, but not during the aura phase

Question 131

Define motor neurone disease?

Answer 131

Motor neuron diseases are a group of progressive neurological disorders that destroy motor neurons, the cells that control voluntary muscle activity such as speaking, walking, breathing, and swallowing. no sensory deficit

Question 132

What is the epidemiology of motor neurone disease?

Answer 132

The lifetime risk of MND is approximately 1 in 400 in the USA and Europe.
There is a slight 2:1 male predominance.
Mean onset at 50-60 years for sporadic cases.
90% of cases are sporadic, while the remaining 10% are familial, often linked to mutations in genes such as SOD1, FUS and C9ORF72.
There is a notable overlap with frontotemporal dementia (FTD) — the most common mutation in MND, C9orf72, is found in 40% of cases of FTD.

Question 133

What causes motor neurone disease?

Answer 133

MND is associated with misfolding of the TDP-43 protein in many cases.
It can be an inherited condition, with several associated genes yet to be identified.
About 2% of total cases are associated with a mutation in the SOD-1 gene.

Question 134

What are the upper motor neurone disease signs and symptoms?

Answer 134

Spasticity
Hyperreflexia
Upgoing plantars (although they are often downgoing in MND).

Question 135

What are the lower motor neurones disease signs and symptoms?

Answer 135

Fasciculations
Muscle atrophy.

Question 136

What are the different motor neurone disease subtypes and do they cause upper or lower motor neurone disease signs?

Answer 136

Question 137

What are the investigations for motor neurones disease?

Answer 137

Investigations for MND primarily aim to rule out treatable differential diagnoses. Key tests include:
Thyroid function tests (TFTs): To exclude thyrotoxicosis syndrome.
Protein electrophoresis: To rule out paraproteinaemias.
MRI of the brain and spinal cord: To assess for brainstem lesions mimicking MND or cervical spondylopathy.
EMG & nerve conduction studies: Important for looking for a myasthenic syndrome, chronic inflammatory demyelinating polyneuropathy, or multifocal mononeuropathy.

Question 138

What is the management for motor neurone disease?

Answer 138

Currently, treatment for MND is primarily supportive, as the only disease-modifying treatment, Riluzole, only extends life expectancy by an average of 3 months.
Non-invasive ventilation can prolong survival in patients with type 2 respiratory failure.

Key management strategies include:
Coordinating treatment via a multidisciplinary team approach.
Providing pain relief with simple analgesia and treating spasticity and contractures with baclofen and botox injections.
Using anticholinergics to manage drooling.
Supporting feeding via an NG or PEG tube as bulbar disease progresses.
Discussing advanced care planning early in disease progression to minimise distress and complications.

Question 139

What is the prognosis of motor neurone disease?

Answer 139

The prognosis for motor neuron disease is generally poor, with life expectancy from diagnosis usually less than 5 years. Most patients succumb to the respiratory complications of the disease.

Question 140

What is Bulbar amyotrophic lateral sclerosis?

Answer 140

This is the variant of motor neuron disease which presents with bulbar symptoms such as slurred speech and difficulty swallowing. On examination, there may be evidence of tongue fasciculations.

Question 141

Define myasthenia gravis?

Answer 141

Myasthenia gravis is an autoimmune disease marked by the production of antibodies that target the postsynaptic nicotinic acetylcholine receptors at the neuromuscular junction. This immune-mediated interference reduces the ability of acetylcholine to trigger muscle contraction, resulting in muscle weakness.

Question 142

epidemiology of myasthenia gravis?

Answer 142

Myasthenia Gravis affects both genders and all age groups, but there is a bimodal distribution with a higher incidence in women under 40 and men over 60.

Question 143

What are the signs and symptoms of myasthenia gravis?

Answer 143

The key feature of weakness in patients with myasthenia gravis is fatigability.
Patients with myasthenia gravis commonly present with:
Fatiguable limb muscle weakness
Ptosis
Diplopia
Facial palsy
Bulbar weakness e.g. dysphagia, dysphonia
Proximal muscle weakness
These signs and typically worsen with prolonged movement or by the end of the day.

Question 144

Which drugs exacerbate myasthenia gravis?

Answer 144

Beta blockers
Lithium
Penicillamine
Gentamicin
Quinolones
Phenytoin

Question 145

What are the key investigations for myasthenia gravis?

Answer 145

Bedside tests: Ice-pack test
Ice is placed over the eye for 2-5 minutes to assess for improvements in ptosis
Blood tests: Testing for serum acetylcholine receptor (anti-AChR) antibody and muscle-specific tyrosine kinase (anti-MusK) antibodies and (rarely) anti-LRP4 antibodies
Imaging investigations: CT scan of the chest to identify thymic hyperplasia or thymoma
Approximately 10% of patients with MG have a thymoma, and it is implicated in the production of autoantibodies
Nerve Conduction Studies/EMG: Repetitive nerve stimulation testing
In a myasthenic crisis, serial pulmonary function tests (spirometry) are performed.
If the forced vital capacity is 15 mL/kg or less, the patient should be considered for mechanical ventilation.

Question 146

What is the management for myasthenia gravis?

Answer 146

Regular review in neurology outpatients
Multidisciplinary team involvement as required (including occupational therapist, physiotherapist, speech and language therapist)
Medical management with immunosuppressive therapy (such as steroids) and anticholinesterase inhibitors (such as pyridostigmine or neostigmine).
Acute cases may require intravenous immunoglobulin (IVIG) or plasmapheresis in severe, steroid-refractory, cases.
Surgical management with thymectomy is indicated in patients with evidence of thymoma AND in patients with non-thymomatous antibody positive n-AChR MG

Question 147

What is the gold standard parameter used to monitor a patient’s respiratory effort with a background of neuromuscular conditions?

Answer 147

Forced Vital Capacity (FVC) is the gold standard parameter to monitor in patients with breathing difficulties with a background of neuromuscular conditions such as myasthenia gravis, motor neurone disease, or Lambert-Eaton syndrome as it is can be reflective of early respiratory muscle dysfunction.

Question 148

Define multiple sclerosis?

Answer 148

Multiple sclerosis is a chronic autoimmune disease, primarily involving the central nervous system, which is marked by the degeneration of the insulating covers of nerve cells in the brain and spinal cord, leading to demyelination and eventual axonal loss.

Question 149

What is the epidemiology of multiple sclerosis?

Answer 149

MS predominantly affects females with a current ratio of 2.3:1 and a mean onset age of 30 years.

Question 150

What causes multiple sclerosis?

Answer 150

The exact cause of multiple sclerosis remains unknown. However, a combination of genetic and environmental factors, including potential viral pathogens, are believed to be contributing factors.
Pathologically, CD4-mediated destruction of oligodendroglial cells and a humoral response to myelin binding protein are key features of the disease.

Question 151

Signs and symptoms of multiple sclerosis?

Answer 151

Multiple sclerosis can cause a wide range of symptoms depending on the affected area of the brain or spinal cord, but common presentations include:
Sensory disturbance, marked by patchy paraesthesia
Optic neuritis, characterised by loss of central vision, loss of red desaturation and painful eye movements
Internuclear ophthalmoplegia, a lesion in the medial longitudinal fasciculus of the brainstem
Subacute cerebellar ataxia
Spastic paraparesis, as seen in transverse myelitis, including Lhermitte’s sign.
Bladder and bowel disturbance
Uhthoff’s sign (electric-like pain down her legs in warm environments)

Question 152

What two groups is multiple sclerosis divided into?

Answer 152

Multiple sclerosis may be divided into two groups:
Relapsing-remitting (which may become secondarily progressive)
Primary progressive.

Relapsing remitting MS makes up 80% of disease at presentation, compared with primary progressive which is <10%.
The remaining 10% fall into a difficult to classify intermediate group of progressive-relapsing disease.

Question 153

Name the things a diagnosis of multiple sclerosis is based on?

Answer 153

At least two of:
Clinical history/examination

Imaging findings
Typically these are periventricular white matter lesions seen on MRI disseminated in time and space

Oligoclonal bands in the CSF - distinct bands of Immunoglobulin (Ig) G
These reflect various immunoglobulins seen on CSF electrophoresis and indicate the presence of an auto-immune process in the CNS.
They are very sensitive for multiple sclerosis although they can also be found in other auto-immune and infectious conditions including Lyme disease, SLE and neurosarcoid.

Visual evoked potential can help further characterise the diagnosis in patients presenting with optic neuropathy

Question 154

Imagine findings in multiple sclerosis:

Answer 154

Question 155

What is the Mcdonald criteria for multiple sclerosis?

Answer 155

Question 156

What is the acute management for multiple sclerosis?

Answer 156

An acute attack of multiple sclerosis should be treated with glucocorticoids involving local neurology services
1g of intravenous methylprednisolone every 24 hours for 3 days is a typical regimen
Infections must first be excluded
Always ensure to check routine bloods and urine dip to rule out any intercurrent infection.
While these interventions does not appear to affect long term outcome, it does appear to reduce the duration and severity of individual attacks.

Question 157

What are the two groups of drugs used for chronic multiple sclerosis?

Answer 157

There are two groups of drugs used in the long term management of relapsing remitting multiple sclerosis: disease modifying therapies (DMTs) and symptomatic therapies.

Question 158

What are the three groups disease modifying therapies are split into for multiple sclerosis?

Answer 158

First-line injectables such as beta-interferon and glatiramer acetate
New oral agents such as dimethyl fumarate, teriflunomide and fingolimod
Biologics such as natalizumab and alemtuzumab.

Question 159

What are the symptomatic therapies for multiple sclerosis?

Answer 159

Physiotherapy
Baclofen and Botox for spasticity
Modafinil and Amantadine and exercise therapy for fatigue
Anticholinergics for bladder dysfunction
SSRIs for depression
Sildenafil for erectile dysfunction
Clonazepam for tremor

Question 160

What is Internuclear ophthalmoplegia?

Answer 160

Internuclear ophthalmoplegia is characterized by an inability to adduct one eye while having nystagmus in the other, and can be a sign of multiple sclerosis which should be investigated with an MRI of the head as first-line.

Question 161

factors that are associated with worse prognosis in Multiple Sclerosis?

Answer 161

Older, male, motor signs at onset, early relapses, many MRI lesions and axonal loss

Question 162

Define Parkinson’s Disease?

Answer 162

Parkinson’s disease (PD) is a neurodegenerative disorder that presents with generalised slowing of movements (bradykinesia), resting tremor and rigidity.

Question 163

What causes Parkinson’s diseases?

Answer 163

The exact cause of Parkinson’s disease is unknown.
It is believed to result from the accumulation of “Lewy bodies”, intracellular inclusions primarily composed of misfolded alpha synuclein.
These bodies form and lead to neuronal death in the dopaminergic cells of the substantia nigra of the basal ganglia, thereby causing the characteristic symptoms.
Both dominant and recessive familial variants of Parkinson’s disease have been identified.

Question 164

What are the core features of Parkinson’s disease?

Answer 164

Bradykinesia
Asymmetric 3-5Hz “pill-rolling” resting tremor
Rigidity
Often noted to be ‘cog-wheeling’ which occurs when the tremor coincidees with rigidity
Gait Disturbance
Small, shuffling steps
Difficult initation and turning ‘en bloc turning’
Hypomimic facies
Micrographia (secondary to a combination of bradykinesia and rigidity)

“TRAP”, which stands for Tremors, Rigidity, Akinesia(Brady) and Postural Instability (Shuffling gait with a stooped posture often with festination).

Question 165

What are the non-motor functions of Parkinson’s

Answer 165

Non-motor features of Parkinson’s disease are also common and may precede onset of motor symptoms by many years. These include:
Autonomic dysfunction, leading to constipation, symptomatic orthostasis (postural hypotension), and erectile dysfunction
Olfactory loss
Constipation
Sleep disorders such as REM behavioural disorder
Psychiatric features including depression, anxiety, and hallucinations

Question 166

What is a late feature of idiopathic Parkinson’s disease?

Answer 166

Postural instability is a late feature of idiopathic Parkinson’s disease. This said, very prominent early autonomic dysfunction should raise the suspicion of Multiple System Atrophy.

Question 167

Early and prominent cognitive dysfunction with Parkinson features should raise suspicions of?

Answer 167

dementia with Lewy bodies (DLB)

Question 168

What are the investigations for Parkinson’s?

Answer 168

Parkinson’s disease is primarily a clinical diagnosis, supported by positive response to treatment trials.
An absolute failure to respond to 1-1.5g of levodopa daily almost excludes a diagnosis of idiopathic Parkinson’s disease.
Other investigations such as MRI Head or a Dopamine Transporter Scan (DaT scan) can be considered in atypical cases.

Question 169

Explain the pharmacological management levodopa for Parkinson’s? Side effects as well

Answer 169

Levodopa is the precurser to dopamine
It is used as a dopamine replacement agent
Should be started in all patients with significant functional impairment according to NICE guidelines
Typically combined with Carbidopa, which decreases side effects and improves CNS bioavailability), oftern referred to as Co-careldopa
Absolute failure to respond to 1-1.5g of levodopa daily virtually excludes a diagnosis of idiopathic Parkinson’s disease, though a response to levodopa does not confirm the diagnosis (as many primary parkinsonian syndromes may also respond).
Side effects can be split into peripheral and central (depending on where the acting dopamine receptor is for the side effect).
Peripheral side effects include:
Postural hypotension
Nausea & vomiting*
With time, and as the underlying disease progresses, levodopa may become a less effective and patients may report end-of-dose effects, where motor activity progressively declines as the previous dose wears off, and on-off phenomena, which manifest as seemingly random fluctuations in drug effect.
One of the most disabling side effects is drug-induced dyskinesia: writhing and uncoordinated movements of the limbs associated with poorly organised dopaminergic control of motor activity.
It typically takes 2-5 years to develop complete loss of response.
*Domperidone is the anti-emetic of choice in those with Parkinson’s disease which has anti-dopaminergic activity but does NOT cross the blood brain barrier.

Question 170

Explain the use of dopamine agonists in Parkinson’s?

Answer 170

Examples include: Ropinirole, rotigotine, Apomorphine. Previous ergot derived formulations (eg cabergoline) as now not widely used as are associated with lung fibrosis.
Have a longer half life than levodopa but are not as potent
Are often used in early disease in those without functional impairment, or late in disease when dyskinesias and motor fluctuations secondary to levodopa is a problem.
Increases activity of dopamine via the mesolimbic pathway which may affect reward centres and lead to unwanted risk-taking behaviours such as gambling.
Apomorphine is the most potent dopamine agonist and is typically given subcutaneously. It works well against motor fluctuations and dyskinesia. Often used late in disease.

Question 171

Explain the use of MAO-B Inhibitors for Parkinson’s?

Answer 171

Examples include: Selegiline, Rasagiline.
Reduce dopamine breakdown peripherally & thus increase central update of levodopa. Often used in combination with levodopa later in disease.
Initial animal studies suggested a “neuroprotective effect” although this has not been seen in human studies.
Can cause serotonin syndrome.

Question 172

Explain the use of COMT inhibitors in Parkinson’s?

Answer 172

COMT inhibitors, or catechol- O -methyltransferase inhibitors include Entacapone and Tolcapone.
Extend the use of levodopa. Useful in wearing off effect in levodopa use.
Tolcapone is more potent than entacapone and can result in hepatotoxicity.

Question 173

Explain the use of amantadine in Parkinson’s?

Answer 173

NMDA receptor antagonist that has an unclear action in Parkinson’s Disease.
Can be used in those suffering from dyskinesia.

Question 174

Explain the use of anticholinergic agents in Parkinson’s?

Answer 174

Examples include: Procyclidine, Trihexyphenidyl
Can be used in those with mild tremors.
Rarely used in clinical practice

Question 175

Explain surgical management in Parkinson’s

Answer 175

Deep Brain Stimulation: typically performed by implanting a stimulating device into a target area of the brain, often the thalamus or the subthalamus

Question 176

Parkinson’s disease patients can experience worsened symptoms when given ? , a common anti-emetic due to its dopamine antagonist properties.

Answer 176

metoclopramide

Question 177

NICE recommends ? as first line for the treatment of hallucinations in patients with Parkinson’s disease and features of psychosis.

Answer 177

Quetiapine

Question 178

How can multiple system atrophy be differentiated from Parkinson’s

Answer 178

Multiple System Atrophy can be differentiated from idiopathic Parkinson’s by its symmetric and atremoulus parkinsonism, constipation (suggesting autonomic dysfunction) and significant postural hypotension, a key feature of MSA.

Question 179

Define peripheral nerve injuries/palsies?

Answer 179

Peripheral nerve injuries, also known as palsies, refer to a range of conditions that result from damage to the peripheral nervous system

Question 180

Name some traumatic causes of peripheral nerve injuries/palsies?

Answer 180

Fractures and dislocations: Direct trauma to the nerves can occur during fractures or dislocations. For instance, radial nerve injury is common in humeral shaft fractures while ulnar nerve can be damaged in elbow dislocations.
Penetrating injuries: These include gunshot wounds, stab wounds or any other sharp object penetrating the skin causing direct damage to the nerves.
Stretch injuries: Excessive stretching of nerves during accidents or sports activities can lead to nerve damage. Brachial plexus injuries from high-speed motor vehicle accidents are typical examples.

Question 181

Name some compressive causes of peripheral nerve injuries/palsies?

Answer 181

Carpal tunnel syndrome: This condition results from compression of median nerve at the wrist due to repetitive movements, obesity or conditions like rheumatoid arthritis leading to characteristic symptoms.
Cubital tunnel syndrome: Compression of ulnar nerve at elbow often due to prolonged bending of elbow like during sleep or work can result in this condition.

Question 182

Name some inflammatory causes of peripheral nerve injuries/palsies?

Answer 182

Vasculitis: Inflammation and necrosis of blood vessels supplying the peripheral nerves can lead to nerve damage. Polyarteritis nodosa is one such example.
Sarcoidosis: This systemic granulomatous disease may involve peripheral nerves although involvement is relatively rare.

Question 183

Name some metabolic causes of peripheral nerve palsies/injuries?

Answer 183

Diabetes mellitus: Chronic hyperglycaemia can lead to peripheral neuropathy, a common complication of diabetes. It usually presents as distal symmetric polyneuropathy.

Question 184

Name some infectious causes of peripheral nerve injuries/palsies?

Answer 184

Lyme disease: Caused by Borrelia burgdorferi, this condition may cause peripheral neuropathy in later stages.
HIV: Peripheral nerve involvement is common in HIV-infected individuals either due to the virus itself or secondary to antiretroviral treatment.

Question 185

Name some neoplastic causes of peripheral nerve injuries/palsies?

Answer 185

Direct invasion: Malignancies like lung cancer, breast cancer or lymphomas can directly invade and damage the nerves.
Paraneoplastic syndromes: Nerve damage can also occur secondary to immune response against tumours in paraneoplastic syndromes.

Question 186

Name some iatrogenic causes of peripheral nerve injuries/palsies?

Answer 186

Surgical procedures: Nerve injuries may occur inadvertently during surgical procedures. For example, axillary nerve injury during shoulder surgery or recurrent laryngeal nerve injury during thyroidectomy.

Question 187

What are the risk factors for peripheral nerve injuries/palsies?

Answer 187

advanced age, presence of comorbid conditions like diabetes or rheumatoid arthritis, certain occupations involving repetitive movements, participation in high-risk sports activities and substance abuse.

Question 188

What 3 categories can peripheral nerve injuries/palsies be grouped into?

Answer 188

neuropraxia, axonotmesis, and neurotmesis

Question 189

Explain the category of neuropraxia?

Answer 189

This is the mildest form of nerve injury characterised by a temporary blockage of nerve conduction without any anatomical disruption to the nerve or its surrounding structures. The hallmark features include:
Transient motor and sensory loss
No Wallerian degeneration observed
Rapid recovery within days to weeks

Question 190

Explain the category of axonotmesis?

Answer 190

This type of injury involves damage to the axon with preservation of the endoneurium, perineurium, and epineurium. It is typically caused by crush or stretch injuries. Axonotmesis is characterised by:
Motor and sensory loss below the level of injury
Wallerian degeneration distal to the site of injury
Potential for regeneration at a rate of 1mm/day with variable recovery outcomes depending on the severity and location of injury

Question 191

Explain the category of neurotmesis?

Answer 191

This represents the most severe form of peripheral nerve injury where there is complete transection or disruption of the nerve fibre including all its encapsulating structures. Features include:
Total motor and sensory loss below level of injury
Complete Wallerian degeneration
No spontaneous recovery; surgical intervention required for potential functional return

Question 192

Explain the sunderland’s classification of Peripheral nerve injuries/palsies

Answer 192

This system provides a more detailed prognosis and guides treatment approaches:
First degree: Equivalent to neuropraxia in Seddon’s classification
Second degree: Equivalent to axonotmesis with preservation of endoneurium in Seddon’s classification
Third degree: Axon and endoneurium are damaged, but perineurium and epineurium remain intact
Fourth degree: Only the epineurium remains intact; surgical intervention often required due to formation of neuroma
Fifth degree: Equivalent to neurotmesis in Seddon’s classification; complete transection requiring surgical repair or grafting

Question 193

The stereotypical presentation of peripheral nerve injuries/palsies revolves around a triad of what?

Answer 193

sensory deficits, motor weakness and autonomic dysfunction

Question 194

What are the sensory deficits in peripheral nerve injuries/palsies?

Answer 194

Patients may report loss of sensation or abnormal sensations, such as numbness, tingling (paraesthesia), or burning pain (dysaesthesia) in the distribution area of the affected nerve.
On examination, there may be reduced or absent tactile discrimination, temperature perception, proprioception and vibratory sensation. The pattern of sensory loss can help determine whether the lesion is mononeuropathy (involving a single nerve) or polyneuropathy (involving multiple nerves).

Question 195

What are the motor weaknesses in peripheral nerve injuries/palsies?

Answer 195

This typically manifests as muscle weakness in the region supplied by the injured nerve. For example, radial nerve palsy might present with wrist drop while ulnar nerve palsy could lead to ‘claw hand’ deformity.
Fine motor skills may be compromised due to impaired dexterity. In chronic cases, muscle atrophy from disuse can occur.
Deep tendon reflexes may be diminished or absent depending on the extent of damage to the motor fibres.

Question 196

What are the automatic dysfunction in peripheral nerve injuries/palsies?

Answer 196

Damage to autonomic fibres can result in a variety of symptoms including changes in skin temperature, sweating abnormalities and trophic changes like hair loss or skin thinning overlying the affected area.
In severe cases involving proximal nerves, patients may experience orthostatic hypotension, bowel/bladder dysfunctions or sexual dysfunction.

Question 197

Name some additional features in peripheral nerve injuries/palsies?

Answer 197

In some cases, patients may experience neuropathic pain which is often described as burning, shooting or stabbing in nature. This can be a result of spontaneous firing of the damaged nerve fibres.
Tinel’s sign (a tingling sensation elicited by tapping over the injured nerve) and Phalen’s test (wrist flexion causing paraesthesia in median nerve distribution) are useful for diagnosing specific nerve injuries such as carpal tunnel syndrome.
Patients with long-standing peripheral nerve injuries may develop compensatory changes like modified gait or posture due to muscle weakness.

Question 198

What are the investigations for peripheral nerve injuries/palsies?

Answer 198

Electrodiagnostic studies: These encompass nerve conduction studies (NCS) and electromyography (EMG). NCS assesses the speed and degree of myelination of nerves while EMG evaluates muscle activity to determine whether there is denervation due to a peripheral nerve injury.
Magnetic Resonance Imaging (MRI): This imaging modality can provide detailed images of nerves and surrounding tissues. It can identify traumatic lesions such as nerve transections, contusions or entrapments, tumours compressing the nerves or infiltrating neuropathies.
High-resolution ultrasound: A non-invasive tool that provides real-time dynamic imaging. It can help in identifying focal nerve enlargements, entrapments or discontinuities.
Blood tests: These may be useful to identify systemic conditions contributing to peripheral neuropathy such as diabetes mellitus, vitamin B12 deficiency, thyroid dysfunction or connective tissue disorders. Specific tests include full blood count, renal function test, liver function test, glucose levels, thyroid function test and serum vitamin B12 levels.

In some cases where diagnosis remains elusive after these investigations, a biopsy may be considered. This could involve skin biopsy for small fibre neuropathy or sural nerve biopsy for suspected vasculitis or amyloidosis.

Question 199

What is the management for peripheral nerve injuries/palsies?

Answer 199

Diagnosis
Detailed history and physical examination remain paramount in diagnosing peripheral nerve injuries.
Evaluate for associated injuries which could impact management.
Electromyography (EMG) and nerve conduction studies may aid in determining the extent of injury and prognosis.
Non-surgical management
Pain control: Non-steroidal anti-inflammatory drugs (NSAIDs), opioids, gabapentin or pregabalin can be used to manage neuropathic pain.
Physiotherapy: Early mobilisation to prevent joint stiffness; strengthening exercises to maintain muscle tone; splinting if required.
Surgical management
Indicated for severe injuries such as Sunderland grade IV and V lesions, failed conservative treatment or presence of sharp penetrating trauma.
Primary repair, nerve grafting or nerve transfers are the mainstay surgical interventions depending on the nature of the injury.
Follow-up and rehabilitation
Routine follow-ups are necessary to monitor progress, modify treatment plans if needed, and assess for complications such as neuroma formation or joint contractures.
A comprehensive rehabilitation plan involving occupational therapy may be required for optimal functional recovery.

Question 200

What are clinical features of ulnar nerve injuries?

Answer 200

• Wasting of hypothenar eminence
• Loss of sensation over medial 1 and a half digits
• Weakness in abductor digiti minimi
• Claw hand
• What levels of the spinal cord are affected?C8-T1

Question 200

What are clinical features of median nerve injuries?

Answer 200

• Wasting of thenar eminence
• Loss of sensation in lateral palmar surface of 3 and a half digits
• Weakness in abductor pollicis brevis
• What condition is the median nerve commonly affected in?Carpal tunnel syndrome
• What levels of the spinal cord are affected?C6-T1

Question 201

What are clinical features of radial nerves injuries?

Answer 201

• Weakness of wrist extension → wrist drop
• Anaesthesia over first dorsal interosseous muscle
• What levels of the spinal cord are affected?C5-T1

Question 202

What are clinical features of Klumpke’s palsy?

Answer 202

• Paralysis of intrinsic muscles of hand
• Loss of sensation in ulnar distribution
• Horner’s syndrome sometimes present
• What levels of the spinal cord are affected?C8-T1

Question 203

What are clinical features of Erb’s palsy?

Answer 203

• Loss of shoulder abduction and elbow flexion
• Arm held internally rotated
• Waiter’s tip
• What levels of the spinal cord are affected?C5-C6

Question 204

What are clinical features of common peroneal nerve injuries?

Answer 204

• Weakness in dorsiflexion and eversion of foot → foot drop
• Sensory loss over dorsum of foot
• Hit in side of knee
• What levels of the spinal cord are affected?L4-S1
• What is a sign of L5 radiculopathy?Weakness of hip abduction and loss of inversion of foot

Question 205

What are clinical features of tibial nerve injuries?

Answer 205

• Inability to invert foot or stand on tiptoe
• Sensory loss on sole of foot
• What levels of the spinal cord are affected?L4-S3

Question 206

What is Guillain-Barre syndrome?

Answer 206

Immune-mediated demyelination of peripheral nervous system often triggered a weeks after an infection (Campylobacter jejuni)

Question 207

What are the symptoms of Guillain-Barré syndrome?

Answer 207

• Back/leg pain initially
• Progressive, symmetrical weakness of all limbs
• Classically ascending weakness of limbs (legs → arms)
• Reflexes are absent

Question 208

What would a lumbar puncture and nerve conduction velocity show?

Answer 208

Rise in protein with normal WCC
Decrease due to demyelination

Question 209

What are radiculopathies?

Answer 209

disorders related to nerve roots, typically manifesting as pain, sensory loss, muscle weakness and diminished reflexes. The pathogenesis of radiculopathies is multifaceted and involves a series of events leading to neuronal dysfunction.

Question 210

What two things cause radiculopathies?

Answer 210

Mechanical Compression: Mechanical forces exerted on the nerve root lead to decreased blood flow and local ischaemia. This results in impaired axonal transport, leading to a build-up of neurotoxic waste products and causing neuronal injury. Subsequent reperfusion leads to oxidative stress exacerbating the injury.
Inflammation: Inflammatory mediators released from degenerated disc material or secondary to systemic conditions like infections or autoimmune diseases can incite an inflammatory response in the nerve root. These mediators include cytokines such as interleukin-1 (IL-1) and tumour necrosis factor-alpha (TNF-alpha), which cause vasodilation, increased vascular permeability and recruitment of inflammatory cells.

The combination of mechanical compression and inflammation leads to oedema and further increases pressure on the nerve root. This vicious cycle perpetuates neuronal damage.

Question 211

What are the sensory symptoms of radiculopathies?

Answer 211

Pain: The hallmark symptom of radiculopathy is radicular pain. This is typically described as sharp, shooting, or electric-like and follows a dermatomal distribution.
Paresthesia: Patients may experience abnormal sensations such as tingling or prickling (pins and needles) in the affected dermatome.
Numbness: Loss of sensation can occur in severe cases where there is significant nerve damage.
Hypersensitivity: Some patients may present with an increased sensitivity to stimuli in the region supplied by the affected nerve root.

Question 212

What are the motor symptoms of radiculopathies?

Answer 212

Muscle Weakness: Depending on which nerve root is compressed, patients may experience weakness in specific muscle groups. For instance, compression of L5 nerve root might result in weakness when trying to raise the big toe and ankle (foot dorsiflexion).
Muscle Atrophy: In chronic cases where there has been ongoing nerve compression, muscle wasting due to lack of innervation can occur.
Fasciculations/Twitching: Spontaneous contractions or twitching of muscles innervated by an irritated nerve root might be observed.

Question 213

What secondary symptoms may patients with radiculopathies present with?

Answer 213

Decreased reflexes: Reflex responses may be diminished or absent in the area served by the affected nerve root.
Gait abnormalities: If lower limb nerves are affected, patients may develop an abnormal gait due to muscle weakness or pain.

Question 214

What is the imaging done for radiculopathies?

Answer 214

Magnetic Resonance Imaging (MRI): This is often the first-line imaging modality for suspected radiculopathy. An MRI provides high-resolution images of the spinal cord, nerve roots, and surrounding structures, enabling direct visualisation of compressive or inflammatory lesions.
Computed Tomography (CT): CT scans are utilised when MRI is contraindicated or unavailable. They provide detailed images of bone structures but are less sensitive in detecting soft tissue abnormalities.
X-ray: While not as detailed as MRI or CT, X-rays can reveal bony abnormalities such as osteophytes or fractures that may be causing nerve compression.

Question 215

Name some other investigations other than imaging one may do for radiculopathies?

Answer 215

Nerve Conduction Studies: These assess the speed at which nerves transmit signals. Slowed conduction suggests damage to myelin sheaths surrounding nerves.
Electromyography: EMG measures electrical activity within muscle fibres, providing insights into how well muscles respond to nerve stimulation. Abnormal findings can suggest nerve root damage.
Serological testing: This can help identify systemic diseases that may cause radiculopathy, such as Lyme disease or vasculitis.
Cerebrospinal fluid (CSF) analysis: In cases of suspected infectious or inflammatory causes of radiculopathy, analysis of CSF obtained via lumbar puncture can provide valuable diagnostic information.

Question 216

Define raised intracranial pressure?

Answer 216

Raised ICP refers to increased pressure within the skull, which can lead to brain damage and death if left untreated

Question 217

What is the Monro-Kellie Doctrine?

Answer 217

the skull is a fixed volume, and any increase in the volume of brain tissue, blood, or cerebrospinal fluid (CSF) must be compensated for by a decrease in another component to maintain normal pressure. Failure to compensate leads to elevated ICP, increasing the risk of coning (herniation of brain tissue) and brainstem death.

Question 218

Name some causes of raised intracranial pressure?

Answer 218

Vascular: Stroke, intracranial hemorrhage (subdural, epidural, subarachnoid).
Infective: Meningitis, encephalitis, brain abscess.
Trauma: Traumatic brain injury.
Autoimmune: Vasculitis.
Metabolic: Hepatic encephalopathy, hypercapnia.
Iatrogenic: Post-surgical or procedural complications.
Neoplastic: Primary or metastatic brain tumors.
Congenital: Hydrocephalus.
Degenerative: Normal pressure hydrocephalus (in elderly patients).
Endocrine: Pituitary adenoma, hypothyroidism (myxedema coma).
Functional: Idiopathic intracranial hypertension (IIH).

Question 219

What are some symptoms of raised intracranial pressure?

Answer 219

Headaches: Worse in the morning, with coughing, straining, or bending over.
Nausea and vomiting: Often early signs of raised ICP.
Visual disturbances: Blurred vision, diplopia, or papilledema (swelling of the optic disc).
Seizures: May occur as ICP rises.
Focal neurological deficits: Varies depending on the underlying cause (e.g., weakness, sensory changes).

Question 220

What are the signs of raised intracranial pressure?

Answer 220

Cranial nerve involvement:
Abducens nerve (CN VI) is most vulnerable due to its long course, leading to lateral gaze palsy.
Third cranial nerve palsy (uncal herniation): Ptosis, fixed dilated pupil, and eye deviation.

Cushing’s triad: A late sign of impending brain herniation, includes:
Bradycardia.
Hypertension.
Irregular breathing.

Question 221

What are the investigations for raised intracranial pressure?

Answer 221

Clinical assessment
History and examination: Look for signs of raised ICP and focal neurological deficits.
Observations: Watch for Cushing’s triad as a sign of impending coning.

Blood tests
Focus on identifying the underlying cause (e.g., infection, metabolic derangements).

Neuroimaging
CT head (CTH): First-line imaging to assess for mass lesions, hemorrhage, or hydrocephalus.
MRI: May be used for further characterization of lesions or if clinical suspicion remains despite normal CT findings.

Question 222

What is the initial management for raised intracranial pressure?

Answer 222

Management begins with a standardized ABCDE approach:
Airway, Breathing, Circulation: Ensure airway patency and adequate oxygenation.
Disability: Assess using the Glasgow Coma Scale (GCS).
Exposure: Check for signs of trauma, infection, or other causes of raised ICP.

Question 223

What is the medical management for raised intracranial pressure?

Answer 223

Seizure management: Detect and treat seizures with anticonvulsants as per the status epilepticus protocol.

Positioning: Elevate the bed to 30-40 degrees to facilitate venous drainage and reduce ICP.

Sedation and airway protection: If GCS < 8, consult an anesthetist for airway management.

Hyperosmolar therapy:
Hypertonic saline is preferred for reducing ICP through osmotic effects.
Mannitol may also be used, but carries risks such as unpredictable pharmacokinetics and potential anaphylaxis.

Control of underlying cause: Treat infection (e.g., antibiotics for meningitis), control metabolic derangements, or manage systemic conditions contributing to raised ICP.

Question 224

What is the surgical management for raised intracranial pressure?

Answer 224

Neurosurgical referral: If the cause is a mass lesion, intracranial hemorrhage, or hydrocephalus, neurosurgical input is required.

Definitive interventions: These may include:
Decompressive craniectomy to relieve pressure.

Ventriculostomy or ventriculoperitoneal (VP) shunt for drainage of CSF in cases of hydrocephalus.

Question 225

What is the monitoring and following of raised intracranial pressure?

Answer 225

Continuous monitoring of GCS and ICP is critical.
Regular neuroimaging may be required to assess the progression of the underlying cause and response to treatment.

Question 226

What is spinal cord compression?

Answer 226

Spinal cord compression (SCC) is a form of myelopathy caused by pressure on the cord by a variety of causes. It causes an upper motor neurone lesion, unlike the lower motor neurone signs seen in cauda equina syndrome, where compression is below the level of L1.

Question 227

What is the leading cause of spinal cord compression in the UK?

Answer 227

Metastatic spinal cord compression (MSCC) is the leading cause in the UK
In around a quarter of patients, MSCC is their first presentation of malignancy

Question 228

What 5 things cause spinal cord compression?

Answer 228

Trauma - typically due to vertebral fractures or dislocation of facet joints; the cord may be severed in significant trauma

Malignancy i.e. MSCC - either due to pathological collapse of vertebrae or compression by growing tumours

Infection including abscess formation and chronic infections such as tuberculosis

Epidural haematoma where blood accumulates in the epidural space, compressing the cord

Intervertebral disc prolapse although this is much more rare than lumbar disc prolapses causing cauda equina syndrome

Question 229

Symptoms of spinal cord compression?

Answer 229

Back pain, which is typically:
Severe
Progressive
Aggravated by straining e.g. coughing
Difficulty walking
Weakness below the level compressed (typically bilateral and symmetrical)
Numbness below the level compressed
Urinary or faecal incontinence
Urinary retention
Constipation

Symptoms and signs of an underlying cause may also be present, e.g. weight loss and fatigue in a patient with MSCC, fevers in a patient with tuberculosis.

Question 230

Signs of spinal cord compression?

Answer 230

Hypertonia
Hyperreflexia (although reflexes may be absent at the level compressed)
Clonus
Upgoing plantars
Sensory loss (a “sensory level”)

Question 231

What is the primary investigation for spinal cord compression?

Answer 231

An MRI whole spine is the key investigation
The whole spine should be imaged as there may be compression at multiple levels
In cases of suspected MSCC, this should be done within 24 hours as per NICE guidelines
Patients with suspected spinal metastases (e.g. back pain) with no neurological signs or symptoms suggestive of MSCC should have their MRI within 1 week
In some cases other imaging modalities may be used, e.g. whole-body CT in the context of major trauma. CT may also be used in patients for whom MRI is contraindicated.

Question 232

What other investigations do we do dependant on signs and symptoms with someone for suspected spinal cord compression?

Answer 232

Do a bladder scan if suspected urinary retention
An ECG and baseline blood tests should be done in anticipation of possible emergency surgical decompression (including a group and save and clotting)
In cases where MSCC is the first presentation of malignancy, further investigations are required to determine where the primary cancer is (guided by history and examination)
This may include further imaging e.g. a CT of the chest, abdomen and pelvis
Bloods may be done for tumour markers e.g. PSA in men
Biopsy is usually required to confirm the diagnosis

Question 233

What is the general management for spinal cord compression?

Answer 233

Management depends on the underlying cause as well as the patient’s background
Patients with traumatic spinal cord injuries should be transferred to a major trauma centre
General principles include:
Immobilise the patient and nurse with spinal precautions (e.g. log-rolling)
Regular repositioning to prevent pressure ulceration in immobile patients
Analgesia for pain
VTE prophylaxis
Catheterise if in urinary retention
Counselling and rehabilitation is key, with multidisciplinary input (e.g. physiotherapy)

Question 234

What is the management for Metastatic spinal cord compression

Answer 234

Start high-dose steroids (usually 16mg dexamethasone initially) in patients with suspected MSCC - this reduces oedema helping to relieve compression
A proton pump inhibitor (PPI) should also be given to prevent peptic ulceration caused by steroids
Blood glucose should be monitored for hyperglycaemia secondary to steroids
Refer to neurosurgery urgently for consideration of surgical decompression (other options include vertebroplasty or kyphoplasty)
Patients unsuitable for surgery may have radiotherapy for spinal metastases - this can also be used as an adjuvant after surgery
Spinal braces may be used in patients not suitable for surgery to help with pain management and spinal stability
Oncology input is key both for diagnosis in patients without a known malignancy and for ongoing management (e.g. chemotherapy)

Question 235

What is cauda equina syndrome?

Answer 235

Cauda equina syndrome (CES) refers to the signs and symptoms that arise from compression of the bundle of nerve roots that extend from the termination of the spinal cord at the L1/L2 level. These nerves supply the pelvis and lower extremities including the parasympathetic supply to the bladder and bowels.

Question 236

What is the main cause of cauda equina syndrome?

Answer 236

The most common cause of cauda equina syndrome is lumbar disc herniation at the L4/L5 or L5/S1 level.

Question 237

What are some other causes of cauda equina syndrome?

Answer 237

Malignancies (either primary or metastatic)
Infection (abscesses, TB)
Trauma including spinal manipulation
Lumbar stenosis
Spondylolisthesis
Congenital causes (e.g. spina bifida)
Epidural haematoma

Question 238

Name some symptoms of cauda equina syndrome?

Answer 238

Lower back pain
Radicular pain - often asymmetrical
Leg weakness
Difficulty walking
Saddle anaesthesia (e.g. unable to feel toilet paper when wiping)
Bowel incontinence or constipation
Urinary incontinence or retention
Erectile dysfunction or sexual dysfunction

Question 239

Signs of a lower motor neurone lesion:

Answer 239

Loss of lower limb power
Hypotonia in the lower limbs
Hyporeflexia in the lower limbs
Sensory loss or paraesthesias in the legs
Reduced perianal sensation
Loss of anal tone
The bladder may be palpable and dull to percussion in urinary retention

Question 240

What are the key investigations for cauda equina syndrome?

Answer 240

The key investigation is an urgent whole spine MRI (to be done within 24 hours of presentation)
This aims to confirm the diagnosis, localise the compression and investigate for a cause
CT myelography is an alternative for patients who are unable to have an MRI
A bladder scan may be required if urinary retention is suspected
Patients should have baseline investigations to prepare them for possible surgery (e.g. an ECG, bloods including a clotting screen and group and save)
Other investigations may be indicated depending on the suspected underlying cause, e.g. further imaging and biopsy if malignancy is suspected, inflammatory markers and blood cultures if infection is suspected)

Question 241

What is the management for cauda equina syndrome?

Answer 241

An emergency neurosurgical or spinal referral should be made (depending on local pathways) for consideration of surgical decompression, ideally within 24 hours of presentation
Keep the patient nil by mouth
Patients in urinary retention should be catheterised
Analgesia for back and radicular pain
Patients with malignant CES should also be started on steroids (usually 16 mg dexamethasone daily in divided doses with PPI cover)
Additional treatments may be indicated based on the cause of CES (e.g. antibiotics if the compression is due to an abscess)
VTE prophylaxis is important due to immobility (although anticoagulation should not be started in patients going for surgery due to bleeding risk)

Question 242

What are the complications for cauda equina syndrome?

Answer 242

Permanent paralysis of the lower limbs
Sensory loss
Bladder and bowel dysfunction
Sexual dysfunction
Pressure ulcers
Venous thromboembolism due to immobility

Question 243

Define a subarachnoid haemorrhage?

Answer 243

A Subarachnoid Haemorrhage (SAH) occurs when blood accumulates in the subarachnoid space, typically due to the rupture of an aneurysm or trauma.

Question 244

What is the incidence and prevalence of subarachnoid haemorrhages?

Answer 244

Incidence: SAH occurs in approximately 6-9 per 100,000 people annually.

Prevalence: The majority of non-traumatic SAH cases (~80%) are caused by berry aneurysms, most commonly on the posterior communicating artery.

Question 245

How can subarachnoid haemorrhages be classified?

Answer 245

Traumatic and non-traumatic haemorrhages

Question 246

Explain non-traumatic subarachnoid haemorrhages:

Answer 246

The most common cause is a ruptured berry aneurysm.
Other causes include: - Arteriovenous malformations (AVMs) - Arterial dissection - Cerebral amyloid angiopathy.

Question 247

Explain traumatic subarachnoid haemorrhages:

Answer 247

Typically caused by head injuries, where blood leaks into the subarachnoid space following trauma.

Question 248

What are the signs and symptoms of subarachnoid haemorrhages:

Answer 248

Thunderclap headache: A sudden, severe headache that reaches maximum intensity in less than 5 minutes.
Often described as “being hit on the back of the head,” commonly with occipital headache localization.
Seizures
Reduced consciousness or loss of consciousness.
Meningism: Neck stiffness and photophobia.
Neurological signs: Falsely localizing signs such as abducens nerve palsy, due to increased intracranial pressure affecting the longest cranial nerve.
Vomiting
Signs of raised intracranial pressure

Question 249

What are the investigations for subarachnoid haemorrhages?

Answer 249

CT head without contrast: The first-line investigation, especially within the first 6 hours of symptom onset, is highly sensitive in detecting SAH.
Lumbar puncture: Used to assess for xanthochromia, a yellow discoloration in the cerebrospinal fluid indicating the breakdown of red blood cells.
Recent NICE guidelines state that if the CT head is done within 6 hours of headache onset and shows no SAH, alternative diagnoses should be considered.
If a CT Head is performed more than 6 hours after symptom onset and the result is negative, then a lumbar puncture can be considered at least 12 hours after symptom onset
CT angiography: To detect any aneurysms or vascular malformations if SAH is confirmed or suspected.

Question 250

A map of the investigations for subarachnoid haemorrhages:

Answer 250

Question 251

What is the medical management for subarachnoid haemorrhages?

Answer 251

Nimodipine is used to prevent delayed cerebral ischemia caused by vasospasm, which can occur after SAH.
Blood pressure management: Hypertension is not aggressively treated unless necessary to reduce the risk of rebleeding.

Question 252

What is the radiological management for subarachnoid haemorrhages?

Answer 252

Endovascular coiling is often preferred over surgical clipping due to better outcomes, but the choice depends on the aneurysm’s location and characteristics.

Clipping may still be performed, particularly for aneurysms that are unsuitable for coiling.

Question 253

What is the prognosis for subarachnoid haemorrhages?

Answer 253

Mortality is around 50% overall, though many of these deaths occur before patients reach the hospital.
Among patients with a Glasgow Coma Scale (GCS) score of >14 on admission, the survival rate is greater than 90%, with low morbidity.

Question 254

what is an Aneurysmal rebleed?

Answer 254

Burst aneurysms account for around 85% of non-traumatic subarachnoid haemorrhages and rebleeding is an early complication with the majority occurring within 12 hours of the initial bleed. Aneursymal rebleeding carries very high morbidity and mortality rates and should be suspected in any patient with a sudden worsening of neurological symptoms after initial presentation.

Question 255

Name an important condition complication of Subarachnoid haemorrhage?

Answer 255

Hydrocephalus
Clotted blood and CSF blockage can cause fluid build-up in the subarachnoid space leading to hydrocephalus. This serious complication usually requires shunting to alleviate increased intracranial pressure.

Question 256

Name a risk factor for non-traumtic subarachnoid haemorrhages?

Answer 256

Risk factors include hypertension (HTN), smoking, excessive alcohol consumption, and adult polycystic kidney disease (ADPCKD), with the latter being a risk factor due to the formation of berry aneurysms.

Question 257

Management for intracranial haemorrhages in patients on Warfarin

Answer 257

Intracranial haemorrhages in patients on Warfarin should be managed by immediate cessation of Warfarin, followed by administration of Vitamin K and Prothrombin complex concentrate to facilitate haemostasis.

Question 258

Define a subdural haemorrhage?

Answer 258

A subdural haematoma is characterised by the accumulation of venous blood in the potential space between the dura mater and arachnoid mater of the brain.

Question 259

What causes subdural haemorrhages:

Answer 259

Subdural haematomas typically occur in elderly individuals, particularly those over 65 years of age. It is often a consequence of minor trauma, leading to shearing forces that tear bridging veins between the cortex and dura mater. These forces commonly arise from minor head traumas but can also occur spontaneously in patients with bleeding disorders, anticoagulant therapy, or chronic alcohol use.

Question 260

Risk factors for subdural haemorrhages:

Answer 260

Advancing age (>65 years old)
Bleeding disorders or anticoagulant therapy
Chronic alcohol use
Trauma.

Question 261

What are the signs and symptoms of subdural haemorrhages:

Answer 261

Clinical presentation of a subdural haematoma varies but may include:
Headache
Nausea or vomiting
Confusion
Fluctuating GCS
Behavioural change.

Question 262

What is the main investigation for subdural haemorrhages?

Answer 262

CT scan

Question 263

Describe how a subdural haemorrhage clot changes on CT over time:

Answer 263

Hyperacute phase (<1 hour): The clot may appear isodense, with underlying cerebral oedema.
Acute phase (<3 days): The clot appears as a crescent-shaped hyperdense extra-axial collection over the affected hemisphere.
Sub-acute phase (3 days to 3 weeks): The clot appears more isodense compared to the adjacent cortex, making identification more difficult. Contrast-enhanced CT or MRI can aid identification. There may be associated mass effect causing midline shift and sulcal effacement.
Chronic phase (>3 weeks): The haematoma appears hypodense relative to the adjacent cortex.

Question 264

What are the further investigations one can do for sub-dural haemorrhage?

Answer 264

Further investigations may include:
Routine blood tests including FBC, Renal Profile, Liver Function Tests
Clotting profile (to assess for coagulopathy)

Question 265

What is the management for subdural haemorrhage?

Answer 265

Management of a subdural haematoma depends on the stage, patient’s premorbid baseline, and functional status. Many cases are managed conservatively, especially if there is no midline shift or cerebral oedema. However, for more severe cases, neurosurgical referral is required.

Conservative management: If no significant midline shift or cerebral oedema.

Surgical management: In cases where intervention is necessary, options include:
Craniotomy: Typically for acute haemorrhages.
Burr holes: Typically for chronic haemorrhages.

Question 266

Define a tension type headache:

Answer 266

Tension-type headache is the most common primary headache disorder, which is not associated with another underlying condition (which would be a secondary headache).

Question 267

How does The International Classification of Headache Disorders (ICHD) categorise tension-type headache?

Answer 267

according to the frequency of attacks:
Infrequent episodic — less than 1 day of headache per month (usually self-limiting).
Frequent episodic — at least 10 episodes of headache occurring on fewer than 15 days per month on average, for more than 3 months.
Chronic — this evolves from frequent episodic attacks, with 15 days or more of headache per month, for more than 3 months, in the absence of medication overuse.

Question 268

What are the key distinguishing features of tension type headaches?

Answer 268

Bilateral, non-pulsatile pain: Unlike migraines, which are often unilateral and throbbing, tension headaches typically involve a dull, aching pain that affects both sides of the head.
Tightness sensation: Described as a constant pressure, often likened to having a tight band or vice around the head.
Scalp muscle tenderness: A hallmark of tension headaches, often identified when the scalp, neck, or shoulder muscles are palpated.
Patients do not typically experience nausea, vomiting, or sensitivity to light and sound, which are more common in migraine sufferers.

Question 269

What is the management of tension type headaches?

Answer 269

Effective management of tension headaches often involves a combination of pharmacological and non-pharmacological strategies:
Analgesia: Treatment often follows the WHO pain ladder, starting with over-the-counter medications like Paracetamol or non-steroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen. In cases where these are insufficient, stronger medications or combination therapies may be considered under the GP’s guidance.
Stress management: Since tension headaches are frequently linked to stress, addressing lifestyle factors, such as stress reduction techniques, regular physical activity, and improved posture, plays a critical role. Cognitive behavioural therapy (CBT) and relaxation exercises may be helpful for chronic sufferers.
Physical therapy: Techniques such as massage or muscle relaxation exercises can alleviate scalp and neck muscle tension that often accompanies tension headaches.

Question 270

Define transient ischaemic attack:

Answer 270

A Transient Ischaemic Attack (TIA), colloquially known as a “mini-stroke”, represents a sudden focal neurological deficit of vascular origin, characterised by symptom resolution typically within an hour. The absence of an acute infarct on imaging differentiates it from a stroke

Question 271

What are the risk factors for transient ischaemic attack?

Answer 271

Hypertension
Diabetes mellitus
High cholesterol
Atrial fibrillation
Carotid stenosis
Smoking
Family history of cardiovascular disease/stroke
History of cardio-embolic events

Question 272

What are the signs and symptoms of transient iscahemic attacks?

Answer 272

Patients typically present with a sudden onset of focal neurological deficits which may include:
Speech difficulty (dysphasia)
Arm or leg weakness
Sensory changes
Ataxia, vertigo or loss of balance
Visual disturbance: Homonymous hemianopia, diplopia
Symptoms of TIA are transient, with most resolving within 1 hour.

Question 273

What are the investigations for transient ischaemic attacks?

Answer 273

Neuroimaging
The preferred modality is MRI to assess for any evidence of ischaemia, haemorrhage or consider alternative pathologies
Carotid ultrasound (looking for carotid stenosis
Echocardiogram (looking for cardiac thrombous)
24 hour tape (looking for atrial fibrillation)
Blood tests (including glucose, lipid profile, clotting factors)

Question 274

What is the management for a transient ischaemic attack?

Answer 274

ideally to be seen within 24 hours of onset of symptoms. The aim of management is to reduce the future risk of stroke and includes:
Lifestyle modifications (smoking cessation, regular exercise, healthy diet)
Control of vascular risk factors (hypertension, diabetes, dyslipidaemia)
Initiation of antiplatelet therapy (e.g., aspirin, clopidogrel) unless contraindicated - Aspirin should be given immediately for a suspected TIA unless contraindicated 300mg
In selected cases, endarterectomy or stenting of the carotid artery might be indicated:
70% stenosis according European Carotid Surgery Trialists’ Collaborative Group criteria (ECST) or
50% according to North American Symptomatic Carotid Endarterectomy Trial criteria (NASCT)
When a carotid artery is totally occluded, carotid revascularisation is not indicated.

Question 275

Define trigeminal neuralgia?

Answer 275

Trigeminal neuralgia is a chronic pain condition characterized by severe, sudden, and brief bouts of shooting or stabbing pain that follow the distribution of one or more divisions of the trigeminal nerve, affecting the patient’s facial region.

Question 276

How can trigeminal neuralgia be classified?

Answer 276

Trigeminal neuralgia can be classified as either primary (idiopathic) or secondary, with the latter being associated with identifiable etiological factors

Question 277

What are some known causes of trigeminal neuralgia?

Answer 277

Known causes include:
Malignancy: can lead to nerve compression or infiltration, resulting in pain
Arteriovenous malformation: abnormal, tangled blood vessels can compress the trigeminal nerve
Multiple sclerosis: demyelination in this condition can affect the trigeminal nerve
Sarcoidosis: granulomatous lesions can affect the trigeminal nerve
Lyme disease: infection and subsequent inflammation can affect the trigeminal nerve

Question 278

What are the signs and symptoms of trigeminal neuralgia?

Answer 278

Unilateral facial pain that is sudden, severe, and brief.
The pain is often described as shooting or stabbing,
Triggered by lightly touching the affected side of the face, eating, or wind blowing on the face.

Question 279

What are the investigations for trigeminal neuralgia?

Answer 279

Trigeminal neuralgia is largely a clinical diagnosis, but investigations may be performed to rule out other causes of facial pain.
Neuroimaging such as MRI can be used to exclude secondary causes, including tumours or vascular compression.

Question 280

What is the medical management of trigeminal neuralgia?

Answer 280

Medical management typically includes;
Carbamazepine (first-line treatment)
Phenytoin
Lamotrigine
Gabapentin

Question 281

What is the surgical management for trigeminal neuralgia?

Answer 281

Microvascular decompression: a procedure to remove or relocate blood vessels that are in contact with the trigeminal root
Treatment of the underlying cause: such as removing a tumour or addressing an arteriovenous malformation
Alcohol or glycerol injections: used to damage the trigeminal nerve and reduce pain signals

Question 282

What’s an important consideration of people on carbmazepine?

Answer 282

Carbamazepine is an enzyme inducer, meaning it accelerates the metabolism of other drugs, including hormonal contraceptives like the combined oral contraceptive pill (COCP). This interaction decreases the effectiveness of the contraceptive, increasing the risk of unintended pregnancy. Patients on carbamazepine should be advised to use alternative or additional forms of contraception, such as barrier methods, or consider switching to a non-enzyme-inducing contraceptive method.

Question 283

Define wernicke’s encephalopathy?

Answer 283

Wernicke’s encephalopathy is a neurological disorder caused by thiamine (vitamin B1) deficiency, manifesting in a triad of specific clinical symptoms: ataxia, confusion, and ocular abnormalities.

Question 284

Which certain populations is wernicke’s encephalopathy higher in?

Answer 284

The condition exhibits a higher prevalence in certain populations: 12.5% in individuals with a history of alcohol dependence, 10% in those with AIDS, and 6% in bone marrow transplant recipients.

Question 285

What are the primary causes of Wernicke’s encephalopathy?

Answer 285

Chronic alcohol abuse: Alcohol interferes with thiamine absorption and utilization.
Malnutrition: This can occur due to inadequate dietary intake, malabsorption disorders, or increased requirements.
Bariatric surgery: Rapid weight loss and reduced nutrient absorption can lead to thiamine deficiency.
Hyperemesis gravidarum: Persistent severe vomiting in pregnancy may lead to nutrient deficiencies, including thiamine.

Question 286

What is the characteristic triad of symptoms in Wernicke’s encephalopathy

Answer 286

a characteristic triad of symptoms:
Ataxia: Unsteady and uncoordinated movements
Confusion: Disorientation and difficulty with attention
Ocular abnormalities: This can include gaze-evoked nystagmus, spontaneous upbeat nystagmus, and horizontal or vertical ophthalmoplegia.

Question 287

What are the investigations for wernicke’s encephalopathy?

Answer 287

To confirm a diagnosis of Wernicke’s encephalopathy, the following investigations may be undertaken:
Neurological examination: Assessment of the characteristic triad of symptoms.
MRI Head: May show characteristic changes in specific brain regions, such as the mammillary bodies and periaqueductal area.
Blood tests: Although not definitive, they can reveal low thiamine levels and other signs of malnutrition or alcohol abuse.

Question 288

Define an ischaemic stroke:

Answer 288

An ischaemic stroke describes a sudden onset focal neurological deficit secondary to focal brain ischaemia, with symptoms lasting >24 hours (or with evidence of infarction on imaging).

Question 289

What causes an ischaemic stroke?

Answer 289

Ischaemic stroke occurs when blood supply in a cerebral vascular territory is reduced secondary to stenosis or complete occlusion of a cerebral artery.

Question 290

What is an ischaemic penumbra?

Answer 290

describes the cerebral area surrounding the ischaemic event where there is ischaemia without necrosis. This area is amenable to recovery with thrombolysis.

Question 291

What are the different ischaemic strokes caused by?

Answer 291

25% are caused by intracranial small vessel atherosclerosis.
50% are caused by large vessel atherosclerosis e.g. carotid artery stenosis.
Typically results from thrombus formation on the atherosclerotic plaque, and subsequent embolism of the thrombus to a smaller cerebral artery.
20% of ischaemic strokes are cardio-embolic
e.g. in atrial fibrillation there is stasis of blood flow in the left atrium, predisposing to thrombus formation in the left atrium, and subsequent embolisation to the brain.
Rare causes of ischaemic stroke include primary vascular causes (such as vasculitis and arterial dissection) and haematological causes (prothrombotic states).

Question 292

Name some stroke factors:

Answer 292

Age
Male Sex
Family History
Hypertension
Smoking
Diabetes
Atrial fibrillation
High cholesterol
Obesity
Migraine

Question 293

What is the most common classification for stroke?

Answer 293

Bamford/Oxford Stroke Classification System

Question 294

A total anterior circulation infarct (TACI) is defined by:

Answer 294

Contralateral hemiplegia or hemiparesis, AND
Contralateral homonymous hemianopia, AND
Higher cerebral dysfunction (e.g. aphasia, neglect) A TACI involves the anterior AND middle cerebral arteries on the affected side.

Question 295

A partial anterior circulation infarct (PACI) is defined by:

Answer 295

2 of the above, OR
Higher cerebral dysfunction alone.
A PACI involves the anterior OR middle cerebral artery on the affected side.

Question 296

A lacunar infarct (LACI) is defined by

Answer 296

a pure motor stroke, pure sensory stroke, sensorimotor stroke, ataxic hemiparesis or dysarthria-clumsy hand syndrome. A LACI affects small deep perforating arteries, typically supplying internal capsule or thalamus.

dysarthria-clumsy hand syndrome’, a lacunar syndrome caused by infarct to the genu (basis pontis) of the internal capsule. This syndrome is characterised by dysarthria and contralateral weakness of the hand.

Question 297

A posterior circulation infarct (POCI) is defined by:

Answer 297

Cerebellar dysfunction, OR
Conjugate eye movement disorder, OR
Bilateral motor/sensory deficit, OR
Ipsilateral cranial nerve palsy with contralateral motor/sensory deficit, OR
Cortical blindness/isolated hemianopia.

Treatment with alteplase if within 4.5hrs
A POCI involves the vertebrobasilar arteries and associated branches (supplying the cerebellum, brainstem, and occipital lobe).

Question 298

Basilar artery occlusion is more likely to present with ?

Answer 298

locked in syndrome (quadriparesis with preserved consciousness and ocular movements), loss of consciousness, or sudden death.

Question 299

Anterior inferior cerebellar artery results in lateral pontine syndrome?

Answer 299

a condition similar to the lateral medullary syndrome but with additional involvement of pontine cranial nerve nuclei. It leads to cerebellar ataxia, vertigo, hearing loss as well as ipsilateral facial weakness

Question 300

Wallenberg’s syndrome (lateral medullary syndrome) causes:

Answer 300

Vestibulocerebellar symptoms: vertigo, falling to the side of the lesion,
multidirectional nystagmus, and diplopia

Autonomic dysfunction: ipsilateral Horner’s syndrome, hiccups

Sensory symptoms: loss of pain and temperature sensation over the ipsilateral face, loss of pain and temperature sensation over the contralateral side of the body (this is due to the involvement of the spinal trigeminal nucleus)

Ipsilateral bulbar muscle weakness: hoarseness, dysphagia, dysphonia, dysarthria.

Question 301

Weber’s syndrome/medial midbrain syndrome (paramedian branches of the upper basilar and proximal posterior cerebral arteries): causes

Answer 301

an ipsilateral oculomotor nerve palsy and contralateral hemiparesis.

Question 302

What is the management of acute ischaemic stroke?

Answer 302

Patients should be approached in the DR ABCDE manner.

CT Head should be perfomed on arrival to the emergency department to distinguish ischaemic from haemorrhagic stroke.
If no evidence of ischaemic stroke, a CT

Angiogram and CT perfusion (in select patients) should be performed to assess for evidence of large vessel occlusion and a salvageable ischaemic penumbra

Question 303

What medication do we consider in acute ischaemic stroke?

Answer 303

Thrombolysis with Alteplase (tissue plasminogen activator) is considered if:
The patient presents within 4.5 hours of symptom onset (Up to 9 hours in select patients with good baseline and favourable perfusion imaging)
No contraindications such as:
Recent head trauma
Recent surgery
Systolic blood Pressure above 185
Currently taking oral anticoagulation
Raised INR (local guidelines vary)

If hyper-acute treatments are offered, antiplatelets are usually started 24 hours after the treatment following a repeat CT Head that excludes any haemorrhagic transformation.

Question 304

What surgical procedure is considered in acute ischaemic stroke?

Answer 304

Mechanical Thrombectomy is usually considered in patients with:
Anterior Circulation Stroke (evidence base is poorer for posterior circulation stroke)
Evidence of large vessel occlusion (ideally proximal up to distal M1)
Good functional baseline
Favourable perfusion criteria indicating salvageable tissue
Presenting within 6 hours (Up to 24 hours in select patients with good baseline and favourable perfusion imaging)

Question 305

If hyper-acute treatments are not offered for ischaemic stroke, patients are started on ?

Answer 305

an antiplatelet agent such as Aspirin or Clopidogrel (local guidelines vary)

Question 306

What are the ischaemic stroke post acute investigations?

Answer 306

MRI Head with Diffusion Weighted Imaging (DWI) is the gold standard test to confirm the presence of an acute ischaemic stroke, which can be present within a few minutes of stroke onset. Due to logistical challenges of acute MRI scanning, this is normally performed within 24 hours following initial hyperacute treatment.

Investigations in the post-hyperacute phase aim to further define the cause of the stroke and to quantify vascular risk factors.

These include:
Carotid ultrasound (to identify critical carotid artery stenosis)
24 to 72 hour cardiac monitoring to assess for evidence of atrial fibrillation
CT/MR angiography (to identify intracranial and extracranial stenosis)
Echocardiogram (if a cardio-embolic source is suspected).
In young patients further investigation e.g. a vasculitis screen or thrombophilia screen may be necessary.
HbA1c and Serum Lipids to optimise other cardiovascular risk factors

Question 307

What is chronic stroke management?

Answer 307

Rehabilitation and supportive management will include an MDT approach with involvement of physiotherapy, occupational therapy, speech and language therapy, and neurorehabiliation.

The key steps in secondary stroke prevention can be remembered by the mnemonic HALTSS:

Hypertension:
Studies show there is no benefit in lowering the blood pressure acutely (as this may impair cerebral perfusion) unless there is malignant hypertension (systolic blood pressure >180 mmHg). Anti-hypertensive therapy should, however, be initiated 2 weeks post-stroke.

Antiplatelet therapy:
Patients should be administered Clopidogrel 75 mg once daily for long-term antiplatelet therapy. In patients with ischaemic stroke secondary to atrial fibrillation, however, warfarin (target INR 2-3. or a direct oral anticoagulant (such as Rivaroxaban or Apixiban) is initiated 2 weeks post-stroke.

Lipid-lowering therapy:
Patients should be prescribed high dose atorvastatin 20-80 mg once nightly (irrespective of cholesterol level this lowers the risk of repeat stroke).

Tobacco
Offer smoking cessation support.

Sugar:
Patients should be screened for diabetes and managed appropriately.

Surgery:
Patients with ipsilateral carotid artery stenosis more than 50% should be referred for carotid endarterectomy. Patients with over 70% stenosis have the most benefit from endarterectomy

Question 308

What is the rule of 4 for cortical strokes?

Answer 308

4 motor syndromes are midline:
- Medial Longitudinal fascicles (eye motor)
-Motor Tract of the UMN (corticospinal tract)
-Medial meniscus (proprioception/vibration)
-Motor nuclei of CN

4 sensory syndromes are side (lateral)
- spinothalamic tract (pain + temp)
- spinocerebellar tract (RAM)
-sympathetic chain (dilation, sweating)
- sensory CN nuclei

Cranial nerve lesions are ipsilateral, except trochlear

Question 309

Which blood vessels supply different part of the brainstem?

Answer 309

Midbrain = PCA
Lateral Pons = AICA
Medial pons = Basilar
Lateral medulla = PICA
Medial medulla = ASA

Question 310

Broca area’s is located in which part of the brain?

Answer 310

inferior frontal gyrus

Question 311

What is conduction aphasia?

Answer 311

This patient has presented with a conduction aphasia, demonstrated by their inability to repeat words and incorrect substitution. The arcuate fasciculus is most commonly affected in such aphasias as it connects Wernicke’s and Broca’s areas.

Question 312

If a patient comes in with a stroke and indigestion what medication do we prescribe?

Answer 312

patient has severe dyspepsia; therefore, aspirin is contraindicated. Clopidogrel has been appropriately commenced.
The patient is also on omeprazole for severe dyspepsia. Omeprazole reduces the antiplatelet function of clopidogrel, rendering it clinically less effective.
Lansoprazole is a PPI which does not have a drug interaction with clopidogrel. It is safe to use for the management of severe oesophagitis.

Question 313

A 54 year old man presents with a dense left hemiplegia, left facial droop, left gaze palsy and left homonymous hemianopia. He is neglecting the left side on examination. CT head on admission showed a right middle cerebral artery (MCA) infarct. His Glasgow coma scale (GCS) drops from E3V4M6 TO E2V2M5. Repeat CT head showed a massive infarction affecting 70% of the right MCA territory with evidence of midline shift.
Given the likely diagnosis, what intervention should this patient be considered for?

Answer 313

In young stroke patients with severe symptoms, extended infarcts affecting greater than 50% of the middle cerebral artery territory, and reduced consciousness, decompressive hemicraniectomy can increase survival rates despite minimal improvement in disability.

Question 314

Acronym for CEREBELLAR signs of stroke

Answer 314

Dysdiachokinesia, Ataxia, Nystagmus, Intention tremor, Slurred speech, Hypotonia

Question 315

In individuals with embolic infarcts confined to one hemisphere of the brain, carotid artery stenosis is a common causative factor.

Answer 315

Carotid artery stenosis leads to showers of embolic infarcts arranged in this way. This is because, in the majority of people, the left internal carotid artery (ICA) supplies the left hemisphere of the brain, and the right ICA supplies the right. Initially, carotid artery dopplers will show the stenosis, with subsequent CT or MR angiography to confirm the degree. Intervention may be offered in the form of carotid stenting or endarterectomy, depending on the functional status of the patient.

Question 316

Define a haemorrhagic stroke?

Answer 316

Haemorrhagic stroke is a cerebrovascular event that occurs when there is bleeding into the brain parenchyma, ventricles or subarachnoid space. This may lead to haematoma formation with raised intracranial pressure, as well as neuronal damage.

Question 317

Risk factors for haemorrhagic stroke?

Answer 317

Older age
Male sex
Family history of haemorrhagic stroke
Haemophilia and other bleeding disorders
Cerebral amyloid angiopathy (CAA)
Hypertension
Anticoagulation therapy
Recreational drugs such as cocaine and amphetamines
Arteriovenous malformations (particularly in younger patients)

Question 318

Signs and symptoms for hemorrhagic stroke?

Answer 318

Severe headache
Altered consciousness, ranging from drowsiness to coma
Vomiting
Nuchal rigidity
Focal neurologic signs e.g. weakness, visual loss, sensory changes
Aphasia
Seizures
Hypertension

Question 319

What bedside tests for hemorrhagic stroke?

Answer 319

Capillary blood glucose as maintaining normoglycaemia improves outcomes
Blood pressure is important to monitor as a potential cause and during the management blood pressure control is key
ECG as there may be cardiac complications of haemorrhagic stroke e.g. arrhythmias
Urine toxicology screen if illicit drug use is suspected

Question 320

What blood tests for hemorrhagic stroke?

Answer 320

FBC and CRP for inflammatory markers
Coagulation screen to identify any bleeding diathesis
U&Es to identify any renal impairment that may be associated with hypertension
LFTs as a baseline

Question 321

What imaging for hemorrhagic stoke?

Answer 321

Non Contrast CT head is the key investigation to identify haemorrhagic stroke
MRI head may be done in some cases to give more detail, identify an underlying cause and plan treatment
MR angiography or digital subtraction angiography can help identify any vascular abnormalities that may have caused the haemorrhage

Question 322

What is the management for haemorrhagic strokes?

Answer 322

Emergency management of a haemorrhagic stroke should involve urgently contacting the stroke team +/- critical care (for example in cases with reduced consciousness)
Urgent neurosurgical referral may be required e.g. for haematoma evacuation
Reverse any anticoagulation treatment or clotting disorders
Rapid blood pressure lowering should be initiated in patients with a systolic blood pressure of 150-220 mmHg
This is usually with IV antihypertensives such as GTN or labetalol
Aim for a systolic blood pressure of below 140 mmHg, but without a fall of > 60 mmHg in the first hour of treatment
This excludes patients with an underlying structural cause (e.g. an aneurysm), a GCS below 6 and those who are going for early neurosurgery
Make patients nil by mouth until they have had a swallowing assessment
Assess hydration and give fluids if dry
Management of subarachnoid haemorrhage and raised intracranial pressure is addressed in a separate chapter

Question 323

What are the complications of hemorrhagic stroke?

Answer 323

Decreased mobility e.g. hemiparesis or hemiplegia
Seizures
Delirium
Hydrocephalus due to bleeding into the ventricles
Cardiac complications e.g. arrhythmias
Recurrence of haemorrhagic stroke
Increased risk of infection e.g. aspiration pneumonia
Increased risk of falls in the longer-term
Incontinence
Neuropathic or musculoskeletal pain
Fatigue
Cognitive decline

Question 324

Headache red flags:

Answer 324

type on onset: abrupt and severe
temporal: progressively severe or increasing frequency
pattern: significant change
neurological signs: meningsm, focal signs, confusion, altered LOC
systemic: abdominal examinations, fever, weight loss
triggers: posture, valsalvar, coughing, exertion
secondary risk factors: systemic disease, cancer, HIV, 3rd trimester pregnancy, post party, recent head injury

Question 325

From 12hrs up to 2 weeks xanthochromia is a reliable test for a SAH by doing a lumbar puncture.

Answer 325

:(

Question 326

Results from a lumbar puncture in meningitis:

Answer 326