NEURO - clinical Flashcards
(326 cards)
1
Q
What sign on a CT head can sometimes be seen which indicates an ischaemic stroke?
A
Dense MCA sign –> visible immediately, shows the responsible arterial clot
2
Q
Cerebellar stroke symptoms
A
- Problems with moving/walking –> cerebellum is part of brain responsible for balance
- Vertigo –> ‘room spinning’
- muscle weakness or tremors
- headache
- nausea and vomiting
- hearing and vision problems
3
Q
Principles of management for an intracranial bleed
A
- Immediate CT head to establish diagnosis
- Bloods: FBC (for platelets) and a coagulation screen
.
1. Admission to a specialist stroke centre/neurosurgery - If GCS decreased –> consider intubation, ventialtion, and intensive care
- Address any bleeding risk –> reversal agents for anticoagulation, platelet transfusions
- Correct severe hypertension, but avoid hypotension
- Surgical options –> craniotomy (section of skull removed), Burr holes (small holes drilled into skull to drain blood)
4
Q
Reversal agents for:
- Warfarin
- Heparin/LMWH
- Apixaban, edoxaban, and rivaroxaban
- Dabigatran
A
- Warfarin –> PCC (prothrombin complex concentrate) for rapid reversal + Vitamin K
- Heparin/LMWH –> protamine sulfate
- Apixaban, edoxaban, and rivaroxaban –> andexanet alfa
- Dabigatran –> idarucizunab
5
Q
For patient’s on anticoagulant therapy (eg. Warfarin), what is the INR target range? + what value indicates a risk of bleeding?
A
- 2.0-3.0
- > 4.9 is high risk of bleeding
6
Q
Investigations for a suspected subarachnoid haemorrhage + what investigation would you do after confirming the diagnosis to locate the source of the bleeding?
A
- non-contrast CT head (1st-line) - needs to be done < 6hrs onset of symptoms, otherwise less reliable
- Lumbar puncture (wait at least 12hrs after onset of symptoms) –> raised RBC + xanthochromia
. - CT angiography - used after confirming diagnosis to locate the source of the bleeding
7
Q
When investigating a SAH, why should a lumbar puncture be performed at least 12hrs after onset?
A
to allow for Xanthochromia to form –> RBC breakdown (bilirubin)
8
Q
Subarachnoid haemorrhage - Acute management
A
- Refer to neurosurgery –> endovascular coiling OR neurosurgical clipping to repair aneurysm
- Nimodipine (Ca channel blocker) –> used to prevent vasospasm (a common complication following a SAH, which can result in brain ischaemia)
- Supportive: analgesia +/- address any bleeding risks
9
Q
Vasospasm is a common complication following a subarachnoid haemorrhage, name two other complications that can occur
A
- Hydrocephalus –> treatment options include lumbar puncture, external ventricular drain, or a ventriculoperitoneal (VP) shunt
- Seizures –> treated with anti-epileptic drugs
10
Q
What is multiple sclerosis (MS)?
A
a chronic and progressive autoimmune condition involving demyelination in the central nervous system (oligodendrocytes), where the immune system attacks the myelin sheath of neurones
11
Q
Who is most commonly affected by MS?
A
Young adults (under 50 years), with a higher prevalence in women
12
Q
What is the role of myelin in the nervous system + which cells provide myelin in the CNS and the PNS?
A
Myelin covers the axons of neurones and helps electrical impulses travel faster
.
- CNS –> Oligodendrocytes
- PNS –> Schwann cells
13
Q
What happens in MS at a cellular level?
A
Inflammation and immune cell infiltration cause damage to the myelin in the CNS, affecting the electrical signals moving along the neurones
14
Q
What is a characteristic feature of MS lesions?
A
They are “disseminated in time and space” –> meaning damage caused by the disease is spread across different areas of the CNS (brain and spinal cord) at different points in time
15
Q
In multiple sclerosis, what happens to myelin in early VS late disease?
A
- Early disease –> remyelination can occur, leading to symptom resolution
- Late disease –> remyelination is incomplete and symptoms become more permanent
16
Q
What is the most common initial presentation of multiple sclerosis (MS) + features
A
Optic neuritis
- central scotoma
- pain with eye movement
- impaired colour vision
- relative afferent pupillary defect (RAPD)
17
Q
Describe a relative afferent pupillary defect (RAPD)
A
where the pupil in the affected eye constricts more when shining a light in the contralateral eye than when shining it in the affected eye
18
Q
How is optic neuritis managed?
A
Urgent ophthalmology input and high-dose steroids
19
Q
What cranial nerves are affected in MS leading to diplopia (double vision) and nystagmus?
A
CN III (oculomotor), CN IV (trochlear), and CN VI (abducens)
20
Q
What is internuclear ophthalmoplegia?
A
A lesion in the medial longitudinal fasciculus causing impaired adduction in one eye and nystagmus in the contralateral abducting eye
- the nerve fibres of the medial longitudinal fasciculus connect the cranial nerve nuclei (“internuclear”) that control eye movements (3rd, 4th, and 6th cranial nerve nuclei)
21
Q
What is a conjugate lateral gaze disorder?
A
A lesion in CN VI (abducens) causing failure of the affected eye to abduct when looking laterally
- eg. in a lesion involving the left eye, when looking to the left, the right eye will adduct (move towards the nose), and the left eye will remain in the middle
22
Q
What focal motor symptoms can MS present with + what focal sensory symptoms can MS present with?
A
Focal motor symptoms –> limb paralysis, incontinence, Horner’s syndrome, facial nerve palsy
.
Focal sensory symptoms –> trigeminal neuralgia, numbness, paraesthesia (pins and needles), Lhermitte’s sign
23
Q
What is Lhermitte’s sign (a feature of MS)?
A
an electric shock sensation that travels down the spine and into the limbs when flexing the neck –> indicates disease in the cervical spinal cord in the dorsal column
24
Q
What is transverse myelitis?
A
a site of inflammation in the spinal cord, causing sensory and motor symptoms depending on lesion location
25
What are the two types of ataxia seen in MS?
- Sensory ataxia --> *due to loss of proprioception (+ve Romberg’s test)*
- Cerebellar ataxia --> *due to a lesion in the cerebellum, causing coordination problems*
26
What is clinically isolated syndrome (CIS) in MS?
CIS describes the first episode of demyelination and neurological signs/symptoms --> may or may not progress to MS
27
What is the most common disease pattern in multiple sclerosis + what are the other two disease patterns?
Relapsing-remitting MS (RRMS) --> *characterised by episodes of disease and neurological symptoms, followed by recovery*
- can be classified as active (new symptoms are developing, or new lesions are appearing on the MRI) or worsening (there is an overall worsening of disability over time)
.
- Secondary progressive MS --> *where there was relapsing-remitting disease, but now there is a progressive worsening of symptoms with incomplete remissions*
- Primary progressive MS --> *involves worsening disease and neurological symptoms from the point of diagnosis, without relapses and remissions*
28
What investigations can support an MS diagnosis?
- MRI scans --> *detects demyelinating lesions*
- Lumbar puncture --> *detects oligoclonal bands in CSF*
29
How is MS managed generally + how are acute relapses managed?
General management:
- MDT (neruologists, nurses, physiotherapists, and occupational therapists)
- Disease-modifying therapies --> *to reduce relapses and slow disease progression*
.
Acute relapses:
- Oral steroids (500mg) OD for 5 days
- IV steroids (1g) OD for 3-5 days (if oral steroids fail or for severe relapses)
30
What are some symptomatic treatments for MS?
- Exercise --> maintain activity and strength
- Fatigue management --> amantadine, modafinil, SSRIs
- Neuropathic pain --> Amitriptyline, gabapentin
- Depression --> SSRIs
- Urge incontinence --> Antimuscarinics (e.g., solifenacin)
- Spasticity --> Baclofen, gabapentin
- Oscillopsia --> Gabapentin, memantine
31
What is motor neurone disease (MND) + does MND affect sensory neurones?
MND is a progressive and eventually fatal condition where the motor neurones stop functioning
.
There is NO effect on sensory neurones
32
What is the most common type of MND + which famous scientist had this condition?
Amyotrophic lateral sclerosis (ALS) --> *Stephen Hawking had ALS*
33
What is the second most common form of motor neurone disease?
Progressive bulbar palsy --> *affects the muscles of talking and swallowing (the bulbar muscles)*
34
What are the common intial symptoms of MND?
- Insidious, progressive muscle weakness, clumsiness, dropping objects, tripping, and slurred speech (dysarthria)
- with the absence of sensory symptoms
35
Signs of lower motor neurone disease
- Muscle wasting
- Reduced tone
- Fasciculations (twitches in the muscles)
-Reduced reflexes
36
Signs of upper motor neurone disease
- Increased tone or spasticity
- Brisk reflexes
- Upgoing plantar reflex
37
How is MND diagnosed?
Clinically by a specialist --> often after excluding other conditions (diagnosis is often delayed)
38
In MND, there are no effective treatments for halting or reversing the progression of the disease. What medication can slow ALS progression and extend survival by a few months?
Riluzole
39
How is breathing supported in MND?
Non-invasive ventilation (NIV) when respiratory muscles weaken
40
What symptomatic treatments are available for MND?
- spasticity
- excessive saliva
- anxiety-induced breathlessness
- spasticity --> baclofen
- excessive saliva --> antimuscarinics
- anxiety-induced breathlessness --> benzodiazapines
41
What is the main cause of death in MND?
Respiratory failure (due to weakened respiratory muscles) or pneumonia
42
What is Parkinson's disease?
a condition where there is a progressive reduction in dopamine in the basal ganglia --> *leading to disorders of movement*
43
What is the classic triad of Parkinson's disease symptoms?
- Resting tremor (a tremor that is worse at rest)
- Rigidity (resisting passive movement)
- Bradykinesia (slowness of movement)
44
What are the main functions of the basal ganglia?
- coordinating habitual movements
- controlling voluntary movements
-learning specific movement patterns
45
How does the tremor in Parkinson’s disease typically present?
- worse on one side and has a 4-6 Hz frequency
- "pill-rolling" appearance
- worse at rest, improves with movement
- exaggerated by distraction --> *eg. performing a task with the other hand exaggerates the tremor"
46
What is cogwheel rigidity?
a jerky resistance to passive movement, typical in Parkinson’s disease
47
How does bradykinesia present in Parkinson’s disease?
- Micrographia (handwriting gets smaller and smaller)
- Shuffling gait with festination (small steps with rapid frequency of steps to compensate for small steps and avoid falling)
- Difficulty initiating movement (pt might freeze and struggle to get moving again)
- Difficulty turning (lots of little steps to turn)
- Hypomimia (reduced facial movements and facial expressions)
48
In Parkinson's disease, other than the classic triad of symptoms (resting tremore, rigidity, and bradykinesia), what are some other features?
- Depression
- Sleep disturbance and insomnia
- Loss of the sense of smell (anosmia)
- Postural instability (increasing the risk of falls)
- Cognitive impairment and memory problems
49
Parkinson's disease VS Benign Essential Tremor
50
What is multiple system atrophy?
A Parkinson’s-plus syndrome, where there is degeneration of the basal ganglia (causing Parkinson's presentation) and degeneration in other areas (leading to autonomic dysfunction and cerebellar dysfunction)
- autonomic dysfunction --> *postural hypotension, constipation, abnormal sweating, and sexual dysfunction*
- cerebellar dysfunction --> *causing ataxia*
51
What is dementia with Lewy bodies?
A Parkinson’s-plus syndrome, which is a type of dementia associated with features of Parkinsonism, it causes a cognitive decline with associated symptoms of visual hallucinations, delusions, REM sleep disorder, and fluctuating consciousness
52
Parkinson's disease, describe each of the below...
- Multiple system atrophy
- Dementia with Lewy bodies
Multiple system atrophy:
- where neurones of various systems in the brain degenerate, including the basal ganglia
- degeneration in the basal ganglia leads to a Parkinson's presentation
- degeneration in other areas leads to autonomic dysfunction and cerebellar dysfunction (causing ataxia)
.
Dementia with Lewy bodies:
- type of dementia associated with features of Parkinsonism
- causes a progressive decline
53
Other than multisystem atrophy and dementia with Lewy bodies, what are two other Parkinsons-plus syndromes?
- Progressive supranuclear palsy
- Corticobasal degeneration
54
How is Parkinson's disease diagnosed?
Clinically based on history and examination findings
- NICE recommend the 'UK Parkinson’s Disease Society Brain Bank Clinical Diagnostic Criteria'
55
What is the main goal of Parkinson’s disease management + what are the main drug classes used to treat Parkinson's disease?
- to control symptoms and minimise side effects, as there is no cure
- pts may describe themselves as "on" when the medications are acting and "off" when the medications wear out, they are experiencing symptoms and their next dose is due
.
- Levodopa (combined with peripheral decarboxylase inhibitors)
- COMT inhibitors
- Dopamine agonists
- Monoamine oxidase-B inhibitors
56
What is levodopa, and why is it combined with other drugs?
- a synthetic dopamine that is usually combined with a peripheral decarboxylase inhibitor (eg. carbidopa or benserazide)
- which stops it from beings metabolised in the body before it reaches the brain
.
- levodopa becomes less effective over time, so other drugs can be used in combination to reduce the dose of levodopa and increase effectiveness of treatment
57
What are examples of levodopa combination drugs?
- Co-beneldopa ("Madopar") = levodopa + benserazide
- Co-careldopa ("Sinemet") = levodopa + carbidopa
58
What is the main side effect of levodopa + what medication can be given to manage this?
Dyskinesia (abnormal movements associated with excessive motor activity)
- examples include --> *dystonia (abnormal postures), chorea (jerky, random movements), athetosis (twisting/writhing movements)*
.
Amantadine (glutamate antagonist) can be used to manage dyskinesia associated with levodopa
59
What is the role of COMT inhibitors (e.g., entacapone) in Parkinson’s disease?
They slow the breakdown of the levodopa in the brain --> therefore extending the effective duration of the levodopa
- the COMT enzyme metabolises levodopa in both the body and brain
60
What is the role of dopamine agonists in Parkinson’s disease + give examples of dopamine agonists
They mimic the action of dopamine in the basal ganglia, stimulating the dopamine receptors --> they can be used to delay levodopa use or be used in combination with levodopa to reduce the required dose
.
- bromocriptine
- pergolide
- cabergoline
61
What is the role of monoamine oxidase-B inhibitors (e.g., selegiline, rasagiline) in Parkinson’s disease?
They block dopamine breakdown and therefore helping to increase the circulating dopamine
- monoamine oxidase enzymes break down neurotransmitters such as dopamine, serotonin, and adrenaline --> *monoamine oxidase-B is more specific to dopamine*
.
- they are typically used to delay the use of levodopa, then in combination with levodopa to reduce the "end of dose" worsening symptoms
62
What is benign essential tremor?
a common condition in older adults, causing a fine tremor in voluntary muscles (especially the hands)
63
What areas of the body can be affected by benign essential tremor?
- Hands (most common)
- Head
- Jaw
- Vocal cords (causing vocal tremor)
64
What are the key features of benign essential tremor?
- Fine tremor (6-12 Hz)
- Symmetrical
- Worse with voluntary movement
- Exacerbated by stress, fatigue, or caffeine
- Improved by alcohol
- Absent during sleep
65
What are key differential diagnoses for a tremor?
- Parkinson’s disease
- Multiple sclerosis
- Huntington’s chorea
- Benign essential tremor
- Hyperthyroidism
- Fever
- Dopamine antagonists (e.g. antipsychotics)
66
What is the management of benign essential tremor?
No treatment is required unless it causes functional or psychological distress
.
Symptomatic treatment:
- Propranolol (a non-selective beta blocker)
- Primidone (a barbiturate anti-epileptic medication)
67
What are important differential diagnoses for seizures?
- Vasovagal syncope (fainting)
- Pseudoseizures (non-epileptic attacks)
- Cardiac syncope (e.g., arrhythmias or structural heart disease)
- Hypoglycaemia
- Hemiplegic migraine
- Transient ischaemic attack
- Alcohol withdrawal seizures --> *occurs in pts with a hx of chronic alcohol use who stop their alcohol intake too abruptly*
68
What investigations are used in epilepsy diagnosis?
- EEG (can do prolonged EEG monitoring) --> *detects typical epilepsy patterns + supports diagnosis*
- MRI brain --> *identifies structural pathology (eg. tumours, lesions)*
.
Additional tests:
- ECG -> *rule out cardiac arrhythmias*
- Blood glucose --> *rule out hypoglycaemia and diabetes*
- Serum electrolytes --> *electrolyte imbalances can cause seizures*
- Blood/urine cultures + lumbar puncture --> *if infection suspected (eg. sepsis, encephalitis, meningitis)*
69
What are key safety precautions for patients with epilepsy?
- Driving restrictions --> *seizure-free for one year before driving*
- Showers instead of baths (drowning risk)
- Caution with swimming, heights, traffic, dangerous equipment
70
Epilepsy management
71
How does sodium valproate work?
by increasing the activity of gamma-aminobutyric acid (GABA), which has a calming effect on the brain
72
What are the key side effects of sodium valproate?
- Teratogenic (harmful in pregnancy) --> *can cause neural tube defects and developmental delay*
- Liver damage and hepatitis
- Hair loss
- Tremor
- Reduce fertility
73
What are 2 features of the 'Valproate Pregnancy Prevention Programme'?
- involves ensuring effective contraception
- annual risk acknowledgement form
74
What is status epilepticus?
a medical emergency defined as either:
- a seizure lasting more than 5 minutes
- multiple seizures without regaining consciousness in the interim
75
How is status epilepticus managed?
ABCDE approach:
- secure airway
- give high-concentration oxygen
- check blood glucose
- gain IV access (insert cannula)
76
What is the first-line medical treatment for status epilepticus?
A benzodiazepine (repeat after 5-10 minutes if seizure continues)
.
Options:
- IV lorazepam (4mg)
- Buccal midazolam (10mg)
- Rectal diazepam (10mg)
77
What are second-line and third-line options for status epilepticus?
Second-line (after 2 benzodiazepine doses):
- IV levetiracetam
- IV phenytoin
- IV sodium valproate
.
Third-line:
- phenobarbital
- general anaesthesia
78
What are the risks if epilepsy is not controlled (either the patient isn't adhering to anti-epileptic medication OR anti-epileptic medication isn't working)?
In around 30% of patients, seizures may remain refractory to treatment --> *these individuals are at increased risk of sudden unexpected death in epilepsy (SUDEP), injuries from falls or accidents during seizures, and psychosocial difficulties*
- SUDEP accounts for up to 17% of all deaths in people with epilepsy, and is most common in those with poorly controlled generalised tonic-clonic seizures
79
What are the two main categories of pain?
- Acute pain (new onset)
- Chronic pain (present for ≥ 3 months)
80
What are the two aspects of the pain experience?
- Sensory (pain perception) - *sensory signal transmitted from the pain receptor ("it is a sharp sensation, likely a needle")*
- Affective (emotional response) - *unpleasant emotional reaction to the pain ("it is excruitating, I can't bear it")*
81
What is pain threshold + give an example of how pain threshold can be measured?
The point at which sensory input is reported as painful
.
- for example, different temperatures can be applied to the skin to measure the point at which the heat is interpreted as pain --> a higher temperature indicates a higher sensory threshold for pain
82
What is allodynia?
Pain experienced with sensory inputs that do not normally cause pain (e.g. light touch)
83
How is pain tolerance different from pain threshold?
- The level of pain an individual can endure before it affects their behaviour, influenced by biological, psychological, and social factors.
- eg. two people might experience a similar pain, but one will get on with normal activities and the other will take time off work and seek help etc.
84
What are the two types of pain fibres and their characteristics?
- C fibres (unmyelinated and small diameter) – *transmit signals slowly and produce dull and diffuse pain sensations*
- A-delta fibres (myelinated and larger diameter) – *transmit signals fast and produce sharp and localised pain sensations*
.
(the signal then travels in the CNS, up the spinal cord (mainly spinothalamic and spinoreticular tract) to the brain where it is interpreted as pain, mainly in the thalamus and cortex)
85
What tracts transmit pain signals in the spinal cord?
Spinothalamic tract and spinoreticular tract
86
What are the three main sensory inputs that generate pain signals?
- Mechanical (eg. pressure)
- Heat
- Chemical (e.g., prostaglandins)
87
What is referred pain?
Pain perceived at a location away from the actual site of tissue damage (e.g., left arm pain during a heart attack)
88
What is neuropathic pain?
Pain caused by abnormal functioning or damage of sensory nerves.
89
What are the common methods of measuring pain?
- Visual Analogue Scale (VAS) - *pain is rated along a horizontal line, and you end up with a numerical value to represent the pain (eg. 75mm along a 100mm line)*
- Numerical Rating Scale (NRS) - *0-10 scale (where 0 is no pain at all, and 10 is the worst pain imaginable)*
- Graphical Rating Scale - *series of faces going from happy to unhappy (helpful in children or learning disabilities)*
90
What are the three steps of the WHO analgesic ladder?
- Step 1: Non-opioid medications --> *paracetamol and NSAIDs*
- Step 2: Weak opioids --> *codeine and tramadol*
- Step 3: Strong opioids --> *morphine, oxycodone, fentanyl and buprenorphine*
91
What are common adjuvant medications for pain management?
- Antidepressants (e.g., amitriptyline, duloxetine)
- Anticonvulsants (e.g., gabapentin, pregabalin).
92
What is a common side effect of long-term use of analgesic medication?
Medication overuse headache
93
What are the key side effects of NSAIDs?
- Gastritis with dyspepsia (indigestion)
- Stomach ulcers
- Exacerbation of asthma
- Hypertension
- Renal impairment
- small cardiovascular risk
94
What are contraindications for NSAIDs?
- Asthma
- Renal impairment
- Heart disease
- Uncontrolled hypertension
- Stomach ulcers
95
What medication is often co-prescribed with NSAIDs to reduce the risk of gastrointestinal side effects?
Proton pump inhibitors (eg. omeprazole or lansoprazole)
96
What are the key side effects of opioids?
- Constipation
- Skin itching (pruritus)
- Nausea
- Altered mental state (sedation, cognitive impairment or confusion)
- Respiratory depression (usually only with larger doses)
97
What medication is used to reverse opioid overdose?
Naloxone
98
What is the standard approach for opioid use in palliative care?
- Background opioids (e.g., 12-hourly modified-release oral morphine)
(opioid patches can also be used for background analgesia --> buprenorphine patches, fentanyl patches)
.
- Rescue doses for breakthrough pain (e.g., immediate-release oral morphine solution) - *rescue dose is 1/6 of 24hr background dose*
99
How do you calculate a breakthrough dose for a patient on 30mg morphine every 12 hours?
The total 24-hour dose is 60mg, so the breakthrough dose is 10mg
(rescue dose is 1/6 of 24hr background dose)
100
What are the approximate opioid conversions equivalent to 10mg oral morphine?
101
What is chronic pain + What are the two categories that NICE separates chronic pain into
Chronic pain = pain that has been present or reoccurs for more than three months
.
- Chronic primary pain – where no underlying condition can adequately explain the pain
- Chronic secondary pain – where an underlying condition can explain the pain (e.g., arthritis)
102
What are the recommended treatments for chronic primary pain (NICE 2021)?
- Exercise programs
- Acceptance and commitment therapy (ACT)
- Cognitive behavioural therapy (CBT)
- Acupuncture
- Antidepressants (e.g., amitriptyline, duloxetine or an SSRI)
103
What treatments should NOT be started for chronic primary pain (NICE 2021)?
- Paracetamol
- NSAIDs
- Opiates
- Anti-epileptic drugs (e.g. pregabalin or gabapentin)
.
(essentially avoid all forms of analgesia, except for antidepressants)
104
What are common causes of neuropathic pain?
- Post-herpetic neuralgia from shingles is in the distribution of a dermatome and usually on the trunk
- Nerve damage from surgery
- Multiple sclerosis
- Diabetic neuralgia (typically affecting the feet)
- Trigeminal neuralgia
- Complex regional pain syndrome
105
What tool assesses neuropathic pain?
DN4 questionnaire (scored out of 10) --> * ≥ 4 suggests neuropathic pain*
106
What are first-line treatments for neuropathic pain?
- Amitriptyline – *tricyclic antidepressant*
- Duloxetine – *SNRI antidepressant*
- Gabapentin – *anticonvulsant*
- Pregabalin – *anticonvulsant*
.
(note: only one neuropathic medication is used at a time, and tramadol may be used only as a short-term rescue for flares)
107
What is the first-line treatment for trigeminal neuralgia?
Carbamazepine
108
What is complex regional pain syndrome + what are key features of complex regional pain syndrome?
A condition with neuropathic pain, abnormal sensations, skin changes - *often triggered by an injury and isolated to one limb*
.
Key features:
- hypersensitivity
- allodynia
- intermittent swelling
- colour/temperature changes
- skin flushing
- abnormal sweating
109
How is complex regional pain syndrome treated?
Guided by a pain specialist, similar to neuropathic pain management.
110
What is facial nerve palsy?
Isolated dysfunction of the facial nerve, presenting with unilateral facial weakness
111
Where does the facial nerve exit the brainstem + What structures does the facial nerve pass through before reaching the face?
- At the cerebellopontine angle
- The temporal bone and the parotid gland.
112
What are the five branches of the facial nerve?
TZBMC
- Temporal
- Zygomatic
- Buccal
- Marginal mandibular
- Cervical
113
What are the motor functions of the facial nerve?
- Facial expression
- Stapedius muscle in the inner ear
- Posterior digastric, stylohyoid, and platysma muscles
114
What is the sensory function of the facial nerve?
taste from the anterior 2/3 of the tongue
115
What parasympathetic functions does the facial nerve provide?
- Submandibular and sublingual salivary glands
- Lacrimal gland (stimulating tear production)
116
What are causes of unilateral UMN facial nerve palsy?
- Stroke (CVA)
- Brain tumours
117
What is Bell’s palsy?
An idiopathic unilateral LMN facial nerve palsy that fully recovers over several weeks
(1/3 are left with residual weakness)
118
What is the recommended treatment for Bell’s palsy within 72 hours of onset (NICE 2023)?
Prednisolone:
- 50mg for 10 days
OR
- 60mg for 5 days, followed by a 5-day taper (reducing by 10mg/day)
119
Why do Bell’s palsy patients require eye drops + what should patients do to protect their eye at night?
- To prevent exposure keratopathy due to incomplete eyelid closure
- Tape the eye closed
120
What causes Ramsay-Hunt syndrome?
Reactivation of the varicella-zoster virus (VZV)
121
What are key features of Ramsay-Hunt syndrome?
- Unilateral LMN facial nerve palsy
- Painful vesicular rash in the ear canal, pinna, or around the ear (can extend to the anterior 2/3 of the tongue and hard palate)
(look for a facial nerve palsy + vesicular rash near the ear)
122
How is Ramsay-Hunt syndrome treated?
- Aciclovir
- Prednisolone
- Lubricating eye drops
123
How do brain tumours present?
- They may be asymptomatic when small but cause progressive focal neurological symptoms as they grow
- often present with symptoms/signs of raised intracranial pressure (intracranial hypertension) - *a growing tumour takes up room within the skull, leaving less space for the other contents (such as CSF), causing a rise in pressure within the intracranial space*
124
A common exam scenario is an unusual change in personality and behaviour, where in the brain is the tumour?
Frontal lobe tumour --> *frontal lobe is responsible for personality and higher level decision-making*
125
What symptoms suggest raised intracranial pressure (intracranial hypertension)?
- Constant headache (worse at night/on waking) + worse on coughing, straining, or bending forward
- Vomiting
- Papilloedema
- Altered mental state
- Visual field defects
- Seizures (partial)
- unilateral ptosis
- 3rd and 6th cranial nerve palsies
126
What is papilloedema + what causes of papilloedema
- Swelling of the optic disc due to raised intracranial pressure, seen on fundoscopy
- the sheath around the optic nerve is connected with the subarchnoid space, the raised CSF pressure flows into the optic nerve sheath, increasing the pressure around the optic nerve behind the optic disc causing the optic disc to bulge forward
127
How are gliomas (tumours of glial cells in the brain or spinal cord) graded?
Gliomas are graded from 1 to 4:
- Grade 1 is the most benign (possibly curable with surgery)
- Grade 4 is the most malignant (e.g., glioblastoma multiforme).
128
What are the three main types of gliomas?
- Astrocytoma (most aggressive = glioblastoma)
- Oligodendroglioma
- Ependymoma
129
What are meningiomas?
Usually benign tumours of the meninges, but they cause mass effect which can lead to raised ICP and neurological symptoms
130
Which cancers most commonly metastasise to the brain?
- Lung
- Breast
- Renal cell carcinoma
- Melanoma
131
How do pituitary tumours present?
- Bitemporal hemianopia (pituitary sits close to optic chiasm)
- Hormone excess (e.g., acromegaly, Cushing’s disease, hyperprolactinaemia, thyrotoxicosis), or hormone deficiency
132
How can pituitary tumours be managed?
- Trans-sphenoidal surgery (through the nose and sphenoid bone)
- Radiotherapy
- Bromocriptine to block excess prolactin
- Somatostatin analogues (e.g., octreotide) to block excess growth hormone
133
What is an acoustic neuroma (vestibular schwannoma)?
benign tumour of the Schwann cells that surround the auditory nerve (vestibulocochlear nerve), at the cerebellopontine angle
134
What condition are bilateral acoustic neuromas associated with?
Neurofibromatosis type 2
135
How do acoustic neuromas (vestibular schwannomas) present?
- Unilateral sensorineural hearing loss (often the first symptom)
- Unilateral tinnitus
- Dizziness or imbalance
- Sensation of fullness in the ear
- Facial nerve palsy (if the tumour grows large enough to compress the facial nerve)
136
What is the first-line investigation for a brain tumour?
MRI scan
(biopsy gives definitive histological diagnosis - *usually obtained during surgery*)
137
What are the main management options for a brain tumour?
Depends on type and grade and is guided by the MDT:
- Surgery (partial or total removal)
- Chemotherapy
- Radiotherapy (to reduce the growth)
- Palliative care
.
(biopsy gives the definitive histological diagnosis --> *this is usually obtained during surgery*)
138
What is Huntington’s disease?
An autosomal dominant genetic disorder causing progressive neurological dysfunction
139
What genetic mutation causes Huntington’s disease?
A trinucleotide repeat disorder involvig a genetic mutation in the HTT gene on chromosome 4, which codes for the huntingtin (HTT) protein
140
What are nucleotides and what are trinucleotide repeats?
- Nucleotides are the building blocks of DNA (adenine, cytosine, guanine and thymine)
- Sequences of three nucleotides repeated multiple times in DNA (e.g., CAGCAGCAGCAGCAG)
141
Other than Huntington's disease, what are other examples of trinucleotide repeat disorders?
- Fragile X syndrome
- Spinocerebellar ataxia
- Myotonic dystrophy
- Friedrich ataxia
142
What is genetic anticipation and what neurological condition displays genetic anticipation?
Anticipation is a feature of trinucleotide repeat disorders, where successive generations have more repeats in the gene, resulting in:
- Earlier age of onset
- Increased severity of disease
.
Huntington's chorea displays genetic anticipation
143
What are the key symptoms of Huntington’s chorea?
Presents with an insidious, progressive worsening of symptoms:
- Cognitive decline
- Psychiatric symptoms
- Chorea (involuntary, irregular body movements)
- Dystonia (abnormal muscle tone leading to abnormal postures)
- Rigidity (increased resistance to passive movement of a joint)
- Eye movement disorders
- Dysarthria (speech difficulties)
- Dysphagia (swallowing difficulties)
144
How is Huntington’s disease diagnosed?
Genetic testing at a specialist centre with pre and post-test counselling
145
What is the management approach in Huntington's disease?
There are no treatment options for slowing or stopping the progression of the disease.
.
Management involves:
- MDT
- physiotherapy
- speech therapy
- Tetrabenazine may be used for chorea symptoms
- Antidepressants (eg. SSRIs) for depression
- Advanced directives
- End-of-life care
(Genetic counseling is important too)
146
What % chance does the child of someone with Huntington's disease have of inheriting the faulty gene?
50% --> *autosomal dominant*
147
In Huntington's disease, what is the life expectancy after the onset of symptoms?
10-20 years
148
What is a common cause of death in patients with Huntington's disease?
Aspiration pneumonia or suicide
149
What is myasthenia gravis?
An autoimmune condition affecting the neuromuscular junction, causing muscle weakness that worsens with activity and improves with rest
150
At what ages does myasthenia gravis typically affect men and women?
Women under 40, men over 60
151
What is myasthenia gravis associated with?
- Thymomas (thymus gland tumours)
- 10-20% of patients with myasthenia gravis have a thymoma, and 30% of thymoma patients develop myasthenia gravis
152
What antibodies are most commonly involved in myasthenia gravis?
Acetylcholine receptor (AChR) antibodies
153
What is the mechanism of myasthenia gravis?
- Acetylcholine receptor (AChR) antibodies bind to the postsynaptic acetylcholine receptors, blocking them and preventing stimulation by acetylcholine
- the more the receptors are used during muscle activity, the more they become blocked
- with rest, the receptors are cleared, and the symptoms improve
- (these antibodies also activate the complement system within the NMJ, leading to cell damage at the postsynaptic membrane, further worsening symptoms)
.
- axons release a neurotransmitter called acetylcholine from the presynaptic membrane, which then travels across the synapse and attaches to receptors on the postsynaptic membrane, stimulating muscle contraction
154
Other than acetylcholine (AChR) receptor antibodies, what other antibodies can cause myasthenia gravis?
- Muscle-specific kinase (MuSK) antibodies
- Low-density lipoprotein receptor-related protein 4 (LRP4) antibodies
.
(MuSK and LRP4 are important proteins for the creation and organisation of the acetylcholine receptor --> *destruction of these proteins leads to inadequate acetylcholine receptors*)
155
What is a key feature of myasthenia gravis + what are some other symptoms that are common in myasthenia gravis?
- Weakness that worsens with use and improves with rest
.
Symptoms most affect the proximal muscles and the small muscles of the head and neck:
- Difficulty climbing stairs, standing from a seat or raising their hands above their head
- Diplopia --> *weakness of extraocular muscles*
- Ptosis --> *eyelid weakness*
- Weakness in facial movements
- Dysphagia (difficulty swallowing)
- Fatigue in jaw when chewing
- Slurred speech
156
What are 3 ways to elicit fatiguability in the muscles to assess for myasthenia gravis
- Repeated blinking will exacerbate ptosis
- Prolonged upward gazing will exacerbate diplopia on further testing
- Repeated abduction of one arm 20 times will result in unilateral weakness when comparing both sides
157
What antibodies are tested for in myasthenia gravis?
- AChR antibodies (85%)
- MuSK antibodies (<10%)
- LRP4 antibodies (<5%)
158
What imaging is used to check for a thymoma (in patients with myasthenia gravis)?
CT or MRI of the thymus
159
What is the edrophonium test (used when there is doubt about a diagnosis of myasthenia gravis)?
- Pts are given IV edrophonium chloride (or neostigmine) - *a short-acting acetylcholinesterase inhibitor*
- Normally, cholinesterase enzymes in the NMJ break down acetylcholine
- Edrophonium blocks these enzymes, reducing the breakdown of acetylcholine
- as a result, the level of acetylcholine at the NMJ rises, temporarily relieving the weakness
- a positive result suggest a diagnosis of myasthenia gravis
160
What are the treatment options for myasthenia gravis?
- Pyridostigmine - *a cholinesterase inhibitor that prolongs the action of acetylcholine and improves symptoms*
- Immunosuppression (e.g., prednisolone or azathioprine) - *suppresses the production of antibodies*
.
- Thymectomy can improve symptoms, even in patients without a thymoma
- Rituximab (a monoclonal antibody against B cells) is considered where other treatments fail
161
What is a myasthenic crisis?
- an acute worsening of symptoms in a patient with myasthenia gravis, often triggered by another illness (such as a respiratory tract infection)
- respiratory muscle weakness can lead to respiratory failure
162
How is myasthenic crisis managed?
- patients may require NIV or mechanical ventilation for respiratory failure
- IV immunoglobulins and plasmapheresis
163
What is Lambert-Eaton myasthenic syndrome (LEMS)?
an autoimmune condition affecting the neuromuscular junction, often associated with small-cell lung cancer (SCLC)
164
How does LEMS differ from myasthenia gravis in presentation?
- LEMS symptoms tend to be more insidious and less pronounced
- Muscle strength and reflexes improve with activity, whereas in myasthenia gravis, symptoms worsen with use
165
What is the usual cause of Lambert-Eaton myasthenic syndrome?
usually a paraneoplastic syndrome associated with small-cell lung cancer (SCLC), but it can also occur as a primary autoimmune disorder without malignancy
166
What is the pathophysiology of Lambert-Eaton myasthenic syndrome?
- caused by antibodies against voltage-gated calcium channels in the presynaptic membrane of the NMJ
- leading to reduced acetylcholine release into the synapse, resulting in a weaker signal and reduced muscle contraction
.
(these antibodies may be produced in response to small cell lung cancer cells that express voltage-gated calcium channels)
(voltage-gated calcium channels are responsible for assisting the release of acetylcholine into the synapse of the NMJ)
167
What are the key symptoms of Lambert-Eaton myasthenic syndrome (LEMS)?
- Proximal muscle weakness --> *causing difficulty climbing stairs, standing from a seat, or raising the arms overhead*
- Autonomic dysfunction --> *causing dry mouth, blurred vision, impotence and dizziness*
- Reduced or absent tendon reflexes
168
How do symptoms of Lambert-Eaton myasthenic syndrome (LEMS) change with activity?
Muscle strength and reflexes improve after periods of muscle contraction, unlike myasthenia gravis, where symptoms worsen with use
169
How is Lambert-Eaton myasthenic syndrome (LEMS) managed?
- Screen for underlying malignancy (small cell lung cancer)
- Amifampridine - *blocks voltage-gated potassium channels in the presynaptic membrane, which prolongs depolarisation and assists calcium channels in carrying out their action*
.
Other options:
- Pyridostigmine (cholinesterase inhibitor)
- Immunosuppressants (e.g. prednisolone or azathioprine)
- IV immunoglobulins
- Plasmapheresis
170
What is Charcot-Marie-Tooth disease?
An inherited disease affecting peripheral motor and sensory neurons, also called hereditary motor and sensory neuropathy
171
What is the inheritance pattern of most Charcot-Marie-Tooth mutations?
Autosomal dominant
172
At what age do symptoms of Charcot-Marie-Tooth usually appear?
Usually before age 10 but can be delayed until 40 or later
173
What are the key clinical features of Charcot-Marie-Tooth disease?
- High foot arches (pes cavus)
- Distal muscle wasting --> *causing “inverted champagne bottle legs”*
- Lower leg weakness (particularly loss of ankle dorsiflexion) --> *high stepping gait due to foot drop*
- Weakness in the hands
- Reduced tendon reflexes
- Reduced muscle tone
- Peripheral sensory loss
174
What is peripheral neuropathy?
Reduced sensory and motor function in the peripheral nerves, often in a “glove and stocking” distribution
175
What mnemonic can be used to remember other causes of peripheral neuropathy?
ABCDE mnemonic:
- A – Alcohol
- B – B12 deficiency
- C – Cancer (e.g., myeloma) and Chronic kidney disease
- D – Diabetes and Drugs (e.g., isoniazid, amiodarone, leflunomide, cisplatin)
- E – Every vasculitis
176
Why is Charcot-Marie-Tooth a common OSCE scenario?
It is a relatively common condition with clear signs and stable patients, making it useful for examining peripheral neuropathy
177
Is there a cure for Charcot-Marie-Tooth disease?
No, treatment is supportive
178
What specialists are involved in the management of Charcot-Marie-Tooth?
- Neurologists and geneticists --> *to make the diagnosis*
- Physiotherapists --> *to maintain muscle strength and joint range of motion*
- Occupational therapists --> *to assist with daily activities*
- Podiatrists --> *to help with foot symptoms and suggest insoles and other orthoses to improve symptoms*
- Orthopaedic surgeons --> *for severe joint deformities*
179
What medication can be used for neuropathic pain in Charcot-Marie-Tooth?
Amitriptyline
180
What is Guillain-Barré syndrome?
An acute paralytic polyneuropathy affecting the peripheral nervous system, causing acute, symmetrical, ascending weakness +/- sensory symptoms
181
What typically triggers Guillain-Barré syndrome?
An infection, particularly Campylobacter jejuni, cytomegalovirus (CMV), and Epstein-Barr virus (EBV).
182
What is the underlying mechanism of Guillain-Barré syndrome?
- Molecular mimicry --> *B cells produce antibodies against the antigens on the triggering pathogen, which mistakenly attack proteins on peripheral neurons*
- (the antibodies produced also match proteins on the peripheral neurones)
183
What parts of the neuron can be affected in Guillain-Barré syndrome?
The myelin sheath or the axon itself
184
When do symptoms of Guillain-Barré syndrome typically start?
Within four weeks of the triggering infection
185
How does the weakness in Guillain-Barré syndrome progress?
- Symmetrical, ascending weakness, starting in the feet and moving upwards
- symptoms peak within 2-4 weeks, then there is a recovery period that can last months to years
186
What are the key neurological features of Guillain-Barré syndrome + what additional complications can occur in Guillain-Barre syndrome?
- Symmetrical ascending weakness
- Reduced reflexes
- Peripheral sensory loss or neuropathic pain
.
Additional complications:
- Cranial nerve involvement --> *facial weakness*
- Autonomic dysfunction --> *urinary retention, ileus, arrhythmias*
187
How is Guillain-Barré syndrome diagnosed + what 2 investigations support the diagnosis of Guillain-Barre syndrome?
Diagnosis is made clinically using the Brighton criteria, supported by investigations
.
- Nerve conduction studies - *show reduced signal*
- Lumbar puncture (CSF analysis) --> *shows raised protein with normal cell count and glucose*
188
What is the first-line treatment for Guillain-Barre syndrome + what is an alternative treatment?
- 1st-line --> IV immunoglobulins (IVIG)
- Alternative --> Plasmapheresis (plasma exchange)
189
What supportive measures are important in Guillain-Barré syndrome?
- VTE prophylaxis (pulmonary embolism is a leading cause of death)
- Intensive care support in severe cases (intubation and ventilation if respiratory failure occurs)
190
How long does recovery from Guillain-Barré syndrome typically take + what are the possible long-term outcomes?
Months to years, with some patients continuing to regain function up to five years after the acute illness
.
- most patients either make a full recovery or are left with minor symptoms
- some are left with significant disability
- mortality is around 5% --> *usually ue to respiratory or cardiovascular complications*
191
What is neurofibromatosis?
A genetic condition causing benign nerve tumours (neuromas) throughout the nervous system, these tumours can cause neurological and structural problems
192
Which type of neurofibromatosis is more common?
Neurofibromatosis type 1 (NF1) is more common than Neurofibromatosis type 2 (NF2)
193
What gene is affected in neurofibromatosis type 1 (NF1), and what does it code for?
NF1 gene on chromosome 17, coding for neurofibromin, which is a tumour suppressor protein
194
What is the inheritance pattern of neurofibromatosis type 1 (NF1)?
Autosomal dominant
195
The diagnostic criteria for neurofibromatosis type 1 are based on the features, what is the mnemonic for the features of NF1?
"CRABBING":
- C – Café-au-lait spots (>15mm is significant in adults)
- R – Relative with NF1
- A – Axillary or inguinal freckling
- BB – Bony dysplasia, such as Bowing of a long bone (or sphenoid wing dysplasia)
- I – Iris hamartomas (Lisch nodules – yellow-brown spots on the iris)
- N – Neurofibromas (skin-coloured raised nodules)
- G – Glioma of the optic pathway
.
(genetic testing also supports a diagnosis of NF1)
196
What is a plexiform neurofibroma, and why is it significant?
A large, irregular neurofibroma containing multiple cell types --> *a single plexiform neurofibroma is significant for NF1*
197
What is the treatment for NF1?
No cure – management involves monitoring, symptom control, and treating complications
198
What are key complications of NF1?
- Neurological – Migraines, epilepsy, learning disability, behavioural problems (e.g., ADHD)
- Cardiovascular – Renal artery stenosis, causing hypertension
- Musculoskeletal – Scoliosis
- Ophthalmology – Optic gliomas, leading to vision loss
- Oncology – Malignant peripheral nerve sheath tumours (MPNST), gastrointestinal stromal tumours (GIST), brain and spinal cord tumours, increased cancer risk (breast cancer, leukaemia)
199
What two unique tumours are strongly associated with NF1?
Malignant peripheral nerve sheath tumours (MPNST) and gastrointestinal stromal tumours (GIST)
200
What gene is affected in NF2, and what does it code for?
NF2 gene on chromosome 22, coding for merlin, which is a tumour suppressor protein in Schwann cells
201
What is the inheritance pattern of NF2?
Autosomal dominant
202
What type of tumours are most strongly associated with NF2?
Schwannomas (benign tumours of Schwann cells), particularly bilateral acoustic neuromas
203
What nerve is affected by acoustic neuromas in NF2?
The auditory nerve (vestibulocochlear nerve, CN VIII), leading to hearing loss and balance issues
204
What is the main treatment option for NF2?
Surgical resection of tumours, though this carries a risk of permanent nerve damage
205
What exam clue suggests NF2?
Bilateral acoustic neuromas are almost always due to NF2
206
What are key red flags associated with headaches?
- Fever, photophobia, or neck stiffness --> *meningitis, encephalitis, or brain abscesses*
- Raised ICP signs --> *worse on coughing/straining, worse on standing/lying/bending over, vomiting*
- Visual disturbance --> *GCA, glaucoma, or tumours*
- New neurological symptoms --> *haemorrhage, tumours*
- sudden-onset occipital headache --> *subarachnoid haemorrhage*
- Hx of trauma --> *intracranial haemorrhage*
- Hx of cancer --> *brain metastases*
- Pregnancy --> *preeclampsia*
207
Why is fundoscopy important in headaches?
To check for papilloedema, which suggests raised ICP due to a brain tumour, idiopathic intracranial hypertension, or an intracranial bleed
208
What are the typical features of a tension headache?
- Mild ache or pressure
- Band-like pattern around the head
- Gradual onset and resolution
- No visual changes
209
What are common triggers for tension headaches?
- Stress
- Depression
- Alcohol
- Skipping meals
- Dehydration
210
What is defined as a chronic tension-type headache?
occurring on more than 15 days per month for at least 3 months
211
How are tension headaches managed?
- Reassurance
- Simple analgesia (e.g., ibuprofen, paracetamol)
- Amitriptyline (first-line for chronic/frequent cases)
212
What are key features of a sinusitis headache?
- Facial pain and pressure
- Follows a recent viral URTI
- Tenderness/swelling over affected sinuses
(due to inflammation of paranasal sinuses)
213
How is sinusitis managed?
- Usually self-limiting (caused by an URTI and resolves in 2–3 weeks)
- >10 days duration --> Steroid nasal spray or phenoxymethylpenicillin
214
What is the key management step for medication-overuse headaches?
Withdraw analgesia, though this can be difficult in patients with chronic pain
215
What causes hormonal headaches?
Low oestrogen levels
- features are similar to migraines
216
When do hormonal headaches commonly occur?
- 2 days before to 3 days into menstruation
- Perimenopausal period
- Early pregnancy (headaches in second half of pregnancy should be investigated for pre-eclampsia)
217
What are treatment options for hormonal headaches?
Triptans and NSAIDs (e.g., mefenamic acid)
218
What is cervical spondylosis?
Degenerative changes in the cervical spine, causing neck pain and headaches
- pain is worse with movement
219
What is trigeminal neuralgia?
Intense facial pain in the distribution of the trigeminal nerve (CN V)
220
What are the three branches of the trigeminal nerve?
- Ophthalmic (V1)
- Maxillary (V2)
- Mandibular (V3)
221
What are the characteristics of trigeminal neuralgia pain?
- Sudden onset, lasting seconds to hours
- Electric-shock, shooting, stabbing, or burning pain
- Triggered by touch, talking, eating, shaving, or cold
- Unilateral in > 90% of cases
222
What condition is trigeminal neuralgia associated with?
Multiple sclerosis (MS)
223
What is the first-line treatment for trigeminal neuralgia?
Carbamazepine
224
Who is most affected by migraines?
More common in women, and most prevalent in teenagers and young adults
225
What are the four main types of migraine?
- Migraine without aura
- Migraine with aura
- Silent migraine (migraine with aura but without a headache)
- Hemiplegic migraine
226
What are the five stages of a migraine?
- Premonitory/prodromal stage (can begin several days before the headache) --> *yawning, fatigue, mood change*
- Aura (lasting up to 60 minutes) --> *visual/sensory/language changes*
- Headache stage (lasts 4 to 72 hours) --> *throbbing pain, photophobia, nausea*
- Resolution stage (gradual fade or relieved by vomiting/sleep)
- Postdromal or recovery phase
.
(these stages vary between patients, some patients may only experience one or two of the stages)
227
What are the key features of migraine headaches?
- Unilateral (rarely bilateral)
- Moderate-severe intensity
- Pounding/throbbing pain
- Photophobia, phonophobia, osmophobia
- Aura (visual changes)
- Nausea & vomiting
228
What are the most common symptoms of migraine aura?
- Visual symptoms --> *sparks, blurred vision, lines, loss of visual fields (eg. scotoma)*
- Sensory symptoms --> *tingling, numbness*
- Language symptoms --> *dysphasia*
229
What is the defining feature of a hemiplegic migraine + what are other symptoms that can occur in hemiplegic migraine?
- Unilateral limb weakness (hemiplegia)
.
- Other symptoms --> *ataxia, impaired consciousness*
230
What is familial hemiplegic migraine?
An autosomal dominant genetic condition where hemiplegic migraines run in families
231
What condition is important to rule out/exclude in hemiplegic migraine?
Hemiplegic migraines mimic stroke or TIA, requiring urgent assessment
232
What are common migraine triggers?
- Stress
- Bright lights
- Strong smells
- Certain foods (e.g., chocolate, cheese and caffeine)
- Dehydration
- Menstruation
- Disrupted sleep
- Trauma
233
What are general measures for migraine attacks?
Rest in a dark, quiet room and sleep
234
What are the medical options for acute migraine attacks?
- NSAIDs (e.g., ibuprofen, naproxen)
- Paracetamol
- Triptans (e.g., sumatriptan)
- Antiemetics if nausea/vomiting (e.g., metoclopramide, prochlorperazine)
235
Why are opiates avoided in migraine treatment?
They can worsen migraines and lead to medication-overuse headaches
236
How do triptans work?
- 5-HT receptor agonists - *they bind to and stimulate serotonin receptors (specifically 5-HT1B and 5-HT1D)*
MOA:
1. cranial vasoconstriction
2. inhibiting the transmission of pain signals
3. inhibiting the release of inflammatory neuropeptides
237
When should triptans be taken for an acute migraine attack + what should be done if a triptan does not work for an attack?
- As soon as the headache starts (not during aura)
- Do not take a second dose for the same attack
238
What are contraindications to triptan use?
- Hypertension
- Coronary artery disease
- Previous stroke, TIA, or MI
239
What is the purpose of a headache diary?
Helps identify triggers and assess treatment response
240
What are first-line medications for migraine prophylaxis?
- Propranolol (non-selective beta-blocker)
- Amitriptyline (tricyclic antidepressant)
- Topiramate (teratogenic --> contraception needed)
241
What lifestyle-based options are suggested for prophylaxis of migraines?
- Cognitive behavioural therapy
- Mindfulness and meditation
- Acupuncture
- Vitamin B2 (riboflavin)
242
What prophylactic options are available for menstrual migraines?
Prophylactic triptans (e.g., frovatriptan, zolmitriptan) taken from 2 days before to 3 days after menstruation
243
How does migraine impact stroke risk?
Slightly increased stroke risk, especially with aura
244
Why is the combined contraceptive pill contraindicated in migraine with aura?
It further increases stroke risk and is contraindicated
245
What are cluster headaches?
Severe, unilateral headaches centred around the eye, occurring in clusters of attacks
246
Why are cluster headaches called "cluster" headaches?
Attacks come in clusters, occurring multiple times a day for weeks or months, followed by pain-free periods lasting months or years
247
How long do cluster headache attacks last?
Between 15 minutes and 3 hours
248
What is the typical patient profile for cluster headaches?
- 30–50-year-old male
- Smoker
- Possible triggers: alcohol, strong smells, exercise
249
How severe is the pain in cluster headaches?
Extremely severe – sometimes called "suicide headaches" due to intensity
250
What are the typical unilateral symptoms associated with cluster headaches?
- Red, swollen, and watering eye
- Miosis (pupil constriction)
- Ptosis (eyelid drooping)
- Nasal discharge
- Facial sweating
251
What are the first-line treatments for an acute cluster headache attack?
- Subcutaneous or intranasal sumatriptan
- High-flow 100% oxygen (may be kept at home)
252
What is the first-line prophylactic treatment for cluster headaches + what are some other prophylactic options for cluster headaches?
- 1st-line --> Verapamil
.
Other prophylactic options:
- Occipital nerve block
- Prednisolone (e.g., a short course to break the cycle during clusters)
- Lithium
253
What is encephalitis?
Inflammation of the brain, caused by infective or non-infective (autoimmune) mechanisms
254
What is the most common cause of encephalitis?
Viral infection, particularly herpes simplex virus (HSV)
255
What are the common viral causes of encephalitis? (include in children and neonates
- In children --> HSV-1 from cold sores
- In neonates --> HSV-2 from genital herpes (contracted during birth)
.
Other viral causes include:
- Varicella zoster virus (VZV) – *associated with chickenpox*
- Cytomegalovirus (CMV) – *associated with immunodeficiency*
- Epstein-Barr virus (infectious mononucleosis), enterovirus, adenovirus, and influenza virus
- Polio, mumps, rubella, measles viruses - *ask about vaccination history*
256
What are the key symptoms of encephalitis?
- Altered consciousness
- Altered cognition
- Unusual behaviour
- Acute onset of focal neurological symptoms
- Acute onset of focal seizures
- Fever
257
What key investigations are needed for encephalitis?
1. Lumbar puncture – *CSF for viral PCR testing*
2. CT scan – *if lumbar puncture is contraindicated*
3. MRI scan – *for detailed brain imaging*
4. EEG – *helpful in mild or ambiguous cases*
5. Swabs – *throat or vesicle swabs to identify causative organism*
6. HIV testing – *recommended for all encephalitis cases*
258
When is a lumbar puncture contraindicated?
- GCS <9
- Haemodynamically unstable
- Active seizures or post-ictal state
259
What is the first-line antiviral for suspected encephalitis?
IV Aciclovir (treats HSV and VZV)
260
What antiviral is used for cytomegalovirus (CMV) encephalitis?
Ganciclovir
261
Why is a repeat lumbar puncture performed in the management of encephalitis?
To ensure successful treatment before stopping antivirals
262
What is the treatment for non-HSV viral encephalitis?
Supportive management (no specific antiviral)
263
What are potential long-term complications of encephalitis?
- Fatigue and prolonged recovery
- Personality & mood changes
- Memory & cognition issues
- Learning disability
- Headaches & chronic pain
- Movement disorders & sensory disturbance
- Seizures
- Hormonal imbalances
264
CSF findings in meningitis
think about it --> bacteria swimming in the CSF will release proteins and use up glucose
265
What are the common causes of brain abscesses?
- Extension of sepsis (middle ear, sinuses)
- Trauma or surgery to the scalp
- Penetrating head injuries
- Embolic events from endocarditis
266
What are the key symptoms of a brain abscess?
Smyptoms depend on site of the abscess, abscesses have a considerable mass effect in the brain and raised ICP is common:
- Headache (dull, persistent)
- Fever (may be absent, not typically swinging pyrexia)
- Focal neurology (depends on location, e.g., oculomotor or abducens nerve palsy secondary to raised ICP)
- Signs of raised ICP (nausea, papilloedema, seizures)
267
What is the key investigation for diagnosing a brain abscess?
CT brain scan (used for assessment and diagnosis) OR MRI brain
.
(image shows an MRI brain showing ring-enhancing lesion in the right frontal lobe with surrounding oedema)
268
What are the key management steps for a brain abscess?
- Surgery – Craniotomy to debride the abscess cavity
- IV Antibiotics – 3rd-generation cephalosporin + metronidazole
- Intracranial pressure management – Dexamethasone
269
What motor neurons are affected in amyotrophic lateral sclerosis (ALS)?
Both upper motor neurons (corticospinal tracts) and lower motor neurons
270
What are the key clinical signs of ALS?
Combination of upper motor neuron (UMN) signs (spasticity, hyperreflexia) and lower motor neuron (LMN) signs (muscle wasting, fasciculations)
271
Which part of the spinal cord is affected in poliomyelitis?
Anterior horns, leading to lower motor neuron signs
272
What are the affected spinal tracts in Brown-Séquard syndrome + clinical features associated?
Brown-Séquard Syndrome (spinal cord hemisection)
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1. Lateral corticospinal tract --> *Ipsilateral spastic paresis below the lesion*
2. Dorsal columns --> *Ipsilateral loss of proprioception & vibration sensation*
3. Lateral spinothalamic tract --> *Contralateral loss of pain & temperature sensation*
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What is the cause of subacute combined degeneration of the spinal cord?
Vitamin B12 and E deficiency
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What are the affected spinal tracts in Subacute combined degeneration of the spinal cord + clinical features associated?
1. Lateral corticospinal tracts --> *Bilateral spastic paresis*
2. Dorsal columns --> *Bilateral loss of proprioception and vibration sensation*
3. Spinocerebellar tracts --> *Bilateral limb ataxia*
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How does Friedreich’s ataxia present compared to subacute combined degeneration?
Same spinal tracts affected, but also includes cerebellar ataxia (e.g. intention tremor)
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What are the affected spinal tracts in anterior spinal artery occlusion + clinical features associated?
1. Lateral corticospinal tracts --> *Bilateral spastic paresis*
2. Lateral spinothalamic tracts --> *Bilateral loss of pain and temperature sensation*
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What are the affected spinal tracts in syringomyelia + clinical features associated?
1. Ventral horns --> *Flacid paresis (typically affecting the intrinsic hand muscles)*
2. Lateral spinothalamic tract --> *Loss of pain and temperature sensation*
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How does MS affect the spinal cord?
Asymmetrical, varying spinal tracts affected --> *causing a combination of motor, sensory, and ataxia symptoms*
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What spinal tracts are affected in neurosyphilis (tabes dorsalis) + associated clinical features
Dorsal columns --> *Loss of proprioception and vibration sensation*
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What is spinal stenosis?
Narrowing of the spinal canal, leading to compression of the spinal cord or nerve roots
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Which regions of the spine are most commonly affected by spinal stenosis?
Cervical and lumbar spine, with lumbar spinal stenosis being the most common
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What age group is most commonly affected by spinal stenosis?
Patients older than 60 years due to degenerative changes
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What are the three types of spinal stenosis?
1. Central stenosis – *narrowing of the central spinal canal*
2. Lateral stenosis – *narrowing of the nerve root canals*
3. Foraminal stenosis – *narrowing of the intervertebral foramina*
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What are some common causes of spinal stenosis?
- Congenital spinal stenosis
- Degenerative changes (facet joint changes, disc disease, bone spurs)
- Herniated discs
- Thickening of ligamenta flava or posterior longitudinal ligament
- Spinal fractures
- Spondylolisthesis (anterior vertebral displacement)
- Tumours
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How do symptoms of spinal stenosis typically present?
Gradual onset of symptoms that worsen with standing and walking, but improve with sitting and spinal flexion
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What is the key symptom of lumbar spinal stenosis with central stenosis?
Intermittent neurogenic claudication (also called pseudoclaudication)
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What are the symptoms of intermittent neurogenic claudication?
- Lower back pain
- Buttock and leg pain
- Leg weakness
- Symptoms relieved by bending forward (shopping cart sign --> *symptoms relieved by bending over shopping cart as this flexes the spine*)
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How can intermittent neurogenic claudication be differentiated from peripheral arterial disease (PAD)?
- Spinal stenosis: Normal pulses & ankle-brachial pressure index (ABPI), relieved by spinal flexion, back pain present
- PAD: Reduced pulses & ABPI, worsened by exertion, not relieved by posture changes, no back pain
.
ABPI and CT angiogram can both be used to help differentiate
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What symptoms are associated with lateral or foraminal stenosis?
Sciatica (radiculopathy) with motor and sensory symptoms
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What are the red flag symptoms of severe spinal stenosis?
Cauda equina syndrome (saddle anaesthesia, incontinence, sexual dysfunction)
- requiring emergency management
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What is the primary imaging investigation for diagnosing spinal stenosis?
MRI
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What are the management options for spinal stenosis?
1. Conservative --> *exercise and weight loss, analgesia, physiotherapy*
2. Decompression surgery (when conservative treatment fails) --> *laminectomy - partial/complete removal of the lamina from affected vertebra*
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What is cauda equina syndrome?
A surgical emergency caused by compression of the cauda equina nerve roots, requiring urgent decompression to prevent permanent neurological damage
- however, even with immediate decompression, patients may still not regain full function
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What is the cauda equina?
- a collection of nerve roots that travel through the spinal canal after the spinal cord terminates around L2/L3
- the spinal cord tapers down at the end in a section called the conus medullaris
- the nerve roots then exit either side of the spinal column at their respective vertebral level
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What functions do the cauda equina nerves supply?
- Sensation --> *to the lower limbs, perineum, bladder and rectum*
- Motor innervation --> *to the lower limbs, and the anal/urethral sphincters*
- Parasympathetic innervation --> *bladder and rectum control*
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What is the most common cause of cauda equina syndrome, and what are some other causes?
Herniated disc
.
Other:
- Tumours (especially metastases)
- Spondylolisthesis (vertebral displacement)
- Abscess (infection)
- Trauma
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What are the key red flag symptoms of cauda equina syndrome?
-Saddle anaesthesia (loss of sensation in the perineum – around the genitals and anus)
- Loss of bladder/rectal sensation (not knowing when they are 'full')
- Urinary retention or incontinence
- Faecal incontinence
- Bilateral sciatica
- Bilateral or severe motor weakness in the legs
- Reduced anal tone on PR examination
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How is cauda equina syndrome diagnosed + what is the management of cauda equina syndrome?
1. Immediate hospital admission
2. Urgent MRI scan - *to confirm or exclude cauda equina syndrome*
3. Neurosurgical input for emergency lumbar decompression surgery
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What are potential long-term complications of cauda equina syndrome, even after decompression surgery?
- Bladder, bowel, or sexual dysfunction
- Leg weakness
- Persistent sensory deficits
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How is MSCC (metastatic spinal cord compression) different from cauda equina syndrome?
- MSCC involves compression of the spinal cord (before the end of the spinal cord and the start of the cauda equina)
- MSCC therefore presents with UMN signs --> *increased tone, brisk reflexes, and upgoing plantar responses)*
- Whereas cauda equina presents with LMN signs (as the nerves being compressed are lower motor neurons that have already exited the spinal cord) --> *reduced tone and reduced reflexes*
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What are the key clinical features of MSCC (metastatic spinal cord compression)?
MSCC presents similarly to cauda equina, with back pain and motor/sensory signs and symptoms
- a key feature is back pain that is worse on coughing or straining
- MSCC also presents with UMN signs (increased tone, brisk reflexes, upgoing plantars)
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What is the emergency management of MSCC (metastatic spinal cord compression)?
1. High-dose dexamethasone (to reduce swelling and relieve compression)
2. Analgesia
3. Surgery, radiotherapy, or chemotherapy depending on cause
.
(a specialist MSCC coordinator should be involved to arrange imaging and treatment)
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What are the main cancers that metastasise to the bones (eg. spinal metastases)?
- Prostate
- Renal
- Thyroid
- Breast
- Lung
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What are the main risk factors for degenerative cervical myelopathy (DCM)?
- Smoking (affects intervertebral discs)
- Genetics
- Occupation (high axial loading)
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Why is degenerative cervical myelopathy (DCM) difficult to diagnose early + what is a key warning sign for DCM?
- Symptoms are subtle, variable, and fluctuate day to day, leading to delays in recognition
.
- Worsening, deteriorating, or new symptoms in a progressive manner
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What symptoms can DCM (degenerative cervical myelopathy) cause?
- Pain (neck, upper or lower limbs)
- Loss of motor function (loss of digital dexterity - preventing simple tasks such as doing up buttons/holding a fork, arm or leg weakness/stiffness - leading to impaired gait/balance)
- Loss of sensory function (numbness)
- Loss of autonomic function (urinary/faecal incontinence and/or impotence) --> *does not necessarily suggest cauda equina syndrome in the absence of other hallmark signs*
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What is Hoffmann’s sign, and what does it indicate?
a reflex test to assess for cervical myelopathy --> *flicking a finger causes involuntary twitching of other fingers on the same hand*
- a positive Hoffmann’s sign suggests cervical myelopathy
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What is the gold-standard investigation for DCM (degenerative cervical myelopathy)?
MRI of the cervical spine
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What is the key principle in managing DCM (degenerative cervical myelopathy) + what is the only effective treatment for DCM?
- Early referral to specialist spinal services (neurosurgery or orthopaedics) is essential to prevent permanent spinal cord damage
.
- Decompressive surgery is the only effective treatment (prevents progression)
.
(note: physiotherapy should only be initiated by specialist services, as manipulation can cause more spinal damage)
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Do cerebellar lesions cause ipsilateral or contralateral signs?
Ipsilateral signs
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What mnemonic is used to remember cerebellar disease symptoms?
DANISH:
- D - Dysdiadochokinesia, Dysmetria (past-pointing), patients may appear 'Drunk'
- A - Ataxia (limb, truncal)
- N - Nystamus (horizontal = ipsilateral hemisphere lesion)
- I - Intention tremour
- S - Slurred staccato speech (scanning dysarthria)
- H - Hypotonia
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Name some causes of cerebellar syndrome
- chronic alcohol abuse - *leading to cerebellar atrophy*
- stroke
- multiple sclerosis - *cerebellar demyelination*
- tumours - *cerebellar metastases, primary brain tumours (eg. cerebellar hemangioma)*
- drugs and toxins - *phenytoin, lead poisoning*
- herediatry ataxias - *Friedreich's ataxia, ataxic telangiectasia*
- paraneoplastic syndromes - *eg. secondary to lung cancer*
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What is hydrocephalus?
a condition in which there is an excessive volume of cerebrospinal (CSF) fluid within the ventricular system of the brain, caused by an imbalance between CSF production and absorption
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What are common symptoms of hydrocephalus?
Symptoms are due to raised intracranial pressure, which include:
- Headache (worse in morning/lying down/during Valsalva maneuver)
- Nausea and vomiting
- Papilloedema
- Coma (severe cases)
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How does hydrocephalus present in infants?
Since infants have skull sutures that are not yet fused, the rise in ICP caused by hydrocephalus will cause an increase in head circumference
- bulging of the anterior fontanelle
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What are the two categories of hydrocephalus?
Obstructive ('non-communicating') hydrocephalus:
- caused by a structural blockage that impedes CSF flow
- causes: * tumours, acute haemorrhage (e.g., subarachnoid or intraventricular haemorrhage), and developmental abnormalities (e.g., aqueduct stenosis)*
.
Non-obstructive ('communicating') hydrocephalus:
- due to an imbalance of CSF production absorption
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What is the first-line investigation for hydrocephalus?
CT head --> *fast and provides adequate resolution of brain and ventricles*
.
(MRI can be used if more details are needed - eg. underlying lesion)
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What role does lumbar puncture play in hydrocephalus?
*when should lumbar puncture not be used?
Lumbar puncture is both diagnostic and therapeutic - *allows you to sample CSF, measure opening pressure, but also to drain CSF to reduce the pressure*
.
*Lumbar puncture must not be used in obstructive hydrocephalus since the difference of cranial and spinal pressures induced by the drainage of CSF will cause brain herniation
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What are the treatment options for hydrocephalus?
- External ventricular drain (EVD) - *used in acute, severe hydrocephalus, CSF is drained into a bag at bedside*
- Ventriculoperitoneal (VP) shunt - *long-term CSF diversion technique that drains CSF from ventricles to peritoneum*
- Obstructive hydrocephalus --> *surgical removal of obstructing pathology*
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What is normal pressure hydrocephalus?
- a unique form of non-obstructive hydrocephalus, characterised by large ventricles, but normal ICP
- it is a reversible cause of dementia seen in elderly patients (can be secondary to reduced CSF absorption at the arachnoid villi, due to head injury, SAH, or meningitis)
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What is the classic triad of symptoms seen in normal pressure hydrocephalus?
- urinary incontinence
- dementia and bradyphrenia
- gait abnormality (may be similar to Parkinson's disease)
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What is the typical imaging finding in normal pressure hydrocephalus?
Imaging shows hydrocephalus with ventriculomegaly in the absence of, or out of proportion to, sulcal enlargement
.
(*CT scan of an elderly male patient showing hydrocephalus, with dilatation of the 3rd ventricle and lateral ventricels, with the characteristic absence of sulcal enlargement*)
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What is the main management option for normal pressure hydrocephalus + what are the potential complications in which 10% of patients will experience?
Ventriculoperitoneal (VP) shunting
.
10% of pts will experience complications such as:
- seizures
- infection
- intracerebral haemorrhages
324
Explain why in 'coning' of the brain, there is hypertension and bradycardia
- The brain autoregulates its blood supply, as ICP rises the systemic circulation will display changes to try and meet the perfusion needs of the brain --> usually this will involve hypertension
- As ICP rises further, the brain will be compressed, cranial nerve palsies may be seen and compression of essential centres in the brain stem will occur --> when the cardiac centre is involved bradycardia will often develop
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