Neurology Flashcards
1
Q
What is the glasgow coma scale?
A
When there are different motor responses on each side, the best motor response is used for scoring.
2
Q
What is status elipticus?
A
Status epilepticus is a medical emergency that refers to a prolonged seizure or a series of seizures without full recovery of consciousness between them. Specifically, it is defined as:
A seizure lasting more than 5 minutes.
Two or more seizures occurring without the person regaining full consciousness between them.
3
Q
What is the management of status elipticus?
A
ABC
airway adjunct
oxygen
check blood glucose
First-line drugs are benzodiazepines
in the prehospital setting PR diazepam or buccal midazolam may be given
in hospital IV lorazepam is generally used. This may be repeated once after 5-10minutes
If ongoing (or ‘established’) status it is appropriate to start a second-line agent such as levetiracetam, phenytoin or sodium valproate
NICE state ‘Take into account that levetiracetam may be quicker to administer and have fewer adverse effects than the alternative options.’
If no response (‘refractory status’) within 45 minutes from onset, then the best way to achieve rapid control of seizure activity is induction of general anaesthesia or phenobarbital
4
Q
What are the complications of meningitis?
A
Neurological sequalae
sensorineural hearing loss (most common)
seizures
focal neurological deficit
infective
sepsis
intracerebral abscess
pressure
brain herniation
hydrocephalus
Patients with meningococcal meningitis are at risk of Waterhouse-Friderichsen syndrome (adrenal insufficiency secondary to adrenal haemorrhage).
5
Q
What is the most common complication of meningitis?
A
Sensorineural hearing loss
6
Q
What lumbar puncture results suggest meningitis?
A
High white cell count, high protein and low glucose which are the typical findings of bacterial meningitis.
There is also the high opening pressure and the cloudy CSF which suggest infection.
7
Q
What is hoffmans sign?
A
Hoffmann’s sign is a pathological reflex where the thumb and index finger of the patient involuntarily flex when the examiner gently flicks or taps the nail of the middle finger (or sometimes the thumb) in a downward direction. indicate the presence of upper motor neuron dysfunction
8
Q
What is Degenerative cervical myelopathy?
A
Degenerative cervical myelopathy (DCM) is a progressive neurological condition caused by spinal cord compression in the cervical spine (the neck region) due to degenerative changes in the spine.
9
Q
What are the symptoms of DCM?
A
DCM symptoms can include any combination of [1]:
Pain (affecting the neck, upper or lower limbs)
Loss of motor function (loss of digital dexterity, preventing simple tasks such as holding a fork or doing up their shirt buttons, arm or leg weakness/stiffness leading to impaired gait and imbalance
Loss of sensory function causing numbness
Loss of autonomic function (urinary or faecal incontinence and/or impotence) - these can occur and do not necessarily suggest cauda equina syndrome in the absence of other hallmarks of that condition
Hoffman’s sign: is a reflex test to assess for cervical myelopathy. It is performed by gently flicking one finger on a patient’s hand. A positive test results in reflex twitching of the other fingers on the same hand in response to the flick.
10
Q
Kernigs sign?
A
Kernigs sign refers to painful knee extension, from a position of hip flexion and knee flexion. It suggest meningeal irritation e.g. meningitis, subarachnoid haemorrhage.
Straight leg raise: this is positively associated with radicular pathology such as disc herniation. The patient feels pain in the back when the leg is raised between 30-60 degrees.
The ankle brachial pressure index [ABPI] is a simple method of assessing the peripheral circulation. It is calculated by dividing systolic blood pressure in the ankle by the the systolic blood pressure in the arm. These are equal in health (ABPI = 1). The ABPI is reduced in peripheral vascular disease.
Tinels test includes tapping over the volar surface of the wrist joint i.e. over the carpal tunnel. This can reproduce paraesthesias in patients with carpal tunnel syndrome.
Limited external rotation of the shoulder 73%
This patient is likely to have adhesive capsulitis. Patients have global restriction of shoulder movements, in at least two axes, though external rotation is classically described as the most affected and painful.
11
Q
Features of Brown sequard syndrome
A
Unilateral spastic paresis and loss of proprioception/vibration sensation with loss of pain and temperature sensation on the opposite side
Tracts affected
1. Lateral corticospinal tract
2. Dorsal columns
3. Lateral spinothalamic tract
- Ipsilateral spastic paresis below lesion
- Ipsilateral loss of proprioception and vibration sensation
- Contralateral loss of pain and temperature sensation
12
Q
How does idiopathic intercrainal hypertension present?
A
The patient presents with a chronic headache that worsens in the morning and upon coughing or straining, which suggests elevated intracranial pressure. A CT scan of the head rules out a mass lesion. Clinical examination confirms papilloedema. Given her multiple risk factors for idiopathic intracranial hypertension’including contraceptive pill use, age, gender, and weight’this is the most likely diagnosis.
Features
headache
blurred vision
papilloedema (usually present)
enlarged blind spot
sixth nerve palsy may be present
13
Q
What are the risk factors for Idiopathic intercranial hypertension?
A
Risk factors
obesity
female sex
pregnancy
drugs
combined oral contraceptive pill
steroids
tetracyclines
retinoids (isotretinoin, tretinoin) / vitamin A
lithium
14
Q
What is the management of Idiopathic intercranial hypertension?
A
Management
weight loss
whilst diet and exercise are important, medications such as semaglitide and topiramate may be considered by specialists. Topiramate is particularly beneficial as it also inhibits carbonic anhydrase
carbonic anhydrase inhibitors e.g. acetazolamide
topiramate is also used, and has the added benefit of causing weight loss in most patients
repeated lumbar puncture may be used as a temporary measure but is not suitable for longer-term management
surgery: optic nerve sheath decompression and fenestration may be needed to prevent damage to the optic nerve. A lumboperitoneal or ventriculoperitoneal shunt may also be performed to reduce intracranial pressure
15
Q
What is normal pressure hydrocephalus?
A
Normal pressure hydrocephalus is a reversible cause of dementia seen in elderly patients. It is thought to be secondary to reduced CSF absorption at the arachnoid villi. These changes may be secondary to head injury, subarachnoid haemorrhage or meningitis.
16
Q
What is the classical triad seen in normal pressure hydrocephalus?
A
A classical triad of features is seen
urinary incontinence
dementia and bradyphrenia
gait abnormality (may be similar to Parkinson’s disease)
It is thought around 60% of patients will have all 3 features at the time of diagnosis. Symptoms typically develop over a few months.
17
Q
What is seen in imaging of normal pressure hydrocephalus?
A
hydrocephalus with ventriculomegaly in the absence of, or out of proportion to, sulcal enlargement
18
Q
What is the management of normal pressure hydrocephalus?
A
Management
ventriculoperitoneal shunting
around 10% of patients who have shunts experience significant complications such as seizures, infection and intracerebral haemorrhages
19
Q
What is Spastic paraparesis?
A
Spastic paraparesis describes a upper motor neuron pattern of weakness in the lower limbs
20
Q
What are the causes of Spastic paraparesis?
A
Causes
demyelination e.g. multiple sclerosis
cord compression: trauma, tumour
parasagittal meningioma
tropical spastic paraparesis
transverse myelitis e.g. HIV
syringomyelia
hereditary spastic paraplegia
osteoarthritis of the cervical spine
21
Q
What is amaurosis fugax?
A
amaurosis fugax means ‘transient darkening’ and it is used by doctors to describe a temporary loss of vision through one eye, which returns to normal afterwards. This is usually due to a temporary disturbance of the blood flow to the back of the eye.
22
Q
What are the feautures of Internuclear ophthalmoplegia?
A
impaired adduction of the eye on the same side as the lesion
horizontal nystagmus of the abducting eye on the contralateral side
23
Q
What is typically spared in motor neuron disease?
A
Motor neurone disease (MND), also known as amyotrophic lateral sclerosis (ALS), occurs when motor neurons in the brain and spinal cord degenerate. This typically leads to progressive weakness of limb, respiratory, and bulbar musculature.
The extraocular muscles are seemingly preserved in most MND patients, even until the terminal stage. Notably, eye movements and blinking are usually the last modes of communication available to terminal MND patients. The eye muscles appear to be better equipped to maintain their muscle-nerve contacts and are thereby less affected.
24
Q
What are the clues which point towards a diagnosis of motor neuron disease?
A
asymmetric limb weakness is the most common presentation of ALS
the mixture of lower motor neuron and upper motor neuron signs
wasting of the small hand muscles/tibialis anterior is common
fasciculations
the absence of sensory signs/symptoms
vague sensory symptoms may occur early in the disease (e.g. limb pain) but ‘never’ sensory signs
Other features
doesn’t affect external ocular muscles
no cerebellar signs
abdominal reflexes are usually preserved and sphincter dysfunction if present is a late feature
25
What is motor neuron disease?
Motor neuron disease is a neurological condition of unknown cause which can present with both upper and lower motor neuron signs. It rarely presents before 40 years and various patterns of disease are recognised including amyotrophic lateral sclerosis (ALS), progressive muscular atrophy and bulbar palsy.
26
What is the presentation of a basilar artery infarct?
This patient is presenting with locked-in syndrome which can occur in a basilar artery infarct. This blood vessel supplies the cerebellum, thalamus, occipital lobe, and brainstem. These strokes are rare and often devastating. There are 3 main presentations of patients experiencing a basilar artery infarct:
An acute decreased GCS and advanced motor symptoms.
Insidious, gradual deterioration in GCS and motor symptoms with a subsequent sudden advanced decrease in GCS and motor symptoms.
A 'herald hemiparesis' with associated headache and vision changes prior to the onset of permanent symptoms of motor loss.
27
Draw the circle of willis
28
What does Anterior cerebral artery infarct cause?
Contralateral hemiparesis and sensory loss, lower extremity > upper
29
What does middle cerebral artery infarct cause?
Contralateral hemiparesis and sensory loss, upper extremity > lower
Contralateral homonymous hemianopia
Aphasia
30
What does Posterior cerebral artery infarct cause?
Contralateral homonymous hemianopia with macular sparing
Visual agnosia
31
What does Weber's syndrome (branches of the posterior cerebral artery that supply the midbrain) cause?
Ipsilateral CN III palsy
Contralateral weakness of upper and lower extremity
32
What does Posterior inferior cerebellar artery (lateral medullary syndrome, Wallenberg syndrome) infarct cause?
Ipsilateral: facial pain and temperature loss
Contralateral: limb/torso pain and temperature loss
Ataxia, nystagmus
33
What does Anterior inferior cerebellar artery (lateral pontine syndrome) infarct cause?
Symptoms are similar to Wallenberg's (see above), but:
Ipsilateral: facial paralysis and deafness
34
What does Retinal/ophthalmic artery infarct cause?
Amaurosis fugax
35
How do lacunar strokes present?
present with either isolated hemiparesis, hemisensory loss or hemiparesis with limb ataxia
strong association with hypertension
common sites include the basal ganglia, thalamus and internal capsule
36
What is Mononeuritis multiplex?
Mononeuritis multiplex is simultaneous or sequential involvement of individual non-contiguous nerve trunks. It typically presents with acute or subacute loss of sensory and motor function of individual nerves. The pattern of involvement is asymmetric, however, as the disease progresses, deficit(s) becomes more confluent and symmetrical, making it difficult to differentiate from polyneuropathy.
37
What lesion is Homonymous hemianopia
incongruous defects
lesion of optic tract
38
What lesion is Homonymous hemianopia congruous defects:
lesion of optic radiation or occipital cortex
39
What lesion is Homonymous macula sparing:
lesion of occipital cortex
40
Homonymous quadrantanopias*
superior
lesion of the inferior optic radiations in the temporal lobe (Meyer's loop)
mnemonic = PITS (Parietal-Inferior, Temporal-Superior)
Right Superior Quadrantanopia Lesion Left Temporal Lobe - CVA
41
Homonymous quadrantanopias inferior
inferior: lesion of the superior optic radiations in the parietal lobe
mnemonic = PITS (Parietal-Inferior, Temporal-Superior)
42
Bitemporal hemianopia
lesion of optic chiasm
43
Bitemporal hemianopia
lesion of optic chiasm
upper quadrant defect > lower quadrant defect =
inferior chiasmal compression, commonly a pituitary tumour
44
Bitemporal hemianopia
lesion of optic chiasm
lower quadrant defect > upper quadrant defect =
superior chiasmal compression, commonly a craniopharyngioma
45
What is Wernicke's encephalopathy ?
Wernicke's encephalopathy is a neuropsychiatric disorder caused by thiamine deficiency which is most commonly seen in alcoholics.
46
What are the rarer causes of Wernicke's encephalopathy?
Rarer causes include persistent vomiting, aոοrеxiа nervosa, stomach cancer, and dietary deficiency.
47
What is the classic triad seen in Wernicke's encephalopathy?
A classic triad of ophthalmoplegia/nystagmus, ataxia and encephalopathy may occur.
Confusion, ataxia, nystagmus/ophthalmoplegia
48
What is the treatment of Wernicke's encephalopathy?
Treatment is with urgent replacement of thiamine
give Pabrinex (IV B/C vitamins)
49
If Wernickes encephalopathy is not treated what may develop?
Korsakoff's syndrome
50
What are the features of Korsakoff's syndrome ?
This is termed Wernicke-Korsakoff syndrome and is characterised by the addition of antero- and retrograde amnesia and confabulation in addition to the above symptoms.
51
What are the features of Wernickes encephalopathy?
oculomotor dysfunction
nystagmus (the most common ocular sign)
ophthalmoplegia: lateral rectus palsy, conjugate gaze palsy
gait ataxia
encephalopathy: confusion, disorientation, indifference, and inattentiveness
peripheral sensory neuropathy
52
What are the investigations for wernickes encephalopathy?
decreased red cell transketolase
MRI
53
What is the acute management for seizures?
check the airway and apply oxygen if appropriate
place the patient in the recovery position
if the seizure is prolonged give benzodiazepines
BNF recommend dose for rectal diazepam, repeated once after 10-15 minutes if necessary
Neonate 1.25 - 2.5 mg
Child 1 month - 1 year 5 mg
Child 2 years - 11 years 5 - 10 mg
Child 12 years - 17 years 10 mg
Adult 10 - 20 mg (max. 30 mg)
Elderly 10 mg (max. 15 mg)
Midazolam oromucosal solution may also be used:
Neonate 300 mcg/kg (unlicensced)
Child 1 - 2 months 300 mcg/kg (max. 2.5mg, unlicensced)
Child 3 - 11 months 2.5 mg
Child 1 - 4 years 5 mg
Child 5 - 9 years 7.5 mg
Child 10 - 17 years 10 mg
Adult 10 mg (unlicensced)
54
Features of third nerve palsy?
eye is deviated 'down and out'
ptosis
pupil may be dilated (sometimes called a 'surgical' third nerve palsy)
55
What are the causes of third nerve palsy?
Causes
diabetes mellitus
vasculitis e.g. temporal arteritis, SLE
false localizing sign* due to uncal herniation through tentorium if raised ICP
posterior communicating artery aneurysm
pupil dilated
often associated pain
cavernous sinus thrombosis
Weber's syndrome: ipsilateral third nerve palsy with contralateral hemiplegia -caused by midbrain strokes
other possible causes: amyloid, multiple sclerosis
*this term is usually associated with sixth nerve palsies but it may be used for a variety of neurological presentations
56
Should Anti parkinson drugs be stopped on acute admission?
Levodopa and other antiparkinsons drugs are 'critical' medicines which should not be stopped on acute admissions and must be delivered on time
57
Acute withdrawal of levodopa can precipitate to..
neuroleptic malignant syndrome
58
Painful third nerve palsy =
posterior communicating artery aneurysm
59
What is the first line treatment for parkinsons?
if the motor symptoms are affecting the patient's quality of life: levodopa
if the motor symptoms are not affecting the patient's quality of life: dopamine agonist (non-ergot derived), levodopa or monoamine oxidase B (MAO-B) inhibitor
60
What is the mechanism of action of Levadopa and what is it taken with?
Levodopa by various routes crosses the blood brain barrier, is decarboxylated to form dopamine
nearly always combined with a decarboxylase inhibitor (e.g. carbidopa or benserazide)
this prevents the peripheral metabolism of levodopa to dopamine outside of the brain and hence can reduce side effects
61
What are the side effects of Levadopa?
common adverse effects:
dry mouth
anorexia
palpitations
postural hypotension
psychosis
some adverse effects are due to the difficulty in achieving a steady dose of levodopa
end-of-dose wearing off: symptoms often worsen towards the end of dosage interval. This results in a decline of motor activity
'on-off' phenomenon: large variations in motor performance, with normal function during the 'on' period, and weakness and restricted mobility during the 'off' period
dyskinesias at peak dose: dystonia, chorea and athetosis (involuntary writhing movements)
these effects may worsen over time with - clinicians therefore may limit doses until necessary
it is important not to acutely stop levodopa, for example, if a patient is admitted to hospital
if a patient with Parkinson's disease cannot take levodopa orally, they can be given a dopamine agonist patch as rescue medication to prevent acute dystonia
62
What are the Dopamine receptor agonists?
Bromocriptine, cabergoline and pergolide (ergots) are orally active drugs that work on D1 and D2 receptors. They have limiting side effects – fibrotic reactions.
* Pramipexole and ropinirole are D2/3 selective receptor agonists that are better tolerated. Short half-life in plasma (6-8hrs) could be a problem
* Rotigotine newer agent delivered by a transdermal patch
* Apomorphine given by injection sometimes given to control the ‘off-effect’ of levodopa
63
What are the side effects of Dopamine receptor agonists?
ergot-derived dopamine receptor agonists (bromocriptine, cabergoline) have been associated with pulmonary, retroperitoneal and cardiac fibrosis. The Committee on Safety of Medicines advice that an echocardiogram, ESR, creatinine and chest x-ray should be obtained prior to treatment and patients should be closely monitored
patients should be warned about the potential for dopamine receptor agonists to cause impulse control disorders and excessive daytime somnolence
more likely than levodopa to cause hallucinations in older patients. Nasal congestion and postural hypotension are also seen in some patients
64
What are the MAO-B (Monoamine Oxidase-B) inhibitors and their mechanism of action?
e.g. selegiline, Rasagiline
inhibits the breakdown of dopamine secreted by the dopaminergic neurons
Combination with levodopa is more effective in relieving symptoms and prolonging life.
65
What is the mechanism of action of amantadine?
Antiviral drug discovered to be beneficial (1969) in PD.
* Increased dopamine release or direct action on dopamine receptors is
primarily responsible for its therapeutic effect.
* Less effective than levodopa or bromocriptine and action declines with time.
66
What are the side effects of amantadine?
side-effects include ataxia, slurred speech, confusion, dizziness and livedo reticularis
67
What are the COMT (Catechol-O-Methyl Transferase) inhibitors and mechanism of action?
e.g. entacapone, tolcapone
COMT is an enzyme involved in the breakdown of dopamine, and hence may be used as an adjunct to levodopa therapy
used in conjunction with levodopa in patients with established PD
68
What are the Acetylcholine Antagonists and mechanism of action?
block cholinergic receptors
now used more to treat drug-induced parkinsonism rather than idiopathic Parkinson's disease
help tremor and rigidity
e.g. procyclidine, benzotropine, trihexyphenidyl (benzhexol)
Muscarinic acetylcholine receptors exert an inhibitory effect on dopaminergic nerves, suppression of which compensates for a lack of dopamine.
69
What are the side effects of What are the Acetylcholine Antagonists ?
Trihexyphenidyl (Benzhexol), Orphenadrine and procyclidine can all be used, with usual anti-cholinergic side effects (e.g. dry mouth, constipation, impaired vision, urinary retention).
70
What are the features of sagittal sinus thrombosis?
may present with seizures and hemiplegia
parasagittal biparietal or bifrontal haemorrhagic infarctions are sometimes seen
'empty delta sign' seen on venography
71
What are the features of Sagittal sinus thrombosis?
may present with seizures and hemiplegia
parasagittal biparietal or bifrontal haemorrhagic infarctions are sometimes seen
'empty delta sign' seen on venography
72
What are the features of cavernous sinus thrombosis?
other causes of cavernous sinus syndrome: local infection (e.g. sinusitis), neoplasia, trauma
periorbital erythema and oedema
ophthalmoplegia: 6th nerve damage typically occurs before 3rd & 4th
trigeminal nerve involvement may lead to hyperaesthesia of upper face and eye pain
central retinal vein thrombosis
73
What are the features of lateral sinus thrombosis?
6th and 7th cranial nerve palsies
74
What is a rare but recognised adverse effect of lamotrigine therapy?
Stevens-Johnson syndrome
75
What are the features of PD?
Bradykinesia
poverty of movement also seen, sometimes referred to as hypokinesia
short, shuffling steps with reduced arm swinging
difficulty in initiating movement
Tremor
most marked at rest, 3-5 Hz
worse when stressed or tired, improves with voluntary movement
typically 'pill-rolling', i.e. in the thumb and index finger
Rigidity
lead pipe
cogwheel: due to superimposed tremor
Other characteristic features
mask-like facies
flexed posture
micrographia
drooling of saliva
psychiatric features: depression is the most common feature (affects about 40%); dementia, psychosis and sleep disturbances may also occur
impaired olfaction
REM sleep behaviour disorder
fatigue
autonomic dysfunction:
postural hypotension
76
What are the features of drug induced PD?
Drug-induced parkinsonism has slightly different features to Parkinson's disease:
motor symptoms are generally rapid onset and bilateral
rigidity and rest tremor are uncommon
77
What medication should be started if encephalitis is suspected?
Start aciclovir in all cases of suspected encephalitis
78
What is Myasthenia gravis?
Myasthenia gravis is an autoimmune disorder resulting in insufficient functioning acetylcholine receptors. Antibodies to acetylcholine receptors are seen in 85-90% of cases*. Myasthenia is more common in women (2:1)
79
What are the key features of Myasthenia gravis ?
The key feature is muscle fatigability - muscles become progressively weaker during periods of activity and slowly improve after periods of rest:
extraocular muscle weakness: diplopia
proximal muscle weakness: face, neck, limb girdle
ptosis
dysphagia
80
What is myasthenia gravis associated with?
Associations
thymomas in 15%
autoimmune disorders: pernicious anaemia, autoimmune thyroid disorders, rheumatoid, SLE
thymic hyperplasia in 50-70%
81
What are the investigations for myasthenia gravis?
single fibre electromyography: high sensitivity (92-100%)
CT thorax to exclude thymoma
CK normal
antibodies to acetylcholine receptors
positive in around 85-90% of patients
n the remaining patients, about about 40% are positive for anti-muscle-specific tyrosine kinase antibodies
Tensilon test: IV edrophonium reduces muscle weakness temporarily - not commonly used any more due to the risk of cardiac arrhythmia
82
What are the management for myasthenia gravis?
long-acting acetylcholinesterase inhibitors
pyridostigmine is first-line
immunosuppression is usually not started at diagnosis, but the majority of patients eventually require it in addition to long-acting acetylcholinesterase inhibitors:
prednisolone initially
azathioprine, cyclosporine, mycophenolate mofetil may also be used
thymectomy
83
What is the management for myasthenic crisis?
plasmapheresis
intravenous immunoglobulins
84
What is the firstline radiological investigation for suspected stroke?
non-contrast CT head scan
85
What is Bells palsy?
Bell's palsy may be defined as an acute, unilateral, idiopathic, facial nerve paralysis. The aetiology is unknown although the role of the herpes simplex virus has been investigated previously. The peak incidence is 20-40 years and the condition is more common in pregnant women.
86
What are the features of bells palsy?
lower motor neuron facial nerve palsy → forehead affected
in contrast, an upper motor neuron lesion 'spares' the upper face
patients may also notice
post-auricular pain (may precede paralysis)
altered taste
dry eyes
hyperacusis
87
What is the management of bells palsy?
Management
in the past a variety of treatment options have been proposed including no treatment, prednisolone only and a combination of antivirals and prednisolone
there is consensus that all patients should receive oral prednisolone within 72 hours of onset of Bell's palsy
there is an ongoing debate as to the value of adding in antiviral medications
Antiviral treatments alone are not recommended
UpToDate recommends the addition of antivirals for severe facial palsy
eye care is important to prevent exposure keratopathy
prescription of artificial tears and eye lubricants should be considered
If they are unable to close the eye at bedtime, they should tape it closed using microporous tape
88
What are the features of Wernicke's (receptive) aphasia?
Due to a lesion of the superior temporal gyrus. It is typically supplied by the inferior division of the left MCA
This area 'forms' the speech before 'sending it' to Broca's area. Lesions result in sentences that make no sense, word substitution and neologisms but speech remains fluent - 'word salad'
Comprehension is impaired
89
What are the features of Broca's (expressive) aphasia?
Due to a lesion of the inferior frontal gyrus. It is typically supplied by the superior division of the left MCA
Speech is non-fluent, laboured, and halting. Repetition is impaired
Comprehension is normal
90
What are the features of Conduction aphasia?
Classically due to a stroke affecting the arcuate fasiculus - the connection between Wernicke's and Broca's area
Speech is fluent but repetition is poor. Aware of the errors they are making
Comprehension is normal
91
What are the features of Global aphasia?
Large lesion affecting all 3 of the above areas resulting in severe expressive and receptive aphasia
May still be able to communicate using gestures
92
What is the management of acute ischaemic stroke?
1.blood pressure control should be considered for patients who present with an acute ischaemic stroke, if they present within 6 hours and have a systolic blood pressure > 150 mmHg
aspirin 300mg orally or rectally should be given as soon as possible if a haemorrhagic stroke has been excluded
2.with regards to atrial fibrillation, the RCP state: 'anticoagulants should not be started until brain imaging has excluded haemorrhage, and usually not until 14 days have passed from the onset of an ischaemic stroke'
3.if the cholesterol is > 3.5 mmol/l patients should be commenced on a statin. Many physicians will delay treatment until after at least 48 hours due to the risk of haemorrhagic transformation
The standard criteria for thrombolysis with alteplase or tenecteplase are as follows:
it is administered within 4.5 hours of onset of stroke symptoms
haemorrhage has been definitively excluded (i.e. Imaging has been performed)
Blood pressure should be lowered to 185/110 mmHg before thrombolysis.
93
What are the absolute contradictions for thrombolysis?
Previous intracranial haemorrhage
- Seizure at onset of stroke
- Intracranial neoplasm
- Suspected subarachnoid haemorrhage
- Stroke or traumatic brain injury in preceding 3 months
- Lumbar puncture in preceding 7 days
- Gastrointestinal haemorrhage in preceding 3 weeks
- Active bleeding
- Oesophageal varices
- Uncontrolled hypertension >200/120mmHg
94
What are the relative contradictions for thrombolysis?
Pregnancy
- Concurrent anticoagulation (INR >1.7)
- Haemorrhagic diathesis
- Active diabetic haemorrhagic retinopathy
- Suspected intracardiac thrombus
- Major surgery / trauma in the preceding 2 weeks
95
Thrombectomy for acute ischaemic stroke
NICE recommend a pre-stroke functional status of less than 3 on the modified Rankin scale and a score of more than 5 on the National Institutes of Health Stroke Scale (NIHSS)
Offer thrombectomy as soon as possible and within 6 hours of symptom onset, together with intravenous thrombolysis (if within 4.5 hours), to people who have:
acute ischaemic stroke and
confirmed occlusion of the proximal anterior circulation demonstrated by computed tomographic angiography (CTA) or magnetic resonance angiography (MRA)
Offer thrombectomy as soon as possible to people who were last known to be well between 6 hours and 24 hours previously (including wake-up strokes):
confirmed occlusion of the proximal anterior circulation demonstrated by CTA or MRA and
if there is the potential to salvage brain tissue, as shown by imaging such as CT perfusion or diffusion-weighted MRI sequences showing limited infarct core volume
Consider thrombectomy together with intravenous thrombolysis (if within 4.5 hours) as soon as possible for people last known to be well up to 24 hours previously (including wake-up strokes):
who have acute ischaemic stroke and confirmed occlusion of the proximal posterior circulation (that is, basilar or posterior cerebral artery) demonstrated by CTA or MRA and
if there is the potential to salvage brain tissue, as shown by imaging such as CT perfusion or diffusion-weighted MRI sequences showing limited infarct core volume
96
What is the secondary prevention of acute ischaemic stroke?
Recommendations from NICE include:
clopidogrel is now recommended by NICE ahead of combination use of aspirin plus modified-release (MR) dipyridamole in people who have had an ischaemic stroke
aspirin is recommended after an ischaemic stroke only if clopidogrel is contraindicated or not tolerated
97
What does the radial nerve supply?
The radial nerve supplies the extensor muscle group.
98
What does the ulnar nerve supply?
The interossei are supplied by the ulnar nerve.
99
Which nerve is responsible for sensory deficit overlying the point of deltoid insertion into the humerus.
axillary nerve
99
What is the origin of the brachial plexus?
Anterior rami of C5 to T1
99
Difference between syncopal episodes and seizures
Syncopal episodes are associated with a rapid recovery and short post-ictal period. Seizures are associated with a far greater post-ictal period
100
What conditions have an association with epilepsy?
cerebral palsy: around 30% have epilepsy
tuberous sclerosis
mitochondrial diseases
101
What are non epileptic causes of recurrent seizures?
Febrile convulsions
Alcohol withdrawal seizures
Psychogenic non-epileptic seizures
102
What are Psychogenic non-epileptic seizures?
previously termed pseudoseizures, this term describes patients who present with epileptic-like seizures but do not have characteristic electrical discharges
patients may have a history of mental health problems or a personality disorder
103
What are the features of medication overuse headaches?
present for 15 days or more per month
developed or worsened whilst taking regular symptomatic medication
patients using opioids and triptans are at most risk
may be psychiatric co-morbidity
This question aims to tackle two key aspects of medication overuse headache (MOH) diagnosis.
The first key concept is that patients that have been taking opioid analgesia (codeine or co-codamol in particular) for an extended period, such as this patient, are at risk of MOH. For opioids and triptans, this overuse is defined as 'using the medication on 10 days or more per month, for 3 months or more'. However, a history of regular medication overuse only forms part of the definition of MOH, and the diagnosis remains 'probable MOH'.
The second key concept is that in order to have a definitive diagnosis of MOH, the history of overuse alone is not sufficient - the patient's symptoms must resolve (or revert back to their original pattern) within 2 months of stopping the causative medication.
MOH usually presents with a tension-type headache, but it can also have migraine-like characteristics, or may even flip between the two within the same day. Overall, however, unilateral headache with visual disturbance is more in keeping with a diagnosis of migraine, and would not be a defining feature of MOH diagnosis. Keep in mind though that patients who have frequent migraines and take regular triptans are also at risk of developing medication overuse headaches.
Medication overuse headaches are generally worse after exercise.
Medication overuse headaches are generally worst in the morning.
104
Vision worse going down stairs, vertical diplopia, classically noticed when reading a book or going downstairs
trochlear nerve palsy
vertical diplopia
classically noticed when reading a book or going downstairs
subjective tilting of objects (torsional diplopia)
the patient may develop a head tilt, which they may or may not be aware of
when looking straight ahead, the affected eye appears to deviate upwards and is rotated outwards
105
What is the acute management of migraines?
first-line: offer combination therapy with
an oral triptan and an NSAID, or
an oral triptan and paracetamol
for young people aged 12-17 years consider a nasal triptan in preference to an oral triptan
if the above measures are not effective or not tolerated offer a non-oral preparation of metoclopramide or prochlorperazine and consider adding a non-oral NSAID or triptan
caution should be exercised when prescribing metoclopramide to young patients as acute dystonic reactions may develop
It should be noted that as a general rule 5-HT receptor agonists are used in the acute treatment of migraine whilst 5-HT receptor antagonists are used in prophylaxis.
106
What is given for migraine prophylaxis?
prophylaxis should generally be given if 'Migraine attacks are having a significant impact on quality of life and daily function, for example they occur frequently (more than once a week on average) or are prolonged and severe despite optimal acute treatment'
options:
propranolol
topiramate: should be avoided in women of childbearing age as it may be teratogenic and it can reduce the effectiveness of hormonal contraceptives
amitriptyline
if these measures fail NICE recommends 'a course of up to 10 sessions of acupuncture over 5-8 weeks'
NICE recommend: 'Advise people with migraine that riboflavin (400 mg once a day) may be effective in reducing migraine frequency and intensity for some people'
for women with predictable menstrual migraine treatment NICE recommend either frovatriptan (2.5 mg twice a day) or zolmitriptan (2.5 mg twice or three times a day) as a type of 'mini-prophylaxis'
treatment options that may be considered by specialists, but fall outside the NICE guidelines:
candesartan: recommended by the British Association for the Study of Headache guidelines
monoclonal antibodies directed against the calcitonin gene-related peptide (CGRP) receptor: examples include erenumab
pizotifen is no longer recommended. Adverse effects such as weight gain & drowsiness are common
107
What are the DVLA rules for driving epilepsy/seizures?
Epilepsy/seizures - all patient must not drive and must inform the DVLA
first unprovoked/isolated seizure: 6 months off if there are no relevant structural abnormalities on brain imaging and no definite epileptiform activity on EEG. If these conditions are not met then this is increased to 12 months
for patients with established epilepsy or those with multiple unprovoked seizures:
may qualify for a driving licence if they have been free from any seizure for 12 months
if there have been no seizures for 5 years (with medication if necessary) a 'til 70 licence is usually restored
withdrawawl of epilepsy medication: should not drive whilst anti-epilepsy medication is being withdrawn and for 6 months after the last dose
108
Difference between TIA and stroke?
Stroke:
A stroke is a medical emergency that results in permanent damage to the brain due to prolonged disruption of blood flow.
The symptoms of a stroke last longer than 24 hours and typically do not resolve on their own.
In some cases, a stroke can cause irreversible brain damage and result in lasting neurological deficits such as paralysis, speech difficulties, or cognitive impairment.
TIA (Transient Ischemic Attack):
A TIA, often called a "mini-stroke," is characterized by temporary neurological symptoms that usually last for a few minutes to a few hours (typically less than 24 hours).
The symptoms of a TIA resolve completely within a short time, without causing permanent brain damage.
A TIA is a warning sign that a full stroke could occur in the future, and it should be taken very seriously.
109
What is the mechanism of action of Pyridostigmine ?
Pyridostigmine is a long-acting acetylcholinesterase inhibitor that reduces the breakdown of acetylcholine in the neuromuscular junction, temporarily improving symptoms of myasthenia gravis
used in the treatment of myasthenia gravis
110
What are the features of cluster headache?
-intense sharp, stabbing pain around one eye
pain typical occurs once or twice a day, each episode lasting 15 mins - 2 hours
the patient is restless and agitated during an attack due to the severity
clusters typically last 4-12 weeks
accompanied by redness, lacrimation, lid swelling
nasal stuffiness
miosis and ptosis in a minority
Episodic, intense, unilateral eye pain, lacrimation, restless → ?cluster headache
111
What is the management of a cluster headache?
NICE recommend seeking specialist advice from a neurologist if a patient develops cluster headaches
acute
100% oxygen (80% response rate within 15 minutes)
subcutaneous triptan (75% response rate within 15 minutes)
112
What is the prophylaxis of cluster headache?
verapamil is the drug of choice
there is also some evidence to support a tapering dose of prednisolone
113
What are the investigations for cluster headache?
most patients will have neuroimaging - underlying brain lesions are sometimes found even if the clinical symptoms are typical for cluster headache
MRI with gadolinium contrast is the investigation of choice
114
postural instability, impairment of vertical gaze, parkinsonism, frontal lobe dysfunction=
Progressive supranuclear palsy
115
What is the management of Post-lumbar puncture headache?
supportive initially (analgesia, rest)
if pain continues for more than 72 hours then specific treatment is indicated, to prevent subdural haematoma
treatment options include: blood patch, epidural saline and intravenous caffeine
116
The most characteristic feature of a common peroneal nerve lesion is...
foot drop.
117
What are features of common peroneal nerve lesion?
The most characteristic feature of a common peroneal nerve lesion is foot drop.
Other features include:
weakness of foot dorsiflexion
weakness of foot eversion
weakness of extensor hallucis longus
sensory loss over the dorsum of the foot and the lower lateral part of the leg
wasting of the anterior tibial and peroneal muscles
118
What is given first-line for spasticity in multiple sclerosis?
baclofen and gabapentin are first-line
119
A woman of childbearing age who presents with generalised tonic-clonic seizures should be offered
lamotrigine or levetiracetam
Ladies of childbearing age can have Lamotrigine or Levetiracetam. However, it's worth noting that lamotrigine can exacerbate myoclonic seizures.
120
What is the treatment for Generalised tonic-clonic seizures?
males: sodium valproate
females: lamotrigine or levetiracetam
girls aged under 10 years and who are unlikely to need treatment when they are old enough to have children or women who are unable to have children may be offered sodium valproate first-line
121
What is the treatment for Focal seizures?
first line: lamotrigine or levetiracetam
second line: carbamazepine, oxcarbazepine or zonisamide
122
What is the treatment for Absence seizures (Petit mal)?
first line: ethosuximide
second line:
male: sodium valproate
female: lamotrigine or levetiracetam
carbamazepine may exacerbate absence seizures
123
What is the treatment for Myoclonic seizures?
males: sodium valproate
females: levetiracetam
124
What is the treatment for Tonic or atonic seizures?
males: sodium valproate
females: lamotrigine
125
Jacksonian movement (clonic movements travelling proximally) indicates
frontal lobe epilepsy
Head/leg movements, posturing, post-ictal weakness, Jacksonian march
126
Temporal lobe seizures are associated with
aura, lip smacking and clothes plucking.
May occur with or without impairment of consciousness or awareness
An aura occurs in most patients
typically a rising epigastric sensation
also psychic or experiential phenomena, such as dejà vu, jamais vu
less commonly hallucinations (auditory/gustatory/olfactory)
Seizures typically last around one minute
automatisms (e.g. lip smacking/grabbing/plucking) are common
127
Occipital seizures are associated with
visual abnormalities.Floaters/flashes
128
Parietal seizures are associated with
sensory abnormalities.Paraesthesia
129
What is Juvenile myoclonic epilepsy?
Juvenile myoclonic epilepsy is a genetic generalised epilepsy syndrome including absence, myoclonic and generalised tonic-clonic seizures.
130
What is Guillain-Barre syndrome?
Guillain-Barre syndrome describes an immune-mediated demyelination of the peripheral nervous system often triggered by an infection (classically Campylobacter jejuni).
Initial symptoms
around 65% of patients experience back/leg pain in the initial stages of the illness
The characteristic features of Guillain-Barre syndrome is progressive, symmetrical weakness of all the limbs.
the weakness is classically ascending i.e. the legs are affected first
reflexes are reduced or absent
sensory symptoms tend to be mild (e.g. distal paraesthesia) with very few sensory signs
131
How do pontine haemorrhages present?
Reduced GCS, paralysis and bilateral pin point pupils
may also have signs of seizure, tetraparesis, and cranial nerve palsy.
132
What investigation should be used to view demyelinating lesions?
MRI with contrast
133
What is Pituitary apoplexy?
Sudden enlargement of a pituitary tumour (usually non-functioning macroadenoma) secondary to haemorrhage or infarction.
134
What are the features of Pituitary apoplexy?
Features
sudden onset headache similar to that seen in subarachnoid haemorrhage
vomiting
neck stiffness
visual field defects: classically bitemporal superior quadrantic defect
extraocular nerve palsies
features of pituitary insufficiency
e.g. hypotension/hyponatraemia secondary to hypoadrenalism
135
What are the Precipitating factors for pituitary apoplexy?
hypertension
pregnancy
trauma
anticoagulation
136
How does extradural haemorrhages present?
classic history is patient who initially loses, briefly regains and then loses again consciousness after a low-impact head injury - the lucid interval
low-impact' trauma (e.g. a blow to the head or a fall)
As the haematoma expands the uncus of the temporal lobe herniates around the tentorium cerebelli and the patient develops a fixed and dilated pupil due to the compression of the parasympathetic fibers of the third cranial nerve.
137
What is the most common bleeding site in extradural haemorrhage?
middle meningeal artery underlying the pterion - the weakest point of the cranium.
Temporal region tenderness
138
How does extradural haematomas look on imaging?
On imaging, an extradural haematoma appears as a biconvex (or lentiform), hyperdense collection around the surface of the brain. They are limited by the suture lines of the skull.
139
You want to prescribe an antiemetic to a 19-year-old female who is having a migraine attack. Which medication is most likely to precipitate extrapyramidal side-effects?
Metoclopramide. Metoclopramide is a dopamine antagonist
140
What are the causes of ataxic gate?
mnemonic 'pastries'
P - Posterior fossa tumour
A - Alcohol
S - Multiple sclerosis
T - Trauma
R - Rare causes
I - Inherited (e.g. Friedreich's ataxia)
E - Epilepsy treatments
S - Stroke
141
Cerebellar vermis lesions cause
gait ataxia
142
Cerebellar hemisphere lesions cause
peripheral ('finger-nose ataxia')
143
What blood test can differentiate true seizures from pseudoseizures?
Prolactin
as prolactin is specifically raised following true epileptiform seizures, compared to pseudoseizures
144
What medication needs to be given before CSF drainage using an external ventricular drain?
IV mannitol
IV mannitol is an osmotic diuretic that may be used in patients with raised ICP
145
What is the causes of Thoracic outlet syndrome ?
Thoracic outlet syndrome (TOS) is a disorder involving compression of brachial plexus, subclavian artery or vein at the site of the thoracic outlet.
146
What drugs may exacerbate myasthenia:
penicillamine
quinidine, procainamide
beta-blockers
lithium
phenytoin
antibiotics: gentamicin, macrolides, quinolones, tetracyclines
147
Features of vestibular schwannoma
The classical history of vestibular schwannoma includes a combination of vertigo, hearing loss, tinnitus and an absent corneal reflex. Features can be predicted by the affected cranial nerves:
cranial nerve VIII: vertigo, unilateral sensorineural hearing loss, unilateral tinnitus
cranial nerve V: absent corneal reflex
cranial nerve VII: facial palsy
148
What is the stepwise treatment of paediatric status elipticus?
Alongside other supportive measures, medical treatment proceeds in a stepwise manner:
1 Buccal midazolam/ IV lorazepam
2 IV lorazepam
3 IV phenytoin, levetiracetam or sodium valproate
4 Rapid sequence induction of anaesthesia using thiopental sodium
Since the child has already been given two doses of IV lorazepam, the most appropriate next step is IV phenytoin. It must be noted that if the child had previously been diagnosed with epilepsy and was already on phenytoin, then phenobarbital would be used instead.
149
What s the preferred way to support nutrition in patents with motor neuron disease
Percutaneous gastrostomy tube (PEG)
150
What is Autonomic dysreflexia?
This clinical syndrome occurs in patients who have had a spinal cord injury at, or above T6 spinal level. Briefly, afferent signals, most commonly triggered by faecal impaction or urinary retention (but many other triggers have been reported) cause a sympathetic spinal reflex via thoracolumbar outflow. The usual, centrally mediated, parasympathetic response however is prevented by the cord lesion.
151
What are features of Autonomic dysreflexia?
Features
the result is an unbalanced physiological response, characterised by extreme hypertension
flushing and sweating above the level of the cord lesion
agitation
in untreated cases, severe consequences of extreme hypertension have been reported, e.g. haemorrhagic stroke.
152
Headache red flags?
Vomiting more than once with no other cause.
New neurological deficit (motor or sensory).
Reduction in conscious level (as measured by the Glasgow coma score).
Valsalva (associated with coughing or sneezing) or positional headaches.
Progressive headache with a fever.
153
What is the management for neuropathic pain?
first-line treatment*: amitriptyline, duloxetine, gabapentin or pregabalin
if the first-line drug treatment does not work try one of the other 3 drugs
in contrast to standard analgesics, drugs for neuropathic pain are typically used as monotherapy, i.e. if not working then drugs should be switched, not added
tramadol may be used as 'rescue therapy' for exacerbations of neuropathic pain
topical capsaicin may be used for localised neuropathic pain (e.g. post-herpetic neuralgia)
pain management clinics may be useful in patients with resistant problems
*please note that for some specific conditions the guidance may vary. For example carbamazepine is used first-line for trigeminal neuralgia
154
Common peroneal nerve lesion can cause..
weakness of foot dorsiflexion and foot eversion
155
What are the investigations for Guillain-Barre syndrome?
lumbar puncture
rise in protein with a normal white blood cell count (albuminocytologic dissociation) - found in 66%
nerve conduction studies may be performed
decreased motor nerve conduction velocity (due to demyelination)
prolonged distal motor latency
increased F wave latency
156
How does Syringomyelia classically present?
cape-like loss of pain and temperature sensation due to compression of the spinothalamic tract fibres decussating in the anterior white commissure of the spine
classic examples are of patients who accidentally burn their hands without realising
this is due to the crossing spinothalamic tracts in the anterior commissure of the spinal cord being the first tracts to be affected
157
What is Syringomyelia?
syringomyelia, where a fluid-filled cyst (known as a syrinx) forms within the spinal cord and blocks the cerebrospinal fluid circulation. The syrinx commonly starts out in the cervical area, extending downwards through the spinal cord. It compresses the decussating spinothalamic tract, affecting pain and temperature sensation, and the corticospinal tract, causing weakness.
158
159
What are the causes of Syringomyelia?
a Chiari malformation: strong association
trauma
tumours
idiopathic
160
What is Subacute combined degeneration of the spinal cord?
Subacute combined degeneration of the spinal cord is due to vitamin B12 deficiency resulting in impairment of the dorsal columns, lateral corticospinal tracts and spinocerebellar tracts..
Recreational nitrous oxide inhalation may also result in vitamin B12 deficiency → subacute combined degeneration of the spinal cord.
161
What are features of Subacute combined degeneration of the spinal cord?
dorsal column involvement
distal tingling/burning/sensory loss is symmetrical and tends to affect the legs more than the arms
impaired proprioception and vibration sense
lateral corticospinal tract involvement
muscle weakness, hyperreflexia, and spasticity
upper motor neuron signs typically develop in the legs first
brisk knee reflexes
absent ankle jerks
extensor plantars
spinocerebellar tract involvement
sensory ataxia → gait abnormalities
positive Romberg's sign
162
What are the features of Duchenne muscular dystrophy?
progressive proximal muscle weakness from 5 years
calf pseudohypertrophy
Gower's sign: child uses arms to stand up from a squatted position
30% of patients have intellectual impairment
163
What are the features of Becker muscular dystrophy?
develops after the age of 10 years
intellectual impairment much less common
calf pseudohypertrophy
Gower's sign: child uses arms to stand up from a squatted position
164
sustained a fall on an outstretched hand On examination, you notice that he has lost the ability to abduct his thumb.
Which nerve is most likely to have been injured?
median
