Neurology Flashcards
(147 cards)
1
Q
What are the classifications of stroke
A
Classification
▪ Ischaemic stroke = 85%
• 30 % = Large vessel disease
- Carotid/vertebral/subclavian artery stenosis
- secondary to atherosclerosis →arterial plaque
embolism
• 20% = Cardioembolic
o AF (and other arrhythmias), infective
endocarditis, patent foramen ovale
• 15% = Small vessel disease
o Hypertensive disorders and lipohyalinosis →
occlusion of small penetrating arteries →
lacunar strokes
• 15% = Unknown aetiology
• 5% = Rarer causes
o Hypotension, dissection, venous infarction,
vasculopathy
▪ Haemorrhagic stroke = 10%
▪ Other = 5%
2
Q
Systematically describe the risk factors for stroke
A
COMORBIDITIES
- Hypertension
- AF
- Hypercholesterolaemia
- Diabetes
- Carotid stenosis
LESS COMMON RISK FACTORS
Polycythaemia/thrombocytosis/thrombophilia/hyperviscosity • Antiphospholipid syndrome • Oestrogen supplementation • Vasculitis • Migraine with aura • HIV and neurosyphilis • Amphetamine/cocaine use
LIFESTYLE
smoking
alcohol
obesity and inactivity
3
Q
How does stroke present? (not specific symptoms but what do all strokes have in common)
A
Most common presentation is the acute onset of focal deficits that are persistent
4
Q
What investigation would you undertake for stroke within
(a) the first hour
(b) the first 24hrs
or;
(c) if initial investigations are negative
A
Within 1-hour:
• CT or MRI brain
Often not sensitive for infarction for 24-48 hours
• FBC and BSL
Within 24-hours:
- FBC, BSL, coags, lipids, ESR
- ECG (assess for AF)
- Carotid artery doppler
If unknown cause, consider further investigation:
- CT/MRI angiography
- Echocardiogram
- Telemetry
- Vasculitis, thrombophilia, and antiphospholipid screen
5
Q
In a systematic manner, describe ACUTE management of stroke
A
- ABCDE assessment
▪ Assess for thrombolysis eligibility
▪ Brain imaging (CT or MRI non contrast)
Haemorrhage → neurosurgery consultation
No haemorrhage → thrombolysis + aspirin
▪ Admission to stroke unit - Antiplatelet therapy and anticoagulation• Antiplatelet therapyo Initiate 300mg of aspirin per day:
- Straight away if thrombolysis is contraindicated
- After 24-hours if thrombolysis is given
o Switch to clopidogrel after 2-weeks• Anticoagulants
o Anticoagulants (DOACs or warfarin) are
initiated after 2-weeks if the stroke
is of cardioembolic aetiologyo There is a 2-week wait due to risk of haemorrhagic transformation of ischaemic stroke
▪ Decompressive craniectomy (if needed)
- May be performed in cases of massive MCA
stroke causing >50% infarction of the
MCA vascular territory → raised ICP
6
Q
what kind of a things do they do for a patient in the stroke unit.
A
-Thromboembolism prophylaxis (aspirin → clopidogrel)
• Swallowing assessment
• Assessment and management of medical complications
o Infection, hyper/hypoglycaemia, AF
• Assessment and management of risk factors
• Initiate secondary prophylaxis and give lifestyle advice
• MDT management with physiotherapy, OT, and speech therapy
7
Q
what are the eligibility criteria for thrombolysis in a pt coming in with acute stroke
A
ELIGIBILITY
- Pt with persistent neurological deficit diagnosed as stroke by an experienced medical team
- Haemorrhagic stroke excluded by CT
- Onset of stroke is well established
- thrombolysis can occur within 4.5 hrs
8
Q
what are the exclusion criteria/contraindications for thrombolysis in a pt presenting with acute stroke
A
There are historical, clinical and laboratory exclusion criteria for stroke
HISTORY
- stroke or trauma in the last 3 months
- any ICH in the past
- major surgery within 2 weeks
- GI or GU bleed in last 3 weeks
- arterial puncture in a non compressible site in the last 7 days
- LP in last 7 days
CLINICAL
- rapidly improving neuro signs
- isolated and minor neuro signs
- seizure at the time of stroke if residual symptoms are due to post ictal phenomena
- symptoms suggestive of SAH even if CT is negative
- persistently high BP >185/110
- active bleeding or acute trauma
LABORATORY
- Platelets <100 000
- INR >1.3 on warfarin
- PTT elevated on heparin
- BSL = <2.8 or >26.2
9
Q
List and briefly describe secondary stroke prevention after an acute stroke
A
▪ Antihypertensive therapy
• There is often a transient compensatory rise in BP following a stroke
• BP should be lowered gradually to prevent hypotensive-hypoperfusion
▪ Hypercholesterolaemia management
• Atorvastatin 40mg → aim for total cholesterol <4mmom/L
▪ Lifestyle changes
• Smoking/alcohol cessation, diet change, exercise, weight loss,
▪ Surgery
• Carotid endarterectomy should be performed within 2-week if there is 70-99%
stenosis
▪ Rehabilitation
• Early physiotherapy → relieve spasticity and prevent contractures; assess
eligibility for walking aids
• Speech therapy and further assessment of swallowing
• Psychological support
10
Q
what is the prognosis for a stroke
A
Prognosis
▪ 25% die within 2-years; 10% within 1-month
• Higher for haemorrhage vs. stroke
▪ Improvement of symptoms usually peaks ~12-months post-stroke
11
Q
Which arteries make up the anterior circulation
A
MCA, ACA, Internal carotid, lenticulostriate arteries
12
Q
what are expected presentations of the anterior circulation strokes. List them separately
A
Anterior Circulation Infarcts
Anterior circulation = ACA, MCA, and ophthalmic arteries
Complete MCA stroke (most common)
• Contralateral hemiplegia and facial weakness (arms > legs)
• Hemianopia
• Parietal lobe involvement
o Hemisensory loss
o Neglect syndromes (if non-dominant side
affected)
o Aphasia
Complete ACA stroke • Hemiparesis (leg > arm) • Frontal lobe defects (apathy, apraxia) ▪ Internal carotid artery occlusion • Presents similarly to MCA stroke, but more likely to only have partial signs due to anastomoses
13
Q
What is medial medullary syndrome?
Which types of stroke causes them?
What neurological structures are affected
What are the resulting deficits
A
Medial Medullary Syndrome
• Pathology of the PICA or vertebral artery → damaged hypoglossal nerve,
corticospinal tract, and dorsal column
• Ipsilateral tongue palsy, contralateral hemiparesis, and contralateral impaired
proprioception
14
Q
what presentation would you expect in a Posterior cerebral artery stroke?
A
Homonymous hemianopia (unilateral) or cortical blindness (bilateral) • Thalamus and temporal lobe involvement → confusion and memory impairment
15
Q
Expected presentation in a brainstem infarct
A
Brainstem Infarct
• Variable presentation depending on the affected CN nuclei and spinal cord tracts
• E.g hemiparesis (corticospinal tract), sensory loss (dorsal column/spinothalamic
tracts), facial numbness/weakness, nystagmus, dysarthria/dysphagia (CN
involvement), Horner’s syndrome (sympathetic fibres), coma (reticular formation)
16
Q
what presentation would you expect in a cerebellar infarct?
A
Cerebellar Infarcts
• Can occur by itself or with brainstem involvement
• Ataxia, dysmetria, dysarthria
17
Q
what is the difference between the brain stem and the cerebellum ?
A
The key difference between brainstem and cerebellum is that brainstem is the region of the brain that connects the brain to the spinal cord, while the cerebellum is the middle part of the brain that helps in motor learning, motor coordination, and equilibrium.
18
Q
what syndrome results from basillar artery thrombosis?
A
Locked in syndrome
19
Q
what are lacunar strokes and what major sign on presentation differentiates them from larger artery strokes?
A
Mostly occur due to ischaemic aetiology, with hypertension being the number one risk
factor
▪ Most common = posterior internal capsule infarction
▪ Usually present without cortical signs (hemispatial neglect, aphasia, visual field change, etc.)
→ pure motor or sensory strokes, unilateral ataxia, dysarthria…
20
Q
Define TIA and what are common signs on presentation
A
Definition
▪ Sudden loss of function that usually only lasts for several minutes, with complete recovery
and no associated findings on neural imaging
▪ Presentation is varied, but the most common signs are hemiparesis and aphasia, and
sometime amaurosis fugax (transient loss of vision in one eye)
▪ Consciousness is usually preserved
21
Q
A pt comes in having had a TIA what are potential complications you are worried about
A
Complications
▪ The main complication is the increased risk of vascular event in the future:
• 30% will have a stroke within 5-years (10% in 1-year)
• 15% will have an MI within 5-years
• Highest risk period for having is a stroke is the 2-week period after TIA
o
22
Q
How would you stratify risk of stroke in a patient coming in with TIA
A
Risk stratification and investigations ▪ Risk stratification = ABCD2 score (high-risk for stroke within 48-hours if >6) • A = Age >60 (1 point) • B = BP >140/90 (1 point) • C = Clinical features o Unilateral weakness (2 points) o Isolated change in speech (1 point) • D = Duration of symptoms o >60 minutes (2 points) o <60 minutes (1 point) • D = Diabetes mellitus (1 point)
23
Q
What investigations would you undertake for a patient you suspect has a TIA
A
Within 1-hour:
• CT or MRI brain
Often not sensitive for infarction for 24-48 hours
• FBC and BSL
Within 24-hours:
- FBC, BSL, coags, lipids, ESR
- ECG (assess for AF)
- Carotid artery doppler
If unknown cause, consider further investigation:
- CT/MRI angiography
- Echocardiogram
- Telemetry
- Vasculitis, thrombophilia, and antiphospholipid screen
24
Q
Management of TIA?
A
Management
▪ Assess and manage risk factors
▪ Medical therapy
• 300mg aspirin initially → 100mg ongoing
• Consider statin therapy and anti-hypertensives
▪ Carotid artery stenting/endarterectomy if severe stenosis
25
```
Intracerebral haemorrhage
1. Definition
2. aetiology
3. presentation
reflect on how these 3 aspects differ from stroke
```
Intracerebral Haemorrhage
▪ Definition
• Haemorrhage into the brain parenchyma, usually arising from rupture of small
arteries perfusing the basal ganglia, thalamus, and brainstem
▪ Aetiology
• Most common cause = hypertension
• Other causes = amyloidosis, haemorrhagic conversion of ischaemic stroke, AVM,
drug-use-associated, cancer-related, trauma-related
▪ Presentation
• Sudden-onset focal neurological deficit in the context of headaches and
hypertension
26
Mx of intracerebral haemorrhage
Management
• ABCDE
• Admit to stroke unit
• Investigations = FBC, BSL, and brain CT
• Cease anticoagulants/anti-platelets
• Maintain systolic BP <140 +/- mannitol to decrease ICP
o Nicardipine or nimodipine drip
• Neurosurgical consult → haematoma evacuation +/- ventricular drain
• Monitor for hyponatraemia (cerebral salt wasting and SIADH)
27
Define a SAH
Definition
| • Haemorrhage into the subarachnoid space +/- intraparenchymal haemorrhage
28
What are the brain layers from top to bottom
- skull
- dura mater
- arachnoid mater
- pia mater
- brain parenchyma
29
Aetiology SAH
```
Aetiology
• Berry aneurysm of larger cerebral arteries (70%)
o PcommA + ICA junction (causes painful CN 3 palsy)
o AcommA + ACA junction
o MCA trifurcation
91
• AVM (10%)
• Unknown/other (20%)
```
30
Presentation of SAH
Presentation
• As opposed to intra-cerebral haemorrhage, focal neurological deficits are less
common and are not the sentinel finding
• Severe thunderclap headache, possibly preceded by a sentinel headache several
days/weeks earlier
• Neck stiffness, photophobia and possible focal neurology
31
What investigations would you consider in a subarachnoid haemorrhage and what findings would you expect@
CT non-contrast → ‘chicken sign’
•
LP → yellow CSF (xanthochromia secondary to bilirubin in CSF)
32
Treatment of SAH
Initial work-up and management as per ICH
• Unruptured → endovascular coiling or clipping
• Associated hydrocephalus → ventricular drain
• Maintain BP <140 systolic with nimodipine
• Monitor for hyponatraemia (cerebral salt wasting and SIADH)
• Consider haematoma evacuation
• Consider anti-epileptic prophylaxis if high-risk
33
Subdural haemorrhage aetiology and presentation
Aetiology
• Rupture of bridging veins in the subdural space, usually secondary to minor trauma
in people with risk factors
• Older age and alcohol → cortical atrophy → increased exposure of bridging veins →
more prone to trauma
• Alcohol and falls-risk → increased risk of bridging vein trauma
• Alcohol and anticoagulants → increased bleeding risk
Presentation
• Usually gradual onset over weeks-months with headaches, drowsiness, confusion →
possible focal neurology → coma/death
34
management and investigation of subdural haemorrhage
Investigations
• CT brain → concave (banana-shaped) bleed
Management
• May be managed with ongoing monitoring and serial imaging; even large collections
can resolve spontaneously
35
aetiology and presentation of extradural haemorrhage
Aetiology
• Rupture of the middle meningeal artery, usually secondary to trauma at the pterion
▪ Presentation
• Initial loss of consciousness → lucid interval → decline in function
o Stupor, ipsilateral pupil dilation, contralateral hemiparesis
• Signs of raised ICP and coning
36
what investigations are done in an extradural haemorrhage
Investigations
• CT or MRI → convex (lemon-shaped) bleed that does not cross suture lines
37
What is the management of in an Extradural Haemorrhage?
Urgent surgical decompression
38
What is the Aetiology/pathogenesis in Venous Sinus Thrombosis?
* Thrombosis of the venous sinuses within the brain
* Associated with risks for hypercoagulability (female, OCP, pregnancy, obesity, dehydration, thrombophilia), though can occur idiopathically or secondary to trauma
39
What is the Presentation in Venous Sinus Thrombosis?
* Cortical veins involvement → venous infarct → focal neurology
* Dural veins involvement → raised ICP → headache, papilloedema, eye pain, fever, seizures, coma
40
What are the Investigations in Venous Sinus Thrombosis?
MRI or CT with contrast in the venous phase
41
What is the Management in Venous Sinus Thrombosis?
Initial LMWH therapy → conversion to DOAC or warfarin therapy for 6-months
42
What are the Diagnostic Criteria for Dementia?
o Acquired loss of mental function affecting 2 or more cognitive domains, including:
▪ Episodic memory (acquisition of new information)
▪ Language function
▪ Frontal executive function (ability to make decisions)
▪ Visuospatial function
▪ Apraxia (impaired movement)
o Must cause significant social or occupational impairment
o Must not be better explained by another pathology
▪ Especially delirium, which is typically acute and fluctuating)
43
What is the Epidemiology for Dementia?
>20% of people aged >80 years are affected
44
What is the Assessment in Dementia?
o Bedside cognitive assessments to screen for dementia
▪ Mini-Mental State Examination (MMSE)
• Considered positive if <25
▪ Montreal Cognitive Assessment (MOCA)
• Testing of memory, visuospatial ability (drawing a clock), executive function, attention, language, abstraction, recall, and orientation to time, person, and place
• Positive if <26
▪ Addenbrooke’s Cognitive Examination (ACE)
• Best done as a supplement to the MMSE
o Detailed neuropsychiatric assessment
▪ Performed by a specialist physician, usually a Geriatrician
▪ Depression is a common cause of dementia-like symptoms, and depression also commonly occurs secondary to dementia
o Physical examination to assess for comorbidities and differential diagnoses
▪ Thorough neurological assessment to screen for possible Parkinson’s, brain lesions, delirium, raised ICP, etc.
o Assessment of functional ability
▪ Assessment of the patient alongside a collateral history to determine the severity of their condition and their ability to complete ADLs
▪ Are they a danger to themselves (can they drive, cook, wash themselves, ambulate?)
▪ Are they a danger to others (are they prone to bursts of aggression/violence?)
o Assessment for polypharmacy and substance use
45
What are the Investigations in Dementia?
Investigation of dementia is largely aimed at ruling out differential diagnoses and assessing for reversible causes
o FBC – infection
o Vitamin B12 and thiamine levels
o BSL - hypoglycaemia → dementia-like syndromes
o LFTs – hepatic encephalopathy
o CMP and UECs – electrolyte derangements (especially sodium and calcium)
o TFTs – hypothyroidism can cause cognitive impairment
o HIV serology
o Syphilis serology – assessment for tertiary syphilis
o CT/MRI – assessment of structural lesions or hydrocephalus
▪ Alzheimer’s is associated with hippocampal atrophy, and FTLD is associated with temporal/frontal lobe atrophy
o CSF – assessment for specific proteins or infective aetiology
o EEG
46
What are the types of Dementia?
o Alzheimer’s Disease (64%)
▪ Most common form of dementia
▪ Typical features include:
• Marked reduction in day-to-day memory and ability to learn new things.
Amnesia for more recent events is far more affected than events in the distant past
• Difficulty with finding the right words and naming of objects
• Impaired frontal executive function
• Impaired skilled motor movements
o Normal walking is intact very late into disease progression; hence patients often go wandering and get lost
• Personality change is usually not seen until very late stages (in contrast to other types of dementia)
• Late stage features → myoclonus, reversed sleep-wake cycles, impaired swallowing
▪ Associated with atrophy of the temporal lobe and hippocampus on MRI
▪ Definitive diagnosis can only be made post-mortem with the presence of betaamyloid depositions in the cerebrum
o Vascular Dementia (17%)
▪ Dementia that occurs via multiple aetiologies that ultimately result in neurological impairment secondary to multiple small strokes
▪ Risk factors for vascular disease are usually present
▪ Features of apraxia, pyramidal signs, and urinary incontinence are common
o Mixed Dementia (10%)
▪ Mix of multiple forms of dementia, most commonly Alzheimer’s and vascular dementia
o Dementia with Lewy Bodies (4%)
▪ Dementia occurring secondary to deposition of alpha-synuclein-containing Lewy bodies throughout the brain
▪ Associated with early disorders of REM sleep, visual hallucinations, and Parkinsonism – changes in memory usually appear later
o Frontotemporal Lobe Dementia (2%)
▪ Presents in various ways depending on whether the frontal or temporal lobe is more affected
▪ Frontal lobe involvement → personality change, emotional dysregulation/blunting, apathy, disinhibition, and usually preserved memory
▪ Temporal lobe involvement → progressive aphasia (usually Wernicke’s aphasia/receptive aphasia)
▪ Can be diagnosed with asymmetric involvement of the frontal and/or temporal lobes on MRI
▪ More commonly seen in adults <65-years
47
What is the Management for Dementia?
o MDT management
▪ GP, geriatrician, social supports, aged-care nursing, family and patient education
o Assessment and management of reversible/contributory causes
▪ Hormonal/vitamin/electrolyte anomalies, polypharmacy, substance use
o Cholinesterase inhibitors (donepezil or rivastigmine)
▪ Alzheimer’s disease is associated with an ACh deficit in the CNS
▪ Increased neural ACh → improved function
▪ Not effective in FTLD or vascular dementia
o NMDA receptor antagonists (Memantine)
▪ If cholinesterase inhibitors are not effective
o Antipsychotics and antidepressants
▪ Antidepressants should be trialed if depression is present
▪ Anti-psychotics may be necessary in later-stages of disease due to agitation and patient distress
o Advanced care planning
48
What are the Differentials for Dementia?
In addition to the causes mentioned above
o Normal pressure hydrocephalus
▪ Idiopathic hydrocephalus → triad of dementia, urinary incontinence, and gait abnormalities
• “weird, wet, and wobbly”
o Wernicke-Korsakoff Syndrome
▪ Permanent progression from Wernicke’s encephalopathy occurring secondary to thiamine deficiency, usually in chronic alcoholics
▪ Causes severe anterograde and retrograde amnesia, with some confusion, but otherwise intact cognition
o Pseudodementia
▪ Dementia-like symptoms associated with severe major depression
49
What is the Pathogenesis/pathology of Multiple Sclerosis?
▪ T-cell-mediated inflammation of the CNS → CNS demyelination and plaque formation → broad spectrum of clinical manifestations presenting over time
▪ Plaques occur secondary to inflammatory-mediated axonal destruction and oligodendrocyte atrophy
▪ Plaques can occur anywhere in the CNS, but over-represented sites include the optic chiasm, periventricular area, corpus callosum, and brainstem connections
50
What is the Aetiology of Multiple Sclerosis?
▪ Unknown, but there is a suspected genetic susceptibility and an association with EBV and HHV-6 infection
▪ Risk factors = low vitamin D, smoking, and obesity
51
What is the Epidemiology of Multiple Sclerosis?
▪ Increased prevalence in women and Caucasians
| ▪ Peak onset between ages 20-40
52
What are the Classification of presentations in Multiple Sclerosis?
There are various clinical courses that MS can follow, and the courses can change between each other
▪ Relapsing-Remitting = 85%
• MS with acute flares in which there is a decrease in function, but return to baseline (or close to baseline) upon resolution before the next attack
• Progresses into secondary progressive MS in 75% of cases
▪ Secondary Progressive
• Initial relapsing-remitting MS that deteriorates such that there is no return of function back to baseline, and functionality deteriorates continuously with time
▪ Primary Progressive = 10-15%
• Deterioration of function with time from the onset of symptoms
▪ Relapsing Progressive = <5%
• As per primary progressive, but with acute episodes of deteriorating function dispersed throughout
53
What is Multiple Sclerosis
| - what are common manifestations (no need to describe clinical course)
Highly variable presentation of MS between people. The disease is characterised but neurological signs and loss of function that occurs with time and follows one of the above
clinical courses
▪ Optic neuritis (common; often earliest finding)
• Inflammation of the optic nerve → blurred vision, visual field defects, and pain with eye movement
▪ Brainstem involvement
• Cranial nerve palsies, usually 1-8 → facial numbness/weakness, diplopia, vertigo, nystagmus, dysarthria, dysphagia, etc.
• Internuclear Ophthalmoplegia
o Lesion of the medial longitudinal fasciculus → impaired conjugate lateral gaze
o Able to adduct both eyes with convergence (looking at your nose), but unable to adduct the eye ipsilateral to the lesion when looking towards the contralateral direction (the abducting eye will usually have nystagmus)
▪ Spinal cord involvement
• Dorsal column → impaired vibration, fine touch, and proprioception, usually asymmetrical
o Usually the first sensory sign observed in MS
• Corticospinal tract → spasticity and hyper-reflexia +/- Babinski
▪ Cerebellum involvement
• Scanning speech (individual syllables over-pronounced)
• Nystagmus
• Intention tremor
▪ Autonomic involvement
• Bowel and bladder incontinence/retention, and impaired sexual activity
▪ Memory deficits, altered concentration and depression
• Relatively late signs
▪ Other
• Lhermitte’s sign = shooting electrical pain down the spine with neck flexion
• Uhthoff’s phenomenon = temporary exacerbation of neurological symptoms with heat exposure (exercise, fever, hot shower, etc.)
• Pseudobulbar palsy = UMN lesion of CNs 9, 10, and 12 → spasticity of associated muscles and labile emotions
54
What are the Diagnosis/Investigations of Multiple Sclerosis?
▪ Gold standard = Brain and spine MRI → plaque visualization
• Usually periventricular
- Investigations to rule out differentials
• B12, SLE, thyroid pathology, sarcoidosis, HIV, etc.
▪ Diagnosis may be made clinically with multiple lesions separated by space (CNS location) and time
▪ CSF electrophoresis → oligoclonal IgG bands
• Relatively non-specific; associated with a poorer prognosis
55
What is the pharmaceutical medical management of Multiple Sclerosis?
▪ Medical Management
• Acute exacerbation
o High-dose glucocorticoids (500-100 mg/day IV methylprednisolone)
• Maintenance therapy
o Initial induction therapy with strong immunosuppressants →
o Glatiramer, interferon-beta therapy, or natalizumab
56
What is the Prognosis of Multiple Sclerosis?
▪ Very variable depending on age of presentation and specific clinical course
▪ Decreased life expectancy by 7-years on average
▪ Average time to being wheelchair dependent = ~25-years
57
What is the definition of Motor Neurone Disease (MND)?
o MND is an umbrella term for a disease that causes progressive deterioration of UMNs and LMNs with sparing of sensory neurons
o The most common form of MND is Amyotrophic Lateral Sclerosis (ALS)
o Second most common cause = progressive bulbar or pseudobulbar palsy
▪ Spasticity of CNs 7-12 with associated emotional lability (PPP)
58
Who is more likely to hae MND and what is the peak age of onset?
o Men > women
| o Peak age = 50-75
59
What is the Aetiology of Motor Neurone Disease (MND)?
Unknown aetiology, but increased risk with family history of MND, smoking, and heavy metal exposure
60
how does ALS typically present
o Simultaneous involvement of a single limb that shows both UMN and LMN signs. The upper limb is most common, usually the hand
▪ UMN signs = hyper-reflexia, spastic paralysis, weakness, clonus
▪ LMN signs = muscle atrophy, fasciculations, hypo-reflexia, flaccid paralysis
o Gradually progresses to involve the other limbs, trunk, and diaphragm
61
What is the Diagnosis in Motor Neurone Disease (MND)?
o Largely a clinical diagnosis
| o EMG studies consistent with denervation are consistent with MND diagnosis
62
What is the Management of Motor Neurone Disease (MND)?
```
o MDT Management
o Supportive care
▪ Non-invasive ventilation when unable to breathe unassisted
▪ Gastrostomy to assist with feeding
▪ Wheelchair and text-to-speech software
```
63
What is the Prognosis in Motor Neurone Disease (MND)?
Average of 3-years life expectancy from time of diagnosis
64
What is the Pathophysiology in Parkinson’s disease?
▪ Progressive depletion of dopamine-secreting cells in the substantia nigra → dopamine deficiency in the basal ganglia
▪ Occurs secondary to idiopathic accumulation of alpha-synuclein-containing Lewy Bodies
65
What is the Presentation in Parkinson’s disease?
▪ Classic triad of:
▪ Resting tremor
• ‘pill-rolling’ tremor at rest that improves with purposeful movement of the hand, and worsens with distraction
• Classically unilateral/asymmetrical
▪ Rigidity
• E.g. cogwheel rigidity
▪ Akinesia (poverty of movement)
• Slow and small movements – e.g. micrographia and shuffling gait
• Difficulty initiating movements, difficulty turning around on the spot, masked facies
▪ Also associated with impaired sleep and depression
▪ Most commonly seen in middle-age and older men
66
What are the Parkinson’s Plus Syndromes in Parkinson’s disease?
Syndromes commonly associated with Parkinson’s Syndrome
▪ Multiple System Atrophy
▪ Involvement of the autonomic nervous system and cerebellum
▪ Cerebellum → ataxia
▪ ANS → postural hypotension, constipation, diaphoresis, sexual dysfunction, urinary retention/incontinence
▪ Dementia with Lewy Bodies
▪ Form of dementia that is also caused by deposition of alpha synucleincontaining Lewy Bodies
▪ Associated with visual hallucinations, delusions, disorders of REM sleep, and fluctuating consciousness
▪ Progressive Supranuclear Palsy
▪ Most common manifestation
▪ Trunk rigidity, dementia, gaze paresis
▪ Corticobasal Degeneration
67
What is the Diagnosis in Parkinson’s disease?
▪ Diagnosis is usually made clinically by a specialist
▪ MRI may be used to rule out differentials
▪ Ensure features of Parkinsonism are not from anti-psychotics/dopamine antagonists
68
What is the Management of Parkinson’s disease?
▪ MDT Management
▪ GP, neurologist, OT, physiotherapy, psychologist
▪ Medications
▪ Levodopa and Carbidopa
• Levodopa = synthetic dopamine
o Becomes less effective with time, hence its use may be delayed initially and supplemented with adjuvant therapies
• Carbidopa
o Peripheral decarboxylase inhibitor → decreased peripheral conversion of dopamine into NA → increased dopamine effect in the CNS and decreased peripheral side-effects
• Side effects of increased dopamine = dystonia, chorea, and athetosis
▪ COMT Inhibitors (Entacapone)
• Slows the breakdown of levodopa in the CNS
▪ Dopamine Agonists (bromocriptine or cabergoline)
• Less effective than levodopa, usually used before initiating levodopa
▪ MAOIs
• Increased dopamine in the CNS; used as a levodopa adjuvant
▪ Deep Brain Stimulation
69
What is the Description of Seizures (guidelines on describing seizures changed in 2020) of Epilepsy?
1. Is this a seizure?
• Differentiate from other differentials – e.g. syncope, stroke, arrhythmia
2. How did the seizure present?
• Focal, general, focal → general, absent, unknown
3. Is the seizure pattern consistent with a known epilepsy syndrome?
4. Is there an underlying aetiology?
• Structural brain lesion, autoantibodies, electrolyte anomaly, hypoglycaemia
o Older terms that are still commonly used:
▪ Generalised = seizure occurring in both hemispheres with associated loss of consciousness (tonic-clonic, atonic, tonic, clonic, absence)
▪ Focal = electrical activity confined to a defined area in the brain → specific features; may be associated with LOC
▪ Simple = no loss of consciousness
▪ Complex = loss of consciousness
70
What are the Features of Seizure in Epilepsy?
▪ Presentation of seizure varies broadly depending on the type, but common defining features of seizure include:
▪ Post-ictal phase
▪ Tongue biting
▪ Recurrent attacks with a similar pattern
▪ Associated cyanosis
71
What is the Diagnosis/Investigation in Epilepsy?
Diagnosis of epilepsy is largely based on clinical features in addition to excluding differential diagnoses
▪ Baseline bloods – FBC, UECs, BSL, LFTs, VBG, CMP
▪ Assessment for causes – infection, electrolyte anomaly, hypoglycaemia, hypoxia
▪ Baseline bloods before initiating anti-epileptics – LFTs, UECs, FBC
▪ Pregnancy test
▪ Valproate and phenytoin are both teratogenic, must make sure patients are not pregnant before initiating therapy
▪ ECG
▪ Arrhythmias causing syncope or hypoxia
▪ Electrolyte anomalies causing ECG changes
▪ Brain MRI
▪ Most patients will receive this to rule-out a structural cause/stroke
▪ EEG
▪ Most patients will receive an EEG, though it is rarely positive due to the intermittent nature of epilepsy
▪ Positive EEG = focal cortical spikes
72
What is the Management of Epilepsy?
▪ Removal of precipitants/contributors
▪ Drugs, alcohol, lack of sleep, causes of electrolyte anomalies
▪ Patient education
▪ Unable to drive
• Duration is variable, but unless the seizure was provoked or only occurs during sleep, 12-months of being seizure-free is required before being able to drive again
• The duration may be shorter following the first seizure vs. recurring seizures
▪ Do not bathe/swim alone
▪ Does their occupation put them at risk of harm if they have a seizure?
▪ Medication
Medication is not always initiated after a 1st seizure. It is more likely to be initiated if there are EEG anomalies, neurological anomalies, MRI findings, or focal seizures.
▪ Generalised tonic-clonic → sodium valproate
▪ Focal seizures → lamotrigine or carbamazepine
▪ Absence seizures → sodium valproate or ethosuximide
▪ Atonic seizure → sodium valproate
▪ Myoclonic → sodium valproate
▪ In summary:
• Sodium valproate is 1st line therapy in all forms of epilepsy except focal seizures, where lamotrigine or levetiracetam are preferred
• Lamotrigine can be used as an alternative to sodium valproate in most cases (commonly used because it is considered safe in pregnancy)
• Ethosuximide is commonly used for absence seizures
o “It really SUX to have absence seizures!”
▪ Acute Seizure Management (as per paediatrics)
▪ 5 minutes → 10mg IV midazolam, measure BSL, ensure patient safety, get IV access
▪ 10 minutes → repeat midazolam dose
▪ 15 minutes → levetiracetam or phenytoin
▪ 20 minutes → phenytoin or levetiracetam
▪ 25 minutes (status epilepticus) → transfer to ICU for
propofol/ketamine/midazolam +/- intubation
▪ Refractory Management Considerations
▪ Resection of epileptogenic foci in the brain
▪ Hemispherectomy
▪ Deep brain stimulation or vagus nerve stimulation
73
What are the Side-Effects of Anti-Epileptics of Epilepsy?
▪ Sodium valproate → Teratogenic, hepatitis, hair loss, tremor
▪ Carbamazepine → agranulocytosis, aplastic anaemia, induction of cP450 enzymes
▪ Phenytoin → antagonist of vitamin D and B12, also teratogenic
▪ Ethosuximide → rash and nightmares
▪ Lamotrigine → steven-Johnson syndrome, DRESS syndrome, leukopaenia
74
Epilepsy in summary
* Valproate or lamotrigine will manage most seizures (lamotrigine can be used in pregnancy)
* Lamotrigine or Keppra (levetiracetam) are 1st line for focal seizures
* Ethosuximide is 1st line for absence seizures
75
What are the UMN vs. LMN Lesions in Facial Nerve Palsy?
▪ UMN lesions → no forehead sparing; there is paralysis of the entire contralateral face
▪ LMN lesions → forehead sparing; there is paralysis of the ipsilateral lower half of the face
▪ Unilateral palsy → stroke, tumour, Bell’s palsy
▪ Bilateral palsy → motor neuron disease, Pseudobulbar palsy
76
What is Bell’s Palsy in Facial Nerve Palsy?
▪ Idiopathic unilateral LMN palsy with acute onset
▪ Recovery occurs by itself over weeks, sometimes lasting up to 12 months
▪ May have residual weakness after resolution
▪ Management
▪ Consider 5-10 days of oral prednisolone
▪ Lubricating eye drops and eye-patch (protection from corneal abrasions)
77
What is Ramsay-Hunt Syndrome in Facial Nerve Palsy?
▪ Facial nerve palsy occurring secondary to shingles infection (VZV) in the CN 7 DRG
▪ Palsy occurs during active disease and is associated with a vesicular rash in the CN-7 distribution around the ear. Also associated with paraesthesias
▪ Management
▪ As per shingles → acyclovir if within 72-hours of onset
▪ Consider oral prednisolone
▪ Eye drops and eye patch
78
What are Other Causes of Facial Nerve Palsy?
▪ Infection → AOM, HIV, Lyme disease
▪ Systemic disease → DM, sarcoidosis, leukaemia, MS, GBS
▪ May occur as part of mononeuritis multiplex
▪ Tumours → vestibular schwannoma, parotid mass, cholesteatoma
▪ Trauma/surgery
79
Peripheral Neuropathy Aetiology = ABCDE
```
o A = Alcohol
o B = B12 deficiency
o C = Cancer and CKD (uraemia → CKD)
o D = Diabetes and Drugs
▪ Isoniazid, amiodarone, cisplatin
o E = Every vasculitis
```
80
What is the Presentation of Brain tumours?
▪ Raised ICP
o Papilloedema
o Headache → constant, worse at night and on waking, worse when leaning forward, associated with vomiting
o Altered mental status
o Seizures
o CN signs → altered vision, ptosis, 3rd and 6th nerve palsies
▪ Site-specific features
o Cortex → motor/sensory signs, sight changes, altered memory
o Brain stem → autonomic lability, CN signs
o Spinal cord → radiculopathies
▪ Endocrine components
o If there is a functional adenoma, or if a space-occupying lesion is affecting endocrine release
o Most common = prolactinoma
81
What are the Tumour Types of Brain tumours?
▪ Gliomas
o Highly malignant and deadly cancer of glial cells
o From worst to best prognosis
▪ Astrocytoma, oligodendroglioma, ependymoma
o Grade 4 astrocytoma = Glioblastoma Multiforme
▪ Meningiomas
o Most common brain tumour
o Benign mass in the brain or spinal cord formed by meningeal tissue
o Main complications = compression and raised ICP
▪ Pituitary Tumours
o Associated with optic chiasm compression → bitemporal hemianopia
o Can be a functional adenoma → secretion of pituitary hormone (most commonly prolactin)
o Can be a non-functioning adenoma → hypopituitarism
▪ Vestibular Schwannoma (Acoustic Neuroma)
o Tumour of schwann cells occurring at the cerebellopontine angle
o Associated with compression of CN 8 +/- CN 7 → hearing impairment, tinnitus, impaired balance, and possible facial nerve palsy
o Very slow growing
o May be bilateral with neurofibromatosis type 2
▪ Malignant Tumours
o Cancers of the CNS occurring secondary to metastasis, most commonly from the lungs, breast, prostate, and colon/rectum
82
What is the Management of Brain tumours?
▪ Highly variable and dependent on the size, type, and location of the cancer → chemotherapy, radiation therapy, surgery, palliation
▪ Pituitary tumours
o Non-functioning microadenoma → ongoing monitoring without treatment
o Non-functioning macroadenoma → trans-sphenoidal hypophysectomy +/- radiotherapy
o Prolactinoma
▪ Small → dopamine agonists (cabergoline or bromocriptine)
▪ Large → trans-sphenoidal hypophysectomy +/- radiotherapy
83
What is the Pathogenesis in Huntington Chorea/Disease?
▪ Autosomal dominant disease → neuronal loss in the striatum, thalamus, and cortex
▪ Occurs due to a trinucleotide repeat disorder that increases in severity with subsequent generations → earlier onset of disease
84
What is the Presentation in Huntington Chorea/Disease?
▪ Early Features (increased movement)
o Changes in mood and cognition
o Psychiatric manifestations
o Chorea, athetosis, hyperkinetic movements, and hyper-reflexia
▪ Late features (decreased movement)
o Bradykinesia
o Dysarthria and dysphagia, akinetic mutism
o Dementia, aggression, depression, and psychosis
85
What is the Diagnosis of Huntington Chorea/Disease?
Presence of trinucleotide repeat of the HTT gene on chromosome 4
86
What is the Management of Huntington Chorea/Disease?
▪ MDT management
o Speech/language therapy, genetic counselling, GP, neurologist, physiotherapist, OT
▪ Medications (symptomatic management)
o Anti-psychotics, dopamine antagonists, SSRIs, benzodiazepines
▪ Advanced care planning
87
What is the Prognosis of Huntington Chorea/Disease?
▪ Life expectancy of 15-20 years after diagnosis
▪ High rate of suicide
▪ Death often occurs secondary to pneumonia
88
What is the Pathophysiology of Myasthenia gravis?
▪ Autoimmune production of acetylcholine receptor antibodies that bind to post-synaptic receptors at neuromuscular junctions → impaired binding of Ach to the receptors → impaired muscle contraction
▪ Antibodies also activate the complement system → damage of the post-synaptic membrane at NMJs → worsening of symptoms
▪ There is a strong link between thymus gland tumours (thymoma) and MG
89
What is the Presentation of Myasthenia gravis?
Severity of disease is highly variable between patients
▪ Peak age = ~40 in women and ~60 in men
▪ Characteristic feature of the disease is weakness of muscles that gets worse with repeated movement and improves with rest
▪ Worsening with movement occurs due to decreased ACh with successive muscle contractions, and subsequent predominance of antibodies binding to ACh receptors
▪ The muscle that are mainly affected are the small proximal muscle of the head and neck, this is because they undergo more contractions throughout the day than do larger muscles
▪ Extraocular muscles → diplopia, that is worse at the end of the day
▪ Ptosis → may be induced with repeated blinking
▪ Facial muscle weakness
▪ Weakened swallowing and mastication
▪ Slurred speech
▪ Symptoms can affect respiratory muscles and may lead to respiratory failure when triggered by respiratory illness
90
What is the Diagnosis of Myasthenia gravis?
▪ Testing for associated antibodies
▪ ACh-receptors antibodies, MuSK antibodies, or LRP4 antibodies
▪ CT or MRI to assess for a thymoma
▪ Edrophonium test (rarely used anymore)
▪ Acetylcholinesterase inhibitor that increases ACh concentration at the NMJ → temporary improvement of weakness
91
What is the Management of Myasthenia gravis?
▪ Medications – specialists may use one of the following:
▪ Reversible acetylcholinesterase inhibitors (neostigmine)
▪ Immunosuppression (corticosteroids or azathioprine)
▪ Rituximab (anti-B-cell antibody → decreased auto-antibody production)
▪ Thymectomy (if thymoma is present)
92
What is the Myasthenic Crisis in Myasthenia gravis?
▪ Severe life-threatening complication of the MG in which there is severe weakness of the respiratory muscles → respiratory failure
▪ Usually occurs secondary to a respiratory tract infection
▪ BiPAP, and sometimes intubation, is often required
▪ Immunomodulatory therapy +/- IVIg +/- plasma exchange may be necessary
93
What is the Pathophysiology of Lambert-Eaton Syndrome?
Pathophysiology
▪ Production of autoantibodies against calcium channels at the pre-synaptic terminal of NMJs
→ decreased release of ACh → muscle weakness
▪ Highly associated with small cell lung cancer, hence it is considered to be a paraneoplastic
syndrome
o
94
what is the typical presentation for Lambert Eaton Syndrome?
Presentation
▪ In contrast to MG, muscle weakness is present at rest and improves with increasing
movements
▪ Hence, muscle weakness predominantly affects the larger muscles that are moved less, i.e.
the proximal muscles of the arms and legs
▪ The small muscles of the eyes, face and throat can also be affected → diplopia, ptosis,
dysphagia, and slurred speech
▪ There is also associated ANS involvement → dry mouth, blurred vision, dizziness, and
constipation
▪ Reflexes are typically reduced/absent at rest, and will improve following repeated
contractions
95
Management of Lambert Eaton Syndrome?
Management
▪ Screening and management of small cell lung cancer
▪ Amifampridine → potassium channel blockade at NMJs → improved ACh release
▪ Immunomodulatory therapy → immunosuppression, IVIg, plasmapheresis
96
Pathophysiology of Charcot-Marie tooth disease?
Pathophysiology
▪ Inherited disease affecting the motor and sensory nerves via dysfunction of the myelin or
axons
▪ Various different possible genetic mutations, most of them being autosomal dominant
97
Charcot Marie tooth disease typical presentation?
Presentation
Usually presents <10-years, but some mutations may delay presentation to >40
▪ Variable presentation, but common features include any of the following:
▪ High foot arch (pes cavus)
▪ Distal muscle wasting (hands and legs)
▪ Lower leg weakness → weak/absent ankle dorsiflexion
▪ Poor muscle tone and hyporeflexia
▪ Peripheral neuropathy
o
98
Management of Charcot Marie tooth disease
Management
▪ MDT management (supportive)
▪ GP, neurologists, geneticists +/- orthopaedic surgery
▪ Physiotherapy, OT, podiatry
99
Pathophysiology of neurofibromatosis?
Pathophysiology
o Autosomal dominant condition that causes benign nerve tumours (neuromas) to
develop throughout the nervous system
o There are 2 types of neurofibromatosis depending on which gene is affected (NF1 vs
NF2 gene)
100
What are the clinical features and the diagnostic criteria for neurofibromatosis 1 ?
Neurofibromatosis 1
o Wide range of clinical features, with at least 2 of the 7 features below needing to be
present to meet diagnostic criteria. Symptoms = CRABBING
▪ C = café-au-lait spots (>5) – light-brown patches of skin
▪ R = relative with neurofibromatosis 1
108
▪ A = axillary or inguinal freckles
▪ BB = bone dysplasia (long bone bowing or sphenoid wing dysplasia)
▪ I = iris hamartomas (>1) – yellow brown discolouration of the iris
▪ N = neurofibromas (2 or more) – lumps on/under the skin
▪ G = glioma of the optic nerve
o
101
What are the common complications for neurofibromatosis 1?
```
Complications
▪ Migraines, epilepsy, brain/spinal cord tumours
▪ Malignant nerve sheath tumours
▪ Increased risk of leukaemia and breast cancer
▪ Renal artery stenosis
▪ GIT stromal tumours
▪ Learning and behavioural issues (ADHD)
▪ Scoliosis
o
```
102
What is the management for neurofibromatosis 1
Management
| ▪ Supportive care and monitoring for complications
103
Neurofibromatosis 2 pathology, common manifestations
• Neurofibromatosis 2
o Mainly affects Schwann cells (myelinating cells of the peripheral nervous system)
o Most common manifestation is unilateral/bilateral vestibular schwannomas (acoustic
neuromas) of CN8 → hearing loss, tinnitus, and balance problems +/- facial nerve palsy
o Schwannomas can also occur elsewhere in the brain and SC → symptoms depending on
site of compression
o
104
Investigations and management of neurofibromatosis 2?
```
Management
▪ Possible surgical resection
• Investigations
o Genetic testing (if diagnosis cannot be made clinically)
o CT/MRI of brain/SC
```
105
What is the pathophysiology of GBS?
Pathophysiology
▪ Acute paralytic neuropathy of the peripheral nervous system occurring secondary to
antibody production against infection by C. Jejuni, CMV, or EBV → myelin sheath damage of
motor nerve axons via molecular mimicry
106
What is the typical presentation of GBS?
o Presentation
▪ Symptoms start within 4 weeks of infection, peak at 2-4 weeks following symptom-onset,
and then spontaneously resolves over a period that can take months to years
▪ Symmetrical ascending weakness starting with the feet and progressing proximally to
involve the trunk and arms (glove and stocking distribution → full-body involvement)
▪ As the disease progresses there may be areflexia/hyporeflexia, paralysis of respiratory
muscles, and paralysis of cranial nerves
▪ Less associated with sensory changes, though a degree of peripheral neuropathy +/-
neuropathic pain can occur
107
How is GBS diagnosed?
Diagnosis
▪ Diagnosis is made clinically using the Brighton criteria
▪ Generation of a risk likelihood based on clinical findings
▪ Supportive investigations
▪ Nerve conduction studies → reduced signal in affected nerves
▪ LP → normal cell count and glucose, raised protein
o
108
Management of GBS?
```
Management
▪ Supportive care
▪ VTE prophylaxis
▪ Immunomodulatory therapy
▪ IVIg or plasma exchange
▪ NB: No efficacy for steroids in management of GBS
▪ Monitoring and management of respiratory failure → intubation and ventilation
o
```
109
Prognosis of GBS diagnosis?
Prognosis
▪ 80% will make a full recovery
▪ 15% will be left with persistent neurological disability
▪ 5% will die
110
Pathogenesis of tuberous sclerosis
Pathogenesis
▪ Genetic condition characterised by the development of hamartomas at various sites
throughout the body
▪ Hamartoma = benign cell growth of the tissue that they originate from →
dysfunction secondary to compression/mass effect
▪ Commonly involved sites include the skin, brain, lungs, heart, kidneys, and eyes
111
Presentation of tuberous sclerosis?
Presentation
Most common presentation is in childhood with epilepsy and associated skin findings
▪ Skin features
• Ash leaf spots – depigmented areas of skin in the shape of an ash leaf
• Shagreen patches – thickened/dimpled pigmented patches of skin
• Angiofibromas – skin coloured or pigmented papules on the nose and cheeks
• Subungual fibromata – fibromas (circular painless lumps) under the nail bed →
gradual displacement of the nail
Café-au-lait spots
• Poliosis – isolated patch of white hair on the
head/eyebrows/beard
▪ Neurological signs
• Epilepsy
• Learning disability
• Developmental delay
▪ Other
• Cardiac rhabdomyomas
• CNS gliomas
• Polycystic kidneys
• Retinal hamartomas
112
Management of tuberous sclerosis?
Management
▪ Supportive MDT management
▪ Epilepsy management
113
red flag signs for headaches and what they may allude to?
Red Flags
▪ Sings of raised ICP → Dilated pupil, Cushing’s triad, worse on waking, leaning forward, or
straining, papilloedema
▪ Meningism → photophobia, fever, neck stiffness
▪ Stroke → focal neurological signs, dizziness
▪ Subarachnoid haemorrhage → thunderclap headache
▪ Temporal arteritis or glaucoma → visual changes
▪ Pre-eclampsia → headache during pregnancy
114
briefly describe tension headaches include triggers and management?
Tension Headache
▪ Normal headache associated with a band-like tightness/pain around the head, often
secondary to muscle tension
▪ Triggers
▪ Stress, depression, alcohol, skipping meals, dehydration
▪ Management
▪ Reassurance and simple analgesia
115
What are secondary headaches and what are possible aetiologies?
Secondary Headache
▪ Present similarly to tension headaches but have a clear precipitant
▪ Aetiology
▪ Medical conditions (infections, OSA, pre-eclampsia), alcohol, carbon monoxide
poisoning, head injury
116
What is trigeminal neuralgia and how is it managed?
Trigeminal Neuralgia
▪ Intense, paroxysmal, acute-onset facial pain in the distribution of a single branch of the
trigeminal nerve
▪ May be idiopathic, but also associated with compression and multiple sclerosis
▪ Management
▪ Carbamazepine and possible surgical decompression (if clear compression
identified on MRI)
117
Presentation of cluster headaches?
Presentation
▪ Severe unilateral headaches presenting with unbearable pain in and around the eye
▪ Other typical associations include:
▪ Red, swollen, and watery eye
▪ Pupil constriction and miosis
▪ Nasal discharge
▪ Facial diaphoresis
▪ Typically occur in clusters, with attacks happening 3-4 times per day for several weeksmonths, then asymptomatic periods that may last several years
▪ Typical episodes last between 15 minutes and 3 hours
118
Management of cluster headaches?
Management
Acute episodes
▪ High flow oxygen and subcutaneous sumatriptan
Prophylaxis
▪ Verapamil or prednisolone take at the start of a cluster period
119
List migraine types
Migraine Types
▪ Migraine without aura
▪ Migraine with aura
▪ Silent migraine (aura without headache)
▪ Hemiplegic migraine
▪ Rare form of migraine that can mimic a stroke
▪ Typical migraine-like headache with associated hemiplegia and ataxia that is of
sudden or gradual onset +/- decreased level of consciousness
120
what are typical presentation features of migraine?
Presentation
Headaches usually last between 4-72 hours, may be associated with triggers, and an aura.
Typical features of the headache include:
▪ Moderate to severe intensity pain with a pounding/throbbing quality
▪ Often unilateral
▪ Associated photophobia and phonophobia
▪ Nausea and vomiting
▪ May have an associated aura
▪ Neurological manifestations that usually precede migraines
▪ They can be highly variable in their manifestation, but the most common
features are visual changes, typically visual field deficits, blurred visions,
scotomas, and other visual phenomena
121
Detail the clinical course of migraines?
Clinical course
▪ Prodromal stage (patients may report feeling slightly off in the days leading up to a
migraine)
▪ Aura (usually lasts ~60-minutes)
▪ Headache
▪ Resolution of symptoms → hangover-like recovery phase for 1-2 days afterwards
122
What are common triggers of migraine?
Triggers
▪ Different people will have different triggers, and migraines can still occur in the absence of
triggers
▪ Common triggers → stress, bright lights, strong smells, certain foods, dehydration,
menstruation, poor sleep, trauma
123
Detail the acute management of migraines?
Acute management
▪ Rest in a dark and quiet room
▪ Simple analgesia +/- anti-emetics
▪ Triptans may be used in some cases (intranasal or SC sumatriptan)
• Triptans are 5HT serotonin receptors agonists that work via an unclear
mechanism to abort migraines in their early phase
• May be prescribed to individuals with recurrent, debilitating migraines
that are refractory to conservative methods of management
124
Describe migraine prophylaxis?
Prophylaxis
▪ Prophylaxis with propranolol, amitriptyline, or topiramate are commonly used
in severe/frequent migraines
▪ Other measures with proven benefit include acupuncture and riboflavin
supplementation
125
What is the pathophysiology of cavernous sinus syndrome?
Pathophysiology
o Increased pressure within the cavernous sinus the brain → compression of structures
within the cavernous sinus
o Cavernous sinus contents = CN 3, 4, 5 (V1 + V2), and 6, and the internal carotid artery
126
Describe the aetiology of sinus cavernous syndrome?
• Aetiology
o Cavernous sinus thrombosis (secondary to sinusitis and spread of contiguous infection)
o Cavernous sinus tumours
o Carotid artery fistula or aneurysm
o Pituitary tumours (cavernous sinus is situated around the pituitary)
•
127
Presentation of sinus cavernous syndrome ?
```
Presentation
o Conjunctival swelling and proptosis
o CN palsies → painful ophthalmoplegia, absent corneal reflex, and impaired upper facial
sensation
o Possible Horner’s syndrome
```
128
Management of sinus cavernous syndrome?
Management
| o Neural imaging to identify the aetiology → disease-specific management
129
Mononeuritis multiplex brief summary
- description
- aetiology
Description
o 2 or more isolated mononeuropathies occurring simultaneously
o E.g. foot drop and sciatic neuropathy
Aetiology = conditions that can cause axonal injury
o Diabetes mellitus, RA, vasculitis, SLE, Lyme disease, amyloidosis, HIV
•
130
Diagnosis and treatment of mononeuritis multiplex?
Diagnosis and treatment is based on identifying and treating the underlying condition in
addition to physiotherapy/OT and adequate pain management
• Must rule out differential diagnoses
o Spinal cord compression, MS, polyneuropathy, Tabes dorsalis, trauma
131
Pathophysiology of diabetic neuropathy
Pathophysiology
o Chronic hyperglycaemia → glycation of axonal proteins → destruction of peripheral
nerves
132
List the types of neuropathy?
1. peripheral neuropathy
2. mononeuropathy
3. autonomic neuropathy
133
Describe peripheral sensory neuropathy?
o Peripheral sensory neuropathy (most common)
▪ Progressive symmetrical loss of sensation in the extremities that progresses in
a ‘glove-and-stocking’ distribution
▪ Associated paraesthesias and burning sensations
▪ Screening is performed with regular monofilament and pinprick tests, as well as
vibration assessment with a tuning fork
134
List and briefly describe common mononeuropathies?
Mononeuropathies (Mononeuritis multiplex)
▪ Most common = CN 3 palsy, though CN 4 and 6 can also be affected
▪ Peripheral mononeuropathies
• Commonly affected nerves = median, ulnar, and common peroneal
nerves → hand/wrist dysfunction and/or foot drop
▪ Truncal neuropathy
• Chest pain secondary to involvement of intercostal nerves
▪ Diabetic lumbosacral plexopathy
• Severe deep thigh pain and weakness associated with muscle atrophy
oh the thigh and hip
135
List and describe common manifestations of autonomic neuropathy
Autonomic Neuropathy
Urogenital dysfunction
• Erectile dysfunction
• Bladder dysfunction (retention, poor emptying, overflow incontinence)
Cardiovascular dysfunction
• Silent MI (asymptomatic MI)
• Poor responsiveness of HR
• Orthostatic hypotension
• Arrhythmias and persistent tachycardia
GIT dysfunction
• Gastroparesis → risk of hypoglycaemia, nausea, bloating, anorexia
• Diarrhoea, constipation, and incontinence
Other
• Sweat gland dysfunction → heat intolerance
• Pupillary dysfunction → impaired vision
• Impaired sensation and physiological response to hypoglycaemia →
increased risk of hypoglycaemia
136
Management of autonomic neuropathy
```
Management
o Regular screening by GP and podiatrist +/- neurologist
o Optimisation of BSL
o Neuropathic pain management
▪ Pregabalin/gabapentin, TCAs, SNRIs
o Management of specific complications
115
▪ E.g. sildenafil for erectile dysfunction or intermittent catheterisation for urinary
retention
```
137
Describe glaucoma?
Description
o Group of eye diseases causing acute or chronic destruction of the optic nerve, usually
secondary to increased intra-ocular pressure
o It is the second leading cause of blindness in the developed world behind macular
degeneration
138
Glaucoma anatomy and pathophysiology?
Anatomy/Pathophysiology
o The anterior portion of the eye consists of the anterior chamber (between the cornea
and the iris), and the posterior chamber (between the lens and the iris)
o Aqueous humour (the fluid in the anterior chamber) is produced by the ciliary body
(attachment site of the suspensory ligaments of the lens) in the posterior chamber
o Aqueous humour circulates from the ciliary body in the posterior chamber → anterior
chamber via the iris → trabecular meshwork → canal of Schlemm → general circulation
o The increase in intraocular pressure associated with glaucoma occurs secondary to
impaired flow of aqueous humour out of the anterior chamber of the eye → fluid
accumulation → raised IOP → compression of the optic nerve
o NB: normal IOP is 10-21 mmHg
o NB: the ‘angle’ in glaucoma describes the space between the iris and trabecular
meshwork. In open angle glaucoma, the angle is open (i.e. there is space between the
iris and meshwork, but the meshwork is pathologically blocked), whereas in closed
angle glaucoma, the angle is closed (i.e. aqueous humour flow is obstructed secondary
to compression of the iris against the meshwork)
139
what is an open angle glaucoma?
o Raised IOP occurs secondary to clogging of the trabecular meshwork, this may occur
idiopathically, or secondary to inflammation (e.g. uveitis), vitreal haemorrhage, retinal
detachment, or chemical injury
o Risk factors → older age, family history, black ethnicity, and near-sightedness
140
Presentation of open angle glaucoma?
Presentation
▪ Initially asymptomatic, onset of symptoms is generally gradual
▪ Mild headaches and impaired night vision → progressive bilateral field loss,
starting peripherally and moving centrally (progressive tunnel vision), blurred
vision, and halos occurring around lights
141
Diagnostics of open angle glaucoma?
Diagnostics
▪ Slit-lamp examination
• Direct visualization of the eye to assess for optic nerve compression
and assessment of the angle between the meshwork and iris
116
▪ Tonometry (Goldmann applantation tonometry = gold standard)
• Device used to assess intraocular pressure
▪ Visual field assessment
• Assessment of peripheral vision loss
142
Management of open angle glaucoma?
Management
▪ Medications to decrease IOP (begun when IOP >24mmHg)
• 1
st line = latanoprost eye drops (prostaglandin analogue)
o Causes increased aqueous flow through the canal of Schlemm
• Other options:
o Beta-blockers (timolol) → decreased fluid production
o Carbonic anhydrase inhibitors (dorzolamide) → decreased
production
o Alpha-2 agonists → reduced production and increased flow
▪ Surgery (refractory to medical management)
• Creation of a new channel in the anterior chamber for aqueous humour
to exit
143
What is an acute angle closure glaucoma?
Acute Angle Closure Glaucoma (less common; acute presentation)
o Ophthalmic emergency that occurs secondary to acute bulging of the iris → blockage of
the trabecular meshwork → acute, severe increase in IOP → permanent blindness if left
untreated
▪ Raised IOP is particularly severe in the posterior chamber → further bulging of
the iris → further compression
144
Acute angle closure glaucoma cause?
Cause
▪ Certain risk factors → older age, female, family history, Asian ethnicity, shallow
anterior chamber
▪ Medications → noradrenalin, anticholinergics, TCAs
145
Presentation of acute angle closure glaucoma?
Presentation
▪ Sudden-onset symptoms, including:
• Unilateral red and painful eye with a hazy cornea
• Eye appear to be bulging, and is hard when palpated
• Frontal headaches, nausea and vomiting
• Blurred vision and halos around the eye
• Mid-dilated, irregular, and unresponsive pupil
146
Investigation for acute angle closure glaucoma?
Diagnostics
▪ Slit-lamp examination
• Direct visualization of the eye to assess for optic nerve compression
and assessment of the angle between the meshwork and iris
116
▪ Tonometry (Goldmann applantation tonometry = gold standard)
• Device used to assess intraocular pressure
▪ Visual field assessment
• Assessment of peripheral vision loss
147
What is the management for acute angle closure glaucoma?
```
Management
Acute care
• Urgent ophthalmology review
• Lay the patient on the back
• Administer pilocarpine eye drops (cholinergic agent) and oral
acetazolamide
• Provide analgesia and anti-emetics
```
Secondary care
• Consider use of other agents to reduce IOP → glycerol/mannitol or
other agents used for management of open angle glaucoma
Laser iridotomy
o Definitive treatment that is often required
o A hole is made between the anterior and posterior chambers to
decompress the posterior chamber
