3a Neurology Flashcards
What is neuromyelitis optica (Devic’s syndrome)?
Transverse myelitis + optic neuritis
Anti-aquaporin 4 positive (NMO IgG)
Normal CSF and MRI
Admit to hospital and give IV steroids + plasma exchange
Immunosuppression in long-term: AZT/MTX/rituximab
What is transverse myelitis?
Inflammation of spinal cord causing bilateral leg weakness and numbness, pain, flexor spasm and incontinence.
ADMIT as emergency
What are the management options for MS?
Acute: IV methylprednisolone
Long-term RRMS
- IFN beta or galatiramer
- Dimethyl fumarate/fingolimod/teriflunomide
- Natalizumab or alemtuzumab
SPMS: siponimod
Symptoms: baclofen (spasticity), gabapentin, flu vaccine
What is Miller Fisher Syndrome and Bickerstaff’s Brainstem Encephalitis?
NO weakness
Miller-Fisher Syndrome (form of GBS)
- Areflexia
- Ataxia
- Opthalmoplegia
Bickerstaff’s Brainstem Encephalitis
- MFS + Babinski positive + drowsiness/altered GCS
What are the main features of Guillain-Barré syndrome?
Symmetrical, ascending and affecting the proximal muscles worst and affecting distal muscles earliest
Weakness, hyporeflexia, hypotonia
Pain (aching/throbbing around shoulders, back, buttocks, thighs) and numbness
Can have paraesthesia
Autonomic dysfunction (hyper/hypotension, tachy/bradycardia)
CN 7 palsy, opthalmoplegia, diplopia
Dysarthria, dyspnoea, dysphagia
What are the investigations and results in Guillain-Barré syndrome?
LFT (raised transaminase)
Spirometry (low FVC)
CSF analysis (normal WCC, cytoalbuminologic dissociation = lots of proteins)
EMG (slowed conduction)
ECG (ST depression, widened QRS, AV block)
When would GBS go to ITU?
FVC < 1.5L or < 90% predicted
Severe autonomic dysfunction
What are the risks for GBS?
Post-partum
Hodgkin’s lymphoma
Respiratory or GI infection
Vaccinations
What are the complications of GBS?
SIADH
Respiratory failure
Muscle wasting
VTE
Aspiration pneumonia
Permanent paralysis/weakness
What are the risk factors for developing WKS?
Hyperemesis gravidarum
Malnutrition (homelessness, alcoholism, malabsorption - coeliac/IBD, short bowel, starvation)
CKD and HF
Increased use (AIDS, malignancy, hyperthyroidism)
Bariatric surgery
Laparotomy and TPN
Old age
What are the investigations and results in WKS?
Low B1
Red cell transketolase (low)
Pyruvate (raised)
How would you manage migraines?
Acute:
- NSAIDs +/- triptans +/- antiemetics
Prophylactic:
- Propranolol or topiramate
- Amitriptylline or acupuncture
- Botox, riboflavin or biologics
- Menstrual = progestogen contraception or mefenamic acid
How would you manage trigeminal neuralgia?
Acute:
- Carbamazepine
Prophylactic:
- Rhizotomy
- Surgical decompression
- Botox
How would you manage cluster headaches?
Acute:
- O2 + triptans
+/- lidocaine + metoclopramide
Prophylactic:
- Verapamil
How would you manage GCA?
Refer to secondary care urgently
Acute:
- IV methylprednisolone or PO prednisolone depending on severity
Long-term:
- PO prednisolone
- Steroid-sparing: tocilizumab, methotrexate
What are some causes of trigeminal neuralgia?
Tumours
AVM
Aneurysm
Superior cerebellar artery compression
Cysts
MS
Sarcoidosis
Chiari malformation
What medications are used in generalised epilepsy?
Tonic-clonic: valproate or lamotrigine/levetiracetam
Tonic/atonic: valproate or lamotrigine
Myoclonic: valproate or levetiracetam
Absence: ethosuximide or valproate/lamotrigine/levetiracetam
What medications are used in focal epilepsy?
Lamotrigine/levetiracetam
Carbamazepine
What are the causes of extradural haemorrhage?
Rupture of middle meningeal artery or vein
Tear in dural venous sinuses
Lumbar puncture or spinal anaesthesia (epidural)
What do you see on CT with an extradural?
Bi-convex lentiform shape ‘D’
What are some complications of an EDH?
Death
Disability
Headaches
Spasticity, neuropathic pain and urinary dysfunction if spinal
Seizures
How would you manage an EDH?
- A to E assessment and stabilise
- Refer to neurosurgery for drainage (craniotomy, craniectomy, Burr hole)
- Reduce ICP by: tilting bed, sedating, giving hypertonic saline and mannitol
What are some causes of a subdural haemorrhage?
Ruptured bridging veins or cortical artery bleed
NAI (shaken baby syndrome = retinal haemorrhage + subdural haemorrhage + encephalopathy)
Trauma (including acceleration-deceleration injury and falls)
AVM, aneurysms
CSF hypoperfusion
Dural metastases
Risks: extremes of age, alcoholism, clotting disorder
How would a SDH appear on CT?
Concave/crescent-shaped
What would suggest raised ICP?
UMN signs
Decrease in GCS
Focal neurological deficits (weakness, numbness, impaired speech in form of dysphasia and dysarthria)
Decreased consciousness and confusion
Personality change
CN 3 and 6 palsies
Fixed-dilated pupil
Nausea and vomiting
Abnormal gait/ataxia
Cushing’s reflex: raised BP + low HR + irregular RR
Papilloedema
Seizures
Diplopia
Impaired upward gaze
What is the Bamford (Oxford) Classification?
Partial (2/3) and total (3/3) anterior circulation stroke
- Homonymous hemianopia
- Unilateral weakness or sensory loss
- Higher cognitive dysfunction
Lacunar stroke
- Pure sensory
- Pure motor
- Mixed sensory motor
- Ataxic hemiparesis
Posterior circulation stroke
- Crossed signs
- Bilateral weakness or sensory loss
- Cerebellar signs
- Homonymous hemianopia with macular sparing or isolated homonymous hemianopia
- Conjugate eye movement disorder
When will you consider a carotid endarterectomy/stent?
Stenosis of carotids must be at least:
- NASCET > 50%
- ECST > 70%
What is Terson’s syndrome?
Vitreous haemorrhage
What are some specific complications of an ischaemic stroke?
Secondary haemorrhagic transformation, malignant MCA syndrome
What are some specific complications of a haemorrhagic stroke?
Hypopituitarism, seizures, hydrocephalus, low sodium (?SIADH), re-bleed, vasospasm
Why is a painful CN3 palsy important?
Indicates a PComm artery aneurysm or SAH
What causes SAH?
Ruptured berry aneurysm
Trauma
AVM
Vertebral artery dissection/rupture
What are the features of SAH?
Sudden-onset occipital headache, collapse/decreased GCS and consciousness
N&V
Seizures
Focal deficits and raised ICP
Neck stiffness due to meningeal irritation
What is Cushing’s triad?
Hypertension with widened pulse pressure (systolic is raised)
Bradycardia
Irregular respirations
What is the imaging of choice in TIA?
Diffusion weighted MRI
What is Huntington’s disease? What neurones are lost in Huntington’s?
Huntington’s disease is an AD inherited CAG repeat affecting chromosome 4
- Also exhibits anticipation so later generations are more affected
- Decrease GABA and ACh neurones in the caudate nucleus and putamen
- Hence less inhibition of the thalamus
What are the main features of Huntington’s?
Triad: psychiatric + chorea + dementia
Prodrome of depression/apathy/personality change
Presentation
- Chorea (semi-purposeful jerky movements that cease with sleep)
- Psychiatric: apathy/depression/anxiety, dementia, obsessions and compulsions
- Abnormal eye movements + diplopia + nystagmus
- Dysarthria and dysphagia
- Movement: dystonia, bradykinesia, ataxia
Late:
- Babinski +
- Spasticity
How would you treat Huntington’s?
Chorea: tetrabenazine or atypical antipsychotics
Psychosis: atypical antipsychotics (olanzapine, risperidone, quetiapine)
Depression: SSRI/SNRI
Bradykinesia: L-DOPA or dopamine agonists
What are some Parkinson’s + syndromes?
Progressive supranuclear palsy
- Symmetrical onset
- Impaired upward gaze
- RIGIDITY > tremor
- Problems with speech and swallow
- Early postural instability
Multi-system atrophy
- Early autonomic dysfunction (falls, postural hypotension, incontinence)
- RIGIDITY > tremor
- Cerebellar + pyramidal (UMN) signs
Cortico-basal degeneration
- Akinetic rigidity
- Cortical sensory loss
What are a few side effects of dopamine agonists?
Pathological gambling // compulsive behaviours
Drowsiness
Hallucinations
What are some side effects of L-DOPA?
Dyskinesia
Painful dystonia
Postural hypotension and falls
Psychosis (remember increased dopamine causes psychosis)
N&V
Dry mouth
On-off effect
What is Parkinson’s disease and what are the main categories of Parkinson’s symptoms?
What is Parkinson’s disease?
- Loss of dopaminergic and ACh neurons in the zona compacta of substantia nigra
- Lack of dopamine favours indirect pathway = less thalamic stimulation
- Also has Lewy bodies (eosinophilia bodies - alpha synuclein, ubiquitin and neurofilament)
Triad: bradykinesia + rigidity + resting tremor (3-5Hz)
Movement: refer to examination findings, small writing, soft voice, freezing at doors, difficulty initiating movements, difficulty buttoning clothes, drooling
Psychological: obsessions, compulsions, depression, anxiety, apathy, hallucinations/delusions
Cognitive: late dementia
Sleep: vivid dreams, REM sleep disorder, acting out dreams
Autonomic: constipation, anosmia, hypotension, late falls, late incontinence
ADLs: late loss of eating and drinking
How would you treat Parkinson’s?
Movement: L-DOPA + benserazide/carbidopa.
- DA: ropinirole, pramipexole, rotigotine
- COMT-i: tolcapone, entacapone
- MAOB-i: rasagiline, selegiline
- Amantadine
Drooling: glycopyronium, atropine, hyoscine, amitriptyline
Sleep: melanin?
Postural hypotension: midodrine or fludrocortisone
Daytime sleepiness : modafinil
Dementia: rivastigmine/galantamine/donepezil > memantine
Depression: SSRI
BPSD: quetiapine or clozapine (NOT haloperidol or risperidone)
What are the positive findings on examination of a Parkinson’s patient?
Contralateral synkinesis
Rigidity and bradykinesia
Decrease in amplitude when tapping toes or fingers
Slow festinant gait and shuffling steps
Difficulty turning
Reduced arm swing
Slow blinking and blank face
Resting tremor
What do you not see in early Parkinson’s?
Incontinence
Dementia
Bilateral involvement
Falls
When would you admit MG to ITU?
- Negative inspiratory pressure < 20 cmH2O
- FVC < 1L
How would you manage myasthenia gravis?
Pyridostigmine
Steroids + AZT
Mycophenolate/cyclophosphamide/ciclosporin/MTX
Rituximab/plasma exchange/IV Ig
Thymectomy
ITU = intubate + ventilate + plasma exchange
What are the features of myasthenia gravis?
Painless fatiguable weakness + normal sensation + normal reflexes + tone
No autonomic features
Weakness affects proximal muscles worst
Areas: face and eyes/neck > limbs > trunk
Dysphagia, dysarthria, dysphonia, ptosis, dyspnoea, myasthenic snarl
Diplopia, LR weakness, inability to sustain upward gaze
What is a cholinergic crisis?
Overtreatment of MG
Diarrhoea
Bradycardia and hypotension
Weakness
Miosis
Sweating and salivation
Give atrophine or propantheline
What is associated with MG?
Thymoma or thymic hyperplasia
HLA B8 and DR3
Other autoimmune conditions: DM, thyroid, coeliac, Addison’s, RA, NMO, MS, SLE, pernicious anaemia
What is MG?
Autoimmune NMJ disorder where antibodies are formed against ACh-receptors on post-synaptic membrane of skeletal muscle (damage and endocytosis)
Antibodies: anti-ACh receptor, anti-MUSK / muscle specific tyrosine kinase, anti-LPR4 / low density lipoprotein receptor related protein
What is the most important in assessing respiratory failure/myasthenic crisis in MG?
FVC goes before saturations and blood gases
CO2 is raised before O2 drops
Associated with anti-MUSK antibodies
What is associated with Lambert-Eaton syndrome?
Small cell lung cancer
Other malignancies lymphosarcoma, breast, stomach, colon, prostate, bladder
What are the main features of Lambert-Eaton syndrome?
Autonomic dysfunction + weakness (gait > eyes/face)
Weakness: waddling gait, difficulty washing hair
Autonomic: hypotension, erectile dysfunction, dry eyes and mouth
Areflexia + normal sensation
Repeated use increases response due to potentiation
Which antibodies are involved in LES?
Anti-P/Q types VGCC antibodies
What pattern of weakness differentiates MG from LES?
MG starts in the eyes/face while LES starts in the extremities
MG has normal reflexes while LES is areflexic
MG does not have autonomic involvement
LES can increase strength with effort whilst MG loses strength
What is Duchenne’s muscular dystrophy?
X-linked recessive causing lack of dystrophin production leading to necrosis of myofibres = muscle weakness (everywhere is affected)
Presents in early childhood with
- Waddling gait + toe walking + walking up the stairs with 2 feet on each step
- Clumsiness and falling
- Delayed milestones (including language)
- Gower’s sign
- Pseudohypertrophy of calves
Associated with dilated cardiomyopathy and scoliosis
Management
- Prednisolone or ataluren if still mobile
- Ventilation
- Supportive management later on
Raised CK
Genetic testing and muscle biopsy
What is myotonic dystrophy? How is it inherited?
Sustained muscle contractions + cataracts + facial weakness (i.e., opened mouth)
AD inheritance with anticipation!
What is Emery-Dreyfus dystrophy?
Progressive weakness + lots of contractures + cardiac involvement
What are a few important points about Friedreich’s ataxia?
Autosomal recessive trinucleotide repeat disorder affecting GAA (frataxin) on chromosome 9
DCML loss + dysarthria + ataxia + absent ankle reflexes but Babinski +
- Think cerebellar + corticospinal + DCML
Distal wasting in LL + pes cavus
Associated with HOCM, kyphoscoliosis and T1DM
What are a few features of fascioscapulohumeral dystrophy?
Weakness in face, shoulders and arms
Sleeps with eyes partially opened and cannot puff cheeks
What is idiopathic intracranial hypertension? Give me a short summary.
Risk factors
Presentation
Management
- Weight loss
- Acetazolamide
- Optic nerve fenestration (sight preserving)
When do you get neck stiffness?
Meningitis
SAH
What are some triggers for cluster headaches?
Alcohol
Lack of sleep
Histamine
Strong smells
Histamine
Nitroglycerin
What is a chronic cluster headache?
Headache free interval < 3 months
OR
1 year without remission
What are some features of a cluster headache?
Unilateral
Increased PSNS: miosis, lacrimation, rhinorrhoea, ptosis
Red swollen eye
Very severe pain behind the eye and in temporal-orbital region
Restlessness/agitation
15 minutes to 3 hours (can recur multiple times a day)
Can you summarise GCA?
ANCA - granulomatous vasculitis affecting medium and large vessels
Associated with polymyalgia rheumatica
Presentation
- Eyes: loss of colour vision, loss of visual acuity, diplopia, amaurosis fugax
- Pain: severe, temporal-occipital region, worse at night, sharp scalp tenderness precedes pain!, jaw claudication
- Systemic symptoms: fever, fatigue, loss of appetite, weight loss
- Optic disc: pale, oedematous
- Retinal haemorrhages on fundoscopy
- Artery: prominent, beaded, non-pulsatile, can be tender
Investigations:
- Biopsy: giant cell infiltrates
- CRP and ESR: very raised
- ANCA: negative
- FBC: NN anaemia
- USS: halo appearance
How would you describe a migraine aura?
NOT hemiplegia/weakness
Occurring before onset of headache
- Dysarthria
- Scotomas or fortification spectrum
- Tingling/paraesthesia/numbness
Prodrome:
- Irritability
- Cravings
- Sensitive to lights/sounds/smells
What are some triggers for migraines?
Chocolate, cheese, caffeine
Alcohol
COCP/HRT
Hunger
Menstruation
Stress
Dehydration
Lack of sleep
Sounds/smells/lights
?Patent foramen ovale
How would someone with migraines describe their headache?
4-72 hours
Subacute onset
Unilateral - frontotemporal
Throbbing/pulsatile pain that is moderate-severe
Nausea/vomiting
Photophobia/phonophobia
Worse with movement
N.B., If no aura, need 5 migraine without aura to get diagnosis
What would you NOT give someone with migraine + aura?
COCP = increased VTE and stroke risk
What are some complications of migraine?
Migrainous infarction
Status migrainosus
Stroke/VTE
Can you summarise tension headache in 5 sentences?
Risk factors include stress/anxiety/depression and poor posture
Bilateral tight/pressing pain that is gradual in onset and described as being mild-moderate
Does not have photophobia/phonophobia or N&V and not worse with activity
Managed with regular painkillers: NSAIDs (ibuprofen/naproxen) or paracetamol
Prophylaxis: amitriptyline, CBT, acupuncture
What is chronic tension headache?
> 15 days a month
What are the most common medication-overuse headache?
Overuse
- 15 or more days/month of simple painkillers (PCM, NSAIDS)
- 10 or more days/month of stronger painkillers (triptans, ergotamines)
Types:
- Migraines (most common)
- Tension headaches
You would manage by going cold-turkey with painkillers (except if using opiates - wean slowly to prevent withdrawal) then aim to start prophylaxis!
What is status epilepticus? How would you manage status epilepticus?
Seizure lasting > 5 minutes or recurrent seizures without regaining consciousness
What to do?
- 1st: benzodiazepine (PR diazepam, buccal midazolam, IV lorazepam) - give 2nd dose + 999 if no response to initial dose
- 2nd: IV phenytoin/valproate/levetiracetam
- 3rd: phenobarbital or general anaesthesia
Also:
- A to E assessment
- Get O2 saturations and capillary glucose
- Bloods: CRP, FBC, blood gases, blood glucose, antiepileptic levels, electrolyte panel, U&E, toxicology
What are some risk factors for developing seizures?
Head injury
Stroke
Dementia syndromes
Neurofibromatosis or tuberous sclerosis or Dravet’s
Electrolyte imbalance (especially sodium, magnesium and calcium)
Hypoxia
Hypoglycaemia
Infection (meningitis, encephalitis, TB, toxoplasmosis)
Prematurity
Cerebral palsy
Epilepsy syndromes
Complex febrile seizures
Tumours
Raised ICP (hydrocephalus, tumours, haematoma)
What happens to prolactin after a seizure?
Can be raised
How would someone with WKS present?
Wernicke’s encephalopathy
- Ataxia
- Confusion
- Eyes (nystagmus, opthalmoplegia)
Korsakoff’s pyschosis
- Agnosia
- Flattened affect/apathy
- Amnesia (retrograde and anterograde)
- Confabulation
- Disorientation
- Inattention
- Telescoping of events (old events seem new to them)
What is Wallernberg’s syndrome?
Horner’s syndrome (ptosis, miosis, anhidrosis)
Diplopia
Dizziness
Dysarthria
Found in PICA strokes or lateral medullary syndrome
How would you manage a TIA?
Aspirin 300mg if not already on aspirin and refer to secondary care within
- 24 hours if presenting < 7 days
- 7 days if presenting > 7 days
- Do not use ABCD2 to stratify risk of stroke
No driving for 1 month
Investigations
- Diffusion weighted MRI
- Carotid USS and doppler
Management (long-term)
- Clopidogrel + statin + ACE-i/ARB
- If NASCET > 50% or ECST > 70% then carotid endarterectomy
How would you manage an ischaemic stroke?
CT head ASAP
MR angio or CT angio prior to thrombectomy
If thrombolysis, ensure BP < 185/110 mmHg and CT head 24 hours after procedure + withhold aspirin for 24 hours
Acutely
- Aspirin 300mg and continued for 14 days or until discharge whichever is sooner
- If presenting < 4.5 hours = alteplase
- If presenting < 6 hours = thrombectomy (if modified Rankin < 3 and NIHSS > 5)
- If longer than that just give aspirin only
- Only lower BP if encephalopathy or malignant hypertension or dissection or end-organ damage due to raised BP
Long-term (no driving for 1 month)
- Consider endarterectomy based on NASCET or ECST
- Clopidogrel + statin + ACE-i/ARB
- If AF, use warfarin/DOAC > clopidogrel
- If venous sinus thrombosis: warfarin
Other investigations
- USS and Doppler carotids
- FBC, U&E, LFT, glucose, cholesterol
- Vasculitis screen depending on age of patient
How would you manage a haemorrhagic stroke (SAH)?
CT head + lumbar puncture (xanthochromia - earliest 6 hours post-stroke)
MR angio/CT angio
What next?
- Stop anticoagulants and reverse if possible
- Nimodipine/labetalol/nitroprusside to prevent vasospasm
- Refer to neurosurgery for coiling (preferred) or clipping of aneurysm
- Manage raised ICP
= Tilt bed, sedate and ventilate
= Hypertonic saline
= Mannitol
Maybe
- If < 6 hour and systolic BP 150-220 then lower to 140-160 mmHg
What are some contraindications to thrombolysis?
INR > 1.7
Haemorrhagic stroke
Major surgery in last 14 days
BP > 185/110 mmHg
Head injury
What are some symptoms of posterior circulation stroke?
Crossed signs
Bilateral weakness/sensory loss
Cerebellar signs (nystagmus, ataxia, vertigo, N&V, dysarthria, intention tremor)
Isolated homonymous hemianopia (or homonymous hemianopia with macular sparing)
Inter-nuclear ophthalmoplegia (inability to adduct 1 eye, unaffected eye demonstrates nystagmus)
Others:
- Decreased GCS
- Collapse
- Diplopia
- Dysphagia
How would a basilar artery stroke present?
Stepwise deterioration
‘Locked in’ syndrome
How would an anterior circulation stroke present?
Homonymous hemianopia
Cortical dysfunction (e.g., dysphasia, visuospatial ability, emotional control, executive function)
Contralateral weakness and/or sensory loss
Weakness causing
- Dysphagia
- Dysarthria
- Facial droop (including ptosis)
How would lateral medullary syndrome present (Wallenberg)?
How would a posterior inferior cerebellar artery stroke present?
More sensory (vs AICA)
How would an anterior inferior cerebellar artery stroke present?
More motor (vs PICA)
What are some risk factors for an ischaemic stroke?
What are some risk factors for a haemorrhagic stroke?
How would a subarachnoid haemorrhage present?
What is a subarachnoid haemorrhage?
- Bleed into subarachnoid space
Headache
- Sudden-onset very severe occipital headache
Meningism - neck stiffness due to meningeal irritation (no fever)
Collapse/loss of consciousness
Painful CN3 and fixed-dilated pupil
Raised ICP
- Seizures
- N&V
- Ataxia
- Personality change
- Loss of higher order function (dysphasia, drowsiness, confusion)
- Decreased GCS
- Weakness/numbness
- CN3/6 palsy
- Papilloedema
Vitreous haemorrhage
How would a CN3 palsy present?
Down and out pupil
Fixed dilated pupil if PSNS fibres are compressed
What is Lambert-Eaton syndrome?
Antibodies form against P/Q types VGCC affecting depolarisation
- Presynaptic
- Affects NMJ and autonomic ganglia
Management:
- Amifampridine (3,4-diaminopyridine)
- Pyridostigmine
- Treat underlying malignancy
When would you admit someone with MG to ITU?
FVC < 1L
NIP < 20 cm H20
Indicates respiratory failure
What are the common causes of death in Huntington’s disease?
Suicide
Pneumonia/respiratory failure
Cardiomyopathy
Can you tell me about normal pressure hydrocephalus?
A form of communicating hydrocephalus with dilated ventricles and normal CSF pressure on LP (< 20 cmH2O)
Triad: incontinence + cognitive impairment + gait apraxia
Severe: spasticity, hyperreflexia (UMN!)
Investigations
- MRI (enlarged ventricles, normal cortex) // especially temporal horns of lateral ventricles
- Large volume LP
- Lumbar infusion (intrathecal infusion - abnormal sustained rise)
Management
- VP shunt (definitive)
- Acetazolamide
- Large volume LP
What are some risk factors for developing NPH?
Think outflow obstruction
- Tumours
- Haemorrhagic stroke
- Meningitis
How would raised ICP hydrocephalus present in an infant/young child?
Sun setting sign (bilateral downward deviation of eyes - pupils partially covered by lower eyelid)
Macewen sign tapping pterion = hyperresonant sound
Swollen fontanelle and widened sutures
- Or large head
Irritability, lethargy, personality change
Not feeding, vomiting
Papilloedema, seizures
How would raised ICP hydrocephalus present in older children and adults?
Headache, nausea and vomiting
Drowsiness/decreased GCS, irritability and personality change
Cognitive impairment
Seizures
Hypertonia (spasticity)
Papilloedema - blurred vision
CN3 and CN6 palsy
- Fixed dilated pupil
- Lateral gaze palsy
- ? Down and out pupil (but CN6 palsy = no ‘out’)
- DIPLOPIA
Impaired vertical gaze
How would you investigate raised ICP hydrocephalus?
Open fontanelle: USS!
Others: MRI/CT head
How would you manage raised ICP hydrocephalus?
VP shunt or extraventricular drain
Acetazolamide
Repeated LP
What are some causes of hydrocephalus?
Aqueduct stenosis
Atresia of foramina of Magendi and Luschka
Neural tube defects
Agenesis of foramen of Monro
Tumours
Haematoma
What are some causes of raised intracranial pressure?
Meningitis/encephalitis causing oedema
Malignant MCA syndrome
Haematoma (EDH, SDH, SAH)
Tumour
Idiopathic intracranial hypertension
Status epilepticus
Fever
Hypoxia
What is GBS?
Type 4 hypersensitivity, due to molecular mimicry, causing AIDP
- Involves T cells and macrophages
Commonly due to C.jejuni
Can also be: EBV, CMV, HIV, VZV, H.influenzae
How does MS present?
Triad: dysarthria + intention tremor + nystagmus
Lhermitte’s and Uhtoff’s
Transverse myelitis
- Bilateral weakness and pain
- Incontinence
- Flexor spasm
Face
- Trigeminal neuralgia
- CN7 palsy (upper therefore forehead sparing)
- Dysarthria
- Dysphagia
Eyes + optic neuritis
- Dyschromatopsia
- Blurred vision
- Pain
- Oscillating vision
- Diplopia
- LR palsy
- Nystagmus (cerebellar involvement)
Limbs
- Weakness (UMN pattern - spastic, hyperreflexic, Babinski +)
- Paraesthesia
Ears
- Sensorineural deafness
- Vertigo
Cerebellar
- Dysdiadochokinesis
- Ataxia
- Nystagmus
- Intention tremor
- Dysarthria
Psychological
- Depression/anxiety
- Cognitive impairment
- Dementia (late)
Autonomic
- Erectile dysfunction
- Incontinence (urge)
- Constipation
What is MS? Are there any risk factors? How would you investigate for MS?
Pathophysiology
- Demyelinating disease affecting the oligodendrocytes
- T cells identify oligodendrocytes and activate B cells which produce antibodies
- Macrophages recognise antibodies and destroy oligodendrocytes
Risk factors
- Female
- Caucasian
- EBV infection
- Living further from equator (low vitamin D?)
- HLA B8 DR3
- FHx and other autoimmune disease
Investigations
- Diffusion-weighted MRI (paraventricular and spinal white matter plaques)
- CSF analysis (oligoclonal IgG bands not present in serum)
- Anti-aquaporin 4 (negative)
What cells are affected in MS?
Oligodendrocytes
What cells are affected in GBS?
Schwann cells
What would you prescribe to close contacts of meningitis patients? Who is considered a ‘close contact’?
Prophylaxis (aim to give within 24 hours of diagnosis, warn that there is still risk of developing meningitis so safety net)
- Ciprofloxacin
- Rifampicin
- Ceftriaxone
Who? Those within 7 days of onset of illness who are
- Same household
- Communal kitchen (e.g., shared flat/house)
- Close partners (e.g., spouse)
- Bed sharing
How would you manage meningitis?
IM benzylpenicillin pre-hospital and non-blanching rash (no rash then hospital ASAP)
Hospital
- IV cefotaxime + amoxicillin (if < 3 months)
== NOT ceftriaxone as risk of jaundice
- IV ceftriaxone (> 3 months)
- Dexamethasone (> 3 months)
- Antiepileptics + fluids
TB meningitis
- Rifampicin + isoniazid for 12 months (total)
- Pyrazinamide + ethambutol for 2 months given alongside rifampicin and isoniazid
First steps
- A to E
- GIVE fluids to resuscitate if necessary (even if before LP!)
- Do not delay antibiotics just to obtain LP, can still get a reasonable sample if taken soon after antibiotics have been started
Investigations
- Blood cultures, CRP, glucose
- Lumbar puncture + CSF analysis
- ABG
- Coagulation screen due to differentials of petechiae
- N.meningitides PCR
CT is NOT helpful in determining raised ICP
How would you manage meningococcal septicaemia?
IM benzylpenicillin pre-hospital if non-blanching rash
Do NOT give dexamethasone!
Do NOT LP
ALL get IV ceftriaxone even if < 3 months
What organisms are involved in bacterial meningitis?
Neonates: GBS (agalactiae), listeria, E.coli, nisseria meningitides, haemophilus influenzae, streptococcus pneumoniae
Children: nisseria meningitides, haemophilus influenzae, streptococcus pneumoniae
Adults: nisseria meningitides, streptococcus pneumoniae
Elderly: listeria, nisseria meningitides, haemophilus influenzae, streptococcus pneumoniae
How would meningitis present?
Triad: fever + headache + neck stiffness
Photophobia
Nausea and vomiting
Non-blanching petechial rash (indicates co-existing meningococcal septicaemia)
Kernig’s and Brudzinski’s sign
Infants can have bulging fontanelle
Focal neurological deficits
Decreased GCS, seizures and personality change are late signs
Shock: prolonged CRT, mottled skin, cold peripheries, tachy/bradycardia, hypotension
How would bacterial meningitis present?
CSF
- WCC: raised, neutrophil dominant
- Glucose: low (<0.5 serum/plasma)
- Protein: raised (> 1g/L)
- Purulent appearance
How would TB meningitis present?
CSF
- WCC: raised, lymphocyte dominant
- Glucose: low (< 0.5 serum/plasma)
- Protein: raised (> 1g/L)
- Cloudy + fibrin web
How would fungal meningitis present?
CSF
- WCC: raised, lymphocyte dominant
- Glucose: low
- Protein: raised
- Cloudy appearance
How would viral meningitis present?
CSF
- WCC: raised, lymphocyte dominant
- Glucose: normal or slightly low
- Protein: normal or slightly raised
- Can be clear or cloudy
What is encephalitis? How would someone present and how do you manage?
Encephalitis is inflammation of the brain parenchyma
Causes
- Commonly viral: VZV, EBV, CMV, rabies, mumps, rubella, measles, Japanese encephalovirus, HSV, HIV
- Subacute sclerosis panencephalitis = late complication of measles
- Autoimmune: SLE
- TB, Lyme, malaria
Presentation
- Flu-like prodrome
- Triad: fever + headache + altered mental status
- Early personality change and seizures
- Generally abnormal behaviour?
- Confusion/disorientation
- Nausea and vomiting
- Those of raised ICP + focal deficits
Investigations
- CT head (HSV1 shows bilateral medial temporal lobe involvement)
- LP + CSF analysis/PCR if not CI
- Bloods: FBC, CRP, blood cultures, INR, ABG, glucose, electrolyte panel, LFT, U&E
- O2 saturations + BP + neurological examination
Management
- Mainly supportive
- IV acyclovir ASAP
What is dementia? What are the most common causes of dementia?
What is dementia?
- Progressive and irreversible decline in higher cognitive function affecting ADLs
- Higher function: memory, cognition (including visuospatial), language, executive function and personality
- NORMAL consciousness
Causes
- Alzheimer’s
- Vascular dementia
- Lewy body dementia
- Frontotemporal dementia
What are some ‘organic’ causes of dementia?
Endocrine/nutritinonal
- Low B1/thiamine (WKS)
- Low B12
- Low folate
- Cushing’s
- Hypothyroidism
Neurological
- Raised ICP (hydrocephalus, tumours, haematoma)
- MS
- Stroke
- Depression causes ‘pseudodementia’
- PD and HD
Infectious
- Syphilis
- HIV
- CJD
How would Alzheimer’s present? How would you manage patients with Alzheimer’s?
Early
- Memory lapses (forgetting events or appointments)
- Word finding difficulties
Progressive decline in
- Language
- Memory
- Higher order thinking (planning, organising, critical/abstract reasoning)
- Loss of visuospatial ability
- Agnosia/anosognosia/aphasia/apraxia
Late
- Incontinence
- No eating/drinking
- Depression
- Hallucinations/delusions
- Aggression
- Disorientation/wandering
- Apathy
- Echolalia/palilalia
Management
- Rivastigmine/donepezil/galantamine
- Memantine
- BPSD: risperidone or ____
What is frontotemporal dementia and how would patients present? Can we manage using medications?
Semantic
- Retains fluent speech but loses vocabulary
Progressive non-fluent aphasia
- Retains simple comprehension but struggles with speech
- Does not really understand complex sentences but knows individual words
Behavioural
- Classical frontotemporal inappropriate behaviour (disinhibition, impulsive, apathetic, obsessive, compulsive, increased appetite, hyperorality)
Memory and spatial orientation goes late in disease
Do NOT medicate except if managing distress/behavioural difficulties
What is Lewy body dementia and how do we manage?
Presentation
- Fluctuating cognition
- Visual hallucinations
- REM sleep disorder
- Bradykinesia/Parkinsonism
- Dementia
Management
- Rivastigmine/donepezil/galantamine
- Memantine
- BPSD: quetiapine or clozapine
What is vascular dementia? Can we treat vascular dementia?
Dementia caused by vascular events (e.g., infarcts)
Patients typically present with a stepwise decline in cognitive function alongside those of previous stroke
Only give medication if co-existing AD or DLB
Best is to treat risk factors for vascular disease!
What are some causes of delirium?
Constipation/urinary retention
Hypoxia
Infection - UTI, pneumonia, meningitis, encephalitis, peritonitis
Metabolic: hypoglycaemia, hyperglycaemia, Na+, Ca2+, carcinoid, porphyria
Pain
Sleeplessness
Prescriptions
Hypothermia/pyrexia
Organ dysfunction (HF, CKD, liver failure)
Neoplasm/nutrition deficiency B1/3/9/12
Environment change
Drugs/medications
Neurological bits: stroke, SOL (tumour, haematoma, hydrocephalus), MS, PD, Huntington’s, dementia syndromes
Any severe and/or chronic illness
What is delirium and how should we treat delirium?
Delirium
- Acute onset fluctuating consciousness
- Impaired alertness, cognition and attention
Compared to dementia
- Acute onset
- Reversible
- Impaired immediate recall
- Change in alertness/consciousness
- Altered sleep-wake cycle
- Fluctuating course
Non-pharmacological
- Keep environment similar, maintain same team, have familiar items and encourage visiting
- Try to re-orientate the patient
Pharmacological management
- Haloperidol 1st line
- Lorazepam 2nd line
What is the ‘confusion screen’ bloods?
FBC
Blood cultures
CRP?
U&E
LFTs
TFTs
Ca2+
Haematinics
B12
Folate
Glucose
Coagulation
CXR, urine dipstick, CT head, ECG
How would hyperactive and hypoactive delirium present?
Hyperactive
- Aggression/agitation
- Confusion
- Wandering
- Hallucinations and delusions
Hypoactive
- Apathy
- Depression
- Withdrawal
- Slowing of movements
- Difficult to wake
What are the common types of motor neurone disease? Can you describe them to me?
Diagnosis is clinical, management is supportive (apart from riluzole)
Investigations to exclude other causes: creatinine kinase, peripheral neuropathy screen, autoantibodies, MRI, EMG
Amylotrophic lateral sclerosis
- Mix of UMN and LMN signs
- Anterior horn cells + motor cortex are both affected
- Think MND when there is foot drop, split hand sign and gait change!
Progressive bulbar palsy
- LMN signs affecting the face (CN 7-12)
Primary lateral sclerosis
- UMN signs only
- Affects motor cortex Betz cells
Progressive muscular atrophy
- LMN signs only
- Anterior horn cell lesion only
- Distal muscles first
Pseudobulbar palsy
- UMN affecting corticobulbar tract
Can you tell me about Bell’s palsy?
Idiopathic LMN facial nerve palsy
Risk factors: DM, pregnancy
Presentation (unilateral, acute onset)
- Face: drooping, forehead sparing if UMN lesion
= Weakness can cause dysarthria and difficulty eating
- Loss of taste
- Hypersensitivity to sound or hearing loss
- Lack of salivation and lacrimation
Management
- Aim to treat within 72 hours
- Give prednisolone + eye care advice
- If no improvement after 3 weeks = referral to secondary care
What are some other causes of CN7 palsy?
UMN
- Syphilis
- Stroke
- MS
- Tumour
LMN
- Lyme disease
- GBS
- HIV
- VZV/EBV/CMV
- Trauma
- Tumours
- Iatrogenic
What is Ramsay-Hunt syndrome?
LMN CN7 palsy caused by reactivation of VZV virus in the geniculate ganglion
Presentation
- Facial droop without forehead sparing (loss of nasolabial fold, drooling, ectropion, etc.)
- Severe ear pain
- Vesicles visible in pinna/ear canal/palate/anterior 2/3 of tongue
- Vertigo, nystagmus, tinnitus
- Hearing loss OR hypersensitivity to sounds
- Lack of tears/salivation
Management
- Prednisolone + acyclovir within 72 hours (but up to 7 days) of symptom onset
- Eye care
Investigations
- VZV serology
- Audiometry?
Can you tell me about Ménière’s disease, labyrinthitis and BPPV?
Ménière’s disease
Labyrinthitis
BPPV
What is anterior cord syndrome?
Loss of anterior 2/3 of spinal cord
- Pyramidal tracts
- Lateral spinothalamic tracts
Causes
- Anterior spinal artery infarct (thromboembolism, hypotension, dissection, atherosclerosis)
- Direct compression of cord
Presentation (bilateral)
- UMN weakness
- Loss of pain and temperature sensation
- Normal proprioception an fine touch (DCML)
- SNS loss (T1-L2): hypotension
What is cauda equina syndrome?
Compression of the cauda equina (nerve roots below L2)
Causes: disc herniation, metastases, infection, trauma
Common sites: L4/5 or L5/S1
Presentation
- Acute onset
- Lower back pain
- LMN weakness (hypotonia, hyporeflexia) without UMN signs
- Sensory loss (perianal anaesthesia)
- Incontinence
- Foot drop
- Sciatica-type pain
Investigations
- MRI spine
Management
- Refer to neurology/neurosurgery/spinal surgery ASAP
- Dexamethasone if malignant cause
Can you summarise sciatica for me?
What is sciatica?
- L4-S1 = sciatic nerve
- Inflammation/compression of sciatic nerve
Presentation
- Sudden or gradual onset
- Pain in back and buttock radiating down LL in distribution of sciatic nerve
- Tingling and sensory loss
- Weakness
- Positive straight leg raise
Management
- NSAIDs
- Codeine + paracetamol
- Continue with normal activities
Can you summarise cervical spinal stenosis?
Narrowing of the cervical spinal canal causing compression of the spinal cord (myelopathy)
Presentation
- LMN signs below lesion
- UMN signs at level of lesion
- Weakness, numbness, paraesthesia affecting upper and lower limbs
- Loss of fine finger movements
- Neck pain and reduced ROM
- Gait instability
Can you tell me about lumbar spinal stenosis?
Presents with neurogenic claudication
- Cramping pain on exertion affecting lower limbs
- Is worse on extension of the hip joint hence able to climb up stairs but not down
- Claudication is not immediately relieved by rest
- Normal pulses and lack of CVD risk factors
- Can also have numbness and weakness
What problems can you have with your ulnar nerve?
Ulnar nerve - C7-T1
Passes through cubical tunnel in the elbow behind the medial epicondyle
Supplies sensation to medial 1.5 digits
Supplies motor function to flexor carpi ulnaris, small muscles of the hand, medial lumbricals and hypothenar muscles
Presentation
- Claw hand = cannot extend inter phalangeal joints and flex metacarpophalangeal joints
- Tingling, pain and numbness
- Muscle weakness
- Wartenberg’s sign = abduction of 5th digit
- Inability to cross fingers
- Fromet’s sign
Management
- Rest
- Splint
- Decompressive surgery
What can go wrong with the radial nerve?
Radial nerve - C5-T1
Runs with profunda brachial artery in spiral groove of humerus
Supplies
- Sensation to posterior forearm and dorsum of hand
- Motor to brachioradialis, extensors, abductor pollicis longus, supinator and triceps
Wrist drop
Inability to extend fingers
Sensory loss and paraesthesia
How would someone with a median nerve problem present?
Median nerve - C6-T1
Carpal tunnel syndrome
Anterior interosseous syndrome
Can you tell me about foot drop?
Inability to dorsiflex the foot
Can have multiple causes
- Common peroneal nerve palsy (ankle jerk present, weak eversion, sensory loss over dorsum and lower lateral leg )
- L5 radiculopathy (no ankle jerk, weak inversion)
- MS
- MND
- Stroke
- Parkinson’s
What are the components of the Glasgow Coma Scale?
Motor (6)
- Spontaneous
- Localises to pain
- Withdraws from pain
- Abnormal flexion (decorticate)
- Abnormal extension (decerebrate)
- No response
Voice (5)
- Spontaneous, oriented
- Confused speech
- Only saying words
- Only producing sounds
- No response
Eyes open (4)
- Spontaneously
- To voice
- To pain
- No response
What are the different types of peripheral neuropathies?
What are some late features of Alzheimer’s?
Any psychiatric symptoms indicate LATE dementia
Late
- Incontinence
- No eating/drinking
- Depression
- Hallucinations/delusions
- Aggression
- Disorientation/wandering
- Apathy
- Echolalia/palilalia
What are some non-pharmacological treatments for dementia?
Cognitive stimulation
Memory enhancement
Reminiscence therapy
Cognitive rehabilitation
How could you screen for dementia?
GPCOG
TYM
6CIT
10 pointer cognitive screen
6 item screen
Mini cog
Addenbrooke’s
MMSE
What is the pathology behind the different dementias?
Alzheimer’s: neurofibrillary (intra) tangles + beta-amyloid (extra) plaques
- Cortical atrophy = widened sulci and enlarged ventricles
- Neurofibrillary tangles = hyperphosphorylated tau proteins
Lewy body: eosinophilic intracytoplasmic inclusion bodies of ubiquitin/alphasynuclein/neurofilament
Frontotemporal: vacuolisation of fronto-temporal lobes + Pick’s bodies (tau-laden neurones)
What are some risk factors for developing dementia?
Trisomy 21 (amyloid precursor protein on C21)
Older age
Female
Smoking and no exercise
Depression
Low IQ/education
Social isolation
Cerebrovascular disease
FHx (presenilin, amyloid precursor protein, ApoE4)
How would you manage MND?
Refer urgently to neurology
Riluzole (anti-glutamatergic) + supportive management
How would common peroneal nerve palsy present?
Risk factors
- Sitting cross-legged
- Obstetric stirrups
- Fibula injury
Presentation
- Foot drop
- Ankle jerk reflex present
- Weak eversion
- Normal inversion
- Sensory loss over dorsum and anterolateral aspect of the leg
How would L5 radiculopathy present?
Foot drop
Abesent ankle jerk reflexes
Weak inversion
Normal eversion
Can you summarise essential tremor?
Presentation
- Tremor during movement
- Improves with alcohol
- Absent during sleep
- All other domains are normal
Tremor
- Fine
- Symmetrical
Management
- Propranolol
How would Brown-Sequard’s syndrome present?
Contralateral loss of pain and temperature sensation
Ipsilateral spastic paresis and loss of proprioception and fine touch
What is spinal cord compression? Can you tell me some common causes? How would patients present?
Causes: disc prolapse, disc herniation, osteophyte formation, abscess, malignancy, fracture
Malignancy: breast, thyroid, lung, prostate, myeloma
Presentation
- UMN at level
- LMN below level
- Sensory loss few levels beneath
- Also has numbness, paraesthesia and pain
- Can have autonomic dysfunction + neurogenic shock
Anterior interosseus syndrome
Motor nerve to FDP and FPL (no sensory loss)
- Compression occurs in elbow or forearm
- Abnormal opposition of thumb
- Weak flexion of thumb, 2nd and 3rd digits (DIPJ)
Carpal tunnel syndrome
Risk factors: pregnancy, hypothyroidism, acromegaly, repetitive strain injury/trauma, RA, CKD, F>M, 50s, anything taking up space
Presentation
- Aching pain in wrist and hand radiating along distribution of median nerve (lateral 2.5 digits palmar)
= Worse at night = wake and shake
- Paraesthesia, tingling and numbness
- Weakness of thenar muscles
= Ape hand = no precision grip
- Phalen’s, Tinel’s and median nerve compression +
Investigations
- Electroneurography
Management
- Splint
- Steroid injection
- Decompressive surgery
