Neurology Flashcards
What are the most common types of dementia?
- Alzheimer’s
- Vascular
- Dementia with Lewy-body
- Frontotemporal dementia (a.k.a Pick’s disease)
Describe the pathophysiology of Alzheimer’s
- Mostly affects temporal lobes
- Senile plaques (deposits of beta-amyloid outside of neurons)
- Neurofibrillary tangles (aggregation of hyperphosphorylated tau proteins inside neurons which cause necrosis of neural tissue)
What are key clinical features of Alzheimer’s?
- Early impairment of memory
- Short-term memory loss/difficultly learning new information
- 4 A’s = amnesia, aphasia, agnosia, apraxia
What are investigations for Alzheimer’s?
- Cognitive assessment
- Memory assessment
- Bloods (TFTs/B12)
- CSF Tau studies
- CT/MRI
What medications can be used for patients with dementia?
Mostly for Alzheimer’s:
- Acetylcholinesterase inhibitors e.g. donepezil, rivastigmine, galantamine
- N-methyl-D-aspartic acid receptor antagonists (NMDA) e.g. memantine (for memory loss)
- Antipsychotics
What is the difference between dementia and delirium?
Dementia = slowly progressive changes with limited fluctuation. Attention is usually intact and very early memories may be preserved
Delirium = acute, transient and usually reversible changes. Often an associated acute illness
What are the clinical features of delirium?
- Acute onset
- Fluctuating symptoms
- Disturbance in awareness and attention
- Disturbance in cognition
- Evidence of an organic cause
What are clinical features of hypoactive delirium?
- Lethargy
- Apathy
- Excessive sleeping
- Inattention
- Withdrawn
- Motor retardation
- Drowsy
- Unrousable
What are clinical features of hyperactive delirium?
- Agitation
- Aggression
- Restlessness
- Rapidly distracted
- Wandering
- Delusions
- Hallucinations
What are the investigations for delirium?
- Bloods (FBC/U&Es/TFTs/LFTs/B12 and folate/coagulation and INR/calcium/glucose/blood cultures)
- Urine dipstick
- MRI/CT
- CXR
What criteria is used for delirium?
DSM-5 criteria:
- Disturbance in awareness
- Acute onset
- Disturbance in cognition
- Not better explained by a pre-existing established or evolving neurocognitive disorder
- Absence of severely reduced GCS
- Evidence of organic cause
What is the management for delirium?
- Determine/treat underlying cause
- Rapid tranquilisation (benzodiazepines e.g. lorazepam/antipsychotics e.g. haloperidol, olanzapine)
- De-escalation methods (maintain adequate distance/move to safe, low-stimulant environment/use non-threatening verbal and non-verbal techniques/involve relatives or people close to patient)
Describe the pathophysiology of vascular dementia
- Subcortical VD (disease affected small vessels of brain)
- Stroke-related VD (following large cortical stroke)
- Single/multi-infarct VD (following single/multiple small strokes)
Describe the pathophysiology of Lewy-Body dementia
- If dementia symptoms 12 months before motor symptoms
- Histopathological findings of intracytoplasmic inclusions (Lewy bodies) that contain alpha-synuclein
- Lewy bodies lead to reduced levels of acetylcholine and dopamine in the brain
Describe the pathophysiology of frontotemporal dementia
- Tissue deposition of aggregated proteins (phosphorylated tau or transactive response DNA-binding protein 43)
- Atrophy around frontal/temporal lobes
What are general clinical features of dementia?
- Slow onset sx
- Lack of insight
- Cognitive impairment
- Behavioural and psychological sx
- Decreased ability to carry out ADLs
What are key clinical features of vascular dementia?
- Stepwise decline in function
- Gait/attention/personality changes
- Focal neurological symptoms e.g. aphasia/weakness
What are key clinical features of Lewy-Body dementia?
- Fluctuating cognitive impairment
- Parkinsonism sx (tremor/rigidity/bradykinesia/postural instability)
- Falls/syncope/hallucinations
- Sleep disturbances/restlessness at night
What are key clinical features of frontotemporal dementia?
- Personality changes and behavioural disturbances (disinhibition)
- Memory and perception relatively preserved
- Stereotypical, repetitive, compulsive behaviour/emotional blunting/abnormal eating/language problems
What is sundowning?
Increase in certain symptoms (e.g. distress/agitation/hallucinations/delusions) in dementia patients that often occur in the late afternoon/evening
What are investigations for dementia?
- Exclude alternative diagnoses
- Cognitive assessments
- Bloods
- ECG
- Virology
- Syphilis testing
- CXR
- CT/MRI head
What are some differential diagnoses for dementia?
- Depression
- Drugs with anticholinergic effects
- Delirium
What is the management for dementia?
- Assess capacity
- Inform DVLA
- Cognitive stimulation therapy
- Cognitive rehabilitation
- Reminiscence work
- Admiral nurses
- Reduce risk factors (e.g. for VD) - stop smoking/exercise/statins/etc.
- Medications
What are risk factors for Parkinson’s?
- Age
- Male
- Pesticide exposure
Describe the pathophysiology of Parkinson’s
- Basal ganglia responsible for coordinating habitual movements
- Substantia nigra = part of basal ganglia that produce dopamine (needed for functioning of basal ganglia)
- Parkinson’s = gradual fall in production of dopamine
What are the clinical features of Parkinson’s?
- Unilateral symptoms (bilateral suggests drug-induced)
- Resting ‘pill rolling’ tremor better on voluntary movement
- Cogwheel rigidity
- Bradykinesia (shuffling gait/small handwriting/hypomimia)
What investigation can differentiate Parkinson’s Disease and benign essential tremor?
DAT scan:
- Normal in tremor
What is the management for Parkinson’s?
- FIRST LINE = Levodopa + peripheral decarboxylase inhibitors (Carbidopa/benserazide)
- Catechol-o-methyltransferase (COMT) inhibitors e.g. entacapone
- Dopamine agonists e.g. bromocriptine/cabergoline/pergolide
- Monoamine oxidase-B inhibitors e.g. selegiline/rasagiline
What are the side effects of excess dopamine?
- Dyskinesias (dystonia/chorea/athetosis)
- Treat with amantadine (glutamate antagonist)
What is an essential tremor and what is it associated with?
A type of action tremor (fine tremor) associated with older age
What are the clinical features of essential tremor?
- Fine tremor in hands/head/jaw/vocals
- Symmetrical
- More prominent with voluntary movement
- Worse when tired/stressed/caffeine
- Improved by alcohol
- Absent during sleep
What is the management for essential tremor?
- No treatment required if not causing issues
- Symptomatic relief = propranolol, primidone
Describe the pathophysiology of Huntington’s disease
- Autosomal dominant
- Increased CAG repeats on Huntington (HTT) gene on chromosome 4
- Caudate nucleus atrophy
- Inhibitory neurones in corpus striatum degenerate
- Lack of GABA
- Anticipation (successive generations have more repeats)
What are the clinical features of Huntington’s disease?
- Typically presents around 30-50
- Chorea
- Eye movement disorders
- Dysarthria
- Dysphagia
What are the investigations for Huntington’s disease?
- Genetic test
- MRI/CT = loss of striatal volume
What is the management for Huntington’s disease?
Symptomatic relief:
- Antipsychotics e.g. olanzapine
- Benzodiazepines e.g. diazepam
- Dopamine-depleting agents e.g. tetrabenzine
What are the complications of Huntington’s disease?
- Life expectancy = approx. 15-20 years after onset of sx
- Death due to respiratory disease
- Suicide
What are the most common types of motor neurone disease and what are the risk factors?
- Amyotrophic lateral sclerosis (ALS)
- Progressive bulbar palsy
- Genetics
- Smoking
- Exposure to heavy metals
- Pesticides
Describe the pathophysiology of motor neurone disease
- Loss of neurones in motor cortex, cranial nerve nuclei and anterior horn cells
- ALS = motor cortex, anterior horn (UMN + LMN)
- PBP = CN IX-XII (UMN + LMN)
- Progressive muscular atrophy = Anterior horn (LMN)
- Primary lateral sclerosis = UMN
What are the clinical features of motor neurone disease?
- No sensory sx
- Progressive weakness of muscles in limbs/trunk/face/speech –> clumsiness
- Often first noted in upper limbs
- Increased fatigue when exercising
- Dysphagia/chewing difficulties
What are the specific clinical features of upper and lower motor neurone disease?
Upper MND = hypertonia/spasticity, clonus, brisk reflexes
Lower MND = muscle wasting, hypotonia, fasciculations, reduced reflexes
What is the management for motor neurone disease?
- ALS = riluzole (slows progression)
- Non-invasive ventilations
- End of life care
What are risk factors for multiple sclerosis?
- Female
- Genetics
- Epstein-Barr virus
- Low vitamin D
- Smoking
- Obesity
Describe the pathophysiology of multiple sclerosis
- Affects CNS (oligodendrocytes)
- Inflammation and infiltration of immune cells damages myelin
- Causes demyelination
What are the clinical features of multiple sclerosis?
- Optic neuritis due to demyelination of optic nerve (reduced vision, central scotoma, pain on movement, impaired colour vision, pupil defect)
- Eye movement abnormalities due to lesions in abducens nerve (diplopia, internuclear ophthalmoplegia, conjugate lateral gaze disorder)
- Weakness (Bell’s palsy, Horner’s, limb paralysis, incontinence)
- Sensory sx (trigeminal neuralgia, numbness, MS hug, paraesthesia, Lhermitte’s sign (electric shock))
- Ataxia
What is optic neuritis?
Inflammatory optic neuropathy affecting one or both optic nerves
- Typical (a.k.a demyelinating) is the most common type
What are the clinical features of optic neuritis?
- Reduced acuity
- RAPD (relative afferent pupillary defect)
- Dyschromatopsia (colour blindness where 2/3 fundamental colours seen)
- Typical patient = Young, Caucasian woman presenting with acute unilateral vision loss associated with painful eye movements and colour desaturation
What are the investigations for multiple sclerosis?
- McDonald’s criteria
- MRI = lesions/areas of T2 hyperintensity
- LP = oligoclonal bands in CSF
- Fundoscopy = often normal but may show optic disc swelling (optic neuritis)
What is McDonald’s criteria?
For MS diagnosis
- 2+ attacks, 2+ lesions
- 1+ attacks,1 lesion, dissemination in space on MR
- 1 attack, 2 lesions, dissemination in time on MR
- 1 attack, 1 lesion, dissemination in space and time
- 1 year of disease progression, at least 2 out of 3 criteria (dissemination in space in the brain/dissemination in space in the spinal cord based on 2 or more T2 lesions/positive CSF)
What does disseminated in space mean for McDonald’s criteria (MS)?
In different regions = juxtacortical, subcortical, infratentorial and spinal cord
What is the management for multiple sclerosis?
- Steroids
- Interleukins/inflammatory cytokines/immune cells
- Symptomatic relief (amitriptyline/antidepressants/anticholinergics e.g. tolterodine, oxybutynin/anti-spasticity e.g. baclofen/gabapentin)
What is given to patients experiencing a relapse of MS?
500mg methylprednisolone orally for 5 days or IV for 3 days
What does myasthenia gravis have an association with?
Thymomas (tumours of thymus gland)
Describe the pathophysiology of myasthenia gravis
- Acetylcholinesterase receptor (Ach-R) antibodies
- Muscle-specific kinase (MuSK) antibodies
- Low-density lipoprotein receptor-related protein 4 (LRP4) antibodies
- Antibodies affect acetylcholine receptors and prevent uptake of the neurotransmitter acetylcholine
What are the clinical features of myasthenia gravis?
- Fatiguability
- Weakness that worsens with activity and improves with rest
- Proximal muscles more commonly affected
- Diplopia/ptosis/facial weakness
- Swallowing/chewing difficulty
- Slurred speech
What are the investigations for myasthenia gravis?
- Check fatiguability (repeated blinking/upward gazing/abduction of arm)
- Antibodies
- Edrophonium test
What is the edrophonium test?
- Investigation for myasthenia gravis
- IV dose of edrophonium chloride given
- Blocks enzymes that break down acetylcholine - Symptoms briefly relieved
What is the management for myasthenia gravis?
- Acetylcholinesterase inhibitors e.g. pyridostigmine, neostigmine (long-acting)
- Prednisolone/azathioprine
- Monoclonal antibodies e.g. rituximab, eculizumab
- Thymectomy
What is a complication of myasthenia gravis?
Myasthenia crisis:
- Acute worsening of symptoms often triggered by another illness
- Can lead to respiratory failure
- Management = IV immunoglobulins and plasmapheresis +/- ventilation
When does Guillain-Barre syndrome typically occur?
Following an infection (e.g. gastroenteritis) - associated with C. Jejuni, CMV, EBV
What are the clinical features of Guillain-Barre syndrome?
- History of recent infection
- Symmetrical ascending weakness
- Reduced reflexes
- Peripheral loss of sensation
What are the investigations for Guillain-Barre syndrome?
- Nerve conduction studies = reduced
- LP for CSF = raised protein, normal cell count/glucose
What is the management for Guillain-Barre syndrome?
- IV immunoglobulins
- Supportive care
- Plasmapheresis (alternative to IV Ig)
- VTE prophylaxis
What are the complications of Guillain-Barre syndrome?
- PE –> respiratory failure
- Locked-in syndrome
What are the most common causes of viral meningitis?
Viral meningitis more common and less severe
- Enteroviruses e.g. echovirus, coxsackie
- HSV
- Mumps virus
What are the most common causes of bacterial meningitis?
Bacterial meningitis less common and more severe
- Neonates = group B strep (strep. agalactiae), E. coli, listeria meningitis
- N. meningitis (a.k.a meningococcus), strep. pneumoniae
What are other causes of meningitis?
Fungal = cryptococcus, candida
What are the risk factors for meningitis?
- Student
- Recent travel
- Immunocompromised
- Pregnancy
- Extremes of ages
- Unvaccinated
What are the clinical features of meningitis?
- Fever
- Neck stiffness/pain
- N+V
- Headache
- Photophobia
- Altered consciousness
- Seizures
What is the main feature of meningococcal septicaemia?
- Non blanching rash –> infection has caused DIC and subcutaneous haemorrhage
What are the investigations for meningitis?
- LP for CSF
- Bloods/blood culture
- Kernig’s test
- Brudzinski’s test
How do you differentiate between viral and bacterial meningitis?
LP for CSF
- Appearance = cloudy (bacterial) or clear (viral)
- Protein = very high (bacterial), high/normal (viral)
- Glucose = low (bacterial), normal (viral)
- WCC = high neutrophils (bacterial), high lymphocytes (viral)
- Culture = bacteria (bacterial), negative (viral)
What is Kernig’s test?
Investigation for meningitis
- Patient lies on back with hip flexed and knee bent to 90 degrees
- Slowly try to extend knee whilst keeping hip flexed
- Meningitis = spinal pain/resistance to movement
What is Brudzinski’s test?
Investigation for meningitis
- Patient lies on back
- Lift head and neck off bed and flex chin to chest
- Positive = involuntarily flexion of hip and knees
What is the management for viral meningitis?
Aciclovir
What is the management for bacterial meningitis?
- Meningococcal septicaemia = IM/IV benzylpenicillin
- <3 months = cefotaxime + amoxicillin/ampicillin
- > 3 months = ceftriaxone
- Post exposure prophylaxis = ciprofloxacin/rifampicin
What is the most common complication of bacterial meningitis?
Sensorineural hearing loss - due to damage to the cochlear nerve or inner ear structures during inflammation
What are the most common causes of encephalitis?
- HSV (neonates = HSV-2, children = HSV-1)
- VZV
- CMV
- EBV
What are the clinical features of encephalitis?
- Fever
- Headache
- Encephalopathy
- Altered consciousness
- Altered cognition/behaviour
- Focal seizures
What are the investigations for encephalitis?
- LP for CSF
- CT/MRI
What is the management for encephalitis?
- HSV/VZV = aciclovir
- CMV = ganciclovir
What are the main components of a seizure?
- Prodrome = precedes seizures by hours to days - weird feeling
- Aura = right before - deja vu/strange smells/flashing lights
- Post-ictal = after seizure - headache/confusion/sore tongue/post-ictal Todd’s palsy (temporary weakness in motor cortex)
What are the features of a non epileptic seizure?
- Occurs in abnormal metabolic circumstances e.g. low Na+, hypoxia
- Last longer than epileptic seizures
- Do not occur in sleep
- No incontinence/tongue biting
- No muscle pain
What blood test can differentiate true seizures from pseudoseizures?
Prolactin - raised in true seizures
What is required for a diagnosis of epilepsy?
- At least 2 unprovoked seizures occurring more than 24 hours apart
- 1 unprovoked seizure and a high probability of further seizures
What are the investigations for seizures?
- EEG
- MRI
- ECG
- Bloods/electolytes/glucose/cultures/LP
What is the management for seizures/epilepsy?
Acute:
- Recovery position
- Place something soft under head
- Remove things that could cause injury
- Note start/end time of seizures
- Call ambulance if seizure lasts >5 mins
Medication:
- Sodium valproate/lamotrigine/carbamazepine/levetiracetam/ethosuximide
When is a diagnosis of epilepsy remission given?
Individuals who:
- Had an age-dependent epilepsy syndrome but are now past the applicable age
- Have remained seizure-free for at least 10 years off anti-seizure medications, provided that there are no known risk factors associated with high probability of future seizures
What is the main complication of seizures/epilepsy?
Status epilepticus - when seizure lasts >5 minutes or 2+ seizures without regaining consciousness
Management:
- ABCDE
- Community = buccal midazolam/rectal diazepam
- FIRST LINE = benzodiazepine - IV lorazepam
- After 2 attempts of benzodiazepine = IV phenytoin/sodium valproate/levetiracetam
- Third line = phenobarbital/general anaesthesia
What is sodium valproate used for and what are the side effects?
Epilepsy and bipolar disorder/mania
- Teratogenic
- Liver damage
- Hair loss
- Tremor
What is carbamazepine used for and what are the side effects?
Epilepsy and bipolar disorder/mania
- Agranulocytosis
- Aplastic anaemia
What is ethosuximide used for and what are the side effects?
Epilepsy
- Night tremors
- Rash
- N+V
What is lamotrigine used for and what are the side effects?
Epilepsy and bipolar disorder/mania
- DRESS syndrome = drug reaction with eosinophilia and systemic symptoms
- Leukopenia
- Stevens-Johnson syndrome (rare, severe skin reaction)
Describe tonic clonic seizures
A.k.a grand mal seizures
- Loss of consciousness
- Tonic (muscle tensing) and clonic (muscle jerking)
- Tongue biting/incontinence/groaning/irregular breathing
- First line management = sodium valproate
- Second line management = lamotrigine/carbamazepine
Describe myoclonic seizures
- Sudden brief muscle contractions
- Patient usually conscious
- Often happens in children as part of juvenile myoclonic epilepsy
- First line management = sodium valproate
- Second line management = Lamotrigine/levetiracetam/topiramate
Describe tonic seizures
- Sudden onset of increased muscle tone (entire body stiffens) - fall if patient standing
- Usually only lasts a few seconds
- First line = sodium valproate
- Second line = lamotrigine
Describe atonic seizures
A.k.a drop attacks
- Brief lapses in muscle tone
- Sudden loss of muscle strength
- Part/all of body becomes limp
- Usually last <3 minutes
- First line management = sodium valproate
- Second line management = lamotrigine
Describe absence seizures
- Common in children
- Blank expression/staring into space
- Abrupt return to normal
- Unaware of surroundings and won’t respond during episode
- Typically last 10-20 seconds
- Management = sodium valproate/ethosuximide
Describe temporal lobe seizures
- Affect hearing/speech/memory/emotions
- Hallucinations/memory flashbacks/deja vu/anxiety/automatisms
- First line management = lamotrigine/carbamazepine
- Second line management = sodium valproate/levetiracetam
Describe frontal lobe seizures
- Motor disturbances
- Jacksonian march
- Remains conscious
- Post-ictal Todd’s palsy
What are febrile convulsions and what are the risk factors?
- Seizures that occur in children whilst they’re febrile (only between ages of 6 months and 5 years)
- Fhx
- Socio-economic class
- Winter season
- Zinc/iron deficiency
What are the clinical features of febrile convulsions?
Simple:
- Generalised tonic-clonic seizures
- Last <15 minutes
- Only occur once during febrile illness
Complex:
- Focal seizures
- Last >15 minutes
- Occur multiple times during febrile illness
What is associated with subarachnoid haemorrhages?
Autosomal dominant polycystic kidney disease - berry aneurysms
What are the clinical features of a subarachnoid haemorrhage?
- History of trauma
- Sudden onset
- ‘Thunderclap’ headache
- Loss of consciousness
- N+V
- Confusion
What are the investigations for subarachnoid haemorrhages?
- CT = hyper-attenuation around circle of Willis (‘5 pointed star’ appearance) - suggests aneurysm –> follow with CT angiogram - if more peripheral, suggests due to trauma
- LP = xanthochromia (yellow due to bilirubin breakdown)
What is the management for subarachnoid haemorrhages?
- Immediately stop antiplatelets/anticoagulants
- Endovascular coiling +/- neurosurgical clipping
- IV mannitol for high ICP
What are subdural haemorrhages and what are the risk factors?
Bleeding into subdural space due to ruptured bridging vein
- Anticoagulants
- Accident prone = dementia/elderly/alcoholics/epileptics
- Babies (shaken baby syndrome)
What are the clinical features of a subdural haemorrhage?
- History of trauma
- N+V
- Confusion
- Fluctuating consciousness/drowsiness
- Headache
How can you tell if a bleed in the brain is acute or chronic?
CT:
- Acute - hyperdense (brighter)
- Chronic - hypodense (darker)
What are the investigations for subdural haemorrhages?
CT:
- Haematoma
- Crescent shaped
- Unilateral
- Midline structures shift away
- Cross sutures
What is the management for subdural haemorrhages?
- Immediately stop antiplatelets/anticoagulants
- Clot evacuation to remove haematoma
- Craniotomy
- Burr hole washout
- IV mannitol for high ICP
What are epidural haemorrhages and who are they more common in?
Bleeding into extradural space due to ruptured middle meningeal artery
- Males
- Young people
What are the clinical features of an epidural haemorrhage?
- History of trauma
- Skull fracture
- Lucid interval = initial drowsiness/unconsciousness –> recovery –> rapid deterioration
What are the investigations for epidural haemorrhages?
CT:
- Bi-convex
- Unilateral
- Do not cross sutures
- Midline shift
What is the management for epidural haemorrhages?
- Immediately stop antiplatelets/anticoagulants
- Clot evacuation
- Ligation of bleeding vessel
- IV mannitol for high ICP
What are the clinical features of an intracerebral haemorrhage?
Sudden onset focal neurological symptoms
- Limb/facial weakness
- Dysphasia
- Vision/sensory loss
What are the investigations for intracerebral haemorrhages?
- CT = hyper- attenuation in brain parenchyma, commonly in middle if patient has HTN
- Often associated with subarachnoid haemorrhage as pia is very thin
Which type of stroke is more common?
Ischaemic
What are the risk factors for strokes?
Same as CVD
- Age
- Male
- HTN/hyperlipidaemia
- Diabetes
- Smoking
- Previous TIA
- Heart disease/AF
- COC (ischaemic)
What are the clinical features of a stroke?
- Sudden limb/facial weakness
- Dysphasia
- Visual/sensory loss
- N+V
What are the investigations for haemorrhagic strokes?
- FIRST LINE = CT
- Diffusion-weighted MRI
What is the management for haemorrhagic strokes?
Acute:
- Neurosurgery - evacuate blood
- IV mannitol for high ICP
- Stop anticoagulants
Secondary:
- Anticoagulant
- BP aim of 140/90
- External ventricular drain (if hydrocephalus)
- Rehabilitation (SALT/PT/OT)
What type of infarcts occur in ischaemic strokes?
Cerebral:
- More common
- Occlusion of large blood vessel to cerebrum (e.g. internal carotid artery/middle cerebral artery)
Lacunar:
- Infarcts of smaller blood vessels
- Affected smaller areas e.g. internal capsule/basal ganglia/thalamus/pons
- Produce more specific symptoms
What are the investigations for ischaemic strokes?
- FIRST LINE = Bloods and CT
- Diffusion-weighted MRI
- Carotid USS
What is the Bamford classification?
Categorises ischaemic strokes based on initial presenting features
- Total anterior circulation stroke
- Partial anterior circulation stroke
- Lacunar syndrome
- Posterior circulation syndrome
- Lateral medullary syndrome (Wallenberg’s syndrome)
- Weber’s syndrome
- Basilar artery
What is the Bamford classification criteria for total anterior circulation stroke?
ALL THREE:
- Unilateral weakness (and/or sensory deficit of face/arm/leg)
- Homonymous hemianopia
- Higher cerebral dysfunction (e.g. dysphasia/visuospatial disorder)
What is the Bamford classification criteria for partial anterior circulation stroke?
TWO:
- Unilateral weakness (and/or sensory deficit of face/arm/leg)
- Homonymous hemianopia
- Higher cerebral dysfunction (e.g. dysphasia/visuospatial disorder)
What is the Bamford classification criteria for lacunar syndrome?
ONE OF:
- Pure sensory stroke
- Pure motor stroke
- Sensorimotor stroke
- Ataxic hemiparesis
What is the Bamford classification criteria for posterior circulation syndrome?
ONE OF:
- CN palsy and a contralateral motor/sensory deficit
- Bilateral motor/sensory deficit
- Conjugate eye movement disorder (e.g. gaze palsy)
- Cerebral dysfunction (e.g. ataxia/nystagmus/vertigo)
- Isolated homonymous hemianopia/cortical blindness
What is the Bamford classification criteria for lateral medullary syndrome?
- Ipsilateral ataxia/nystagmus/dysphagia/facial numbness/CN palsy e.g. Horner’s syndrome
- Contralateral limb sensory loss
What is the Bamford classification criteria for Weber’s syndrome?
- Ipsilateral CN III palsy
- Contralateral weakness
What is the Bamford classification criteria for a basilar artery stroke?
‘Locked in’ syndrome
What is the management for ischaemic strokes?
Acute:
- Exclude haemorrhagic stroke
- Oral/rectal aspirin 300mg
- Thrombolysis = IV alteplase (within 4.5 hours of sx onset)
- Mechanical thrombectomy (within 6 hours)
- Aspirin 300mg daily for 2 weeks then clopidogrel
- Warfarin/apixaban
- Rehabilitation (SALT/OT/PT)
What is Wallenberg syndrome/lateral medullary syndrome?
Stroke due to blockage of posterior inferior cerebellar artery
- Causes ischaemia in lateral part of medulla oblongata in brainstem
- Involvement of lateral spinothalamic tract
- Ipsilateral facial pain and loss of temperature sensation
- Contralateral limb/torso pain and loss of temperature sensation
- Ataxia
- Nystagmus
What is lateral pontine syndrome?
Stroke due to blockage of anterior inferior cerebellar artery
- Artery supplies the pons
- Similar presentation to lateral medullary syndrome
- Ipsilateral facial paralysis
- Ipsilateral deafness
What are the clinical features of a stroke affecting the anterior cerebral artery?
- Contralateral hemiparesis and sensory deficits
- Lower extremities worse affected
What are the clinical features of a stroke affecting the middle cerebral artery?
- Contralateral hemiparesis
- Upper extremities worse affected
- Contralateral homonymous hemianopia
- Aphasia
What are the clinical features of a stroke affecting the posterior cerebral artery?
- Contralateral homonymous hemianopia with macular sparing
- Visual agnosia
Weber’s syndrome = branches of the posterior cerebral artery that supply the midbrain
- Ipsilateral CN III palsy (eye points down and out)
- Contralateral weakness of upper/lower extremities
What are the clinical features of a stroke affecting the retinal/ophthalmic artery?
Amaurosis fugax
What are the clinical features of a stroke affecting the basilar artery?
Locked-in syndrome
Describe lacunar strokes
- Strong association with HTN
- Common sites = basal ganglia/thalamus/internal capsule
- Isolated hemiparesis, hemisensory loss or hemiparesis with limb ataxia
What are causes of transient ischaemic attacks?
- Thromboemboli
- Hypoviscosity
- Hypoperfusion
- Vasculitis
What is a crescendo TIA?
- 2 or more TIAs in 1 week
- High risk factor for stroke
What are the clinical features of a transient ischaemic attack?
- Last <24 hours without infarction (typically resolve within 10 minutes)
- Sudden facial/limb weakness
- Dysphasia
- Sensory/visual loss
- N+V
What are the investigations for transient ischaemic attacks?
- Blood glucose (hypoglycaemia can cause focal neurological symptoms)
- CT
- Diffusion-weighted MRI
- Carotid doppler
- ACBD2 risk score (age/BP/clinical features/duration/diabetes)
What is the management for transient ischaemic attacks?
- Aspirin (acute)
- Clopidogrel and atorvastatin (long term prophylaxis)
What are triggers for migraines?
CHOCOLATE:
- Chocolate
- Hangovers
- Orgasms
- Cheese
- Oral contraceptives
- Lie ins
- Alcohol
- Tumult (loud noise)
- Exercise
What are the clinical features of migraines?
- Aura (visual disturbances/somatosensory sx)
- Unilateral pain
- Throbbing-type pain
- Moderate to severe intensity
- Motion sensitivity
- Nausea/vomiting
- Photophobia/phonophobia
What are the investigations for migraines?
- Diagnosis using sx
- CT/MRI
- LP
What is the management for migraines?
Acute:
- Triptans (sumatriptan)
- NSAIDs (naproxen)
- Paracetamol
- Anti-emetics (prochlorperazine)
Secondary:
- Beta blockers (propranolol)
- Anti-convulsants (topiramate)
- TCAs (amitriptyline)
What are the risk factors for cluster headaches?
- Male
- Genetics
- Smoker
- Alcohol
What are the clinical features of cluster headaches?
- Rapid onset of excruciating pain around eye/temples/forehead
- Unilateral and localised pain
- Pain rises to crescendo over a few minutes and lasts for 15-160 minutes
- Watery/bloodshot eyes
- Facial flushing
- Rhinorrhoea
- Miosis
- Ptosis
What is the management for cluster headaches?
Acute:
- Analgesics ineffective
- 15L 100% O2 for 15 mins via non-rebreather mask
- Triptans (sumatriptan)
Secondary:
- Calcium channel blocker (verapamil)
- Prednisolone
- Reduce alcohol intake/stop smoking
What are triggers of tension headaches?
- Stress
- Sleep deprivation
- Bad posture
- Hunger
- Eyestrain
- Anxiety/depression
- Noise
- Dehydration
- Alcohol
What are the clinical features of tension headaches?
- Come on and resolve gradually
- No visual changes/aura
- No vomiting
- No sensitivity to head movement
- Pressure behind eyes
What is the management for tension headaches?
- Avoidance of triggers
- Basic analgesia (aspirin/paracetamol/ibuprofen)
- Relaxation techniques
- Hot towels to local area
What are triggers of trigeminal neuralgia?
- Cold weather
- Spicy food
- Caffeine
- Citrus fruits
What causes trigeminal neuralgia?
Compression of trigeminal nerve and its branches (ophthalmic/maxillary/madibular)
What are the clinical features of trigeminal neuralgia?
- Intense facial pain that comes on spontaneously
- Lasts between a few seconds to hours
- Electricity-like shooting pain
- Majority are unilateral
What is the management for trigeminal neuralgia?
- Carbamazepine
- Surgery to decompress trigeminal nerve
What are the clinical features of idiopathic intracranial hypertension?
Typical patient:
- Obese, young female with headaches and blurred vision
- N+V
- Often eases throughout the day
- Worsens when patient bends down
What does temporal (giant cell) arteritis affect?
The aorta and/or its major branches (carotid and vertebral arteries)
What are the clinical features of temporal arteritis?
- Unilateral headache over temporal area
- Scalp tenderness
- Jaw claudication
- Visual disturbances (blurred vision, diplopia, amaurosis fugax)
What are the investigations for temporal arteritis?
- USS = halo sign of temporal and axillary arteries
- Temporal artery biopsy (gold standard) = shows giant cells and granulomatous inflammation
What is the management for temporal arteritis?
High dose glucocorticoid stat e.g. prednisolone 40mg-60mg
Describe the pathophysiology and clinical features of Horner’s syndrome
Dysfunction of oculosympathetic nerve
- Unilateral ptosis
- Unilateral miosis
- Unilateral anhidrosis
- Enophthalmos
What are the investigations for Horner’s syndrome?
- Examination = visual fields/facial sensation/extra-ocular movements
- Eye drops to make pupils dilated = sympathetically denervated pupil will dilated poorly
- CT/MRI
What is a squint and what are some common causes of it?
Misalignment of the eye a.k.a strabismus
- Hydrocephalus
- Cerebral palsy
- Space-occupying lesions e.g. retinoblastoma
- Trauma
What are the different types of squint?
- Concomitant (difference in control of extraocular muscles)
- Paralytic (paralysis in one/more of extraocular muscles)
- Esotropia/exotropia (inward/outward positioned)
- Hyper/hypotropia (upward/downward)
What is amblyopia?
In squint, lazy eye becomes progressively more passive and has reduced function
What is the main feature of a squint?
Diplopia
What are the investigations for squints?
- Fundoscopy/eye movements/visual acuity
- Hirschberg’s test (pen torch shone and observe reflection in cornea)
- Cover test (cover one eye and observe when moved to other eye)
What is the management for squints?
- Occlusive patch over good eye
- Atropine drops in dominant eye (causes blurred vision)
What is Bell’s palsy?
Acute, unilateral facial nerve weakness/paralysis
What is the facial nerve (CN VII) responsible for?
- Motor = facial expression, stapedius in inner ear, posterior digastric/stylohyoid/platysma muscles
- Sensory = taste from anterior 2/3 of tongue
- Parasympathetic = submandibular/sublingual salivary gland, lacrimal gland
What is the management for Bell’s palsy?
-Often self limiting over several weeks/months
- Prednisolone 50mg for 10 days or 60mg for 5 days followed by 5 day reducing regime
- Lubricating eye drops
What is Ramsay-Hunt syndrome?
- Shingles infection that has affected the facial nerve
- Painful vesicular rash in ear canal/pinna/around ear (may extend to anterior 2/3 of tongue and hard palate)
- Unilateral lower motor neurone facial nerve palsy
What are some risk factors for cerebral palsy?
- Antenatal = maternal infection/trauma during pregnancy
- Perinatal = birth asphyxia (HIE)/pre-term birth
- Postnatal = meningitis/severe neonatal jaundice/head injury
Describe the pathophysiology of the different types of cerebral palsy
- Spastic = UMN damage
- Dyskinetic = basal ganglia damage
- Ataxic = cerebellar damage
- Mixed = all
What are the clinical features of spastic cerebral palsy?
- Hypertonia
- Reduced function
What are the clinical features of dyskinetic cerebral palsy?
- Problems controlling muscle tone
- Hypertonia/hyptonia
- Athetoid movements (abnormal, involuntary movements)
- Oro-motor problems
What are the clinical features of ataxic cerebral palsy?
Problems with coordinated movement
What are the clinical features of cerebral palsy in development?
- Failure to meet milestones
- Hypertonia/hypotonia
- Hand preference below 18 months
- Problems with coordination/speech/walking
- Problems with feeding/swallowing
- Learning difficulties
What are the investigations for cerebral palsy?
Neurological examination:
- Hemiplegic/diplegic gait
- Extended legs with plantarflexion of feet/toes
- Coordination problems
- Hypertonia/brisk reflexes/reduce power (UMN signs)
- Athetoid movements
What is the management for cerebral palsy?
- SALT/PT/OT/dieticians
- Surgery (release contractures/lengthen tendons)
- Muscle relaxants e.g. baclofen
- Anti-epileptics
- Glycopyrronium bromide (for excessive drooling)
What are causes of hypoxic ischaemic encephalopathy?
- Maternal shock
- Intrapartum haemorrhage
- Prolapsed cord
- Nuchal cord (wrapped around baby’s neck)
How are the clinical features of hypoxic ischaemic encephalopathy graded?
Sarnat Staging Grades:
- Mild = poor feeding/general irritability/hyper-alert/resolves within 24 hours
- Moderate = poor feeding/lethargic/hypotonic/seizures/can take weeks to resolve
- Severe = reduced consciousness/apnoeas/flaccid, reduced or absent reflexes
What is the management for hypoxic ischaemic encephalopathy?
Supportive care:
- Neonatal resuscitation
- Ventilation
- Circulatory support
- Nutrition
- Acid base balance
- Treatment of seizures
- Therapeutic hypothermia
What are the most common types of muscular dystrophy and when do they present?
- Duchenne’s = 3-5 years
- Becker’s = 8-12 years
- Myotonic dystrophy
- Facioscapulohumeral muscular dystrophy
- Oculopharyngeal muscular dystrophy
- Limb-girdle muscular dystrophy
- Emery-Dreifuss muscular dystrophy
Describe the pathophysiology of Duchenne’s/Becker’s muscular dystrophy
- X linked recessive
- Defective gene for dystrophin (protein that helps to hold muscles together)
- Becker’s = gene less severely affected than Duchenne’s
What are the clinical features of muscular dystrophy?
- Gower’s sign (stand up from lying down using a specific technique)
- Progressive weakness
- Waddling gait
- Language delay
- Pseudohypertrophy of calves
- Slow/clumsy
What is Gower’s sign?
Specific technique used by patients with proximal muscle weakness (muscular dystrophy) to stand up from lying position:
- Get on hands and knees and push hips up and backwards
- Weight shifted backwards and hands moved to knees
- Legs kept mostly straight and hands walk up legs to get upper body erect
What is the management for Duchenne’s/Becker’s muscular dystrophy?
- Exercise
- Night splints/passive stretching
- Oral steroids
- Creatinine supplementation
What is the life expectancy of Duchenne’s muscular dystrophy?
- 25-35 years
- Usually due to cardiac/respiratory complications
What are the clinical features of a brain abscess?
- Pain
- Headache
- Fever
- Fluctuating consciousness
- Visual disturbance
- Unilateral weakness
- N+V
- Seizures
- Personality changes
- Confusion
- Difficulty moving/speaking
- Neck/back stiffness
What are the investigations for a brain abscess/herniation?
- Bloods
- MRI/CT
What is the management for a brain abscess?
- Abx (cephalosporin + metronidazole)
- Steroids
- Surgery
What are the clinical features of a brain herniation?
- Seizures
- Decreased level of consciousness/coma
- Mydriasis
- Irregular/slow pulse
- Respiratory/cardiac arrest
- Loss of brainstem reflexes (blinking/gagging/pupillary reflex)
- Fever
What is the management for a brain herniation?
- Osmotic diuretics
- Paracetamol (fever)
- Sedation/paralytic agents
- Prophylactic anticonvulsants
- Surgery - decompressive craniectomy
- Hypertonic saline bolus (to decrease ICP)
What is Herpes Zoster?
A.k.a shingles
An infection caused by the reactivation of VZV within dorsal root or cranial nerve ganglia
What is herpes zoster ophthalmicus?
When it affects the ophthalmic branch (V1) of the trigeminal nerve (CN V)
What are the clinical features of shingles?
- Fever
- Headache/photophobia
- Malaise
- Paraesthesia/pain along affected dermatome
- Painful/itchy unilateral maculopapular rash –> vesicular rash –> dries and scabs over
- Hutchinson’s sign
What is Hutchinson’s sign?
Sign of shingles
- Cutaneous lesions on tip/side/root of nose
- Indicates involvement of V1 nasal branch
- Strong predictor of ocular involvement
What are the investigations for shingles?
- Ophthalmological examination (acuity/vision/anterior segment/slit lamp/fundoscopy/extra-ocular muscles)
- Neurological examination (eyelid erythema, oedema/retinitis, optic neuritis/CN palsy/reduced corneal sensation)
- Swabs for viral PCR
What is the management for shingles?
- Antiviral therapy = oral aciclovir 800mg 5x a day for 7-10 days (should be given within 72 hours of rash onset)
- Oral steroids
- Supportive management (cold compress/analgesia/topical lubricants)
What is the main complication of shingles?
Postherpetic neuralgia (PHN):
- Pain that persists in areas where the rash once was located and continues for >90 days after rash onset
What is Ramsay-Hunt syndrome and what are the clinical features?
- Reactivation of varicella zoster virus in the facial nerve (Herpes Zoster Oticus)
- Otalgia
- Hearing loss
- Facial paralysis
What is the cause of malaria and how is it spread?
Plasmodium family of protozoan parasites spread via bites from female anopheles mosquitos
What are the types of malaria?
- Plasmodium falciparum = most common and most severe
- Plasmodium vivax and ovale (can lie dormant for up to 4 years)
- Plasmodium malariae
- Plasmodium knowlesi
What are the clinical features of malaria?
- Fever/sweats/rigors
- Fatigue/myalgia
- Headache
- N+V
- Pallor
- Hepatosplenomegaly
- Jaundice
What are the investigations for malaria?
Malaria blood film
- In order to exclude malaria = require 3 negative samples taken over 3 consecutive days
What is the management for uncomplicated malaria?
- Notify public health
- FIRST LINE = artemether + lumefantrine (Riamet)
- Quinine + doxycycline
- Quinine + clindamycin
- Proguanil + atovaquone (Malarone)
- Chloroquine
- Primaquine
What is chloroquine used for and what are the side effects?
Uncomplicated malaria and malaria prophylaxis
- Increasing rates of resistance
What is primaquine used for and what are the side effects?
Uncomplicated malaria
- Can cause severe haemolysis in patients with G6PD deficiency
What is the management for complicated malaria?
- Notify public health
- HDU/ICU admission
- FIRST LINE = IV artesunate
- IV quinine dihydrochloride
What is prophylactic management for malaria?
- Mosquito spray/nets/barriers
- Antimalarial medication:
- Proguanil + atovaquone (malarone)
- Doxycycline
- Mefloquine
- Chloroquine + proguanil
What is mefloquine used for and what are the side effects?
Prophylactic management of malaria
- Risk of psychiatric side effects e.g. anxiety/depression/abnormal dreams/psychosis/seizures
What is artesunate used for and what are the side effects?
Complicated malaria
- Haemolysis
What is an acoustic neuroma?
A.k.a vestibular schwannoma
- Benign tumour of Schwann cells surrounding the vestibulocochlear nerve (CN VIII)
What are the clinical features of an acoustic neuroma?
- Gradual onset
- Unilateral sensorineural hearing loss
- Unilateral tinnitus
- Dizziness/imbalance
- Sensation of fullness in ear
- Loss of corneal reflex
- Facial nerve palsy (if large)
What are the investigations for acoustic neuromas?
- Audiometry = sensorineural hearing loss
- MRI/CT
What is the management for acoustic neuromas?
- Conservative management
- Surgery
- Radiotherapy
Describe the epidemiology of brain tumours
- Leading cause of childhood cancer deaths in UK
- Almost always primary in children
Which cancers are most likely to metastasise to the brain?
- Lung
- Breast
- Renal cell carcinoma
- Melanoma
- Colon
What is a meningioma?
- Brain tumour growing from cells of meninges
- Usually benign
What is a glioma?
- Tumour of glial cells in brain/spinal cord
- Astrocytoma (astrocytes) - glioblastoma is most malignant
- Oligodendroglioma (oligodendrocytes)
- Ependymoma (ependymal cells)
What are the clinical features of brain tumours?
- Can be asx
- Progressive focal neurological symptoms (depending on location) = limb weakness/balance and coordination issues/memory loss/speech disturbance/behaviour and personality changes/visual disturbances
- Raised ICP (headache worse on waking, coughing, straining, bending forward/vomiting/papilloedema)
What are clinical features of a pituitary tumour?
- Bitemporal hemianopia
- Acromegaly (excess GH)
- Hyperprolactinaemia
- Cushing’s disease (excess ACTH/cortisol)
- Thyrotoxicosis (excess TSH/T3/T4)
What are the investigations for brain tumours?
- CT/MRI
- Biopsy
- Fundoscopy
- Glioma grading (1-4 where 4 is most malignant e.g. glioblastoma multiforme)
What is the investigation for papilloedema (due to raised ICP)?
Fundoscopy:
- Blurring of optic disc margin
- Elevated optic disc
- Loss of venous pulsation
- Engorged retinal veins
- Haemorrhages around optic disc
- Paton’s line
What is the management for brain tumours?
- Surgery
- Chemotherapy/radiotherapy
- Palliative care
What is the management for pituitary tumours?
- Trans-sphenoidal surgery
- Radiotherapy
- Bromocriptine (blocks excess prolactin)
- Somatostatin analogue e.g. octreotide (blocks excess GH)
What are the clinical features of cerebellar disease?
VANISHED:
- Vertigo
- Ataxia
- Nystagmus
- Intention tremor
- Slurred speech
- Hypotonia
- Exaggerated broad based gait
- Dysdiadochokinesis
What is cauda equina syndrome?
Compression of nerve roots of the cauda equina - collection of nerve roots that travel through the spinal canal after the spinal cord terminates at L2/L3:
- Provides sensory innervation to lower limbs/perineum/bladder/rectum
- Provide motor innervation to lower limbs/anal and urethral sphincters
- Provides parasympathetic innervation to bladder/rectum
What are the clinical features of cauda equina syndrome?
- Saddle anaesthesia
- Loss of sensation in bladder/rectum
- Urinary/faecal retention/incontinence
- Bilateral sciatica
- Bilateral/severe motor weakness in legs
- Reduced anal tone
What are the clinical features of spinal cord commpression?
- Myelopathy
- Back/neck/spine pain
- Limb weakness
- Difficulty walking
- Sensory changes e.g. numbness/paraesthesia
- Urinary/faecal retention/incontinence
What are the investigations for spinal cord compression/cauda equina syndrome?
- X-ray/MRI/CT (urgent - within 24 hours)
- Biopsy
What is the management for spinal cord compression/cauda equina syndrome?
- NSAIDs
- Steroid injections
- High dose steroids
- Abx
- Chemotherapy/radiotherapy
- Surgery (lumbar decompression)
- Acupuncture/chiropractic care
- PT
What is anterior cord syndrome and what are common causes?
Incomplete spinal cord injury to the anterior 2/3 of spinal cord
- Anterior spinal artery ischaemia
- Disc herniation
- Trauma
- Tumour
- Epidural collection
What are the clinical features of anterior cord syndrome?
- Weakness/paralysis
- Loss of pain/temperature sensation
- Autonomic dysfunction (orthostatic hypotension)
- Bladder/bowel/sexual dysfunction
- NORMAL 2 point discrimination/proprioception/vibratory senses
What are the investigations and management for anterior cord syndrome
- MRI
- Supportive treatment (poor prognosis)
What is neurofibromatosis?
Genetic condition that causes benign nerve tumours (neuromas) to develop throughout the nervous system
Describe the pathophysiology of neurofibromatosis?
Type 1:
- More common
- NF1 gene found on chromosome 17
- Codes for a protein called neurofibromin (tumour suppressor protein)
- Autosomal dominant
Type 2:
- Associated with acoustic neuromas
- NF2 gene found on chromosome 22
- Codes for a protein called merlin (tumour suppressor protein - important in Schwann cells)
- Mutations lead to schwannomas
- Autosomal dominant
What are the clinical features of neurofibromatosis?
Type 1 - CABBING
- Café-au-lait spots
- Axillary/inguinal freckling
- BB = bony dysplasia (e.g. bowing of long bone/sphenoid wing dysplasia)
- Iris hamartomas (Lisch nodules - yellow/brown spots on iris)
- Neurofibromas (skin-coloured raised nodules/papules with smooth regular surface)
- Glioma of optic pathway
What are the investigations and management for neurofibromatosis?
- Genetic testing
- Monitoring/managing symptoms/treating complications
What is chronic fatigue syndrome also known as?
Myalgic encephalomyelitis
What are the risk factors for chronic fatigue syndrome?
- Female
- Infections
- Immune system problems
- Hormone imbalance
- Genetics
What are the clinical features of chronic fatigue syndrome?
- Extreme tiredness all the time
- Long recovery time following physical activity
- Insomnia
- Thinking/memory/concentration difficulties
- Muscle/joint pain
- Headaches
- Sore throat/flu-like symptoms
- Dizziness/nausea
- Palpitations
What is the criteria for a diagnosis of chronic fatigue syndrome?
- Severe tiredness lasting >6 months
- Other medical conditions have been ruled out
- > 4 symptoms present
What is the management for chronic fatigue syndrome?
- Symptomatic relief
- CBT
- Energy management
- Diet changes/vitamin supplements
- Medications for pain/sleeping
What is narcolepsy and what is it associated with?
Primary sleep disorder in which the brain loses its normal ability to regulate the sleep-wake cycle - strong association with certain HLA-subtypes
Describe the pathophysiology of narcolepsy
Type 1:
- Low levels of orexin (hypocretin) in CSF
- This is a neurotransmitter involved in the regulation of sleep/wakefulness/appetite
- Loss of orexin-secreting neurons in the hypothalamus = narcolepsy
What are the clinical features of narcolepsy?
- Insomnia
- Vivid dreams
- Excessive daytime sleepiness
- Cataplexy (type 1)
- Hypnagogic/hypnopompic hallucinations (dream-like hallucinations)
- Sleep paralysis
What are the investigations for narcolepsy?
- Multiple sleep latency testing EEG (MSLT)
- Polysomnography (PSG)
- CSF orexin levels
What is multiple sleep latency testing EEG (MSLT)?
Investigation for narcolepsy - EEG
- Sleep latency = time taken to fall asleep
- SOREM = sleep-onset REM - REM sleep occurring within 15 minutes of sleep onset (usually occurs over an hour after falling asleep)
What is the management for narcolepsy?
- Scheduled naps
- CNS stimulants e.g. modafinil/dexamphetamine/methylphenidate/pitolisant
- Antidepressants (for cataplexy) e.g. clomipramine, SSRIs, venlafaxine
- Sodium oxybate (sedative)
- Social support
What is cataplexy?
Sudden loss of muscle tone whilst conscious leading to weakness and a loss of voluntary muscle control
What are risk factors for cataplexy?
- Fhx
- Brain injury
Associated with certain conditions: - Type 1 narcolepsy
- Prader-Willi syndrome
- Angelman syndrome
- Niemann-Pick type C disease
What are common triggers for cataplexy?
Sudden, strong emotions e.g. laughter/fear/anger/stress/excitement
What are the clinical features of cataplexy?
ALWAYS FULLY CONSCIOUS - vary in severity:
- Minor = slight drooping of eyelids
- Severe = total body collapse with inability to move/speak/keep eyes opem
What is Wernicke’s encephalopathy?
Acute neurological condition characterised by a triad of confusion, ataxia and oculomotor dysfunction
What is Korsakoff syndrome?
Chronic amnesia syndrome characterised by defects in both anterograde and retrograde memory
What are the causes of Wernicke’s encephalopathy?
Thiamine/vitamin B1 deficiency due to:
- Chronic alcoholism
- Prolonged fasting/starvation
- Anorexia nervosa
- Hyperemesis gravidarum
- Systemic malignancy
- End-stage renal failure
- GI disease/malabsorption
What are the causes of Korsakoff syndrome?
Untreated Wernicke’s encephalopathy
What are clinical features of Wernicke’s encephalopathy?
- Ataxia
- Delirium/confusion
- Ophthalmoplegia/nystagmus
What are clinical features of Korsakoff syndrome?
- Irreversible short term memory loss
- Confabulation
- Time disorientation
How is Wernicke’s encephalopathy/Korsakoff syndrome treated?
IV pabrinex
What are the complications of Wernicke’s encephalopathy?
- Permanent horizontal nystagmus
- Inability to walk
- Deficit in learning/memory
What are the complications of Korsakoff syndrome?
Permanent neurological damage - recovery is rare
- Progressive reduced level of consciousness
- Coma
- Death
What is Meniere’s disease?
Balance disorder caused by excess fluid in a part of the inner ear (labyrinth)
What are some causes of Meniere’s disease?
- Allergies
- Abnormal immune system response
- Abnormal fluid drainage (due to blockage)
- Head injury
- Genetic risk
- Migraines
- Viral infection
Describe the pathophysiology of Meniere’s disease
- Labyrinth made up of bony labyrinth and membranous labyrinth
- Membranous labyrinth encased in bone and contains endolymph
- When head moves, endolymph moves
- Excess endolymph build-up = vertigo and affect hearing
What are the clinical features of Meniere’s disease?
- Vertigo (sweating, N+V)
- Tinnitus
- Loss/reduced hearing
- Loss of ability to hear low frequencies
- Pressure in affected ear
- Loss of balance
- Headaches
What are the investigations for Meniere’s disease?
- Hearing test
- Balance t7est
- MRI
- Electrocochleography (ECOG - measures electrical activity of inner ear)
What is the management for Meniere’s disease?
- No curative treatment
- Surgery
- Medication (allergies/reduce fluid build up/relieve symptoms/improve circulation in inner ear)
- Hearing aids
Describe the pathophysiology of hydrocephalus
Obstruction leading to blocked CSF flow
Aqueductal stenosis:
- Cerebral aqueduct that connects third and fourth ventricles is narrowed
- CSF build-up in lateral/third ventricles
Arnold-Chiari malformation:
- Cerebellum herniates downwards through foramen magnum to block CSF outflow
Babies <2:
- Cranial bones not fused at sutures
- Skull able to expand to fit cranial contents
- Hydrocephalus causes outward pressure on cranial bones
What are the clinical features of hydrocephalus?
- Gait changes
- Poor balance
- Mood changes/depression
- Confusion/memory changes
- Difficulty responding to questions
- Loss of bladder control
What are the clinical features of hydrocephalus in babies <2?
- Enlarged/rapidly increasing head circumference (occipito-frontal circumference)
- Bulging anterior fontanelle
- Poor feeding/vomiting
- Poor tone
- Sleepiness
What is normal pressure hydrocephalus?
Increased CSF in the ventricles without raised ICP - there is no physical obstruction of CSF, the ventricles just dilated
What are the characteristic clinical features of normal pressure hydrocephalus?
- Confusion
- Ataxia
- Urinary incontinence
What is communicating hydrocephalus and what are common causes?
Build up of CSF not due to obstruction but due to an inability if the arachnoid granulations to absorb the CSF
- Meningitis
- SAH
- Cancer
What are the clinical features of raised ICP?
- Reduced alertness/consciousness
- Severe headaches (usually worse in the morning and wake patients up, worse on coughing/straining/bending forward)
- Blurred vision
- N+V
- Changes in behaviour
- Weakness/problems moving/talking
- Lack of energy
What would the observations be in a patient with raised ICP?
Cushing’s triad:
- Widening pulse pressure
- Bradycardia
- Irregular breathing
What are the investigations for hydrocephalus?
- CT/MRI (FIRST LINE)
- LP for CSF (not if raised ICP suspected - contraindicated)
- Temporary drain (see if symptoms improve)
What are normal ICP values?
Adults = 10-15mmHg
Infants = 5-20mmHg
What is the management for hydrocephalus?
- Osmotic diuretics
- Prophylactic anticonvulsants
- Surgery (remove lesion)
- Surgery (decompressive craniectomy)
- VP shunt
What is a VP shunt?
Ventriculoperitoneal shunt - used for hydrocephalus
- Inserted into ventricle in brain and passed under skin from head through to abdomen
- Extra CSF shunted to abdomen where it is absorbed
Describe mononeuropathy
- Only one nerve affected
- Nerve compression due to tumour/cast/prolonged posture/etc.
- E.g. cubital tunnel syndrome = neuropathy of ulnar nerve
- E.g. carpal tunnel syndrome = neuropathy of median nerve
What are the most common non-compression causes of peripheral neuropathy?
DAVID
- Diabetes mellitus
- Alcoholism
- Vitamin B12 deficiency
- Infection/inherited (GBC/Charcot-Marie-Tooth)
- Drugs
What are the clinical features of peripheral neuropathy?
- Numbness/tingling/loss of sensation in hands/feet
- Burning/stabbing/shooting pain in affected areas
- Loss of balance/coordination
- Muscle weakness
What are the investigations for peripheral neuropathy?
- CT/MRI
- Nerve conduction studies
How are peripheral nerve injuries classified?
Sunderland classification system:
- First degree = reversible, local block, no surgery required, usually recovers within hours-weeks
- Second degree = loss of continuity of axons, no surgery required
- Third degree = damage to axons and supporting structures, neurolysis/grafting required
- Fourth degree = damage to axons and surrounding tissues to create scarring that prevents nerve regeneration, surgery/grafting required
- Fifth degree = nerve divided into 2, surgery required
What is the management for peripheral neuropathy?
- Acupuncture/massage therapy
- Medication
- Orthotics
- PT/rehab
- Weight loss
- Surgery/neurolysis/grafting
- Lifestyle changes (better control of diabetes)
What is a radiculopathy and which type is the most common?
Compression of the nerve roots as they exit the spinal cord and spinal column - lumbar radiculopathy is most common
What are common causes of radiculopathies?
- Stenosis (spinal stenosis/foraminal stenosis)
- Bone spurs (OA/trauma/degenerative conditions)
- Disc herniation
- Injury
- Gradual degeneration with age
What is sciatica?
A lumbar radiculopathy involving the sciatic nerve (L4-S3)
Describe the anatomy of the sciatic nerve
- Divides into tibial and common peroneal nerve at knee
- Supplies sensation to lateral lower leg and foot
- Supplies motor function to posterior thigh, lower leg and foot
What are the clinical features of radiculopathies?
- Sharp pain in affected area
- Weakness/loss of reflexes
- Numbness/paraesthesia
- Poor coordination/difficulty walking
What are the clinical features of sciatica?
- Unilateral pain from the buttock radiating down the back of the thigh to below the knee or feet
- ‘Electric/shooting’ pain
- Paraesthesia/numbness
- Motor weakness
- Affected reflexes
- Bilateral sciatica = red flag for cauda equina
What are the investigations for radiculopathies?
- X-ray
- CT/MRI
- Nerve conduction studies
What is the investigation for sciatica?
Sciatic stretch test:
- Patient lies on back with leg straight
- One leg lifted from ankle with knee extended until limit of hip flexion
- Ankle dorsiflexed
- Sciatica-type pain in buttock/posterior thigh indicates sciatic nerve root irritation
- Symptoms improve with knee flexion
What is the management for radiculopathies?
- NSAIDs/opioids/muscle relaxants
- Steroid injections
- Maintain healthy weight
- Good posture whilst sitting/playing sports/exercise/lifting heavy objects
- Lifestyle changes
- PT
- Surgery - laminectomy (remove small section of bone to relieve pressure)
What causes neuroleptic malignant syndrome?
- Adverse reactions to antipsychotics
- Abrupt withdrawal of dopaminergic medication
What are the clinical features of neuroleptic malignant syndrome?
- Rigidity
- Hyperthermia
- Altered mental status
- Autonomic instability
What are the investigations for neuroleptic malignant syndrome?
- Bloods (raised CK/WCC, deranged LFTs/U&Es, metabolic acidosis)
- CT/MRI head
- Infection screen (culture/LP)
What is the management for neuroleptic malignant syndrome?
- Withdraw causative medication
- Supportive treatment
- Dantrolene may be used
- Dopamine agonist (bromocriptine) may be used
What is the main complication of neuroleptic malignant syndrome?
AKI
What is the difference between myopathy and myelopathy?
Myopathy - any disease affecting muscles
Myelopathy - nerve damage inside spinal cord
What pattern of weakness is typically associated with myopathies?
Proximal and asymmetrical
What are the most common causes of delirium?
PINCH ME:
- Pain
- Infection
- Nutrition
- Constipation
- Hydration
- Medication
- Environment