Neuro Flashcards
What are the triad characteristics of Hunting’s disease
Dominant inheritance
Choreoathetosis (involuntary sudden movements often of the muscles in the arms, legs, face, and body)
Dementia
Patients with Huntington’s disease are more likely to experience behavioural changes and depression.
What is the first line medication to treat depression in this patient group
Selective serotonin reuptake inhibitors (SSRIs)
What medication has the most evidence-base to managed the choreoathetosis for Hunting’s Disease
Tetrabenazine
What are the 4 key signs of bacterial meningitis
Fever
Lethargy
Vomiting
Non-blanching rash
An Extradural Haemorrhage is a collection of blood between which layers of the skull?
The inner surface of the skull and the outer layer of the dura
Extradural haemorrhage is commonly caused by a tear in which artery?
Middle meningeal artery
Patient’s with Extradural haemorrhage often present with a palsy of which cranial nerve?
6th cranial nerve (abducen’s nerve)
What is the gold standard investigation to diagnose an extradural haemorrhage
CT head
Demonstrates a hyperdense bi-convex shape and are limited by the cranial sutures (they can’t cross over the sutures).
Egg-shaped, Emergency (arterial bleed)
Young patient with a traumatic head injury that has an ongoing headache. They have a period of improved neurological symptoms and consciousness followed by a rapid decline over hours is a typical Hx of what type of intracranial bleed?
Extradural haemorrhage
Expressive dysphagia i.e. ability to understanding of speech, but difficulty producing words.
Which part of the brain does this deficit localise to?
Broca’s area
Receptive dysphasia i.e. inability to understand speech but able to generate words. Typically present with a patient speaking in “word salad” as a result of their impaired understanding
Which part of the brain does this deficit localise to?
Wernicke’s area
Remember Word Salad - Wernicke’s
First line for the treatment of trigeminal neuralgia
Carbamazepine
What is the classic triad presentation of Devic’s disease (also known as neuromyelitis optica)
Transverse myelitis
Optic neuritis
Positive serum NMO-IgG (antibodies against aquaporin-4)
Typically relapsing-remitting
Serum NMO-IGG (antibodies against aquaporin-4) is highly suggestive of what condition
Devic’s disease (also known as neuromyelitis optica)
MRI reveals a spinal ring-enhancing lesion - what condition is this most likely to be
Spinal epidural abscess
A spinal epidural abscess may present with a spastic paraparesis but would also cause a fever and spinal pain
Voltage-gated potassium channel antibodies in CSF
Specific for what condition
Autoimmune encephalitis
Typically presents with sub-acute cognitive impairment, altered consciousness, seizures, and abnormal movements
Likely Dx:
Fatigable muscle weakness affecting limb muscles, extra-ocular muscles (drooping eyelids, diplopia), facial muscles (difficulty smiling or chewing), and bulbar muscles (change in speech or difficulty swallowing)
Symptoms are worse after prolonged movement or at the end of the day.
Myasthenia Gravis
Myasthenia gravis is an auto-immune disease characterised by antibodies against what kind of receptors?
Nicotinic acetylcholine receptors on muscle fibres.
What antibody is most likely to be positive in myasthenia gravis
Acetylcholine receptor antibody (depends in serum)
If negative or equivocal - check for muscle-specific tyrosine kinase antibodies
If ACh receptor antibody is positive and you now strongly suspect Myasthenia gravis.
What investigation might you consider doing
CT chest as MG is strongly associated with abnormalities of the thymus, and imaging to look for thymic hyperplasia or thymoma is warranted to evaluate the role of thymectomy
What is the first-line treatment in ocular myasthenia gravis
Anticholinesterase inhibitors e.g. pyridostigmine
First-line therapy in generalised myasthenia gravis
Immunosuppressive therapy e.g. prednisolone
AND
Anticholinesterase inhibitors e.g. pyridostigmine
What is used when the anticholinesterase inhibitor do not control symptoms of myasthenia gravis completely
Corticosteroids
A second-line immunosuppressant such as azathioprine is frequently used to reduce the dose of corticosteroid.
What medication can be used to induce temporary remission in severe relapses of myasthenia gravis, particularly where bulbar or respiratory function is compromised or before thymectomy.
Plasmapheresis
OR
Infusion of intravenous immunoglobulin
Function of Acetylcholinesterase inhibitors i.e. anticholinesterases
Inhibit the breakdown of acetylcholine in the synaptic cleft, increasing acetylcholine concentrations in the synaptic cleft, and increasing activation of nicotinic acetylcholine receptors at the neuromuscular junction.
Used in the management of myasthenia gravis
Likely Dx:
Balance issues leading to recurrent falls and vertical gaze palsy
Progressive supranuclear palsy (Parkinson’s plus syndrome)
Presents with parkinsonism with additional features.
What are the triad of characteristic features of Parkinsonism
Unilateral resting tremor
Cogwheeled rigidity
Bradykinesia (slow movements)
What medication can be used in the treatment of essential tremor
Propranolol OR primidone
Once patients are reassured that there is nothing more serious going on, they may elect not to commence pharmacotherapy
When is the disease activity for multiple sclerosis defined as Active
At least two clinically significant relapses occur within the last 2 years
When is the disease activity for multiple sclerosis defined as Highly active
Unchanged/increased relapse rate or by ongoing severe relapses compared with the previous year, despite disease-modifying drug treatment
When is the disease activity for multiple sclerosis defined as Rapidly evolving severe relapsing- remitting
> =2 disabling relapses in 1 year
AND EITHER
> = 1 gadolinium-enhancing lesions on brain magnetic resonance imaging (MRI)
OR
Significant increase in T2 lesion load compared with a previous MRI.
What are the two types of disease patterns for Multiple sclerosis
Relapsing-remitting (which may become secondarily progressive) - most common
Primary progressive.
What would be found in the CSF for multiple sclerosis
Oligoclonal bands
These reflect various immunoglobulins seen on CSF electrophoresis and indicate the presence of an auto-immune process in the CNS
Imaging modality for multiple sclerosis
MRI with contrast- will show periventricular white matter lesions
First line management of an acute relapse of multiple sclerosis
Oral methylprednisolone
IV methylprednisolone should be considered as an alternative if oral methylprednisolone has failed or is not tolerated
What criteria is used to diagnosis multiple sclerosis
McDonald criteria
What are the two groups of drugs used in the long term management of relapsing remitting multiple sclerosis
Disease modifying therapies (DMTs)
AND
Symptomatic therapies e.g. baclofen
What is the single best investigation to confirm early ischaemic stroke
Diffusion-weighted MRI
CT scans are useful for detecting haemorrhagic strokes, but can be normal in the first few hours of an ischaemic stroke
What medication is recommended in the treatment of acute ischaemic stroke if it can be administered within 4.5 hours of symptom onset and if intracranial haemorrhage has been excluded by appropriate imaging techniques
Alteplase
Alteplase is recommended in the treatment of acute ischaemic stroke if it can be administered within how many hours since the symptoms onset (haemorrhagic stroke has already been excluded)
Within 4.5 hours of symptom onset
Patients with ischaemic stroke or transient ischaemic attack associated with atrial fibrillation should be reviewed for what kind of long-term treatment
Anticoagulant
What is the long term treatment following a transient ischaemic attack or an ischaemic stroke (not associated with atrial fibrillation)
Clopidogrel
What is the name of the stroke classification system
Bamford/Oxford classification
What can be performed in patients with anterior circulation strokes within 6 hours of symptom onset
Mechanical Thrombectomy
Mechanical Thrombectomy can be performed in patients with ANTERIOR circulation strokes within how many hours since symptom onset
6 hours
Mechanical Thrombectomy can be performed in patients with POSTERIOR circulation strokes within how many hours since symptom onset
12 hours
If it has been greater than 4.5 hours since symptom onset for ischaemic stroke (therefore not in the time window for alteplase).
What is the best management option
Oral aspirin (treatment dose 300mg) daily for two weeks
If alteplase has been done for ischaemic stroke when should aspirin be started?
Aspirin is usually started 24 hours after the treatment following a repeat CT Head that excludes any new haemorrhagic stroke.
Secondary stroke prevention
Mnemonic HALTSS:
Hypertension: anti-hypertensive therapy should be initiated 2 weeks post-stroke.
Antiplatelet therapy (if stroke associated with AF anticoagulant e.g. Rivaroxaban or Apixiban, otherwise clopidogrel is initiated 2 weeks post-stroke.
Lipid-lowering therapy e.g. atorvastatin
Tobacco (smoking cessation)
Sugar: screened for diabetes and managed appropriately.
Surgery: patients with ipsilateral carotid artery stenosis more than 50% should be referred for carotid endarterectomy.
What is the gold standard investigation in assessing for the underlying cause of subarachnoid haemorrhage
CT angiography of the head
Once the acute condition is managed she needs to be assessed for an underlying cause
What is the gold standard investigation for diagnosing a subarachnoid haemorrhage
CT head
Will show hyperdense material in the subarachnoid space
If the CT head does not confirm the diagnosis of subarachnoid haemorrhage what is the next best investigation?
Lumbar puncture (After least 12 hours after symptom onset)
Will show Xanthochromia (which can lead to pink or yellow coloured CSF) and an increase in cerebrospinal fluid (CSF) bilirubin
Diagnosis of subarachnoid haemorrhage -> CTPA complete.
Refer to neurosurgeons. What options do they have to fix a ruptured aneurysms
Endovascular techniques exist for coiling or stenting SAH bleed.
Surgical techniques include clipping.
What does Romberg’s positive indicate
Positive if there is a significant increase in unsteadiness upon closure of the eyes
Suggests that the ataxia is sensory in nature
What are the 6 features of cerebellar dysfunction
Acronym DANISH:
i) Dysdiadochokinesia (an inability to perform rapid alternating hand movements)
ii) Ataxia (a broad-based, unsteady gait)
iii) Nystagmus (involuntary eye movements)
iv) Intention tremor (seen when the patient is asked to perform the ‘finger-nose test’)
v) Slurred speech
vi) Hypotonia
What is the best description for this motor phenomenon of epilepsy:
Unilateral leg twitching initially which spreads up and causes movement in the trunk and eventual arm
Jacksonian march
Due to the propagation of electrical activity through the primary motor cortex
How would Todd’s paresis present
Focal, often unilateral, weakness following a seizure
Likely Dx:
Unilateral deafness and vertigo occurring later, in addition to left-sided lesions of cranial nerves V, IX and X
Acoustic neuroma
What is the manoeuvre that is diagnostic of benign positional paroxysmal vertigo (BPPV)
Hallpike manoeuvre
The presence of debris in the semicircular canals of the ears causes vertigo upon head movement.
What is the manoeuvre that is first line in treatment benign positional paroxysmal vertigo (BPPV)
Epley manoeuvres treat BPPV by clearing the debris.
Treatment of Ménière’s disease
Antihistamines and bed rest
Treatment of Ramsay Hunt syndrome
Aciclovir and prednisolone.
What are the three medications that most commonly cause ototoxicity
Aminoglycoside antibiotics eg. gentamicin, vancomycin
Loop diuretics eg. furosemide
The uvula will deviate ___ the side of the lesion
AWAY
Tongue will point ______ the side of the lesion
TOWARDS
Polycystic kidney disease can be associated with what kind of aneurysms
Berry aneurysms - which can rupture causing a subarachnoid haemorrhage
Acute Management of suspected acute bacterial meningitis
IV ceftriaxone
Addition of IV amoxicillin in young/old patients to better cover listeria, and IV aciclovir if viral encephalitis is suspected.
Inheritance pattern for Myotonic dystrophy
Autosomal dominant pattern.
Trinucleotide repeat disorder affecting muscle-specific chloride channels
Likely Dx:
Pt in their 20s
Frontal balding
Slow relaxing grip
Gradually worsening weakness in his hands
Myotonic dystrophy
How is myotonic dystrophy diagnosed
Genetic testing
Likely Dx
Ipsilateral impairment of the adducting eye
Nystagmus in the abducting eye
Internuclear ophthalmoplegia (the side of the eye that cannot adduct)
Triad of features for normal-pressure hydrocephalus.
Urinary incontinence
Dementia
Gait abnormality
“Wet, Wobbly and Wacky”
All of which have developed over several months
First-line treatment for normal pressure hydrocephalus
Ventriculo-peritoneal shunting
What measure is used to monitor respiratory involvement in Guillain-Barré syndrome
Forced vital capacity (FVC)
If it is dangerously reduced, intubation and admission to the Intensive Care Unit may be necessary
Management of Guillain-Barré syndrome
Conservative measures e.g. monitoring of ventilation (with serial spirometry and ABG) ± ventilation
Medical management with IV immunoglobulin and, if ineffective, plasmapheresis.
What is the most common cause of medical third (oculomotor) nerve palsy,
Diabetes mellitus
Difference between medial and surgical third nerve palsy
Medical: Painless and down and out pupil
Surgical: Painful and dilated pupil (due to compression of the outer parasympathetic fibres)
What is the one disease modifying treatment available for motor neurone disease
Riluzole (an antiglutamatergic drug which dampens motor nerve firing) has been shown to prolong life by 3 months.
prophylaxis for cluster headache
Verapamil
managing an acute attack of cluster headache
100% oxygen via non-rebreathable mask with a subcutaneous or nasal Triptan
First line management of Trigeminal neuralgia
Carbamazepine
Inability to abduct thumb after a Colle’s fracture
Injury to what nerve?
Median nerve
The median nerve supplies the motor supply for the radial half of the fingers in the hand
Urinary incontinence + gait abnormality + dementia =
normal pressure hydrocephalus
Infected followed by 1-3 weeks of lower back and leg pain along with paraesthesia and weakness is pathognomonic of what condition
Guillain-Barre syndrome
Features of Brown-Sequard syndrome
Caused by lateral hemisection of spinal cord.
Same sided weakness and proprioception/vibration loss
Loss of pain/temperature on the opposite side to the hemisection
Feature of Subdural haemorrhage on CT head
Sickle-shaped
Slow (venous bleed)
Features of a temporal lobe seizure
An aura occurs in most patients
typically a rising epigastric sensation
also psychic or experiential phenomena, such as déjà vu
automatisms (e.g. lip smacking/grabbing/plucking) are common
Obese, young female with headaches / blurred vision - pathognomonic for
idiopathic intracranial hypertension
First line treatment for transient ischaemic attack
Aspirin 300mg immediately with specialist review within 24 hours
Neuroleptic malignant syndrome is a rare but dangerous condition seen in patients taking what medication.
Antipsychotic medication.
Neuroleptic malignant syndrome is a medical emergency.
Management
Immediate cessation of the dopamine antagonist (or restarting or continuing of the dopamine agonist)
AND
Supportive measures (rehydration, cooling, and treatment of rhabdomyolysis if present).
Investigation for Narcolepsy
Multiple sleep latency EEG
Management of Nacrolepsy
Daytime stimulants (e.g. modafinil) and nighttime sodium oxybate
First line medication for Absence seizures
Ethosuximide
Lamotrigine for all epilepsies expect?
Myotonic epilepsy
Carbamazepine is contraindicated in what type of epilepsy?
Juvenile myoclonic epilepsy
Absent epilepsy (worsens the symptoms)
Drugs that cause Stevens-Johnson syndrome
CLASP:
Carbamazepine
Lamotrigine
Allopurinol
Sulfonamide
Phenobarbital
First line management of bells palsy
Oral prednisolone
eye care is important to prevent exposure keratopathy e.g. artificial tears and eye lubricants and taping the eyes closed if they are unable to close the eye at bedtime
‘Cape-like’ features (neck, shoulders and arms):
loss of sensation to temperature but the preservation of light touch, proprioception and vibration
Classic examples are of patients who accidentally burn their hands without realising
Pathognomonic of what condition
Syringomyelia
Caused by a collection of CSF within the spinal cord
Gold standard investigation for syringomyelia
Full spine MRI with contrast to exclude a tumour or tethered cord
DVLA guidelines for a patient with a first unprovoked or isolated seizure if brain imaging and EEG normal
Patients cannot drive for 6 months
Define Status epilepticus
A single seizure lasting >5 minutes
OR
>= 2 seizures within a 5-minute period without the person returning to normal between them
Serotonin syndrome can present in a very similar manner to neuroleptic malignant syndrome (NMS).
One main difference is that serotonin syndrome presents when in comparison to NMS
Serotonin syndrome presents over hours rather than days like in neuroleptic malignant syndrome
WHat is the key differentating symptom when comparing Posterior Inferior Cerebellar Artery stroke to lateral pontine syndrome (in which facial weakness - not numbness - is the key)
PIKACHU –> PICA can’t chew
People with posterior inferior cerebellar artery stroke will have dysphagia
