NeuroSoc Revision doc Flashcards
What is the Parkinsonism triad of symptoms
Rigidity
Bradykinesia
Tremor
What kinds of rigidity can be seen in Parkinsonism
Cog-wheel
Lead pipe
What kind of tremor is seen in Parkinsonism and how can it be made more apparent
Resting pill-rolling tremor
Can be accentuated by asking the patient to perform a task requiring concentration such as counting backwards from 100
Which joints can be flexed to test for rigidity
Elbow and wrist joints can be flexed and extended whilst relaxed
What is a Lewy Body
Intracytoplasmic, eosinophilic, alpha-synuclein containing inclusion
Where do Lewy Bodies tend to affect in Parkinson’s disease
The pars compacta of the substantia nigra
Which neurotransmitter pathway is affected in Parkinson’s
Dopaminergic
What is micrographia
Decreasing amplitude of handwriting.
What disease is micrographia seen in
Parkinson’s
What are 4 non-motor symptoms of Parkinson’s
non-REM sleep disorder, dementia, depression, constipation
What is considered the most effective treatment for Parkinson’s
Levodopa
What is the mode of action of Levodopa
Dopamine precursor that can cross the blood-brain barrier
Name 3 side effects of Levodopa
Hypotension
Nausea
Dyskinesia
What drug can Levodopa be given with
Can be combined with peripheral dopa- decarboxylase inhibitors (carbidopa) to prevent peripheral metabolism of levodopa
Which class of drug, also used for depression, can be given for Parkinson’s
Monoamine oxidase B inhibitors
How do Monoamine oxidase B inhibitors work and give an example
Inhibits monoamine oxidase B, an enzyme responsible for catabolising dopamine to homovanillic acid.
Example: rasagiline
What is dopamine catabolised to by MAO-B
Homovanillic acid
What is a potentially fatal side effect of MAO-B inhibitors
Hypertensive crisis due to tyramine excessive diet
What is a treatment option for those under 70 with Parkinson’s
Dopamine agonists
Why might you prefer giving dopamine agonists to a young Parkinson’s patient rather than Levodopa
Due to the high risk of dyskinesia in younger patients using levodopa.
Give an example of a dopamine agonist
Pramipexole
Name 4 side effects of dopamine agonists
Hypotension
Nausea
Ankle swelling
Hallucinations
What kinds of drugs can cause parkinsonism
Typical Anti-psychotics (since they block dopamine 2 receptors)
Define Lewy Body dementia
Dementia or cognitive symptoms occurring before or at the same time as parkinsonism
Define Parkinson’s disease dementia
Dementia starting more than 1 year after the onset of parkinsonism
What are 3 defining features of Lewy Body dementia
Features of parkinsonism
Visual hallucinations
Cognitive decline
What is multiple system atrophy
Parkinsonism Autonomic dysfunction (resulting in postural hypotension, erectile dysfunction or urinary urgency) ± cerebellar dysfunction
A ‘hot cross bun” appearance of the pons on axial MRI suggests what disease
Multiple system atrophy
What will an MRI show in multiple system atrophy
Putaminal atrophy
MRI showing midbrain atrophy with a ‘hummingbird’ sign on sagittal view should suggest which disease
Progressive supranuclear palsy
What are some symptoms of Progressive supranuclear palsy
Axial akinesia and rigidity, loss of balance and unexplained falls Forgetfulness Dysarthria Loss of eye movements (vertical gaze)
What is an essential tremor
A movement disorder characterized by a postural or action tremor, rather than a resting tremor seen in Parkinson’s
What are intention tremors caused by
Caused by lesions in the cerebellum or its pathways, most commonly seen in multiple sclerosis
What is dystonia
Dystonia is a common movement disorder characterised by involuntary muscle spasms leading to abnormal postures of the affected body part
What is chorea
Chorea consists of continuous spontaneous jerky movements, irregularly timed and randomly distributed.
Which triplet is repeated in Huntington’s
CAG
How many CAG repeats are needed to cause Huntington’s
> 35
How is Huntington’s inherited
Autosomal dominant
Name 3 early signs of Huntington’s
Depression
Apathy
Clumsiness
Huntington’s progresses to cause which 3 symptoms
CHorea
Dementia
Rigidity
Which drug can be given for symptomatic relief in Huntington’s
Tetrabenazine (Anti-Choreic)
How does Tetrabenazine work
Inhibits vesicular monoamine transporter (VMAT)
Rheumatic fever can manifest into which chorea
Sydenham’s chorea
What are tics
Tics are typically relatively brief rapid intermittent stereotyped involuntary movements or sounds
What is the most common syndrome characterised by tics
Gilles de la Tourette syndrome
What is myoclonus
Defined as a sudden brief shock(like involuntary movement caused by muscular contraction or inhibition
What is Hemiballism
Wild flinging/throwing movements of one arm or leg
Why does hemiballism occur
cerebrovascular lesion to the subthalamic nucleus
What is a seizure
A short episode of symptoms caused by a burst of abnormal electrical activity in the brain
What is epilepsy
The ongoing liability to recurrent seizures
What should you always ask patients about in an OSCE if they have presented with a seizure
Preceding Aura
How would a focal seizure in the occipital lobe present
Visual phenomena such as flashing lights, colours or hallucinations
How would a focal seizure in the parietal lobe present
Sensory manifestations can take the form of tingling or numbness or pain.
How would a focal seizure in the frontal lobe present
Motor signs such as stiffness, twitching or spasm
How would a focal seizure in the temporal lobe present
Changes in mood or behaviour
A rising epigastric sensation is a common manifestation in which type of focal seizure
Temporal lobe focal seizure
What is the difference between a simple and a complex focal seizure
Simple = no loss of consciousness
What is an Absence seizure and how long do they typically last
An abrupt sudden loss of consciousness (“the absence”) and of all motor activity rapidly stopped
Usually lasts 10 seconds
Do people fall during an absence seizure
Not usually since muscle tone is preserved
What is a characteristic sign of an absence seizure on EEG
3Hz generalised spike-wave
What is a clonic seizure
During a clonic seizure, the individual’s muscles begin to spasm and jerk. The elbows, legs and head will flex and relax rapidly
What is a tonic seizure
A tonic seizure causes a brief loss of consciousness, the patient falls to the ground and often there is extension of the neck; with upturning of the eyes and arching of the back
What is a tonic-clonic seizure
Tonic phase - muscles become stiff
Clonic phase - muscles will start to contract and relax rapidly
How is epilepsy typically diagnosed
Generally by clinical history
Why is EEG useful in epilepsy
To help support a diagnosis of epilepsy and determine a seizure type
Name 2 first-line drugs for focal seizures
Carbamazepine
Lamotrigine
Name 2 first-line drugs for generalised tonic-clonic seizures
Sodium Valproate
Lamotrigine
Name 2 first-line drugs for generalised absence seizures
Sodium Valproate
Ethosuximide
Name a drug used first-line for myoclonic generalised seizures
Sodium Valproate
If someone has an unclassified type of epilepsy, which drugs would be used first-line and second-line
1st line- Sodium Valproate
2nd line - Lamotrigine
2nd line drug for generalised myoclonic seizures
Levetiracetam
Name 2 second-line drugs for generalised tonic-clonic seizures
Lamotrigine
Levetiracetam
Which drug should you never give for generalised seizures and why
Carbamazepine
Can make them worse
How does Carbamazepine work
Na+ channel inactivation
Which anti-convulsants work by inactivating Na+ channels
Carbamazepine
Phenytoin
How does Lamotrigine work
Blocks voltage-gated Na+ channels
How does Ethosuximide work and when can it be used
Blocks thalamic T- type Ca2+ channels
Used 1st line for absence seizures
Which anticonvulsant is teratogenic
Sodium Valproate
How does Sodium Valproate work
Na+ channel inactivation with increase GABA concentration
Which anti-convulsant can cause Steven-Johnson’s syndrome
Lamotrigine
What is status epilepticus
Status epilepticus is defined as a convulsive seizure which continues for a prolonged period (longer than five minutes)
How would you manage status epilepticus
community - rectal diazepam
hospital - IV lorazepam
What is Motor Neurone Disease
A group of neurodegenerative conditions which affect only the upper and/or lower motor neurones
What is the most common MND type
Amyotrophic Lateral Sclerosis (ALS)
Which chromosome has genes which have been linked to MND
Chromosome 21
Which type of dementia is MND often linked to
Frontotemporal dementia (FTD)
What is the only drug given to those with MND and how does it affect prognosis
Riluzole
Extends life expectancy by 3 months
How does Riluzole work
Blocks glutaminergic neurotransmission in the CNS
Name 3 complications of MND
Depression
Weight loss + malnutrition (2ndary to dysphagia)
Respiratory depression
Name an autosomal recessive muscle disease
McArdle’s disease
What is deficient in McArdle’s disease
Myophosphorylase
What is the mode of inheritance of myotonic dystrophy
Autosomal dominant
What is split hand syndrome and which disease is it linked to
Thenar muscles atrophy but hypothenar muscles are spared
Seen in ALS
A tri-nucleotide repeat on chromosome 19 should suggest which disease
Myotonic Dystrophy
Name 3 characteristic signs of myotonic dystrophy
‘Christmas tree-like’ bilateral cataracts
Early frontal balding
Sternocleidomastoid and distal muscle wasting
In Lambert-Eaton Myasthenic syndrome, what structure are autoantibodies created against
Ca2+ voltage-gated channels at the pre-synaptic terminal
How is LEMS diagnosed
Presence of anti(VGCC antibodies and by characteristic electrophysiological findings using a 20–50 Hz repetitive stimulation
Which imaging scan must be carried out in suspected LEMS and why
CT scan to rule out small cell lung cancer
How is LEMS usually treated
3- 4 diaminopyridine (Amifampridine)
blocks presynaptic Ca2+ channels
Which part of the NMJ does Myasthenia gravis affect
post-synaptic nicotinic acetylcholine receptors
What is a common presentation of myasthenia gravis
Muscle fatiguability
usually of extra-ocular muscles
Are proximal or distal muscles usually affected in myasthenia gravis
Proximal
What is a Myasthenia crisis
weakness of the muscles of respiration causes ventilator failure
What is the treatment for myasthenia crisis
plasmapheresis
IV immunoglobulins
systemic steroids
What is a cholinergic crisis and what kind of people is it common in
common in patient receiving high dose anticholinesterase medication
What kind of hyperplasia is Myasthenia Gravis
Thymus hyperplasia
thymoma
Which connective tissue disease is associated with Myasthenia Gravis
SLE
Name 4 drugs that can exacerbate myasthenia gravis
Gentamicin
Lithium
Beta-blockers
Verapamil
Which antibody can be tested for in Myasthenia Gravis
IgG antibodies against acetylcholine receptor (anti-AChR)
How is myasthenia gravis usually treated
Acetylcholinesterase inhibition using Pyridostigmine
Which bacteria infection can precede Guillan-Barre syndrome
Campylobacter
How is polio spread
Facael-oral transmission
What does a poliomyelitis infection cause destruction of
Destruction of cells in anterior horn of spinal cord (LMN death)
What are the 2 broad categories of pain
Nociceptive pain
Neuropathic pain
What is allodynia
pain produced in response to a stimulus that is normally not painful
What is hyperalgesia
increase in pain response, with lowering of the pain threshold, to a
stimulus that is normally painful
What is the leading cause of dementia
Alzheimer’s disease
Which syndrome increases your risk of developing Alzheimer’s
Down syndrome
Which version of the ApoE gene increases and decreases risk for Alzheimer’s
ApoE4 - increases
ApoE2 - decreases
What happens to the gyri and sulci in Alzheimer’s
Narrowing of gyri
Widening of sulci
What are the plaques and neurofibrillary tangles made up of in Alzheimer’s
B-amyloid plaques
Tau tangles
Which source of cholinergic projections to the brain is often first affected in Alzheimer’s
nucleus basalis of Meynert
What change is seen in CSF biomarkers in Alzheimer’s disease
increase in phosphorylated tau protein and decrease in β-amyloid
What class of drugs can be given for Alzheimer’s disease
Acetylcholinesterase inhibitors
rivastigmine or galantamine
Which drug apart from cholinesterase inhibitors can be given for Alzheimer’s and how does it work
Memantine is a voltage-dependent, non-competitive
(NMDA) receptor antagonist
How could a haemorrhagic stroke occur in Alzheimer’s
due to β-amyloid deposits in cerebral blood vessels that can weaken them (cerebral amyloid angiopathy)
What are the 3 broad variants of frontotemporal dementia
Behavioural
Primary progressive aphasia
(semantic and progressive non-fluent aphasia)
Which scan is particularly useful for showing the changes of Frontotemporal dementia
SPECT scan
What is the second most common form of dementia
Vascular dementia
What will an MRI or SPECT scan show in vascular dementia
Infarcts and white matter changes
What is Creutzfeldt–Jakob disease
A transmissible prion neurodegenerative disease which causes cell death (spongiform changes in the cortex) and astrocytosis
How does Creutzfeldt–Jakob disease present and progress
Rapidly progressing dementia, ataxia and myoclonic jerks
What would CSF analysis show in a patient with Creutzfeldt–Jakob disease
Elevated 14-3-3 protein
What is multiple sclerosis characterised by
Demyelination of white matter areas in the brain
What is the most common phenotype of MS
Relapsing-Remitting
Areas of demyelination are referred to as what in MS
Plaques
Name 3 common areas of demyelination
Optic nerve
Corpus callosum
Periventricular white matter
Name 3 risk factors for MS
Those living in a higher latitude
Female
Previous EBV infection
Which HLA gene is linked to MS
HLA DRB1
What is the most ocular symptom of MS
Optic Neuritis
What is Lhermitte’s sign
Electric shock-like sensation down the upper limbs and
trunk on neck flexion
WHat is Uhthoff’s phenomenon and which disease is it seen in
Exacerbation of current symptoms in hot
environments
seen in MS
What does the mneumonic DANISH stand for and what does it indicate
Indicates Cerebellar dysfunction
Dysdiadochokinesia, Ataxia, Nystagmus,
Intention tremor, Slurred speech and Hypotonia/Heel-shin test
What will an MRI of a brain affected by MS show
Multiple white matter plaques
What will CSF analysis of a patient with MS show
Presence of IgG Oligoclonal bands
What are 3 options for 1st line therapy in terms of disease-modifying therapy for MS
Interferon-beta
Tecfidera
Glatiramer acetate
How is spasticity managed in MS
Baclofen
How is neuropathic pain managed in MS
Gabapentin
How is fatigue managed in MS
Amantadine + modafinil
How is bladder dysfunction managed in MS
Anti-Cholinergic drugs (Oxybutynin)
Name 5 red flags for a headache
Age >55 Immunosuppressed Previous/current malignancy Early morning headache Exacerbated by Valsalva manoeuvre
What does aura refer to in regards to a headache
Aura refers to reversible visual, auditory, motor or language symptoms
including zigzag lines, central or hemianopic scotoma, aphasia and muscle weakness
What are the Trigeminal autonomic cephalgias (TAC)
A group of headache disorders categorised by common features of unilateral pain (often severe) in the distribution of the trigeminal nerve
Name 4 trigeminal autonomic cephalgias
Cluster
Paroxysmal hemicrania
SUNCT
Trigeminal Neuralgia
Which of the trigeminal autonomic cephalgias has an absolute response to Indomethacin
Paroxysmal Hemicrania
What does SUNCT stand for
Short Unilateral Neuralgiform headache with Conjunctival injection Tearing
Name 2 treatments for SUNCT
Lamotrigine
Gabapentin
How is trigeminal neuralgia treated
Carbamazepine
Name 3 vascular disorders which can present as a headache
Subarachnoid haemorrhage
Carotid dissection
Giant cell arteritis
What is Pseudotumor cerebri
idiopathic intracranial hypertension
What is the treatment for idiopathic intracranial hypertension
Weight loss is first line management of IIH and usually is sufficient to reduce ICP.
Acetazolamide (decreases CSF production) can be used as a medical treatment for
IIH
Shunt in refractory cases
What are clinical presenting features of idiopathic intracranial hypertension
A throbbing headache worse in the morning
Bilateral papilloedema.
Nausea and vomiting.
Which nerve palsy can be a presentation of idiopathic intracranial hypertension
Sixth nerve palsy
Which investigations can be carried out for idiopathic intracranial hypertension
MRI or CT
What will be seen upon execution of a lumbar puncture in a patient with idiopathic intracranial hypertension
High opening pressure
What will idiopathic intracranial hypertension show on MRI/CT
Slit-like ventricles
What does meningism refer to
Triad of symptoms:
Photophobia
Neck stiffness
A headache
Where does infection usually spread from to cause meningitis
Sinuses
Nasopharynx
Ear
Which organism affects mainly neonates and elderly, with regards to meningitis
Listeria Monocytogenes
Which organism is most commonly the cause of Meningitis in infants + children/adolescents
N.Meningitidis
Which organism is most commonly the cause of Meningitis in Adults
Strep. Pneumoniae
Which organism is most commonly the cause of Meningitis in those with recent neurosurgery or head trauma
Staph Aureus
What is Waterhouse–Friderichsen syndrome
Waterhouse–Friderichsen syndrome (WFS) is defined as adrenal gland failure due to bleeding into the adrenal glands, commonly caused by severe bacterial infection: Typically it is caused by Neisseria meningitidis
What is Kernig’s sign and what pathology does it suggest
A patient will resist Knee extension when the hip is flexed Suggests meningitis (or at least meningeal irritation)
What is Brudzinksi’s sign and what pathology does it suggest
Neck flexion will cause flexion at the hip and the knee joints Suggests meningitis (or at least meningeal irritation)
What is the name of the test which involves pushing a glass into a purpuric rash to prove it is non-blanching
Tumbler’s test
In a patient with suspected meningitis, which investigation must always be carried out before a lumbar puncture and why
CT scan-
To rule out increased intracranial pressure
must be done before LP so coning does not occur
What will a lumbar puncture show in meningitis with respect to Cells Colour of CSF Protein Glucose
Cloudy turbid CSF
Polymorphs present
Increased protein
Decreased glucose
What is the empirical treatment for community-acquired meningitis
IV Ceftriaxone + Dexamethasone
When would amoxicillin be given alongside Ceftriaxone for meningitis treatment
If Listeria is suspected
When should you suspect Listeria to be the causative organism in meningitis
Neonate or >55 or Immunosuppressed
What is the prophylactic treatment given to those who have been in contact with someone with meningitis
Ciprofloxacin
Rifampicin if refractory to treatment
What will a lumbar puncture show in viral meningitis with regards to: CSF colour Glucose Cells Protein
Clear fluid
Low/normal glucose
Lymphocytes
Normal protein
Name 3 characteristic CSF findings in Tuberculosis meningitis
Clear and culture negative
Lymphocytosis
Fibrin webs
What is Inflammation of the brain parenchyma known as
Encephalitis
What is the most common cause of encephalitis
Herpes simplex
A patient with seizures with focal neurological deficits (aphasia) should make you think of what
Herpes simplex encephalitis
Parotitis in an unvaccinated patient should make you think of which type of encephalitis
Mumps encephalitis
Skin lesions\vesicles in a dermatomal pattern should make you think of which type of encephalitis
Varicella-zoster encephalitis
MRI in HSV encephalitis will show what
Characteristic oedema in temporal lobes
What is the treatment for encephalitis
IV acyclovir
What is a serious consequence of the measles virus involving the brain parenchyma
Subacute sclerosing panencephalitis
Describe CSF findings in a Subarachnoid haemorrhage
Xanthochromia
Describe CSF findings in Narcolepsy
Low or undetectable levels of CSF orexin/hypocretin
Describe CSF findings in Alzheimer’s
High hyper-phosphorylated Tau protein
Low B-amyloid
Describe CSF findings in Multiple sclerosis
IgG oligoclonal bands
