NeuroSoc Revision doc Flashcards

1
Q

What is the Parkinsonism triad of symptoms

A

Rigidity
Bradykinesia
Tremor

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2
Q

What kinds of rigidity can be seen in Parkinsonism

A

Cog-wheel

Lead pipe

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3
Q

What kind of tremor is seen in Parkinsonism and how can it be made more apparent

A

Resting pill-rolling tremor

Can be accentuated by asking the patient to perform a task requiring concentration such as counting backwards from 100

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4
Q

Which joints can be flexed to test for rigidity

A

Elbow and wrist joints can be flexed and extended whilst relaxed

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5
Q

What is a Lewy Body

A

Intracytoplasmic, eosinophilic, alpha-synuclein containing inclusion

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6
Q

Where do Lewy Bodies tend to affect in Parkinson’s disease

A

The pars compacta of the substantia nigra

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7
Q

Which neurotransmitter pathway is affected in Parkinson’s

A

Dopaminergic

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8
Q

What is micrographia

A

Decreasing amplitude of handwriting.

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9
Q

What disease is micrographia seen in

A

Parkinson’s

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10
Q

What are 4 non-motor symptoms of Parkinson’s

A

non-REM sleep disorder, dementia, depression, constipation

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11
Q

What is considered the most effective treatment for Parkinson’s

A

Levodopa

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12
Q

What is the mode of action of Levodopa

A

Dopamine precursor that can cross the blood-brain barrier

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13
Q

Name 3 side effects of Levodopa

A

Hypotension
Nausea
Dyskinesia

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14
Q

What drug can Levodopa be given with

A

Can be combined with peripheral dopa- decarboxylase inhibitors (carbidopa) to prevent peripheral metabolism of levodopa

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15
Q

Which class of drug, also used for depression, can be given for Parkinson’s

A

Monoamine oxidase B inhibitors

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16
Q

How do Monoamine oxidase B inhibitors work and give an example

A

Inhibits monoamine oxidase B, an enzyme responsible for catabolising dopamine to homovanillic acid.
Example: rasagiline

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17
Q

What is dopamine catabolised to by MAO-B

A

Homovanillic acid

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18
Q

What is a potentially fatal side effect of MAO-B inhibitors

A

Hypertensive crisis due to tyramine excessive diet

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19
Q

What is a treatment option for those under 70 with Parkinson’s

A

Dopamine agonists

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20
Q

Why might you prefer giving dopamine agonists to a young Parkinson’s patient rather than Levodopa

A

Due to the high risk of dyskinesia in younger patients using levodopa.

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21
Q

Give an example of a dopamine agonist

A

Pramipexole

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22
Q

Name 4 side effects of dopamine agonists

A

Hypotension
Nausea
Ankle swelling
Hallucinations

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23
Q

What kinds of drugs can cause parkinsonism

A

Typical Anti-psychotics (since they block dopamine 2 receptors)

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24
Q

Define Lewy Body dementia

A

Dementia or cognitive symptoms occurring before or at the same time as parkinsonism

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25
Define Parkinson's disease dementia
Dementia starting more than 1 year after the onset of parkinsonism
26
What are 3 defining features of Lewy Body dementia
Features of parkinsonism Visual hallucinations Cognitive decline
27
What is multiple system atrophy
``` Parkinsonism Autonomic dysfunction (resulting in postural hypotension, erectile dysfunction or urinary urgency) ± cerebellar dysfunction ```
28
A 'hot cross bun" appearance of the pons on axial MRI suggests what disease
Multiple system atrophy
29
What will an MRI show in multiple system atrophy
Putaminal atrophy
30
MRI showing midbrain atrophy with a 'hummingbird' sign on sagittal view should suggest which disease
Progressive supranuclear palsy
31
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) ```
32
What is an essential tremor
A movement disorder characterized by a postural or action tremor, rather than a resting tremor seen in Parkinson’s
33
What are intention tremors caused by
Caused by lesions in the cerebellum or its pathways, most commonly seen in multiple sclerosis
34
What is dystonia
Dystonia is a common movement disorder characterised by involuntary muscle spasms leading to abnormal postures of the affected body part
35
What is chorea
Chorea consists of continuous spontaneous jerky movements, irregularly timed and randomly distributed.
36
Which triplet is repeated in Huntington's
CAG
37
How many CAG repeats are needed to cause Huntington's
>35
38
How is Huntington's inherited
Autosomal dominant
39
Name 3 early signs of Huntington's
Depression Apathy Clumsiness
40
Huntington's progresses to cause which 3 symptoms
CHorea Dementia Rigidity
41
Which drug can be given for symptomatic relief in Huntington's
Tetrabenazine (Anti-Choreic)
42
How does Tetrabenazine work
Inhibits vesicular monoamine transporter (VMAT)
43
Rheumatic fever can manifest into which chorea
Sydenham’s chorea
44
What are tics
Tics are typically relatively brief rapid intermittent stereotyped involuntary movements or sounds
45
What is the most common syndrome characterised by tics
Gilles de la Tourette syndrome
46
What is myoclonus
Defined as a sudden brief shock(like involuntary movement caused by muscular contraction or inhibition
47
What is Hemiballism
Wild flinging/throwing movements of one arm or leg
48
Why does hemiballism occur
cerebrovascular lesion to the subthalamic nucleus
49
What is a seizure
A short episode of symptoms caused by a burst of abnormal electrical activity in the brain
50
What is epilepsy
The ongoing liability to recurrent seizures
51
What should you always ask patients about in an OSCE if they have presented with a seizure
Preceding Aura
52
How would a focal seizure in the occipital lobe present
Visual phenomena such as flashing lights, colours or hallucinations
53
How would a focal seizure in the parietal lobe present
Sensory manifestations can take the form of tingling or numbness or pain.
54
How would a focal seizure in the frontal lobe present
Motor signs such as stiffness, twitching or spasm
55
How would a focal seizure in the temporal lobe present
Changes in mood or behaviour
56
A rising epigastric sensation is a common manifestation in which type of focal seizure
Temporal lobe focal seizure
57
What is the difference between a simple and a complex focal seizure
Simple = no loss of consciousness
58
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
59
Do people fall during an absence seizure
Not usually since muscle tone is preserved
60
What is a characteristic sign of an absence seizure on EEG
3Hz generalised spike-wave
61
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
62
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
63
What is a tonic-clonic seizure
Tonic phase - muscles become stiff | Clonic phase - muscles will start to contract and relax rapidly
64
How is epilepsy typically diagnosed
Generally by clinical history
65
Why is EEG useful in epilepsy
To help support a diagnosis of epilepsy and determine a seizure type
66
Name 2 first-line drugs for focal seizures
Carbamazepine | Lamotrigine
67
Name 2 first-line drugs for generalised tonic-clonic seizures
Sodium Valproate | Lamotrigine
68
Name 2 first-line drugs for generalised absence seizures
Sodium Valproate | Ethosuximide
69
Name a drug used first-line for myoclonic generalised seizures
Sodium Valproate
70
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
71
2nd line drug for generalised myoclonic seizures
Levetiracetam
72
Name 2 second-line drugs for generalised tonic-clonic seizures
Lamotrigine | Levetiracetam
73
Which drug should you never give for generalised seizures and why
Carbamazepine | Can make them worse
74
How does Carbamazepine work
Na+ channel inactivation
75
Which anti-convulsants work by inactivating Na+ channels
Carbamazepine | Phenytoin
76
How does Lamotrigine work
Blocks voltage-gated Na+ channels
77
How does Ethosuximide work and when can it be used
Blocks thalamic T- type Ca2+ channels | Used 1st line for absence seizures
78
Which anticonvulsant is teratogenic
Sodium Valproate
79
How does Sodium Valproate work
Na+ channel inactivation with increase GABA concentration
80
Which anti-convulsant can cause Steven-Johnson's syndrome
Lamotrigine
81
What is status epilepticus
Status epilepticus is defined as a convulsive seizure which continues for a prolonged period (longer than five minutes)
82
How would you manage status epilepticus
community - rectal diazepam | hospital - IV lorazepam
83
What is Motor Neurone Disease
A group of neurodegenerative conditions which affect only the upper and/or lower motor neurones
84
What is the most common MND type
Amyotrophic Lateral Sclerosis (ALS)
85
Which chromosome has genes which have been linked to MND
Chromosome 21
86
Which type of dementia is MND often linked to
Frontotemporal dementia (FTD)
87
What is the only drug given to those with MND and how does it affect prognosis
Riluzole | Extends life expectancy by 3 months
88
How does Riluzole work
Blocks glutaminergic neurotransmission in the CNS
89
Name 3 complications of MND
Depression Weight loss + malnutrition (2ndary to dysphagia) Respiratory depression
90
Name an autosomal recessive muscle disease
McArdle's disease
91
What is deficient in McArdle's disease
Myophosphorylase
92
What is the mode of inheritance of myotonic dystrophy
Autosomal dominant
93
What is split hand syndrome and which disease is it linked to
Thenar muscles atrophy but hypothenar muscles are spared | Seen in ALS
94
A tri-nucleotide repeat on chromosome 19 should suggest which disease
Myotonic Dystrophy
95
Name 3 characteristic signs of myotonic dystrophy
'Christmas tree-like' bilateral cataracts Early frontal balding Sternocleidomastoid and distal muscle wasting
96
In Lambert-Eaton Myasthenic syndrome, what structure are autoantibodies created against
Ca2+ voltage-gated channels at the pre-synaptic terminal
97
How is LEMS diagnosed
Presence of anti(VGCC antibodies and by characteristic electrophysiological findings using a 20–50 Hz repetitive stimulation
98
Which imaging scan must be carried out in suspected LEMS and why
CT scan to rule out small cell lung cancer
99
How is LEMS usually treated
3- 4 diaminopyridine (Amifampridine) | blocks presynaptic Ca2+ channels
100
Which part of the NMJ does Myasthenia gravis affect
post-synaptic nicotinic acetylcholine receptors
101
What is a common presentation of myasthenia gravis
Muscle fatiguability | usually of extra-ocular muscles
102
Are proximal or distal muscles usually affected in myasthenia gravis
Proximal
103
What is a Myasthenia crisis
weakness of the muscles of respiration causes ventilator failure
104
What is the treatment for myasthenia crisis
plasmapheresis IV immunoglobulins systemic steroids
105
What is a cholinergic crisis and what kind of people is it common in
common in patient receiving high dose anticholinesterase medication
106
What kind of hyperplasia is Myasthenia Gravis
Thymus hyperplasia | thymoma
107
Which connective tissue disease is associated with Myasthenia Gravis
SLE
108
Name 4 drugs that can exacerbate myasthenia gravis
Gentamicin Lithium Beta-blockers Verapamil
109
Which antibody can be tested for in Myasthenia Gravis
IgG antibodies against acetylcholine receptor (anti-AChR)
110
How is myasthenia gravis usually treated
Acetylcholinesterase inhibition using Pyridostigmine
111
Which bacteria infection can precede Guillan-Barre syndrome
Campylobacter
112
How is polio spread
Facael-oral transmission
113
What does a poliomyelitis infection cause destruction of
Destruction of cells in anterior horn of spinal cord (LMN death)
114
What are the 2 broad categories of pain
Nociceptive pain | Neuropathic pain
115
What is allodynia
pain produced in response to a stimulus that is normally not painful
116
What is hyperalgesia
increase in pain response, with lowering of the pain threshold, to a stimulus that is normally painful
117
What is the leading cause of dementia
Alzheimer's disease
118
Which syndrome increases your risk of developing Alzheimer's
Down syndrome
119
Which version of the ApoE gene increases and decreases risk for Alzheimer's
ApoE4 - increases | ApoE2 - decreases
120
What happens to the gyri and sulci in Alzheimer's
Narrowing of gyri | Widening of sulci
121
What are the plaques and neurofibrillary tangles made up of in Alzheimer's
B-amyloid plaques | Tau tangles
122
Which source of cholinergic projections to the brain is often first affected in Alzheimer's
nucleus basalis of Meynert
123
What change is seen in CSF biomarkers in Alzheimer's disease
increase in phosphorylated tau protein and decrease in β-amyloid
124
What class of drugs can be given for Alzheimer's disease
Acetylcholinesterase inhibitors | rivastigmine or galantamine
125
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
126
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)
127
What are the 3 broad variants of frontotemporal dementia
Behavioural Primary progressive aphasia (semantic and progressive non-fluent aphasia)
128
Which scan is particularly useful for showing the changes of Frontotemporal dementia
SPECT scan
129
What is the second most common form of dementia
Vascular dementia
130
What will an MRI or SPECT scan show in vascular dementia
Infarcts and white matter changes
131
What is Creutzfeldt–Jakob disease
A transmissible prion neurodegenerative disease which causes cell death (spongiform changes in the cortex) and astrocytosis
132
How does Creutzfeldt–Jakob disease present and progress
Rapidly progressing dementia, ataxia and myoclonic jerks
133
What would CSF analysis show in a patient with Creutzfeldt–Jakob disease
Elevated 14-3-3 protein
134
What is multiple sclerosis characterised by
Demyelination of white matter areas in the brain
135
What is the most common phenotype of MS
Relapsing-Remitting
136
Areas of demyelination are referred to as what in MS
Plaques
137
Name 3 common areas of demyelination
Optic nerve Corpus callosum Periventricular white matter
138
Name 3 risk factors for MS
Those living in a higher latitude Female Previous EBV infection
139
Which HLA gene is linked to MS
HLA DRB1
140
What is the most ocular symptom of MS
Optic Neuritis
141
What is Lhermitte’s sign
Electric shock-like sensation down the upper limbs and | trunk on neck flexion
142
WHat is Uhthoff’s phenomenon and which disease is it seen in
Exacerbation of current symptoms in hot environments seen in MS
143
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
144
What will an MRI of a brain affected by MS show
Multiple white matter plaques
145
What will CSF analysis of a patient with MS show
Presence of IgG Oligoclonal bands
146
What are 3 options for 1st line therapy in terms of disease-modifying therapy for MS
Interferon-beta Tecfidera Glatiramer acetate
147
How is spasticity managed in MS
Baclofen
148
How is neuropathic pain managed in MS
Gabapentin
149
How is fatigue managed in MS
Amantadine + modafinil
150
How is bladder dysfunction managed in MS
Anti-Cholinergic drugs (Oxybutynin)
151
Name 5 red flags for a headache
``` Age >55 Immunosuppressed Previous/current malignancy Early morning headache Exacerbated by Valsalva manoeuvre ```
152
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
153
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
154
Name 4 trigeminal autonomic cephalgias
Cluster Paroxysmal hemicrania SUNCT Trigeminal Neuralgia
155
Which of the trigeminal autonomic cephalgias has an absolute response to Indomethacin
Paroxysmal Hemicrania
156
What does SUNCT stand for
``` Short Unilateral Neuralgiform headache with Conjunctival injection Tearing ```
157
Name 2 treatments for SUNCT
Lamotrigine | Gabapentin
158
How is trigeminal neuralgia treated
Carbamazepine
159
Name 3 vascular disorders which can present as a headache
Subarachnoid haemorrhage Carotid dissection Giant cell arteritis
160
What is Pseudotumor cerebri
idiopathic intracranial hypertension
161
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
162
What are clinical presenting features of idiopathic intracranial hypertension
A throbbing headache worse in the morning Bilateral papilloedema. Nausea and vomiting.
163
Which nerve palsy can be a presentation of idiopathic intracranial hypertension
Sixth nerve palsy
164
Which investigations can be carried out for idiopathic intracranial hypertension
MRI or CT
165
What will be seen upon execution of a lumbar puncture in a patient with idiopathic intracranial hypertension
High opening pressure
166
What will idiopathic intracranial hypertension show on MRI/CT
Slit-like ventricles
167
What does meningism refer to
Triad of symptoms: Photophobia Neck stiffness A headache
168
Where does infection usually spread from to cause meningitis
Sinuses Nasopharynx Ear
169
Which organism affects mainly neonates and elderly, with regards to meningitis
Listeria Monocytogenes
170
Which organism is most commonly the cause of Meningitis in infants + children/adolescents
N.Meningitidis
171
Which organism is most commonly the cause of Meningitis in Adults
Strep. Pneumoniae
172
Which organism is most commonly the cause of Meningitis in those with recent neurosurgery or head trauma
Staph Aureus
173
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
174
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) ```
175
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) ```
176
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
177
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
178
``` 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
179
What is the empirical treatment for community-acquired meningitis
IV Ceftriaxone + Dexamethasone
180
When would amoxicillin be given alongside Ceftriaxone for meningitis treatment
If Listeria is suspected
181
When should you suspect Listeria to be the causative organism in meningitis
Neonate or >55 or Immunosuppressed
182
What is the prophylactic treatment given to those who have been in contact with someone with meningitis
Ciprofloxacin | Rifampicin if refractory to treatment
183
``` 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
184
Name 3 characteristic CSF findings in Tuberculosis meningitis
Clear and culture negative Lymphocytosis Fibrin webs
185
What is Inflammation of the brain parenchyma known as
Encephalitis
186
What is the most common cause of encephalitis
Herpes simplex
187
A patient with seizures with focal neurological deficits (aphasia) should make you think of what
Herpes simplex encephalitis
188
Parotitis in an unvaccinated patient should make you think of which type of encephalitis
Mumps encephalitis
189
Skin lesions\vesicles in a dermatomal pattern should make you think of which type of encephalitis
Varicella-zoster encephalitis
190
MRI in HSV encephalitis will show what
Characteristic oedema in temporal lobes
191
What is the treatment for encephalitis
IV acyclovir
192
What is a serious consequence of the measles virus involving the brain parenchyma
Subacute sclerosing panencephalitis
193
Describe CSF findings in a Subarachnoid haemorrhage
Xanthochromia
194
Describe CSF findings in Narcolepsy
Low or undetectable levels of CSF orexin/hypocretin
195
Describe CSF findings in Alzheimer's
High hyper-phosphorylated Tau protein | Low B-amyloid
196
Describe CSF findings in Multiple sclerosis
IgG oligoclonal bands