Neurology Flashcards
1
Q
What is a stroke?
A
A cerebrovascular accident - hypoperfusion to the brain causing ischaemia and infarction of brain tissue.
2
Q
What causes a stroke?
A
Ischaemic (clots) or haemorrhagic (bleeds).
3
Q
What is a transient ischaemic attack?
A
Sudden onset transient neurological dysfunction secondary to ischaemia without infarction which lasts under 24 hours (usually 5-15 minutes).
4
Q
Why is a TIA significant?
A
Often precedes a stroke.
5
Q
What is a crescendo TIA?
A
Two or more TIAs in one week which makes it high risk of a stroke.
6
Q
How is a TIA and stroke differentiated?
A
After recovery:
-TIA - Symptoms resolve in minutes, always less than 24h and no infarct.
-Stroke - Symptoms last at least 24h with infarction.
7
Q
What causes a TIA?
A
Thrombo-emboli in the carotid artery/major brain vessel.
8
Q
What are the risk factors for stroke?
A
Same as CVD:
Smoking, obesity, T2DM, hypertension, AF, hypercholesterolaemia, previous TIA.
9
Q
How is a stroke diagnosed?
A
Identifying a stroke - FAST (face, arms, speech, time).
-Non-contrast head CT/diffusion weighted MRI:
TIA/ischaemic - Mostly normal.
Haemorrhagic - Hyperdense blood.
10
Q
How is a TIA managed?
A
Start daily aspirin and secondary prevention with statins and clopidogrel.
11
Q
How is a haemorrhagic stroke managed?
A
Referral to neurosurgery for evacuation of blood.
IV mannitol to reduce ICP.
12
Q
How is an ischaemic stroke managed?
A
Within 4h - thrombolysis with alteplase.
If large - thrombectomy.
13
Q
What is the secondary prevention of stroke?
A
Modify risk factors and start stroke rehab.
-Daily clopidogrel and statin.
14
Q
What percentage of strokes are ischaemic?
A
85%.
15
Q
What percentage of strokes are haemorrhagic?
A
15%.
16
Q
What are six risk factors for intercranial bleeds?
A
Head trauma, hypertension, aneurysms, brain tumours, anticoagulants, connective tissue disorders.
17
Q
How do intercranial bleeds present?
A
Sudden onset headache with seizures, vomiting, reduced GCS and other sudden onset neurological symptoms.
-Signs of raised ICP:
Cushing triad - bradycardia, increased PP, irregular breathing.
18
Q
What is amaurosis fugax?
A
Transient loss of vision due to decreased blood flow to retina (retinal/ophthalmic artery occlusion).
19
Q
How are intercranial bleeds diagnosed?
A
CT/MRI of head and referral to neurosurgery for removal.
20
Q
What is the Glasgow coma scale (GCS)?
A
An assessment tool for assessing the level of consciousness based on eyes, verbal and motor response.
21
Q
Explain the GCS.
A
-Eye opening response ranked 1-4 (none, to pain, to speech, spontaneous).
-Speech response ranked 1-5 (none, sounds, bad words, confused conversation, orientated).
-Motor response ranked 1-6 (none, extends, abnormal flexion, flexion, localises pain, obeys).
22
Q
What are the main four locations of intercranial bleeds?
A
Subarachnoid, subdural, extradural and intercranial.
23
Q
Explain an intracerebral haemorrhage.
A
Bleeding into brain tissue, can be located anywhere in brain tissue.
24
Q
What is a subarachnoid haemorrhage?
A
Bleeding into subarachnoid space where CSF is located, between pia and arachnoid matter.
25
What is the main cause of subarachnoid haemorrhages?
Ruptured cerebral aneurysms.
26
How does a subarachnoid haemorrhage present?
Thunderclap headache.
27
What is a subdural haemorrhage?
Bleeds between the dura and arachnoid matter.
28
What causes a subdural haemorrhage?
Rupture of bridging veins of outer meningeal layers.
29
How does a subdural haemorrhage appear on CT scan?
Crescent/banana shaped not limited by cranial structures.
30
What is an extradural haemorrhage?
Bleed between the skull and dura.
31
What causes an extradural haemorrhage?
Rupture of middle meningeal artery.
32
How does an extradural haemorrhage appear on CT scan?
Bi-convex shaped limited by cranial strictures.
33
What are the main two CNS infections?
Meningitis and encephalitis.
34
What is meningitis?
Inflammation of the meninges which line the brain and spinal cord.
35
What causes meningitis?
Viral or bacterial infection.
36
What is meningococcal meningitis?
When bacteria are infecting meninges and CSF around the brain and spinal cord.
37
What is meningococcal septicaemia?
When the the meningococcal bacterial infection is in the bloodstream. This is the cause of the non-blanching rash caused by DIC.
38
What is the most common cause of meningitis?
Viral is more common than bacterial.
39
Explain viral meningitis and it's causes.
More common and less severe.
-Mostly caused by HSV, VZV and enteroviruses (coxsackie).
40
What is the management for viral meningitis?
Usually only supportive. If confirmed HSV meningitis - aciclovir.
41
What are the most common causes of bacterial meningitis in adults?
N. meningitidis and S. pneumoniae.
42
What is the most common cause of bacterial meningitis in neonates? Why?
Group B strep - lives harmlessly on vagina.
43
What is a cause of bacterial meningitis in the very young and very old?
Listeria.
44
Describe N. meningitidis.
Gram negative diplococcus.
-Vaccines available - MenB/C, ACWY.
45
Describe S. pneumoniae.
Gram positive diplococcus in chains.
-Vaccine available - PCV.
46
What are the four risk factors for meningitis?
Extremes of ages, immunocompromised, crowded environment, not vaccinated.
47
What are seven main symptoms of meningitis?
Fever, neck stiffness, photophobia, vomiting, headache, altered consciousness and seizures.
48
How does meningococcal septicaemia present?
Non-blanching rash.
49
How does meningitis present in neonates?
Non-specific: fever, poor feeding and lethargy.
-Lumbar puncture in neonates with fever and lethargy.
50
What are two tests to test for meningeal irritation?
Kernig's test - Can't extend knee when the hip is flexed without pain due to meninges stretching and being irritated.
Brudzinski's test - When the neck is flexed, the knees and hips involuntarily flex.
51
How is meningitis investigated?
Lumbar puncture at L4 and CSF analysis.
-CSF sample also sent for viral PCR testing.
52
What are CSF results in viral meningitis?
Clear, normal or raised protein, normal glucose, high WCC (lymphocytes) and negative bacterial culture.
53
What are the CSF results in bacterial meningitis?
Cloudy, high protein, low glucose, high WCC (neutrophils) and positive bacterial culture.
54
How is bacterial meningitis and meningococcal septicaemia treated in the community?
IM benzylpenicillin and hospital referral.
55
How is bacterial meningitis and meningococcal septicaemia treated in hospital for neonates aged 1-3 months?
IM cefotaxime and amoxicllin.
56
How is bacterial meningitis and meningococcal septicaemia treated in hospital for people aged above 3 months?
Ceftriaxone.
57
How is bacterial meningitis and meningococcal septicaemia treated in hospital if there's a risk of MRSA?
Vancomycin.
58
What is the supportive treatment in hospital for bacterial meningitis?
Dexamethasone - reduces the frequency of hearing loss and neurological deficit.
59
What are five complications of meningitis?
Hearing loss, seizures/epilepsy, cognitive impairment/memory loss, focal neuro symptoms.
60
What is the post exposure prophylaxis treatment to meningitis?
Single dose of ciprofloxacin.
61
What is encephalitis?
Inflammation of the brain.
62
What are the causes of encephalitis?
Infective and non infective.
63
What are the infective causes of encephalitis?
Viral, bacterial is very rare in the UK.
-Mostly HSV-1.
-Others include HSV-2, CMV, EBV, VZV and HIV.
64
What are the two risk factors for encephalitis?
Extremes of age, immunocompromised.
65
How does encephalitis present?
With fever, headache, altered consciousness, altered cognition and unusual behaviour.
-Acute onset focal neuro symptoms and acute inset focal seizures.
66
How is encephalitis diagnosed?
Lumbar puncture and send CSF off for viral PCR testing.
-CT/MRI.
67
How is encephalitis treated?
IV antivirals according to cause:
-Aciclovir for HSV and VZV.
-Ganciclovir for CMV.
68
What are the complications of encephalitis?
Fatigue, personality changes, cognitive changes, learning disability, headaches, movement disorders, seizures.
69
What is multiple sclerosis?
An autoimmune, chronic and progressive condition that involved demyelination of the myelinated neurones of the CNS.
70
When does MS usually present and who is it most common in?
Usually presents in young adults under 50 and is more common in women.
71
When do symptoms improve in MS?
When pregnant or in postpartum period.
72
What are the five main causes of MS?
EBV, genes, low vitamin D, smoking and obesity.
73
What are seven risk factors for MS?
Females, 20-40y, autoimmune disease, family history, EBV, smoking, obesity.
74
What is the key term in MS used to describe the way MS lesions change over time in different locations?
Disseminated in time and space.
75
What are the different disease patterns in MS?
-Relapse-remitting (mc) - episodes of disease followed by recovery.
-Primary progressive - Gradual deterioration without recovery,
-Secondary progressive - Relapse-remitting but now primary progressive.
76
What is usually the first presenting symptom in MS?
Optic neuritis - loss of vision in one eye due to myelinated and inflamed optic nerve.
77
How does MS present?
Paresthesia, blurred vision, numbness with Lhermitte's sign (when neck is flexed - electric shock sensation) that is worsened by heat.
-Bell's palsy, limb weakness, incontinence.
-6th CN lesion - internuclear ophthalmoplegia and conjugated lateral gaze disorder.
78
What are the two main investigations for MS?
MRI of brain and spinal cord showing lesions.
Lumbar puncture which shows oligoclonal bands in CSF.
79
Which criteria has to be satisified for a diagnosis of MS?
McDonald criteria - 2+ attacks, separate events that affect different parts of the CNS (disseminated in time and space).
80
How are acute relapses of MS treated?
IV methylprednisolone.
81
How is MS prophylactically treated to modify the disease?
DMARDs and biologics.
82
What is Guillian-Barre syndrome?
An acute paralytic polyneuropathy that affects the peripheral nervous system.
83
Who does Guillian-Barre syndrome affect?
Males aged 15-30 and 50-70.
84
What causes Guillian-Barre syndrome?
Triggered by an infection:
-C. jejuni (mc), CMV and EBV.
85
Describe the pathophysiology of Guillian-Barre syndrome.
Molecular mimicry - Plasma cells make antibodies against antigens on the pathogen of preceding infection. These antibodies also match the proteins on Schwann cells and attack those.
-Causing demyelination of PNS and polyneuropathy.
86
Describe the clinical course of Guillian-Barre syndrome.
4 weeks before - Gastroenteritis/infection.
Symptoms start.
2-4 weeks - Peak symptoms.
Months-years - Recovery period.
87
Describe the prognosis of Guillian-Barre syndrome.
80% fully recover.
15% left with neurological disability.
5% will die.
88
How does Guillian Barre syndrome present?
Symmetrical ascending weakness and paralysis (feet up).
Reduced reflexes, peripheral loss of sensation.
Respiratory failure in 35%.
89
How is Guillian-Barre syndrome diagnosed?
Nerve conduction studies.
Lumbar puncture - raised protein, normal WCC and glucose (inflammation with no infection).
90
What is the treatment for Guillian-Barre syndrome?
IV Ig for 5 days or plasma exchange.
Supportive care and VTE prophylaxis (PE is a cause of death).
In respiratory failure - intubation, ventilation and ICU admission.
91
What is Parkinson's disease?
Progressive reduction of dopamine in the basal ganglia of the brain which leads to movement disorders which causes problems like shaking and stiffness that get worse over time.
92
Describe the pathophysiology of Parkinson's disease.
Loss of dopaminergic neurones from substantia nigra.
-Presence of Lewy bodies.
93
What are the two most common neurodegenerative disorders?
1. Dementia.
2. Parkinson's.
94
What is the typical Parkinson's patient?
Old man around 70y.
95
What are three risk factors for Parkinson's disease?
Family history, males, higher age.
96
What are the main triad of symptoms in Parkinson's disease?
Resting tremor, rigidity, bradykinesia.
-Usually unilateral.
97
Describe the presenting symptoms of Parkinson's disease.
-Unilateral tremor - pill rolling tremor 4-6 times a second.
-Cogwheel rigidity.
-Bradykinesia - movements get slower and smaller.
98
Describe the bradykinesia in Parkinson's disease.
Slowness of movement:
-Smaller handwriting, shuffling gait, difficulty initiating movement, difficulty turning when stood up, reduced facial movement/expression.
99
What are five other symptoms of Parkinson's disease?
Insomnia, anosmia, postural instability, depression, cognitive impairment.
100
How is Parkinson's diagnosed?
Diagnosed clinically based on presentation and examination - bradykinesia, tremor, rigidity.
101
What is the treatment for Parkinson's disease?
Levodopa and decarboxylase inhibitors (carbidopa) to stop levodopa from being broken down.
-Others:
Monoamine oxidase B inhibitors.
Dopamine agonists (cabergoline/bromocriptine).
102
How successful is the treatment for Parkinson's disease?
Works very well initially but the body soon becomes resistant.
103
What is a key differential diagnosis for Parkinson's disease?
Benign essential tremor.
104
What is benign essential tremor?
Common tremor associated with older age. Mostly in the hands.
105
Compare and contrast benign essential tremor and Parkinson's.
Parkinson's - Asymmetrical.
BET - Symmetrical.
Parkinson's - 4-6Hz.
BET - 5-8Hz.
Parkinson's - Worse at rest.
BET - Improves with rest.
Parkinson's - Improves with intentional movement.
BET - Worse with intentional movement.
Parkinson's - Other Parkinson's features.
BET - No other Parkinson's features.
Parkinson's - No change with alcohol.
BET - Improves with alcohol.
106
What is Huntington's disease?
An autosomal dominant genetic condition with full penetrance that causes progressive deterioration within the nervous system.
107
What is Huntington's disease caused by?
Trinucleotide repeat disorder in HTT gene on chromosome 4.
108
What does Huntington's disease display?
Anticipation - successive generations have more repeats in the gene which leads to earlier age of onset and increased severity of the disease.
109
What is the life expectancy of Huntington's disease?
15-20 years after symptoms start.
110
When does Huntington's disease usually begin to present?
Asymptomatic until ages 30-50.
111
Describe the number of trinucleotide repeats and their effects in Huntington's.
<35 - No Huntington's.
35-55 - Huntington's.
>60 - Severe Huntington's.
112
What causes symptoms in Huntington's disease? (pathophysiology).
1. Caudate nucleus atrophies.
2. Inhibitory neurones in the corpus striatum degenerate.
3. Levels of GABA decrease.
4. Excessive nigrostriatal pathway.
113
How does Huntington's disease present?
Chorea - excessive limb jerking.
Dementia, psychiatric issues, depression.
Eye movement disorders, dysarthria, dysphagia.
114
How is Huntington's disease diagnosed?
Family history of earlier and more severe Huntington's.
Genetic test - 35 CAG repeats on chromosome 4.
115
What is the management of Huntington's disease?
Extensive counselling and breaking bad news.
Speech and language therapy.
End of life care planning.
-Medications:
Antipsychotics, benzodiazepines, dopamine antagonists.
-SSRIs for depression.
116
What is dementia?
Irreversible, progressive decline and impairment of higher brain function (memory, judgement, language, personality).
117
Does dementia occur with consciousness changes?
No, occurs without impairment of consciousness.
118
What are the main four types of dementia and how prevalent are they?
Alzheimer's - 60%.
Vascular - 20%.
Lewy body - 10%.
Frontotemporal - 5%.
119
What is the biggest risk factor for dementia?
Increasing age.
120
Describe Alzheimer's disease and it's pathophysiology.
Most common cause of dementia due to beta amyloid plaques due to breakdown of PPA and tall neurofibrillary tangles (tau proteins) in cerebral cortex.
-This results in the death of brain cells.
121
What is the pattern of cognitive decline in dementia?
Steady decline in cognitive function.
122
What are two risk factors for Alzheimer's disease?
Down's syndrome, ApoE4 mutation.
123
Describe the presentation of Alzheimer's.
Usually after 60, affects all of brain.
Main presenting symptom is memory loss.
-Agnosia, apraxia and aphasia.
124
What is vascular dementia? Who is it more common in?
Dementia due to cerebrovascular damage (stroke, TIA).
More common in men with previous stroke.
125
What is the pattern of cognitive decline in people with vascular dementia?
Stepwise fashion.
126
How does vascular dementia present?
Cognitive impairment with mood disturbances and psychiatric issues.
127
What is Lewy body dementia?
Dementia due to spherical Lewy body proteins deposited in the brain.
128
Describe the link between Lewy body dementia and Parkinson's disease.
Lewy body proteins are present in both, they are more widespread in Lewy body dementia and concentrated in the substantia nigra in Parkinson's.
-Parkinson's before - Parkinson dementia.
-LBD before - Lewy body dementia with Parkinsonism.
129
Describe the presentation of Lewy body dementia.
Often presents with visual hallucinations, Parkinson's symptoms and cognitive decline.
130
What is Frontotemporal dementia?
Neuron damage and death in the frontal and temporal lobes due to deposition of tau proteins.
131
How does Frontotemporal dementia present?
Speech and language - temporal.
Thinking and memory - frontal.
132
How is dementia diagnosed?
MMSE and brain MRI.
133
Describe the MMSE.
Tool to assess mental status and screen for cognitive impairment.
-Out of 30:
>25 is normal.
18-25 is impaired.
<17 is severely impaired.
134
What is the conservative management of dementia?
Social stimulation and exercise.
135
What is the treatment for Alzheimer's disease?
Acetylcholinesterase inhibitor - donepezil.
136
What is the treatment for vascular dementia?
Antihypertensives - ACEi (ramipril).
137
How can headaches be divided?
Into primary and secondary.
138
What are primary headaches?
Those with no pathology - more common.
139
What are secondary headaches?
Secondary to pathology.
140
What are the four most common types of primary headaches?
Tension, migraine, cluster, drug induced (medication overuse).
141
What are six causes of secondary headaches?
GCA, infection, subarachnoid haemorrhage, cerebrovascular disease, eye/ear/sinus pathology.
142
What are seven red flags for headaches?
Fever, photophobia, neck stiffness, new neurological symptoms, dizziness, visual disturbance and vomiting.
143
What are tension headaches?
Most common primary headache with a mild ache across the forehead due to muscle aches.
144
What are tension headaches associated and not associated with?
Associated with stress, depression, alcohol, hunger and dehydration.
NOT associated with visual changes or N+V.
145
What is the treatment of tension headaches?
Simple analgesia (aspirin and paracetamol).
146
Describe the site, character and severity of tension headaches.
Site - Bilateral.
Character - Pressing/tightening.
Severity - Mild/moderate, non disabling.
147
What are cluster headaches?
A rare, disabling unilateral, excruciating stabbing pain headache.
148
How long do each type of primary headache usually last?
Tension - 30m to 7 days.
Cluster - 15m to 3 hours.
Migraine - 4h to 3 days.
149
What are migraines?
Complex neurological condition that causes headaches and associated symptoms. They occur in attacks that follow a typical pattern.
150
What are the types of migraine?
-With aura (visual phenomena - sparks, blurred, lines, blind spots).
-Without aura.
-Silent migraine (with aura, without headache).
-Hemiplegic migraine.
151
What are the triggers of migraines?
CHOCOLATE:
-Chocolate, hangovers, orgasms, cheese, oral contraceptives, lie ins, alcohol, tumult (loud noise), exercise.
152
Explain the five stages of migraine.
Prodromal - mood changes, days before.
Aura - visual changes, minutes before.
Headache - throbbing/pounding up to 3 days.
Resolution - headache fades or relieved by vomiting.
Recovery - headache is gone.
153
What is a hemiplegic migraine?
Mimic strokes - migraines with unilateral weakness.
154
How do migraines typically present?
Unilateral pounding/throbbing headache at moderate to severe intensity.
-Can be bilateral.
-Photophobia, phonophobia, N+V.
155
How are migraines diagnosed?
Clinical unless other pathology is suspected.
-Normal neuro exam.
156
What are the acute management of migraines?
Paracetamol, aspirin, NSAIDs, triptans.
Triptans (5HT agonists) abort migraines when they start.
If N+V - Use anti-emetics such as metoclopramide.
157
What is migraine prophylaxis?
Propranolol, topiramate, amitriptyline.
158
Describe the prognosis of migraines.
They tend to get better over time and people go into remission.
159
What are four indicators of secondary headaches?
Thunderclap headaches, focal neurological deficit, associated systemic features, patients over 50.
160
What is epilepsy? What is the criteria?
Idiopathic cause of seizures.
2 or more episodes more than 24h apart.
161
What are two risk factors for epilepsy?
Familial inherited and dementia.
162
What are seizures?
Transient episodes of abnormal electrical activity in the brain.
163
What are the causes of seizures?
VITAMINDE:
Vascular, infection, trauma, autoimmune, metabolic, idiopathic, neoplasms, dementia/drugs, eclampsia.
164
Describe the pathophysiology of seizures.
The normal balance between GABA and glutamate shifts towards glutamate.
-More excitatory with increased glutamate stimulation and increased GABA inhibition.
165
Describe the stages of epileptic seizures.
Usually last less than 2 minutes:
-Prodrome - Mood changes, days before.
-Aura - Deja vu and automatisms minutes before.
-Ictal seizure.
-Post-ictal period - Confusion, headache, decreased GCS, dysphasia, sore tongue and Todd's paralysis.
166
What are the nine types of seizures?
Tonic clonic, absence, tonic, clonic, myoclonic, atonic, simple focal, complex focal, status epilepticus.
167
Describe tonic-clonic seizures.
Grand mal - tonic (rigidity and fall) and clonic (jerking of limbs).
168
Describe absence seizures.
Petit mal - childhood seizures of staring blankly into space. Last up to 15s.
169
Describe myoclonic seizures.
Jerking of limbs, only last a few seconds.
170
Describe clonic seizures and tonic seizures.
Clonic - Shaking and jerking of limbs (may lose consciousness).
Tonic - Muscles become stiff (may fall).
171
Describe atonic seizures.
All muscles relax and you may fall to the ground.
172
Describe simple focal seizures.
Remain awake and aware - strange feeling, unusual tastes and smells, tingling, intense emotion, stiffness/twitching in part of body.
173
Describe complex focal seizures.
Random body movements - lose awareness and have no memory of it.
-Smacking lips, rubbing hands, noises, moving arms, chewing/swallowing.
174
Describe status epilepticus.
Any seizure that lasts for more than 5m, or a seizure back to back or 3 seizures in one hour.
175
What are the investigations of epilepsy/seizures?
EEG to show electrical patterns.
MRI to show underlying pathology of brain.
ECG to exclude heart problems.
176
What is the management of epilepsy?
Sodium valproate - most forms.
Carbamazepine - focal seizures.
Status epilepticus - benzodiazepines.
177
What is motor neurone disease?
Umbrella term that describes neurodegenerative disorders progressively affecting motor neurones but not sensory neurones.
178
What is the most common type of MND?
Amyotrophic lateral sclerosis (ALS).
179
What are three less common types of MND?
Progressive bulbar palsy, primary lateral sclerosis, progressive muscular atrophy.
180
Describe the pathophysiology of MND.
Affects both UMN and LMN causing both UMN and LMN signs.
-The sensory neurones are spared.
181
What are five risk factors for MND?
Family history, male, smoking, heavy metals, pesticides.
182
What is a typical MND patient?
Older man with affected relative, with mixed UMN and LMN signs.
-No eye signs or sensory signs.
183
What are UMN signs in MND?
Increased muscle tone and spasticity, fast reflexes, upgoing plantar responses (Babinski sign).
184
What are LMN signs in MND?
Muscle wasting, reduced tone, twitching, reduced reflexes.
185
How is MND diagnosed?
Clinical - excluding other conditions.
186
What is the management of MND?
No treatments to stop or reverse disease.
-Riluzole slows progression of the disease.
-Break bad news, end of life care.
187
What are three complications of MND?
Respiratory failure, aspiration pneumonia, swallowing failure.
188
How do patients with MND usually die?
Of pneumonia or respiratory failure.
189
What is myasthenia gravis?
Autoimmune condition against neuromuscular junction post synaptic receptors.
190
Who is affected by myasthenia gravis?
Affects males and females equally bur at different ages:
-Females at 40.
-Males at 60.
191
What is myasthenia gravis linked to?
Thymus gland cancer - thymoma.
-15% of myasthenia gravis have a thymoma.
-30% of patients with a thymoma develop myasthenia gravis.
192
Describe the pathophysiology of myasthenia gravis.
Autoantibodies:
85% Anti Ach-R - Bind to post synaptic receptor and inhibits. More binding during exercise so less effective stimulation of muscle with increased activity, improves at rest.
15% Anti MuSK - MuSK helps synthesise Ach-R resulting in decreased Ach-R expression of post-synaptic membrane.
193
How does myasthenia gravis present? What are 6 signs?
Muscle weakness that is worse with exertion and better with rest which starts at the the head/neck and progresses to lower body.
-Diplopia, ptosis, myasthenia snarl, fatiguability, swallowing difficulties, slurred speech.
194
What are the investigations for myasthenia gravis?
Serology - Anti Ach-R, anti MuSK.
CT/MRI thymus or thymectomy scar.
Edrophonium test - IV edrophonium test.
195
Explain the edrophonium test in myasthenia gravis.
Give IV edrophonium (reversible ACHase inhibitor).
-Normally ACHase in nmj breaks down Ach but edrophonium blocks these and stops the breakdown of Ach. Ach levels increase temporarily and relives the weakness.
This confirms a diagnosis of myasthenia gravis.
196
What is the management of myasthenia gravis?
1st line - ACHase inhibitors (neostigmine, pyridostigmine).
2nd line - Immunosuppression with steroids.
-Thymectomy.
197
What is a serious complication of myasthenia gravis?
Myasthenic gravis - acute symptoms worsening with severe respiratory weakness.
198
How is myasthenic crisis treated?
Plasma exchange or IV Ig.
Ventilation.
199
What is giant cell arteritis?
Large vessel vasculitis.
200
How does a CNI lesion present?
With anosmia (loss of smell).
201
How does a CNII lesion present?
Vision loss.
202
How does a CNIII lesion present?
Ptosis, down and out eye, fixed dilated pupil.
203
How does a CNIV lesion present?
Diplopia looking down.
204
How does a CNVI lesion present?
Adducted eye.
205
How does a CNV lesion present?
Jaw deviates towards affected side, loss of corneal reflex, trigeminal neuralgia.
206
How does a CNVII lesion present?
Facial drop with no forehead sparing.
207
What causes a CNVII lesion?
Bell's palsy, parotid inflammation.
208
How does a CNVIII lesion present and what causes it?
Hearing loss and loss of balance.
Due to skull changes and compression.
209
How do both CNIX and CNX lesions present?
Impaired gag reflex, swallowing, respiratory and vocal issues.
210
How does a CNXI lesion present?
Unable to shrug shoulders.
211
How does a CNXII lesion present?
Tongue deviation towards side of lesion.
212
What is peripheral neuropathy?
Nerve pathology of the peripheral nerves outside of the CNS.
213
What is carpal tunnel syndrome?
Pressure of the median nerve (C6-T1) passing through the carpal tunnel.
214
How does carpal tunnel syndrome present?
Gradual onset weakness of grip with an aching hand/forearm, paresthesia of hand and wasting of thenar eminence.
215
How is carpal tunnel syndrome investigated?
Phalen test - flexing fist for 1 minute which will cause paresthesia and pain.
Tinel test - tapping wrist causes tingling.
Durkan's test - Pressing on carpal tunnel for 30s, paresthesia and pain is positive.
216
What is the management of carpal tunnel syndrome?
1st line - Splint and pain relief.
2nd line - Corticosteroids.
3rd line - Surgical decompression.
217
Describe the epidemiology of carpal tunnel syndrome.
Affects females more than males, affects 5-10% of population.
218
What are six risk factors for carpal tunnel syndrome?
Pregnancy, hypothyroidism, RA, obesity, overuse of wrist (tennis, rowing), diabetes.
219
What is radial nerve palsy? How does it present?
C5-T1 - Presents with wrist drop.
220
What is ulnar nerve palsy? How does it present?
C8-T1 - Presents with claw hand.
221
What is sciatica?
Symptoms due to irritation of the sciatic nerve.
222
What causes sciatica?
L5/L1 lesion due to spinal causes (disc herniation/prolapse) or non-spinal (tumours, pregnancy).
223
How does sciatica present?
Pain from buttock down lateral leg to pinky toe.
224
How is sciatica investigated?
Can't raise leg straight without pain.
Sciatic stretch test - raise legs to point of discomfort, dorsiflex foot, pain = positive.
MRI of spinal cord to investigate intervertebral discs.
225
How is sciatica treated?
Analgesia and physiotherapy.
Neurosurgery for disc herniation/prolapse.
226
What are the main causes of peripheral neuropathy?
DAVID:
Diabetes, alcoholism, vitamin B12 deficiency, infective/inherited, drugs.
227
Where does the spinal cord run from?
C1-T1.
228
How does spinal cord compression present?
Spastic paraparesis, radicular pain at level of compression and sensory loss below level of compression.
229
What are four causes of spinal cord compression?
TB, degenerative disc and vertebral lesions, vertebral neoplasms.
230
What are the investigations and management of spinal cord compression?
Spinal cord MRI/CT.
-Neurosurgery.
231
What is cauda equina syndrome?
Compression below the conus medullaris (L1 - End of spinal cord).
232
What causes cauda equina syndrome?
Commonly lumbar disc herniation.
233
How does cauda equina syndrome present?
Leg weakness with LMN signs, saddle anaesthesia and bowel/bladder dysfunction.
234
What is Charcot-Marie-Tooth syndrome?
Usually an autosomal dominant inherited disease that affects the peripheral motor and sensory nerves, which cases dysfunction in myelin or axons.
235
How does Charcot-Marie-Tooth syndrome usually present?
Pes cavus (high arched feet), distal muscle wasting (inverted champagne bottle legs), weakness in lower legs (loss of ankle dorsiflexion).
Other - hand weakness, reduced tendon reflexes, reduced muscle tone, peripheral sensory loss.
236
What is shingles?
Reactivation of VZV virus after chickenpox. Peripheral nerves attacked via sensory dorsal root.
237
How does shingles present and how is it treated?
Painful rash confined to a dermatome.
Treated with oral aciclovir.
238
What is Duchenne muscular dystrophy?
X-linked recessive mutated dystrophin gene - affects only boys where muscles are replaced with adipose tissue.
239
What is Lambert Eaton syndrome?
Disease with similar features to myasthenia gravis - causes progressive muscle weakness with increased use as a result of damage to nmj.
240
Who does Lambert Eaton syndrome occur in?
Typically in those with SCLC - antibodies against calcium channels in SCLC which also target calcium channels in presynaptic terminals in nmj's - no Ach released.
241
Are primary or secondary brain tumours more common?
Secondary brain tumours are much more common than primary.
242
Which five cancers most commonly spread to the brain?
Lung (SCLC, NSCLC), breast, melanoma, kidney, gastric.
243
What are four types of primary brain tumour?
Astrocytoma, oligodendrocytoma, meningioma, schwannoma.
244
How are astrocytomas graded?
From 1-4.
1 being benign and 4 is glioblastoma.
245
How do brain tumours present?
Increased ICP, focal neurology, headache, seizures, weight loss and lethargy.
246
How are brain tumours investigated and managed?
MRI/CT of head.
-Surgery, chemo, radio and steroids.
247
What is syncope?
Term used to describe the event of temporarily losing consciousness due to disruption of blood flow to the brain often leading to a fall.
248
What are four causes of primary syncope?
Dehydration, hunger, extended standing in warm environment, vasovagal response to stimuli (blood, pain).
249
What are secondary causes of syncope?
Hypoglycaemia, dehydration, anaemia, infection, anaphylaxis, arrhythmias, heart disease.
250
How does syncope present?
Hot/clammy, sweaty, heavy, dizzy/lightheaded, vision going blurry/dark, headache.
251
What investigations are used for syncope?
ECG for arrhythmia (24h if paroxysmal suspected), ECHO for suspected structural heart disease.
Bloods - FBC, U+E and glucose (anaemia, arrhythmias, diabetes).
252
How is syncope managed?
Avoid triggers in primary, management of underlying pathology.
253
Which brain haemorrhagic events are strokes and which ones aren't?
Intracerebral haemorrhage and subarachnoid haemorrhage - STROKES.
Subdural and extradural haematomas - NOT STROKES.
254
How does an ischaemic stroke affecting the ACA present?
Contralateral weakness and sensory loss of lower limb.
255
How does an ischaemic stroke affecting the MCA present?
Contralateral motor weakness and sensory loss.
Speech issues due to Wernicke's and Broca's areas being affected.
256
How do different intercranial bleeds appear on CT scans?
Extradural - Bi-convex shape (lemon).
Subdural - Crescent shape (banana).
Subarachnoid - Star shaped lesion.
257
What are the three main types of seizures?
Primary generalised.
Partial focal.
Partial seizure with secondary generlisation.
258
Compare generalised and focal seizures.
Generalised - Bilateral, affects all brain.
Focal - Confined to one region.
259
How do focal temporal seizures present?
Dysphasia, memory and emotion is affected.
260
How do focal frontal seizures present?
Motor disturbances, Todd's palsy (temporary paralysis), Jacksonian march (tingling/twitching in an area of the body that gets larger).
261
How do focal parietal seizures present?
Paresthesia.
262
How do focal occipital seizures present?
Visual phenomena - spots, lines, flashes.
263
What medication is used when sodium valproate is contraindicated?
Lamotrigine.
