3a Neuro Flashcards
(255 cards)
1
Q
What is MRI with diffusion weighted imaging used for?
A
MRI with diffusion weighted imaging looks at the way water molecules are arranged in the brain. Very sensitive in detecting acute infarction, especially in small strokes. (Stroke ILA)
2
Q
What is a key cause of strokes in young people, associated with movements of the neck?
A
Vertebral artery dissection.
Stroke ILA
3
Q
Is an anterior, middle or posterior circulation stroke more likely to be caused by embolic disease?
A
Posterior circulation strokes. (ILA)
4
Q
85-year-old woman, aortic valve replacement, left sided weakness, temperature, recent tooth extraction. What is the first test you need to do?
A
Blood cultures - Echo important but blood cultures first because guides treatment with antibiotics. (ILA)
5
Q
How would you differentiate between a migraine and subarachnoid haemorrhage?
A
Both cause headache, photophobia and nausea. Subarachnoid haemorrhage can result in vasospasm which can produce focal neurology, but patients are usually sicker. Symptoms resolve within a few days at the most in a migraine but last longer with SAH.
(ILA)
6
Q
What are three cardiovascular causes of amaurosis fugax?
A
Carotid artery atherosclerotic disease
Ophthalmic artery atherosclerosis
Embolism from heart
(ILA)
7
Q
What is Todd’s paresis?
A
Todd’s paresis is temporary focal neurological deficit following an epileptic seizure.
8
Q
What score would you use to assess someone’s risk of stroke after diagnosis with AF?
A
CHADS2 CHF Hypertension Age >75 Diabetes Previous Stroke (2 points) or CHA2DS2VASc
9
Q
How do you determine whether an NG tube is safe to use in a patient?
A
Test the pH of the aspirate from the NG tube. If there is no aspirate or pH is >4, an X ray is needed to confirm the position.
10
Q
How does migraine present?
A
4 - 72 hours. Moderate to severe intensity Pounding or throbbing in nature Usually unilateral Photo and phonophobia \+/- aura (visual changes eg blurring, lines) Nausea and vomiting
Hemiplegia migraine: + hemiplegia, ataxia, changes in consciousness (stroke mimic)
Stages:
- Prodromal <3 days
- Aura <60 mins
- Headache 4-72 hrs
- Resolution - vomiting, sleeping, fading
- Postdromal/recovery phase.
[ztf]
11
Q
What are the red flags to ask about in headache patients?
A
Fever, photophobia, neck stiffness - meningitis/encephalities
New focal neurology - haemorrhage, malignancy, stroke
Dizziness - stroke
Visual disturbance - temporal arteritis, glaucoma
Sudden onset occipital - SAH
Worse on coughing/straining/lying/bending - ICP
Severe enough to wake patient from sleep
Vomiting - ICP or CO poisining
Trauma - ICP
Pregnancy - pre-eclampsia.
[ztf]
12
Q
Give the criteria for diagnosis of migraine without aura.
A
5 or more attacks with the following: 4-72 hours Unilateral/pulsating pain/ aggravated by activity Nausea/vomiting Photo and phonophobia
13
Q
Give the criteria for diagnosis of migraine with aura.
A
> =2 attacks with >=1 fully reversible aura symptom: visual, sensory, speech/language, or motor weakness.
And at least 2 of the following:
At least 1 aura symptom spreads gradually over 5 mins, or at least 2 symptoms occur in succession
Each individual aura symptom lasts 5-60 mins
At least 1 aura symptom is unilateral
Followed within 60 mins by a headache
14
Q
Give 3 triggers for migraine.
A
CHOCOLATE Chocolate Hangovers Orgasms (sexual activity) Cheese + other strong smells Oral contraceptive Lie ins Alcohol Trauma eg head injury, stress Exercise
15
Q
How is migraine managed?
A
Will go away eventually, dark quiet room sleep.
Acute:
Triptans (5HT receptor agonists. eg sumatriptan 50mg)
NSAID/paracetamol
Anti-emetic eg metoclopramide
Prophylaxis: Topiramate (TERATOGENIC - cleft palate) propanolol amitriptyline acupuncture (recommended by NICE) If menstruation-associated, NSAIDs eg mefanamic acid around that time can help prevent.
16
Q
What can cause mild ache across the forehead like a tight band?
A
Tension headaches.
Come on and resolve gradually, no visual changes, common.
Due to stress, depression, alcohol, skipping meals or dehydration. Muscles of frontalis, temporalis and occipitalis. No visual changes.
17
Q
Describe the diagnostic criteria for infrequent episodic tension headache.
A
Probable: Missing one feature of tension headache diagnosis, does not fit a diagnosis of another headache disorder.
Infrequent episodic: <1 day/month
Frequent episodic: 1-14 days/ month, >3 months
Chronic: >15 days/ month, >3 months
A. >=2 of:
-bilateral
-pressing/tightening pain
-mild to moderate intensity
-not aggravated by routine physical activity.
B. No nausea or vomiting, no more than one of photo or phonophobia.
18
Q
How is tension headache treated?
A
Reassure
Analgesia - NSAIDS (eg aspirin, ibuprofen, diclofenac)
Relaxation
Hot towel to local area
19
Q
Describe the classifications of cluster headaches
A
Episodic: >2 cluster periods lasting 7 days to 1 year, pain free for at least 1 month at a time
Chronic: Cluster period for more than 1 year, no remission or remission less than 1 month.
20
Q
Give 3 features of a headache that would suggest it is a cluster headache.
A
Lasts 15-180 mins
Severe, unilateral periorbital pain
Ipsilateral cranial autonomic features: Miosis, ptosis, rhinorrhoea, redness, lacrimation
21
Q
Suggest a treatment for cluster headaches.
A
Acute attack: Subcutaneous sumatriptan, nasal spray zolmitriptan
Prophylactic verapamil, lithium, corticosteroids.
22
Q
Give 3 features of a headache that would suggest temporal arteritis.
A
Jaw claudication
Amaurosis fugax (temporary blindness, usually in one eye)
Temporal artery and scalp tenderness
Malaise
Palpable, tender temporal arteries with reduced pulsation
23
Q
How is temporal arteritis diagnosed?
A
Temporal artery biopsy
Blood tests:
ANCA negative, ESR >50, CRP and ALP raised, Hb lowered.
24
Q
How is temporal arteritis treated?
A
Refer for specialist review, in the meantime:
High dose oral prednisolone (40mg) and low dose aspirin (75mg) plus a PPI for gastroprotection.
25
Give 3 symptoms of a carotid territory stroke.
```
Weakness of leg, face, arm
Amaurosis fugax
Impaired language
Easier to spot - FAST campaign
[3a lecture]
```
26
Give 3 symptoms of a posterior circulation stroke.
```
Dysarthria
Dysphagia
Diplopia
Dizziness
Ataxia
Diplegia (symmetrical weakness)
These symptoms are less obvious than carotid territory stroke symptoms, and can be more devastating.
```
27
Which is the most common type of stroke?
Ischaemic (85%)
28
What would you be looking for in a CT scan if you suspect stroke?
Ischemic - loss of sulci and grey matter differentiation
| Haemorrhagic - blood, showing up white.
29
What is the Oxford stroke classification?
```
(=Bamford).
Clinical classification of patterns of neurological deficit in acute ISCHEMIC stroke:
1. Anterior - partial or total
2. Posterior
3. Lacunar
```
30
Give 3 symptoms of anterior circulation stroke.
Contralateral weakness, sensory loss, inattention
Dysarthria
Dysphasia - receptive and expressive
Homonymous hemianopia/ visual inattention
Higher cortical dysfunction
31
Give 3 features specific to parietal lobe seizures.
Numbness
Tingling
Pins and needles
(The parietal lobe is responsible for orientation, pain, touch, and visual perception). PTS
32
Give 3 features of frontal lobe seizures.
Motor weakness
Dysphasia
Rapid recovery. PTS/ILA
33
Give 3 features of temporal lobe seizures.
```
Deja vu
Dysphasia
Automatisms (being unaware of actions)
Fear
Derealisation
Olfactory/gustatory hallucinations
Post-ictal confusion
```
34
What symptoms would you get if there is a problem with the occipital lobe?
Visual disturbance/ hallucination
35
If a patient presents with ataxia and nystagmus in the left eye, where is the lesion?
Left cerebellum.
36
What is a seizure?
Transient excessive hypersynchronous neuronal changes in the brain.
37
What is epilepsy?
A recurrent tendency towards seizures: either 2 unprovoked seizures, or 1 seizure and EEG evidence of a tendency towards seizures.
38
Where in the brain are complex partial seizures most likely to occur?
Temporal lobe.
39
Give 3 differences between a simple and complex partial seizure.
Simple - awareness unimpaired, no post-ictal symptoms, focal motor, sensory or autonomic symptoms.
Complex: Awareness impaired, may have aura or impaired awareness at onset, post-ictal confusion.
40
What is an absence seizure? How is it differentiated from a 'vacant spell'?
A brief (<10 second) pause in consciousness accompanied by a 3 Hz spike and wave on ECG. Generally occur in childhood, someone stops speaking for a few seconds then continues.
41
When/why is an ECG useful for someone who has had a blackout?
To check for conditions that give rise to transient tachyarrhythmias, which can cause sudden death in young people, and have an abnormal ECG between events. [3a blackouts lecture]
42
Define a tonic-clonic seizure.
Loss of consciousness, limbs stiffen (tonic) then jerk (clonic). Can have only tonic or clonic phase. There is also post-ictal confusion and drowsiness. [ohcm]
43
Define a myoclonic seizure.
Sudden jerk of a limb, face or trunk; patient could be thrown violently to the ground or have a violently disobedient limb.
44
Define an atonic seizure.
Sudden loss of muscle tone causing a fall without LOC.
45
Define idiopathic generalised epilepsy (IGE).
A group of epileptic attack disorders with a strong genetic basis and often no structural brain abnormalities. They consist of typical absences, myoclonic jerks, and generalized tonic-clonic seizures, alone or in varying combinations and severity. [ILA]
46
How would you recognise and treat status epilepticus?
Seizure lasting >30mins but treat as status after 5 mins.
ABCDE, IV lorazepam, ECG, fluids (normal saline)
Note the time
47
How is non-epileptic attack disorder diagnosed?
Symptoms: Uncontrollable symptoms, one part of body, post-ictal emotion. Psychological basis. Exclude learning disability. Normal CNS exam, ECG, MRI and CT. Can co-exist with epilepsy.
48
What is narcolepsy?
Narcolepsy is a disorder in which sleep phenomena affect the waking state, due to a lack of orexin A and B, neuropeptides which maintain wakefulness.
49
What are the symptoms of narcolepsy?
People with narcolepsy usually have a few but not all of these symptoms:
Cataplexy, sleep paralysis, hypnogogic hallucinations, daytime sleepiness.
50
Define daytime sleepiness as a symptom.
Falling asleep with little warning in a situation that may be inappropriate.
51
Define hypnogogic hallucinations.
Vivid, often frightening visual, auditory or tactile hallucinations that occur as someone is falling asleep, probably caused by the overlap between wakefulness and REM sleep.
52
Define sleep paralysis and why it occurs.
The inability to move after waking or before falling asleep. Voluntary muscles are paralysed during sleep, but if this paralysis continues after consciousness is regained or semi-regained, a person may feel paralysed, frightened, suffocated or experience hallucinations.
53
Define cataplexy and why it occurs.
Cataplexy is transient muscle weakness triggered by a strong emotion, often happiness, without LOC. It usually resolves in minutes. It can be partial (face, neck, knees) or severe, causing totally body paralysis/weakness/collapse.
54
What is shingles?
Shingles is a condition in which a painful rash in one unilateral dermatome, malaise and fever are caused by reactivation of the varicella zoster virus.
55
How does shingles present?
Shingles presents as pain in a dermatomal distribution, followed by a few days of malaise and fever.
56
Explain the pathology of shingles.
Shingles is a reactivation of varicella zoster virus. This first infects the respiratory epithelial cells before moving to the lymph nodes in a primary infection. Secondary infection occurs when the virus travels to the liver and spleen, where it infects T cells and then keratinocytes. It travels retrogradely through sensory neurones to the dorsal root and trigeminal nerve ganglia, where it lays dormant. Reactivation may be caused by immunocompromise. When this occurs, the virus travels anterogradely to the skin, causing shingles.
57
How is shingles diagnosed?
Shingles is diagnosed by the way the rash looks. A Tzanck test may be used to look for multinucleated giant cells in the fluid of the vesicles, a blood test for varicella antibodies, and PCR for viral DNA.
58
How is shingles treated?
Treat acute zoster with antivirals: aciclovir or famciclovir. If conjunctiva affected, give acyclovir ointment.
59
What is Wernicke-Korsakoff syndrome?
A spectrum of disorders in which thiamine deficiency causes neuronal degeneration, producing an acute phase called Wernicke encephalopathy, and a chronic phase called Korsakoff psychosis/syndrome. It is seen most commonly in heavy drinkers.
60
What are the functions of thiamine in the body?
When thiamine is converted to thiamine pyrophosphate, the metabolically active form, it has multiple functions:
1. Assists enzymes allowing glucose metabolism
2. Help metabolise lipids and carbohydrates in the brain
3. Maintains normal amino acid and neurotransmitter levels
4. Assists with propagation of impulses along axons
61
Give 3 ways alcohol abuse causes impaired glucose metabolism.
1. Alcohol abuse leads to decreased nutritional intake as alcohol is high calorie, low nutrition. Therefore thiamine deficiency occurs.
2. Alcohol inhibits phosphorylation of thiamine to its metabolically active form thiamine pyrophosphate.
3. Alcohol prevents absorption of thiamine by decreasing gene expression for thiamine transporter 1.
4. Chronic alcohol use leads to cirrhosis, leading to impaired storage of thiamine in the liver.
Therefore thiamine is not able to assist with glucose metabolism.
62
Explain how thiamine deficiency causes neurological symptoms.
Thiamine is not longer able to assist with glucose metabolism, lipid and carbohydrate metabolism in the brain, propagation of neural impulses, and maintenance of amino acid and neurotransmitter levels.
The brain is particularly vulnerable to impaired glucose. This leads to haemorrhage and necrosis in the mamillary bodies, causing confusion; in the cerebellum, causing ataxia, and in the 6th cranial nerve, causing ophthalmoplegia (paralysis of lateral gaze).
63
What is the clinical triad of Wernicke's encephalopathy?
Ophthalmoplegia
Ataxia
Confusion
64
How does Korsakoff syndrome present?
Limbic involvement -> Anterograde and retrograde amnesia
| Confabulation
65
How is Wernicke's encephalopathy treated?
Medical emergency - treat asap to reduce risk of Korsakoff syndrome. Thiamine infusion (IV pabrinex) over a few days.
Add glucose when thiamine is normalised - if it is added before there is enough thiamine pyrophosphate, the glucose will turn to lactic acid and there is a risk of acidosis.
66
What investigations would you do if someone presented with ophthalmoplegia, ataxia and confusion?
Wernicke's encephalopathy is generally diagnosed by clinical impression but can also do:
LFT
Thiamine level blood test
MRI shows degeneration of mammillary bodies.
67
What is Huntington disease?
A rare neurodegenerative disease in which a repeated sequence of DNA causes an abnormal protein, leading to abnormal movements and cognitive problems.
68
How is Huntington disease passed on?
It is autosomal dominant. There is a 50% chance that an affected person will have an affected child. It displayed anticipation: successive generations have more CAG repeats in the gene, resulting in earlier age of onset and increased severity of the disease.
69
Explain the pathology of Huntington disease.
When there are more than 36 repeats of the CAG sequence, glutamine (CAG) aggregates in the putamen and caudate nuclei of the basal ganglia causing neuronal cell death and tissue loss. These areas are involved in the inhibition of movement, so the damage causes movement problems.
70
What is athetosis?
Athetosis, one of the symptoms of Huntington disease, is the name for slow, snake-like movements of the hands.
71
Give 3 symptoms of Huntington disease other than chorea.
```
Psychological problems: Dementia, personality changes, Depression (suicide).
Abnormal eye movements
Loss of co-ordination
Dysarthria and dysphagia
Respiratory disease eg pneumonia.
```
72
What is anticipation in terms of genetics?
Anticipation describes why symptom onset of Huntington disease is earlier with each generation. The child can inherit more repeats than the parent because there is more opportunity for repeat expansion of the CAG sequence.
73
What is the treatment for Huntington disease?
Only the chorea cause by increased dopamine can be treated, not the underlying disease.
Dopamine receptor antagonists eg olanzapine
Tetrabenazine - depletes dopamine.
MDT essential and core of treatment. Do not continue unnecessary medication - SEs vs benefits.
74
What are the cardinal features of parkinsons disease?
Resting tremor
Bradykinesia
Rigidity
[OCHM]
75
Explain the pathophysiology of Parkinson's disease.
Loss of dopaminergic neurons in the substantia nigra, part of the basal ganglia which help with initiation and control of movement. Lewy bodies.
76
Give 3 non-motor symptoms of parkinson's disease.
Autonomic dysfunction (postural hypotension, constipation, urinary frequency/ urgency, dribbling of saliva)
Sleep disturbance
Reduced sense of smell
77
What are Parkinson's plus syndromes?
Syndromes which give rise to parkinsonian features, eg:
Progressive supranuclear palsy (PSP)
Multiple system atrophy (MSA) - autonomic and cerebellar dysfunction
Corticobasal degeneration
Lewy body dementia (LBD) - progressive cognitive decline, hallucinations, REM sleep disorders/fluctuating consciousness.
78
What are the risks associated with sudden withdrawal of parkinsons medications?
Acute akinesia, neuroleptic malignant syndrome.
| Malabsorption can have this effect, eg abdo surgery, gastroenteritis.
79
Describe the first-line pharmacological treatment for parkinson's disease and how it works.
Co-benyldopa/co-careldopa: Contains
Levodopa (dopamine precursor, which can cross the blood brain barrier, and is converted to dopamine in the brain by dopa-decarboxylase) plus carbidopa/benserazide (reduces conversion of levodopa to dopamine outside the CNS so there is more available to enter the brain).
COMT inhibitors eg entacapone slows breakdown of levodopa in the brain to extend its duration.
80
What is Alzheimer's disease?
Alzheimer's disease is a chronic neurodegenerative disease which is the most common cause of dementia in the UK.
81
Describe the presentation of Alzheimer's disease. How does it differ from other types of dementia?
1.Enduring, progressive and global cognitive impairment. Visuo-spatial skill, memory, verbal ability and executive function are impaired. (other dementias affect specific domains. )
2. Anosognosia: lack of insight into the problems caused by the disease.
3. Later features: irritability, mood disturbance, behavioural change, psychosis, agnosia.
4. Cognitive impairment is progressive but non-cognitive symptoms may come and go over months.
5. Often become sedentary and uninterested towards the end.
[ohcm]
82
Explain the pathology of Alzheimer's disease.
1. Environmental and genetic factors play a role.
2. Amyloid precursor protein (APP) is degraded to beta amyloid peptide.
3. This results in progressive neuronal damage, neurofibrillary tangles, amyloid plaques, and loss of acetylcholine.
4. Defective clearance of beta-amyloid plaques by macrophages due to altered macrophage gene expression.
5. Neuronal loss mostly in the hippocampus, amygdala, temporal neocortex and subcortical nuclei.
6. Vascular damage occurs, 95% of AD patients show vascular dementia.
[ohcm]
83
Give 3 risk factors for Alzheimer's disease.
```
1st degree relative with AD
Down's syndrome because APP is coded for on chromosome 21, of which people with Down's have an extra.
APOE E4 allele homozygosity
Vascular RFs: HTN, diabetes, etc
Depression, loneliness (not just living alone)
Low physical/cognitive activity
Smoking
Excessive alcohol consumption
[ohcm]
```
84
What are some acetylcholinesterase inhibitors and what are the side-effects?
Donezepil, rivastigmine, galantamine.
Used for Alzheimer's disease, increase the amount of acetylcholine in the synaptic cleft.
Cholinergic effects: vasodilation, constriction of the pupils, increased secretion of sweat, saliva and tears, bradycardia, mucus secretion in the respiratory tract,
constriction of the airways.
85
Explain the pathology of vascular dementia.
Cognitive decline due to cerebrovascular disease. This could be a major stroke, multiple small unrecognised strokes (multi-infarct) or chronic changes in smaller vessels (subcortical). Risk factors are any vascular ones: HTN, strokes, focal CNS signs.
86
How does vascular dementia present and how is this different to other dementias?
There is a sudden onset, stepwise deterioration in cognitive function, whereas other types of dementia progress more gradually. This can make it hard to recognise.
87
How is vascular dementia managed?
There are no approved drugs to treat VD yet. Cognitive stimulation programmes, multisensory stimulation and art/music therapy are beneficial, as well as the general support for all dementia, eg planning ahead, care co-ordination and practical help around the home.
88
Give five causes of dementia which can be improved.
```
Thiamine deficiency (eg alcohol)
Syphilis
Tumours (meningioma)
Thyroxine/ b12 deficiency
Subdural haematoma
Parkinsons disease
HIV (+/- cryptococcosis)
Normal pressure hydrocephalus
Whipple's disease
Pellagra
```
89
Why would you do a physical examination in someone who presents with memory loss over years?
?Physical cause of cognitive impairment
?Parkinson's disease
RFs eg for vascular dementia
90
What is hydrocephalus?
Hydrocephalus is the buildup of fluid in the brain which could lead to increased intracranial pressure. The cause varies. Presents as acute confusional state.
91
What investigations would you do if you suspect hydrocephalus in someone with acute confusional state?
CT head: Enlarged oval 3rd ventricle (normally slit-like), Temporal horns visible (normally not)
Send CSF for analysis
[caroll teaching]
92
What is normal pressure hydrocephalus?
A syndrome in which there is dilatation of the cerebral ventricles (esp 3rd) and normal CSF pressure at LP.
93
How does normal pressure hydrocephalus present?
Triad: Dementia, Gait ataxia, Urinary incontinence.
Progressive slowing of cognitive and motor function consistent with a pattern of subcortical dementia. Urinary incontinence is a late symptom.
94
What causes normal pressure hydrocephalus?
50% idiopathic
| 50% secondary to mechanical obstruction of CSF movement across the meninges, eg meningitis, SAH, trauma, radiotherapy.
95
Why does NPH cause ataxia?
It is caused by pyramidal upper motor neurone paparesis.
96
How is NPH diagnosed?
CT head scan: increased size of lateral ventricles, cortical thinning.
24 hour ICP monitoring: typical beta pattern.
97
How is hydrocephalus managed?
CSF diversion by:
- Extraventricular drain
- intracranial CSF shunt, usually ventriculo-peritoneal
- Endoscopic third ventriculostomy temporarily if concern about risk of infection with VP shunt.
98
Give 3 risk factors for hydrocephalus.
Anything causing obstruction of CSF travelling across the meninges:
Bacterial meningitis (pus)
Subarachnoid/intraventricular haemorrhage (blood)
Posterior fossa tumour
Spina bifida
[Carol teaching]
99
Why/when is important to use head imaging before an LP?
Eg if the LP is for suspected bacterial meningitis, this is a risk factor for hydrocephalus; if hydrocephalus present, LP can cause herniation. If there is an intracranial mass, LP is also contraindicated,
100
Describe the presentation of a right frontal lobe syndrome.
Contralateral so left side limb weakness
Motor dysphasia for dominant lobe (Broca's area is the most inferior part of the motor strip).
(personality duplicated so only affected in bilateral disease).
[Caroll]
101
Describe the presentation of a left parietal lobe syndrome.
For non-dominant lobe, contralateral so right side sensory disturbance (no limb pain).
For dominant lobe, also higher sensory processing issues: dyslexia, dyscalculia, dysgraphia, astereognosis.
102
Describe the presentation of a dominant temporal lobe syndrome.
Dominant lobe: understanding of speech (superior temporal gyrus) and memory
No clin syndrome for non-dominant lobe
103
Describe the presentation of a left occipital lobe syndrome.
Contralateral so right side visual field issues.
104
What is parasellar syndrome?
Episodic orbital pain caused by inflammation of the cavernous sinus.
105
When would interval imaging be used?
Where an imaging finding is likely to represent a low-grade or benign tumour which is incidental, small, asymptomatic, causing minimal symptoms or symptoms which are unlikely to benefit from surgery, or risks of surgery outweigh benefits. [caroll]
106
Give two issues with interval imaging.
An MRI is usually used for interval imaging.
| Patients may be claustrophobic or have an MRI-incompatible implant. In this case consider CT scanning instead. [caroll]
107
Give 3 indications for neurosurgery to treat tumours.
Symptom management
Prevent likely function- or life-threatening deterioration
When there is diagnostic uncertainty
108
Why are posterior fossa tumours particularly significant?
The posterior fossa is a smaller 'box', bounded by the suboccipital bone and tentorium, so there is a greater risk of hydrocephalus, and compression on the brainstem.
109
Why might incomplete tumour removal be performed?
To maximise preservation of vital neurovascular structures
Or where the boundaries of the tumour are indistinct with respect to the brain
[Caroll]
110
What is multiple sclerosis?
An inflammatory, demyelinating autoimmune disease which damages the myelin sheath of oligodendrocytes in the central nervous system.
111
Describe the epidemiology of MS.
Commonest cause of neurological disability.
Peak incidence at ages 20-40.
Caucasian, Females 3:1.
More common in temperate areas, adults take risk with them but children who move before puberty acquire the risk of where they settle.
112
What causes MS?
```
Unknown! Growing evidence for:
Genetics
EBV
Smoking
Obesity
Low vit D: Early exposure to vitamin D is important, and vitamin D status relates to prevention of MS, and fewer symptoms and lesions on MRI in established MS.
```
113
Describe the typical presentation of MS.
Optic neuritis - rapidly decreasing central vision, VI nerve --> double vision INO, conjugate lateral gaze disorder. (loss of red vision first?)
Numbness/ tingling in limbs
Leg weakness
Brainstem/cerebellar symptoms
114
How is MS diagnosed?
```
Clinical diagnosis
Lesions disseminated in time and space
PPMS: Progressive over 1 year
Exclude other causes
MRI sensitive but not specific for plaques, can demonstrate atypical lesions.
LP: oligoclonal bands in the CSF
```
115
How is MS managed?
Reduce stress, do exercise
Acute relapses: NICE recommends methylprednisolone (steroid) 500mg PO 5 days. This does not change overall prognosis so use sparingly.
DMDs, biologics eg infliximab
Symptomatic
- amitriptyline for neuropathic pain
- SSRIs for depression
- Anticholinergics for urge incontinence (but worsen cognitive impairment)
Baclofen/physio/gabapentin for spasticity.
[zero to finals]
116
What is the pathophysiology of Guillain-Barre syndrome?
GBS is an acute inflammatory demyelinating neuropathy.
'Molecular mimicry' - B cells create antibodies against the antigens on the infective pathogen, which then attack the person's own nerves (myelin and/or axon).
117
Describe the typical presentation of GBS.
Symmetrical ascending muscle weakness, occurring a few weeks after infection (Campylobacter, CMV, EBV)
Starts in the feet and moves upwards. Facial weakness, cranial neuropathies, areflexia, and numbness distally becoming more proximal.
It may advance quickly to paralysis, and the progressive phase is usually 4 weeks followed by recovery as the myelin regrows.
118
How is GBS diagnosed?
Lumbar puncture: CSF shows elevated protein.
Nerve conduction studies: Slow conduction/reduced signal.
Brighton criteria
119
What are the severe complications of GBS?
Severe weakness
Aspiration
Resp failure - use ITU
Autonomic instability - severe sudden hypotension, cardiac arrhythmia.
PE is leading cause of death
80% recover, 15% left with neuro disability, 5% will die.
120
How is GBS managed?
Admit to hospital
Let ITU know in case of resp failure, monitor vital capacity. If <1L, send to ITU
DVT/VTE prophylaxis
Monitor BP and ECG
Monitor swallowing - low threshold for NG feeding
IVIg
Plasmapheresis (= plasma exchange)
121
What is motor neurone disease?
A cluster of major degenerative diseases characterised by selective loss of motor neurones. Most common is ALS.
122
What clinical pattern would be seen in amyotrophic lateral sclerosis?
There is loss of motor neurones in the motor cortex and anterior horn of the cord, so weakness and UMN signs (eg upgoing plantars) and LMN wasting/fasciculation.
[OHCM]
123
A patient has fasciculations of the tongue, hoarse voice and swallowing problems - what could be going on?
Bulbar palsy - disease of cranial nerves IX-XII (bulbar) in the medulla. Lower motor neurone lesion of muscles in tongue.
This can be caused by MND, GBS, polio, MG etc.
[OHCM]
124
Describe the typical presentation of MND.
```
60 y/o man with FHx.
UMN: hypertonia, spasticity, brisk reflexes
LMN: wasting, hypotonia, fasciculations, reduced reflexes.
This can manifest as:
Stumbling spastic gait
Foot drop +/- proximal myopathy
Weak grip - 'door handles difficult'
Shoulder abduction - 'hair washing'
'Clumsiness'
Aspiration pneumonia
NO SENSORY SX.
```
125
What investigations would you do if someone presents with possible MND?
Brain/cord MRI helps exclude structural causes
LP helps exclude inflammatory causes
Neurophysiology can detect subclinical denervation and help exclude mimicking motor neuropathies. [OHCM]
126
What is cerebral palsy?
A number of non-progressive neurological conditions in which damage to the brain causes loss of muscle control.
127
Describe the presentation of CP.
Depends which part of brain affected:
Spastic - 70% of CP. Upper motor neurones cannot receive GABA so are uninhibited, causing hypertonia and therefore tight, stiff muscles.
Dyskinetic/athetoid - damage to basal ganglia causes dystonia and chorea.
Ataxic - cerebellum affected -> ataxia.
128
What causes cerebral palsy?
Neurodevelopmental disease: Prenatal exposure to radiation, post-natal head trauma, infection/hypoxia either pre- or post-natally.
Genetic mutation may also be a cause but is less common.
129
Describe the epidemiology of CP.
1 in around 400 children.
130
Describe the management of CP.
There is no cure.
Multidisciplinary approach - OT, SALT, Physio, Neuro input. Build strength, improve walking, stretch muscles.
Muscle relaxants eg baclofen, diazepam and botulinum toxin help reduce hypertonus, decrease pain.
Surgery may be used to loosen muscles, straighten bones which are misshapen or atrophied, and cut nerve to reduce involuntary movements.
131
What is neurofibromatosis?
A genetic condition that causes neuromas, which are benign CNS tumours.
NF1 (1 in 2500) - AD inherited mutation of NF1 gene on chromosome 17, codes for neurofibromin protein which is a tumour suppressor.
NF2 (rarer).
132
Give 5 signs of NF1.
```
>2 features of:
CRABBING:
Café-au-lait spots
Relative with NF1
Axillary or inguinal frexkes
Bony dysplasia such as Bowing of long bone
Iris hamartomas (Lisch nodules)
Freckling, typically in skin-folds
Neurofibromas - small, purple, gelatinous nodules, may become papillomatous, not painful but may itch.
Glioma of optic nerve
[OHCM]
```
133
What are 3 common complications of NF1?
Complications only occur in 30% of people with NF1.
Renal artery stenosis causing hypertension (6%)
Mild learning disability is common
Local effects:
nerve root compression -> weakness, pain, parasthesiae
GI -> bleeds; obstruction
Bone -> cystic lesions, scoliosis, pseudoarthrosis.
phaeochromocytoma
Malignancy (5%) - peripheral nerve sheath tumour, GI stromal tumour
134
How is NF1 managed?
MDT - geneticist, neuro, surgeon, physio, GP.
Yearly measurement of BP, cutaneous survey.
Excise troublesome dermal neurofibromas (can't realistically remove all).
Genetic counselling - AD inheritance
135
Describe the presentation features of NF2.
Acoustic neuromas -> sensorineural hearing loss, may have tinnitus and vertigo
Tumours are benign but press on local structures and can increase intracranial pressure.
Cataracts
136
What is meningitis?
Inflammation of the pia and arachnoid mater of the meninges, causing a syndrome of headache, fever and neck stiffness.
137
Describe the presentation of meningitis.
Headache, fever and neck stiffness
Photo and phonophobia
Early: Leg pains, Cold peripheries, Pallor
Late: Non-blanching rash if bacterial, Kernig's sign, decreased consciousness, coma, seizures, focal CNS signs.
[ohcm]
138
How is meningitis managed?
Bacterial: abx eg cefotaxime, amoxicillin covers Listeria so use if they are immunocompromised
Monitor for sepsis:
Cap refill- slow
BP - decreases
Disseminated intravascular coagulation
Fever and pulse increase or stay the same.
[ohcm]
139
What causes meningitis?
85% caused by enteroviruses, eg coxsackie A and B, HSV.
Bacterial rarer but more serious: Meningococcus (Neisseria meningitides) or pneumococcus (Strep pneumonia)
Fungal and TB - immunocompromised individuals
Usually haematogenous spread through the blood brain barrier, the choroid plexus being a weak link, but can occur through direct spread (spina bifida, skull fracture).
140
What investigations would you do to diagnose meningitis and what would they show?
Kernig's sign - pain + resistance on passive knee extension with hip fully flexed.
CT scan to exclude SAH
LP is the diagnostic test. CSF pressure raised, glucose concentration decreased, protein increased, WBCs >5. Varies depending on the type of meningitis.
141
What is encephalitis?
Inflammation of the brain parenchyma. Similar to meningitis but confusion and altered mental state more prominent. [PTS]
142
Describe the presentation of encephalitis.
Odd behaviour, decreased consciousness, focal neurology or seizure, preceded by an infectious prodrome (fever, rash, lymphadenopathy, cold sores, conjunctivitis, meningeal signs). History of travel/animal bite.
143
Give 3 causes of encephalitis.
Usually viral and often never identified, so usually treat before cause is known.
Viral: HSV-1 and 2, arboviruses, CMV, EBV, VZV, HIV seroconversion, measles, mumps, rabies, japanese B, West Nile, tick-borne
Non-viral: bacterial meningitis, TB, malaria, listeria, lyme disease, legionella, etc
144
What can cause the symptoms of encephalitis but without infectious prodrome?
Encephalopathy - can be caused by hypoglycaemia, hepatic encephalopathy, DKA, drugs, hypoxic brain injury, uraemia, SLE, beri-beri.
145
What investigations would you do if you suspect encephalitis?
Bloods - blood culture, serum for viral PCR, throat swab, MSU
Contrast CT - focal bilateral temporal lobe involvement suggests HSV encephalitis, meningeal enhancement suggests meningoencephalitis. BEFORE LP.
LP - increased protein and lymphocyte levels in CSF, decreased glucose. Send for viral PCR.
EEG: confirms diagnosis but does not indicate cause
146
How is viral encephalitis managed and what is the prognosis if untreated?
Emergency - untreated mortality 70%
Acyclovir within 30 mins of presentation, 14 days, as empirical treatment for HSV. 21 days if immunosuppressed. Adjust according to eGFR.
Supportive therapy, HDU/ICU if necessary
Symptomatic treatment eg phenytoin for seizures
147
What is Horner's syndrome?
Horner's syndrome, also called oculosympathetic paresis, is when damage to the sympathetic trunk causes a triad of miosis, ptosis and anhidrosis.
148
Describe the underlying pathology of Horner's syndrome
Could be caused by interruption to the sympathetic facial supply at the:
- brainstem (demyelination, vascular disease)
- cord, eg syringomyelia
- thoracic outlet eg Pancoast tumour in pulmonary apex
- path of the internal carotid artery
Idiopathic congenital form is rare.
149
What is a paralytic squint and what causes it?
Acquired defect of movement of the eye due to disease of the III, IV and VI cranial nerves. Also causes double vision. Can be a sign of raised intracranial pressure.
150
What is Bell's palsy?
Idiopathic facial nerve palsy. Isolated, unilateral dysfunction of the facial nerve (CN VII).
151
Describe the presentation of a facial nerve palsy.
Unilateral mouth sagging, drooling, trapped food between gum and cheek, speech difficulty, failure of eye closure leading to dry or watery eye. Patient cannot wrinkle forehead (LMN lesion) or whistle (buccinator)
152
How would you determine the cause of a facial nerve palsy?
```
Is there forehead sparing?
UMN:
Patient CANNOT move forehead on affected side.
Unilateral: stroke, stumour.
Bilat (rare): MND, Pseudobulbar
```
LMN:FOREHEAD SPARED because of bilateral innervation from the contralateral UMN.
Eg Bell's palsy: unilat LMN facial nerve palsy.
Abrupt onset overnight or after nap
Complete unilateral facial weakness at 24-72 hours
Ipsilateral numbness/ pain around ear
Ageusia (decreased taste)
Increased sensitivity to sound (due to stapedius palsy)
153
What cause would you consider if someone has facial palsy and rash?
```
Ramsay hunt syndrome (latent varicella zoster reactivates in facial nerve ganglion) Give prednisolone and acyclovir.
Others:
Lyme disease
Meningitis
TB
```
154
What can cause bilateral facial nerve palsy?
Sarcoid, GBS, lyme disease, trauma
155
How is Bell's palsy diagnosed and managed?
Exclude other causes with blood ESR+glucose and CT/MRI
Treat with prednisolone within 72 hours, otherwise it clears up on its own
Use tape to shut eyes at night/lubricating eye drops.
156
Give 2 risk factors for Bell's palsy.
Pregnancy (x3)
| Diabetes (x5)
157
Describe the presentation of trigeminal neuralgia.
Paroxysms of intense, stabbing, unilateral pain, lasting seconds, in the trigeminal nerve distribution (particularly mandibular and maxillary divisions). Face screws up in pain.
158
What can trigger the pain of trigeminal neuralgia?
Washing affected area, shaving, eating, talking, dental prostheses (innocuous stimuli).
159
14% of trigeminal neuralgias have a secondary cause. Give 3 possible causes.
1. Compression of trigeminal root by anomalous or aneurysmal intracranial vessels or a tumour
2. chronic meningeal inflammation
3. MS
4. zoster
5. skull base malformation (eg Chiari).
160
Give 3 risk factors for trigeminal neuralgia.
Triggers - cold, spicy, caffeine
Asians: Female: Male 2:1
5-10% of people with MS
90% unilateral 10% bilateral
161
How is trigeminal neuralgia managed?
1st line: Carbamazepine > lamotrigine, phenytoin, gabapentin.
2: Surgery decompression or intentional nerve damage
162
What is bovine papular stomatitis?
A disease caused by the BPS virus, occurring worldwide in cattle and causing a mild rash on the hands. It is important because it presents a differential diagnosis for foot-and-mouth disease in people with skin lesions on their hands.
163
What is the pathology of bulbar palsy?
A lower motor neurone lesion of cranial nerves VII- XII causing paralysis of the muscles of the face, larynx, pharynx and tongue.
164
Describe the presentation of bulbar palsy.
```
Tremulous lips
weak fasciculating tongue
drooling
dysphagia
absent palatal movements
dysphonia - rasping if unilateral, nasal if bilateral
articulation difficulty - initially can't say 'r's
[patient.info]
```
165
What can cause bulbar palsy? Give 3 causes
```
MND
Dipheria
Polio
Tumours
Guillain-barré syndrome
```
166
What is the pathology of pseudobulbar palsy?
Lesion of the corticobulbar tracts which contain upper motor neurons from the cortex to CNs V and VII-XII.
167
Describe the presentation of pseudobulbar palsy.
```
Donald duck speech
Unable to protrude tongue
Absent palatal movements
Facial muscle paralysis
Persistent dribbling
Exaggerated reflexes eg jaw jerk
Nasal regurgitation
Dysphonia
Dysphagia
*No tongue wasting
```
168
Give 3 causes of pseudobulbar palsy.
```
CVD eg bilateral cortical infarct
Neurosyphilis
MS
MND
Head injury
```
169
What is a crescendo TIA and what is the significance?
>2 TIAs in a week. high risk of stroke.
170
What is the ROSIER tool? What would ROSIER score 2 indicate?
Recognition Of Stroke in the Emergency Room. Based on clinical features and duration, stroke likely if scoring anything above 0.
171
How would you determine the risk of stroke within 48 hours after TIA?
ADBC2 score: Risk of stroke within following 48 hours.
Age >60 =1
BP >140/90 = 1
Clinical features of unilat weakness =2, dysphasia alone =1
Duration >1h =2, >10mins =1
Diabetes = 1
172
How is stroke managed?
Admit
Exclude hypoglycaemia
CT to exclude bleed
If no bleed give aspirin 300mg stat, continue for 2 weeks
Thrombolysis with alteplase within 4.5 hr. Monitor for complications - eg bleed, using CT.
173
How is carotid stenosis diagnosed and managed?
Dx: Carotid USS
If present:
Endarterectomy to remove plaques or carotid stenting to widen the lumen
174
What imaging would you use to establish the vascular territory affected by stroke?
Diffusion weighted MRI
175
What could cause sudden onset headache, seizures, weakness, vomiting, and/or reduced consciousness in a patient on warfarin?
Intracranial bleed. Cause of 10-20% of strokes.
| [zero to finals]
176
What does a GCS of 8 or below indicate, and what is the minimum GCS score?
Min 3/15 because each category has a minimum of 1.
| 8 or less = may not be able to maintain airway so INTUBATE.
177
What is a subdural haemorrhage?
Rupture of bridging veins in the subdural space of the meninges (between dura and arachnoid mater). On CT has crescent shape and crosses cranial sutures.
178
What would you suspect in an elderly or alcoholic patient with a sudden onset headache? What would you see on CT to confirm this?
Subdural haemorrhage - crescent shape bleed crossing the sutures on CT.
179
How is intracranial haemorrhage managed acutely?
```
CT head
FBC, clotting
admit to stroke unit
Monitor GCS - Consider ICU, intubation and ventilation
Correct any clotting abnormality
Correct severe htn but avoid hypotension
```
180
A 50 year old black woman reports a sudden onset severe pain at the back of the head that came on while she was at the gym, what would you need to exclude?
Subarachnoid haemorrhage. RFs: cocaine, sickle cell, strenuous activity eg weightlifting/sex, black, female, 45-70.
High morbidity and mortality.
Often occipital (back of head)
Do a CT head - will see hyperattenuation (white) in the subarachnoid space.
181
What investigations would you do for suspected SAH?
CT first
If negative, LP. Red cell count will be raised persistently (not just at the start due to the LP trauma)
Xanthochromia - yellow CSF caused by bilirubin
Angiography to locate source of bleeding.
182
How is SAH managed and what are the key complications to address?
Multidisciplinary, may need specialist support.
Surgery - coiling or clipping to repair vessel and prevent re-bleeding
Nimodipine (CCB) to prevent VASOSPASM
LP/shunt for hydrocephalus
AEDs for seizures
183
What is intranuclear ophthalmoplegia and what should you consider as the cause?
Unilateral CN VI (abducens) palsy. Internuclear = nerve fibres between CN III, IV and VI nuclei, so co-ordination of the eye movements is damaged. Ophthalmoplegia = problem with eye muscles.
Can be a presenting sx of MS.
184
What is the most common disease pattern in MS at initial diagnosis?
Relapsing-remitting MS - further classified by whether the disease is active (new sx) and/or worsening (worsening of disability).
Others:
secondary progressive - was originally RR but became progressive
Primary progressive - never relapsed.
185
What is clinically isolated syndrome?
First episode demyelination. May be MS, may not - you need evidence of multiple lesions in space and time to diagnose MS. If lesions are seen on MRI they are more likely to progress to MS.
186
What is optic neuritis and what are the causes?
Unilat reduced vision developing over hours to days. Central scotoma (blind spot), pain on eye movement, impaired colour vision(Red first?), RAPD.
MS (50%)
Other autoimmune: SLE, sarcoidosis
DM
Infection: Syphilis, Measles, mumps, Lyme disease
Refer to ophthalmology for assessment.
Treatment with steroids, recovery takes 2-6 weeks.
187
How is motor neurone disease treated?
Symptomatic:
Riluzole slows progression and extends survival
Death is from resp failure or (aspiration) pneumonia.
Non-invasive ventilation
MDT
advanced directives eg for parenteral feeding, end of life planning
188
What is cogwheel rigidity? What is the most common cause?
Tension in their warm that gives way to movement in small increments. Sign of parkinsons disease.
189
What could cause a fine, symmetrical, 5-8hx tremor which improves at rest and after alcohol?
Benign essential tremor. Most notable in hands but affects head, jaw and voice too. Common in older people.
PD is asymmetrical at first, 4-6hz, worse at rest (pill-rolling to make it go away) and no improvement with alcohol.
Hyperthyroidism
Huntingtons chorea
Fever
Meds eg antipsychotics
190
What are the advantages and disadvantages of dopamine agonists in PD?
Eg bromocryptine, cabergoline.
Delay use of levodopa and reduce dose of levodopa required to control symptoms.
Not as effective as l-dopa, prolonged use causes pulmonary fibrosis.
191
How is benign essential tremor managed?
If causing problems for the patient, can treat with propranolol or primidone (barbiturate AED). If not, don't treat.
192
How is tonic clonic epilepsy managed?
GTCS:
1. sodium valproate (teratogenic)
2. Lamotrigine, carbamazepine
193
How are focal seizures managed?
'other way round' to general:
1. Carbamazepine, lamotrigine
2. Sodium valproate, levetiracetam
194
What are the indications and contraindications of sodium valproate?
Indications
Absence: Valproate = ethosuximide.
Atonic: Valproate > lamotrigine
Myoclonic: Valproate > lamotrigine
CI:
Teratogenic. advice re contraception and avoid in girls and women since 2018.
Liver damage, hepatitis, hair loss, tremor.
195
What are the indications and side effects of carbamazepine?
```
Focal seizures 1st line
SEs:
Agranulocytocis
Aplastic anaemia
P450 inducing --> loads of interactions.
```
196
What are the SEs of phenytoin?
Folate, vit D def (think F for folate and fenytoin)
Folate def --> Megaloblastic anaemia
Vit D def --> Osteomalacia
197
What are the SE of ethosuximide?
Night terrors
| Rashes
198
What are the SEs of lamotrigine?
SJS/DRESS syndrome - life threatening skin rashes
| Leukopaenia
199
What causes neuropathic pain?
```
Abnormal functioning of the sensory nerves delivering painful signals to the brain.
Shingles - dermatomal, truncal
MS
Diabetes - feet
Trigeminal
Complex regional pain syndrome (CRPS)
Iatrogenic - surgery
```
200
What is the DN4 questionnaire?
Assesses characteristics of pain and examination of affected area, score of 4/10 or higher indicated neuropathic pain.
[ztf]
201
How is neuropathic pain treated?
```
1st:
TCA: amitriptyline
SNRI: Duloxetine
Anticonvulsants: gabapentin, pregabalin.
Flares:
Tramadol 'rescue'
Capsaicin (chilli pepper cream) topical
Physio
Psychological input
Trigeminal --> carbamazepine 1st line, refer to specialist if ineffective.
```
202
What can cause change in personality and behaviour, and headache worse at night?
Frontal lobe tumour (frontal lobe does personality and higher level decision making).
203
What investigations would you do in a patient with altered mental state, focal seizures and unilateral ptosis?
These are all features of raised ICP - do fundoscopy to look for papilloedema (optic disc swelling). The retinal vessels should flow straight across a flat surface, so if they 'curve' this suggests elevation of the disc (oedema).
204
Which cancers most commonly metastasise to the brain?
Breast
Bronchi (lung)
Bowel (colorectal)
Prostate
205
What is a glioma and what are the main types?
```
Glial cell tumour of brain/spinal cord.
Astrocytoma - GBM
Oligodendroglioma
Ependymoma
Grades 1-4. Glioblastomas are the worst
```
206
What is a meningioma?
Usually benign tumour of meninges. However can cause mass effect symptoms.
207
What could cause cushing's disease, acromegaly, thyrotoxicosis and bitemporal hemianopia?
Pituitary tumour
Optic chiasm --> bitemporal hemianopia
Can cause hypopituitarism or hyperpituitarism which would cause excess GH.
Prolactin secreting tumours treated with bromocriptine
GH secreting tumours treating with somatostatin analogues eg ocreotide
208
What can cause hearing loss, tinnitus and balance problems?
Acoustic neuroma (vestibular schwannoma)
209
What is myasthenia gravis (MG)?
An autoimmune condition causing progressive fatiguable muscle weakness which improves with rest.
Associated with thymoma (20%)
210
What is the pathophysiology of MG?
Antibodies are produced against acetylcholine receptors, so they are blocked.
So muscle contraction gets worse with use as more receptors are blocked, and better with rest as more receptors are 'freed up'.
Other antibodies:
MuSK (muscle specific kinase) --> inadequate acetylcholine receptors produced and organised
LRP4
211
How does MG present?
Weakness getting worse with use and improved with rest.
| Proximal and small muscles - extraocular, eyelid (ptosis), facial, swallowing, jaw (chewing), talking (dysarthria)
212
What would you look for on examination in MG?
Examination - elicit fatiguability, upward gaze for a long time --> diplopia on eye movement
Unilateral weakness on repeated arm abduction
Look for thymectomy scar
FVC
213
How is MG diagnosed?
Antibodies - Ach receptors in 85%, MuSK in 10%, LRP4 (<5%)
CT/MRI
Edrophonium/neostigmine test - Inhibit cholinesterase enzymes, briefly improve weakness, established MG diagnosis.
214
How is MG treated?
ACh inhibitors - Pyridostigmine, neostigmine
Immunosuppression - steroids, azathioprine
Thymectomy - even in those without thymoma.
If that fails, try MABs: rituximab (B cell) available on NHS; eculizimab (C5) is not.
!!FVC for myasthenic crisis
215
What is myasthenic crisis and how is it treated?
```
Resp failure in MG. Life threatening emergency.
Measure FVC
Rx options:
NIV eg BIPIP
Intubation, ventilation
IVIg
Plasma exchange
```
216
What is Lambert-Eaton Myasthenic syndrome (LEMS)?
Similar to MG but with more insidious milder symptoms, and usually occurs in patients with small cell lung cancer (SCLC).
Autoimmune antibodies are produced against voltage gated calcium channels (VGCCs) in SCLC cells and the presynaptic terminal of the NMJ. The VGCCs are necessary for release of Ach into the synapse, so damaged channels = less Ach release = poor muscle contraction.
217
How does LEMS present?
Proximal muscle weakness, legs
Intraocular muscles - diplopia
Levator muscles - ptosis
Oropharyngeal muscles - dysphagia and dysarthria
Autonomic - dry mouth, blurred vision, impotence, dizziness
Reduced reflexes which improve after maximal muscle contraction, a phenomenon called post-tetanic potentiation.
218
How does LEMS present?
Gradual onset
Proximal muscle weakness, legs
Intraocular muscles - diplopia
Levator muscles - ptosis
Oropharyngeal muscles - dysphagia and dysarthria
Autonomic - dry mouth, blurred vision, impotence, dizziness
Reduced reflexes which improve after maximal muscle contraction, a phenomenon called post-tetanic potentiation.
219
What is Charcot-marie-tooth disease?
Inherited disease that affects peripheral nerves (motor and sensory). Dysfunction of myelin or axons. Most are autosomal dominant mutations.
220
How and when does CMT present?
Sx appear in childhood <10 years but may be delayed until 40 or later.
Pes cavus
'inverted champagne bottle legs' due to distal muscle wasting
Weakness in lower legs, particularly loss of ankle dorsiflexion
Weakness in hands
Reduced reflexes, tone
Peripheral sensory loss
221
Give 5 causes of peripheral neuropathy. (hint: ABCDE)
Alcohol
B12 def
Cancer and Chronic kidney disease (and CMT)
Diabetes and Drugs (isoniazid, amniodarone, cisplatin)
Every vasculitis
222
How is CMT managed?
No cure
MDT - neuro and genetics are involved in dx (and counselling?)
Physio
OT
Podiatrist (charcot foot)
Orthopaedic surgeons to correct joint deformities if causing disability
223
What is the pathophysiology of NF2?
Chromosome 22 AD inherited mutation --> codes for Merlin protein which is a tumour suppressor protein particularly important in Schwann cells (--> Schwannomas).
Asso with bilateral acoustic neuroma
224
What is tuberous sclerosis?
Genetic condition causing multisystem effects but characteristically hamartomas. These are benign neoplastic growths of any tissue - skin, brain, lung, heart, kidney, eye.
225
What is the pathophysiology of tuberous sclerosis?
TSC1 gene on chromosome 9 --> hamartin
TSC2 gene on chromosome 16 --> tuberin
These proteins interact to control size and growth of cells.
Mutation in one of the above --> abnormal cell size and growth
226
What skin signs would you look for in a child with epilepsy?
Tuberous sclerosis signs:
Ash leaf spots - depigmented areas
Shagreen patched - thickened pigmented patched
Angiofibromas - skin coloured papules over nose and cheeps
Subungual fibromata - nail bed lumps
Cafe-au-lait spots
Poliosis - white hair on head, eyebrows, eyelashes
227
What is sinusitis and how does it present?
Headache associated with inflammation in the ethmoidal, maxillary, frontal or sphenoidal sinuses. This usually produces facial pain behind the nose, forehead and eyes. Usually viral and resolves within 2-3 weeks.
228
How is sinusitis treated?
```
Will resolve within 3 weeks
Saline nasal irrigation
Steroid nasal spray if symptoms are prolonged
Abx occasionally required
[zero to finals]
```
229
How does a hormonal headache present?
Low estrogen related
Generic, nonspecific tension-like headache (tight band).
Premenstrual, menopausal, pregnancy first trimester
OCP improves.
230
How do analgesia headaches present?
Nonspecific tension-like headache, after continuous or excessive use of analgesia - need to withdraw to improve the sx.
231
How does cervical spondylosis present?
Degenerative changes in the cervical spine --> Neck pain worse on movement, headache. Exclude spinal cord or nerve root lesions, inflammation, malignancy and infection.
232
What are cluster headaches?
Severe, unbearable (suicide) unilateral headaches, usually around the eye, which come in clusters of attacks eg 3-4 a day for a month, followed by remission of a few years.
30-50 years old, male, smoking
Treated with OXYGEN
233
What can cause a 40 year old man to present with severe pain and a red, swollen eye, miosis, ptosis, nasal discharge and facial sweating?
Cluster headache - very painful headache around the eye.
234
How are cluster headaches managed?
Acute: SC triptan eg sumatriptan, high flow 100% oxygen 15-20 mins. Sometimes given this at home.
Prophylaxis: verapamil, lithium, prednisolone during clusters.
235
What is a radiculopathy?
A compressed nerve in the spine, causing pain, numbness, tingling or weakness in a peripheral nerve distribution. This is a type of LMN lesion occurring after the DRG.
236
What is a myelopathy and how is it different to a radiculopathy?
A lesion in a spinal nerve before it reaches the DRG, therefore UMN lesion, whereas radiculopathy is lower motor neurone. Caused by compression in the spinal cord eg tumour, arthritis, inflammation, ageing, trauma.
237
What symptoms could be caused by a cervical spine radiculopathy?
```
Unilateral peripheral neuropathies
Median nerve - Carpal tunnel
Ulnar nerve - ulnar nerve palsy
Thoracic outlet syndrome
Self-limiting and not recurrent.
Neck pain, stiffness, headache, limb pain and parasthaesia, sensory loss, weakness.
```
238
What symptoms could be caused by a cervical spine myelopathy?
Bilateral
Insidious progression
Motor, sensory and autonomic dysfunction can occur.
Parasthaesia, muscle wasting, lower limb symptoms, heavy legs, poor balance and falls, loss of bowel/bladder control.
Acute spinal cord compression is a neurosurgical emergency.
239
Which nerve is involved in deltoid function and sensation?
C5 nerve root --> axillary nerve, deltoid muscle
240
Which nerve controls sensation over the index finger and controls elbow flexion?
C6 nerve root --> musculocutanous nerve, biceps brachii
241
What neuropathy could cause weakness of elbow extension and loss of sensation to the middle finger?
C7 nerve --> radial/axillary nerves --> triceps brachii
242
What neuropathy could cause weakness of finger flexion and loss of sensation over the little finger?
C8 nerve root/radiculopathy
243
What could cause foot drop and lateral thigh numbness?
Common peroneal nerve - entrapment at the fibular head. Sciatic nerve has 2 main branches: tibial nerve and common peroneal nerve.
L5 nerve root: weakness of hip abduction (gluteal muscles) and pain and sensory loss in the side of the thigh and lower leg, including the dorsum of the foot and toes I-III
[BMJ]
244
What could cause weak finger abduction and inner arm numbness?
T1 nerve root --> ulnar nerve.
245
What is carpal tunnel syndrome and how does it present?
Median nerve entrapment at the wrist caused by pressure or adjacent tissue.
RFs: RA, HM, acromegaly, work/trauma, female, fat, fetus, forties and fifties.
Presents with numbness/pain in palmar thumb, first 2 fingers, worse at night/when holding steering wheel/phone, APB weakness, Tinel's and Phalen's signs, Flick sign - patient making flicking movement of wrist to alleviate symptoms.
246
What is spinal cord syndrome?
Often caused by lesion in the anterior cord: Areflexic, flaccid leg weakness, sphincter disturbance. Pain and temp loss.
DCML preserved.
Can be traumatic or non-traumatic. Resuscitate, MRI/CT.
247
What is a coma?
Unresponsive, unrousable stable lasting >6 hours. Lacks normal sleep-wake cycle, no voluntary actions.
248
How could a brain abscess present?
Coma with focal signs
| Caused by CNS infection - 1-6 month after CNS infection. TB, immunosuppression, CMV, EBV
249
What is a myopathy and what are the main causes?
Myopathy = disorder of muscle fibres --> weakness.
Inherited eg DMD, BMD, Myotonic dystrophy (MD) = weakness and myotonia (delayed relaxation of muscle after contraction). AD inheritance, CTG repeat expansion, anticipation - like HD?! cardiac sx
Acquired:
Infection - HIV
Endocrine - usually proximal. Hyper/hypothroidism, vit D, hyperparathyroidism, hyperadrenalism eg Cushing's, acromegaly
Drugs: statins
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When are statins indicated?
For prevention of ischemic stroke when cholesterol >3.5mmol/L. May increase risk of haemorrhage.
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A man with severe hypertension has a stroke. Would you give an antihypertensive? What would influence this decision?
NICE advise that in the acute management of ischaemic stroke to avoid treatment of hypertension unless there are certain hypertensive complications such as:
hypertensive encephalopathy
hypertensive nephropathy
hypertensive cardiac failure/myocardial infarction
aortic dissection
pre-eclampsia/eclampsia
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What can cause ptosis and a 'down and out' eye?
Oculomotor (III) nerve palsy.
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What can cause an adducted eye?
Abducens nerve (VI) palsy.
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If someone gradually sinks to the ground at the start of a seizures, what would this indicate?
Syncope.
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What is the diagnostic criteria for orthostatic hypotension?
Regardless of symptoms, orthostatic hypotension can be diagnosed if the patient meets these criteria.
Note that if the patient’s BP is normally >160 S, a drop in diastolic BP no longer has bearing on the result,
and systolic must drop by >30 (rather than 20) for the diagnosis.
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