Neurology Flashcards
1
Q
What age-related changes occur in the CNS?
A
- Decrease in brain weight.
- Cerebral cortical atrophy.
- Secondary loss of white matter due to axonal degeneration.
- Structural changes in surviving cortical neurones like neurofibrillary tangles (AD) and reduced size, numbers and dendritic branches,
- Alterations in quantity and distribution of NTs.
- Increase in astrocytes.
- Thickening of the leptomeninges.
- Arteriosclerosis and amyloid angiopathy.
- Compensatory enlargement of lateral ventricles.
- Large and numerous granulations on the brain.
- gyri = narrow; sulci = broad
2
Q
Define dementia.
A
Dementia can be defined as an acquired global impairment of intellect, memory and personality without impairment of consciousness.
3
Q
What are the various causes of dementia?
A
Traumatic: dementia pugilistica
Vitamin: deficiency of B1 (thiamine- WK syndrome), B2, B12
Autoimmune: vasculitis
Neoplasms: & other intracranial lesions (subdural haematoma, hydrocephalus)
Infections: HIV, neurosyphilis, Whipple’s, TB
Substance abuse: drugs and toxins (alcohol, barbiturates, heavy metals)
Hormones: hypothyroidism, hypoparathyroidism (endocrine)
Electrolyte disturbances: liver failure, uraemia (metabolic)
D: depression and degenerative (AD, Huntington’s, Lewy body, Prion, Parkinson’s, frontotemporal lobar)
4
Q
What are the features of dementia?
A
Acquired loss of higher mental function affecting episodic memory, language function, frontal executive function, visuospatial function and apraxia.
severe enough to cause social & occupational impairment
being chronic and stable
5
Q
What are the key areas to test whilst taking a history for dementia?
A
- Memory
- Functional ability
- Personality and frontal lobe function
- Language
- Visuospatial ability
- Psychiatric features
- Tempo of progression
- Family history of dementia
- Alcohol and drug abuse
- Medication
- Other neurological problems
6
Q
What investigations would you conduct to identify treatable causes of dementia?
A
- Blood tests (CBC, ESR, vitamin B12, urea, electrolytes, glucose, liver function, serum calcium, thyroid, HIV serology)
- Brain imaging- MRI & CT
- Detailed neuropsychometric assessment
- CSF measurement
- Genetic tests
- EEG
- Brain biopsy
7
Q
What are the key clinical features of Alzheimer’s Disease?
A
- Episodic memory impairment
- Language impairment: difficulty finding words
- Apraxia
- Agnosia
- Frontal executive function affected
- Parietal presentation: visuospatial difficulties
- Posterior Cortical Atrophy: visual disorientation
- Personality remains intact
- Anosognosia: deficit of self-awareness
- Tempo: insidious onset; gradual progression.
- Late non-cognitive features: myoclonus, seizures, sleep cycle reversal, incontinence, impaired swallowing leading to terminal aspiration pneumonia.
8
Q
What genes are involved in the inheritance of AD?
A
Amyloid precursor protein gene on Chr.21
Presenilin 1 on Chr.14
Presenilin 2 on Chr.9
Genotype e4e4 on the ApoE gene on Chr.19.
9
Q
What pattern of inheritance is seen in AD?
A
Autosomal dominant
10
Q
What environmental risk factors are associated with AD?
A
Increasing age, trauma and vascular risk factors.
Anti-inflammatory drugs are thought to reduce the risk of AD.
11
Q
What are the 4 main histological hallmarks of AD?
A
- Loss of neurones and synapses (temporal and cerebral atrophy- brain weight reduced to 1100g)
- Extracellular senile plaques
- Intracellular neurofibrillary tangles
- Amyloid angiopathy
12
Q
What are the characteristics of Lewy body dementia?
A
- visual hallucinations
- fluctuating cognition (variation in attention and alertness)
- REM sleep behaviour disorder (RBD)
- dysautonomia
- parkinsonism
13
Q
What can be used to temporarily improve cognitive function in DLB?
A
Cholinesterase inhibitors
14
Q
What are the main causes of vascular dementia?
A
- multi-infarct dementia (usually in middle cerebral arterial distribution)
- cerebral small-vessel disease
- post-stroke dementia
15
Q
What are the common signs of vascular dementia?
A
Apraxic gait disorder
Pyramidal signs
urinary incontinence
16
Q
How can vascular dementia be distinguished from AD?
A
Imaging and clinical features
history of TIAs
follows succession of cerebrovascular events
stepwise course
17
Q
What is the most frequent cause of stroke?
A
hemiplegia due to a vascular lesion of the internal capsule
18
Q
What are the 3 arteries that supply blood to the internal capsule?
A
anterior choroidal (from ICA) medial striate (from anterior cerebral) lenticulostriate (from middle cerebral)
19
Q
What are the functions of each cortical lobe?
A
Frontal- higher functioning, personality, motor control, speech production
Temporal- hearing, language comprehension, emotional control, memory
Parietal- sensation- pain, touch, pressure, temperature
Occipital- vision and visual perception
20
Q
Name 4 components of CSF that be measured on doing a lumbar puncture.
A
protein serology, cytology and culture glucose WCC (appearance)
21
Q
What are the cortical territories of the anterior, posterior and middle cerebral arteries?
A
ACA - the medial part of the frontal and the parietal lobe and the anterior portion of the corpus callosum, basal ganglia and internal capsule.
MCA- majority and lateral surface of hemisphere
PCA- midbrain, thalamus, inferomedial part of the temporal lobe, occipital pole, visual cortex, and splenium of the corpus callosum.
22
Q
Name 5 risk factors for TIA/stroke.
A
hypertention diabetes mellitus smoking cardiac disease clotting disorders oral contraceptive pill previous TIA (for stroke)
23
Q
Name 3 clinical features of a stroke in each of the cerebral artery territories.
A
ACA- lower limb paresis, drowsiness, akinetic mutism
MCA- Aphasia, facial droop, contralateral limb weakness and sensory loss
PCA- contralateral homonymous hemianopia, visual agnosia, cortical blindness
24
Q
Name 4 clinical features of posterior circulation strokes.
A
hemi/tetra/facial paresis dysarthria vertigo nausea and vomiting visual disturbance
25
What is the eligibility criteria for a patient to receive thrombolysis in stroke management?
alteplase - also known as tissue plasminogen activator (tPA) - should be administered to all patients presenting with stroke, providing:
Haemorrhagic stroke has been excluded.
The patient presents within four and a half hours of having the event.
Access to specialised services is available.
26
Name one cause of each type of haemorrhage: extradural, subdural and subarachnoid.
extradural- head injury/trauma near pterion region between temporal and parietal bones
subdural- blunt trauma causing rapid acceleration-deceleration of the head
subarachnoid- ruptured berry aneurysm
27
Name two clinical features of each type of haemorrhage: extradural, subdural and subarachnoid.
extradural- seizures, CSF rhinorrhoea
subdural- headache, N&V
subarachnoid- thunderclap headache, altered consciousness
28
Define an epileptic seizure.
An epileptic seizure is a paroxysmal event in which change is behaviour, sensation and cognitive processes are caused by excessive hypersynchronous neuronal discharges in the brain.
29
Name 4 causes of epilepsy.
idiopathic
head injury
CNS infection
CNS neoplasm
30
Name 5 characteristic features of an epileptic seizure.
```
tongue biting
incontinence
transient paraesthesia
deja vu
involuntary jerky movements of limbs
```
31
Name four characteristic features of non-epileptic attacks that distinguish them from epileptic seizures.
1. last longer- upto 20 minutes
2. associated with psychosocial distress
3. dramatic motor phenomena or prolonged atonia
4. ictal crying and speaking
32
Name two types of syncope and describe the characteristic features of each.
orthostatic hypotension
vasovagal syncope
33
Define syncope.
Syncope is a transient loss of consciousness caused by transient global cerebral hypoperfusion characterised by rapid onset, short duration and spontaneous complete recovery.
34
Name the three cardinal features of Parkinson's Disease and the symptoms for each.
1. Bradykinesia- problems with doing up buttons, microphagia, shuffling gait
2. tremor- at rest, may be unilateral
3. rigidity- pain, problems with turning in bed
35
Name 3 common co-morbidities in Parkinson's Disease.
1. Depression and other psychiatric problems
2. Dementia
3. Autonomic dysfunction
36
Describe the three pharmacological strategies for the management of Parkinson’s disease.
dopamine agonists- levodopa
monoamine oxidase B inhibitor- selegiline
cathechol-O-methyltransferase inhibitor
37
Describe the characteristic features of essential tremor.
- uncontrolled shaking movements with no other symptoms
- mostly in arms and legs
- no known cause
- increasing incidence with age
- tends to occur in families
38
What are the three clinical features of normal pressure hydrocephalus?
gait abnormality
urinary incontinence
dementia
39
Define dystonia
Dystonia is a movement disorder that causes involuntary contractions of your muscles. These contractions result in twisting and repetitive movements. a state of abnormal muscle tone resulting in muscular spasm and abnormal posture
40
Describe the clinical features of Huntington’s disease.
```
chorea
behavioural disturbances
psychosis
cognitive impairment
personality change with increasing aggression
depression
bradykinesia
spasticity
clonus
```
41
Describe the clinical features of cerebellar dysfunction.
```
staccato speech
unsteadiness
dysphagia
blurred vision
nystagmus
ataxia
action tremor
dysdiadochokinesia
```
42
Name 2 causes of acquired ataxia.
paraneoplastic cerebellar dysfunction
| multisystem atrophy
43
Name 2 forms of inherited ataxia.
Friedreich's ataxia
| spinocerebellar ataxia 6
44
You are called by the paramedics to inform you that they have been called to see a 65 year old right handed man who presents with a right sided weakness, dysarthria, loss of vision and difficulty speaking. The paramedics inform you that the symptoms came on suddenly at 13:30. It is now 15:00.
What is the likely diagnosis?
What do you do next?
middle cerebral artery stroke
they are eligible for thrombolysis as it's only been an 1.5 hr since onset of symptoms, MCA means can do decompressive craniectomy is increased ICP and clot retrieval - multiple options available
45
What are the risk management strategies for stroke?
dual antiplatelet therapy
statins
antihypertensives
warfarin or NOACs for AF
46
What is amaurosis fugax?
An attack of transient and painless loss of vision in one eye that is indicative of transient retinal ischaemia usually associated with stenosis of ipsilateral carotid artery.
47
What are the first and second line medications for dementia?
1st line: acetylcholine esterase inhibitor- rivastigmine
| 2nd line; NDMA antagonist (anti-glutamate)- memantine
48
Which of the following is a likely consequence of a skull fracture?
```
subarachnoid haemorrhage
subdural haematoma
extradural haematoma
subarachnoid haematoma
cerebellar haemorrhage
```
extradural haematoma
49
What are the red flag symptoms for suspecting a brain tumour?
new headache with hx of cancer
papilloedema
cluster headache
seizure
significantly altered consciousness, memory, confusion, coordination
50
What are the predictive features of a positive temporal artery biopsy for giant cell arteritis?
```
jaw claudication
diplopia
abnormalities on palpation (absent pulse, beaded, enlarged)
raised ESR
anaemia
scalp tenderness
temporal headache
```
51
What are the mechanisms of action of antiepileptic drugs?
1. reduce pre-synaptic excitability by inhibiting voltage-gated sodium and potassium channels
2. reduce neurotransmitter release by inhibiting voltage-gated calcium channels (pregabalin)
3. target GABA receptor, transaminase and transporter.
52
What are the two pathological features of Parkinson's?
loss of dopaminergic neurones in the substantia nigra
| presence of intracellular lewy bodies in neurones
53
What gait disturbances would you expect to see in someone with Parkinson's?
shuffling, festination, unsteadiness on turning, difficulty initiating walking
54
What is bradykinesia?
slowness of initiation of voluntary movement wit progressive reduction in speed or amplitude or repetitive actions
55
What are the motor complications of late-stage PD?
1. wearing off of medication
2. on-dyskinesia: hyperkinetic when drugs work
3. off-dyskinesia: painful dystonic posturing when drugs don't work
4. freezing: unpredictable loss of mobility
56
Give three side effects of dopamine agonists.
tiredness
gambling
hypersexuality
57
What are the 3 preparations of L-dopa?
```
dispersible (morning)
standard release (day time)
slow release (night time)
```
58
What is normal pressure hydrocephalus?
It describes the condition of ventricular dilatation in the absence of increased CSF pressure on lumbar puncture, characterised by a triad of gait abnormality, urinary incontinence and dementia.
59
Where do brain metastases tend to originate from?
```
lung
breast
stomach
prostate
thyroid
kidney
```
60
What is 3rd nerve lesion/palsy?
compression of the oculomotor nerve against the petroclinoid ligament when the temporal lobe uncus herniates caudally. It leads to compression of the parasympathetic fibres causing the ipsilateral pupil to be fixed and dilated.
61
What is the Brown-Sequard syndrome?
It is damage to one half of the spinal cord (cord hemisection) resulting in ipsilateral paralysis and loss of proprioception (as these are carried by the dorsal column and corticospinal tracts which decussate in the medullary pyramids) and contralateral loss of pain and temperature (as these are carried by the spinothalamic tract which decussates immediately on entering the spinal cord at one or two segments above point of entry)
62
How do midline cerebellar lesions present?
truncal and gait ataxia, nystagmus, vertigo, vomiting, obstructive hydrocephalus
63
How do lesions of the cerebellar hemispheres present?
ipsilateral limb ataxia with intention tremor, past point and mild hypotonia.
64
What is ataxia?
It is a neurological sign consisting of a lack of muscle coordination during voluntary movements.
65
What is the main function of the cerebellum?
Coordination and precision of movements on ipsilateral side of body.
66
How does Wernicke's encephalopathy manifest?
acute confusion
ataxia
ophthalmoplegia
67
What causes Wernicke's encephalopathy?
Thiamine deficiency
68
What are the 3 ways by which CNS infections can occur?
1. direct inoculation from trauma, surgery
2. contiguous focus or haematogenous spread to choroid plexus
3. invasion via nerves (herpes simplex)
69
Name 3 contraindications to performing a lumbar puncture.
1. abnormal clotting
2. petechial back rash
3. raised ICP (check for papilloedema)
70
Where in the brain will you find dopaminergic neurones?
Substantia nigra
71
What disease causes a rapidly progressive decline and what other features would you see?
Creutzfeldt-Jacob disease
prion infection causing spongiform encephaopathy
causes rapidly fata demetia - death within 1 year
myoclonic jerks and extra-pyramidal signs
causes - sporadic, infected hosipital infection, familial, blood transfusions in 1995
72
What is Huntington's disease?
autosomal dominant disease with 100% peetrance - trinucleotide expansion repeat of CAG.
symptoms - cognitive decline --> progresses to subcortical dementia, personality change, choreinform involuntary movements, dysarthria, psychiatric disturbance.
genetic test - children must wait until old enough to decide
73
What is the pathophysilogy of HD?
```
reduced GABA (reduced inhibition)
causing dopamine hypersensitvity and increase in dopamine transmission
```
increased stimualtion at thalamus and cortex --> involuntary movements
74
Name 2 medical conditions that can cause psychiatric symptoms?
neurosyphilis - grandiosity, euphoria, mania, personality change
Wilson's disease - copper excess leading to both neuro and psych changes, liver disease and kayser-fleischer rings
75
Name a potentially reversible cause of dementia?
Normal pressure hydrocephalus
triad of - ataxia, dementia and urinary incontinence
causes - idiopathic, SAH, head trauma, meningitis
tx with ventriculoperitoneal shunt
76
What are the features of an ataxc gait?
wide-based
falls
cannot walk heel-to-toe
often worse in the dark or with eyes closed
77
What are the 2 main causes of an ataxic gait?
1. cerebellar problem
| 2. issue with proprioception
78
What are the cerebellar causes of an ataxic gait?
MS
posterior fossa tumour
alcohol
phenytoin toxicity
deficits are ipsilateral to cerebellar lesion
79
What are the features of cerebellar syndrome?
ataxia + nystagmus
80
What are the proprioceptive causes of an ataxic gait?
sensory neuropathies - low B12
| inner ear problems affecting the vestibular system
81
How do you distinguish between a cerebellar and proprioceptive cause of ataxic gait?
walk normally with eyes open, problems start when eyes closed --> proprioceptive
problems exists all the time --> cerebellar
82
What are the features and causes of a circumduction (spastic) gait?
features - stiff gait, circumduction of the legs +/- scuffing of the toe of the shoes
cause - stroke (hemiplegia)
83
What are the features and causes of a shuffling (extra-pyramidal) gait?
features - flexed posture, shuffling feet, postural instability, slow to start
cause - Parkinson's diease, PD+ syndromes, other causes of Parkinsonism such as antipsychotic medications
84
What are the causes of an antalgic gait?
AKA limping
MSK cause - painful limb
85
What are the features and causes of high stepping gait?
features - trip over often, struggle with dorsiflexion of the foot, lift ffeet high whilst walking to avoid tripping over
cause - foot srop (common peroneal nerve pasly)
86
What are the features and causes of Trendelenberg gait?
Features - unstable hip, sound side sags on Tredelenberg test
Causes - congenital hip dislocation, DDH, gluteus medius muscle weakness, superior gluteal nerve damage
87
What are the features and causes of an apraxic gait?
features - glued to the floor when attempting to walk, wide based unsteady gait with a tendency to fall (novice on ice)
causes - normal pressure hydrocephalus, multi-infarct states, Alzheimer's disease
88
How does the onset of an episode of weakness help you assess the cause of it?
Sudden onset - likely to be a vascular event
medium onset - likely to be related to demyelination
insidious onset - slow-growing tumours etc
89
What are the patterns and distributions of muscle weakness?
1. proximal weakness - muscle problem - hair, chair, stairs - struggle to do things close to their trunk
2. distal weakness - nerve problem - nueropathy starts distally and works its way up - glove and stocking distribution
3. symmetrical - genetic or metabolic cause eg. diabetes, muscular dystrophy
4. asymmetrical - vasculitis or inflammatory
5. mononeuropathy - entrapment
6. polyneuropathy - systemic like diabetes
90
What are the features of peripheral neuropathy?
chronic and slowly progressive
length-dependent
sensnory, motor or both
glove and stocking distribution
91
How does mononeuritis multiplex present and what are some causes?
individually erves picked off randomly - wrist drop, leg numbness, foot drop
```
subacute presentation (months)
inflammatory/immune-mediated
```
causes - inflammation of the vasa nervorum can block off the blood supply to the nerve causing sudden deficit
vasculitis (Wegner's, PAN, RA), sarcoidosis
92
Give some examples of entrapment mononeuropathies.
Median nerve at wrist = carpal tunnel syndrome
ulnar nerve at elbow
radial nerve at axilla
common peroneal nerve in leg
93
What causes a myasthenic crisis?
infection
natural part of the disease
under or overdosing of medication
94
How should a myasthenic crisis be treated?
urgent neuro review
monitor breathing - serial FVC measurements
anaesthetic review
95
How does muscular dystrophy present and what clinical test can be done to demonstrate it?
presents in childhood with proximal muscle weakness
can have bulky muscles at first = pseudohypertrophy
then muscle wasting occurs
scoliosis is prominent later on
Gower test = positive
96
How is myopathy investigated?
creatinine kinase
EMG
ESR, CRP
+/- genetic (DMD, Becker)
+/- biopsy
97
What is the definition of coma/brain death?
unarousable unresponsiveness
98
What are the 3 domains used in assessing the Glasgow Coma Scale (GCS)?
1. best eye opening response
2. best verbal response
3. best pain response
99
What are the 4 levels of best eye opening response?
1. spontaenously
2. to speech
3. to pain
4. None
100
What are the 5 levels of best verbal response?
1. orientated in time/place/person
2. confused
3. inappropriate words
4. incomprehensible sounds
5. none
101
What are the 6 levels of best pain response?
1. obeys commands
2. localises pain
3. normal flexion to pain (aka withdraws from pain)
4. abnormal flexion to pain (decorticate response)
5. extends to pain - decerebrate response
6. none
102
What causes a fixed dilated pupil?
3rd nerve palsy
occulomotor nerve comes out of the brain stem and goes over the apex of the petrous part of the temporal bone as goes through the cavernous sinus to supply the eye. This means it's susceptible to being damaged when the brain is swollen, bleeding, trauma, etc.
parasympathetic fibres' job is to constrict the pupil so when they are damaged the pupil is fixed in the dilated position.
103
What is another differential for fixed dilated pupil?
blind eye
104
What are some metabolic causes of coma?
drugs, poisoning - alcohol, tricyclics, carbon monoxide
hypoglycaemia
hyperglycaemia - ketoacidotic or HONK (hyperglycaemic hyperosmotic non-ketotic coma)
hypoxia
CO2 narcosis (COPD) - when people are given too much oxygen, the hypoxic drive to breathe slows down, which means CO2 builds up and can lead to lethal hypercapnia
Septicaemia, hypothermia, myxoedema, Addisonian crisis, hepatic/uraemia encephalopathy due to kidney or liver failure
105
What are some neurological causes of coma?
trauma
infection - meningitis, encephalitis, HSV
vascular - stroke, SAH, hyptertensive encephalopathy
epilepsy - non-convulsive status epilepticus, post-ictal state
106
How is the unconscious patient managed?
ABCDE
IV access
stabilise cervical spine (esp in trauma)
control any seizures - phenytoin
tx potential causes - IV glucose, thiamine, naloxone if pinpoint pupils
brief collateral hx and exam
vital signs and pupils checked often
investigtions - blood, cultures, CXR, CT head
continually re-assess and plan investigations
107
What is the definition of vertigo?
An illusion of movement, often rotary, of the patient or their surroundings
spinning.tilting.veering sideways feeling as if being pushed or pulled
always worse with movement
108
What are the causes of vertigo?
motion sickness
alcohol intoxication
benign paroxysmal positional vertigo - disturbance of crystals in the ear, worse when turning head or rolling over in bed
acute labyrinthitis - severe vertigo, acute onset, usually viral adn settles down
Meniere's disease - triad of vertigo, hearing loss and tinnitus - abnormal fluctuations in endolymphatic fluid, attacks that come and go
Ototoxicity - aminglycoside antibiotics (gentamicin), thiazide diuretics, lithium
Acoustic neuroma - unilateral hearing loss and vertigo causes by Scwannoma in the brain
Traumatic damage involving petrous part of temporal boe
herpes zoster around the external acoustic meatus
109
Which medications are associated with ototoxicity that can present as vertigo?
aminogylcoside antibiotics like gentamicin
thiazide diuretics
lithium
110
What are some features in the history which would point you away from a diagnosis of vertigo?
feeling faint
light-headedness
loss of awareness during attacks
111
How would you investigate vertigo?
hx
tilt table test
MRI scan if suspecting acoustic neuroma
112
How is vertigo managed?
symptomatic tx for dizzinessin acute labyrinthitis - prochlorperazine (stemil)
antihistamines can help with dizziness - cinnarizine
113
How does a headache due to venous sinus thrombosis present?
subacute or sudden headache
papilloedema - do fundoscopy!!
114
What are the symptoms of a sinusitis headache?
dull, constan ache over frontal/maxillary sinuses, may also be felt right in the middle of the nose/forehead
tenderness
post-nasal drip
pain worse when leaning forwards
common with coryza
pain lasts 1-2 weeks
115
How does acute glaucoma present?
elderly, long-sighted people
constant aching pain develops rapidly around one eye, radiating to the forehead
symptoms - markedly reduced vision, visual halos, nausea/vomiting
signs - red congested eye, cloudy cornea, dilated non-responsive pupil (may be oval shaped), decreased acuity
116
What sort of things can precipitate acute glaucoma?
dilating eye-drops
emotional upset
sitting in the dark (cinema)
117
How do you treat acute glaucoma?
immediate expert help
IV acetazolamide - a carbonic anhydrase inhibitor
118
How are migraines treated and prevented?
Tx - NSAID + triptan or paracetamol + triptan
Prophylaxis - topiramate or propranolol
119
How are cluster headaches treated?
acute attack - 100% oxygen for 15 mins + sumatriptan SC
prophylaxis - verapamil
120
How is trigeminal neuralgia treated?
Carbamazepine
121
What are the clinical features and treatment of giant cell arteritis?
exclude in all >50 with headache lasting a few weeks
tender, thickened, pulseless temporal arteries
jaw cluadication, scalp tenderness
ESR>40
tx - stat high dose methylprednisolone
122
What are the main signs of Parkinson's disease?
1. bradykinesia (cardinal sign)
2. rigidity
3. pill-rolling resting tremor
4. shuffling gait
5. loss of postural reflexes
123
What are some red flag symptoms which may lead you to believe that it's not PD but instead a PD+ syndrome?
Early falls
ealy cognitive decline
early bladder and bowel dysfunction
both sides affected equally
124
What are the 4 PD+ syndromes?
1. progressive supranuclear palsy (PSP)
2. cortico-basal degeneration
3. Multi-system atrophy
4. Lewy body dementia
125
What are the features of progressive supranuclear palsy?
```
early falls
early cognitive impairment
occurs above the nuclei of C3, 4 and 6
difficulty moving the eyes
ocular cephalic reflex will be present (caused by supranuclear issue) they tilt/turn their head to look at things rather than moving their eyes
```
126
What are the features of multi-system atrophy?
early bladder and bowel dysfunction
| autonomic involvement i.e. casuing postural hypotension and falls
127
What are the features of Lewy Body dementia?
early visual hallucinations
clouding of consciousness
sleep behaviour disorder
128
What are the extra features of cortico-basal degeneration?
early myoclonic jerks
apraxia
agnosia
alien limb
129
What are the 3 types of tremor and what can cause them?
1. intention - cerebellar issue
2. resting - PD
3. Postural - anxiety, increased adrenaline, slabutamol, valproate, lithium, benign essential tremor
130
What are some neurological causes of altered sensation?
MS
peripheral neuropathy due to DM
GBS - ascending paralysis and numbness
Spinal cord compression - legs, saddle anaesthesia
131
What are some causes of blackouts/LOC?
```
vasovagal - neruocardiogenic syncope
situational syncope
carotid sinus syncope
epilepsy
NEA
drop attacks - cataplexy, hydrocephalus
hypoglycaemia
orthostatic hypertension
anxiety - hyperventilation
factitious blackouts
choking
```
132
What are the vascular causes of unilateral vision loss?
amaurosis fugax
central retinal artery occlusion
central retinal vein occlusion
anterior ischaemic optic neuropathy (think GCA)
stroke affecting the occipital lobe
GCA
vitreous haemorrhage - diabetic retinopathy, CRVO, macular degeneration
133
What are the non-vascular causes of unilateral vision loss?
```
optic neuritis (MS)
retinal detachment - flashes/floaters with decreased vision
acute angle closure glaucoma - painul red eye, N&V
```
134
What investigations would you do in someone presenting with unilateral vision loss?
full eye exam - movements, acuity, fundoscopy
MRI
VEP - visual evoked potentials - helps dx optic neuritis
Fluorescecin angiography - central retinal vein occlusion
tonometry - measures intra-ocular pressure (glaucoma)
USS - ocular USS to look for vitreous haemorrhage/retinal detachment
LP - shows oligoclonal bands in MS
135
What can cause spinal cord compression?
```
malignancy or benign mass
infection (epidural abscess)
disc prolapse
haematoma (esp if on warfarin)
myeloma - rule out in anyone >50 with back pain
```
136
Secondary malignancy is the most common cause of cord compression. What are the 5 cancers which spread to the bone?
```
Breast
Thyroid
Prostate
Kidney
Lung
```
137
What is the ddx for cord compression?
```
transverse myelitis
MS
trauma
dissecting aneurysm
GBS
```
138
What causes dyarthria?
cerebellar disease
extrapyramidal disease i.e. stroke
pseudobulbar palsy - MND, severe MS
bulbar palsy - facial nerve palsy, GBS, MND
139
What is the difference between bulbar and pseudobulbar palsy?
pseudo - affects upper motor neurones
bulbar - affects te lower motor neurone (of C9, 10, 11, 12)
pseudobulbar dysarthria - slow, nasal, effortful "hot potato" voice
bulbar dysarthria - nasal speech due to paralysis of the palate
140
What causes dysphonia (reduced volume of speech due to weakness of respiratory muscles)?
Myasthenia gravis
Gullain Barre Syndrome
Parkinson's disease (dysphonia + dysarthria)
141
How does Broca's dysphasia differ from Wernicke's dysphasia?
Broca's - expressive - non-fluent speech produced with effort and frustration, malformed words, reading and writing are impaired but comprehension is intact.
Wernicke's - receptive - empty, fluent speech, cannot respond to requests. Reading, writing and comprehension are impaired, replies are inappropriate
142
What are some neurological disorders that can cause difficulty swallowing?
```
myasthenia gravis
bulbar palsy
pseudobulbar palsy
syringobulbia (fluid filled cavities in the brain stem)
bulbar poliomyelitis
wilson's disease
Parkinson's disease
stroke
```
143
What are some non-neurological conditions which cause swallowing difficulty?
```
cancer
benign strictures
pharyngeal pouch
achalasia
oesophageal spasm
systemic sclerosis - scleroderma
```
144
What drug can help in the management of urinary retention?
Tamsulosin - alpha-blocker - relaxes muscle in the bladder neck
145
What are the causes of optic neuritis?
MS
infection - lyme, syphilis, HIV
B12 deficiency
arteritis (GCA)
146
How does optic neuritis present?
reduced visual acuity over a few days
pain on moving eye
exacerbated by heat or on exercise
relative afferent pupillary defect
dyschromatopsia - abnormal perception of colours
recovery of vision usually occurs within 6 weeks
147
What are the diagnostic criteria for MS?
2 CNS lesions
with symptoms lasting >24 hours
```
disseminated in time (>1 month apart)
and space (clinically or on MRI)
```
MCDONALD CRITERIA
148
What are the 4 subtypes of MS?
1. relapsing remitting
2. primary progressive
3. secondary progressive
4. benign
149
What are the typical symptoms of MS?
- visual loss (optic neuritis)
- pyramidal weakness, spastic paraparesis
- sensory disturbance
- cerebellar symptoms - nystagmus, vertigo, tremor, ataxia, dysarthria
- bladder involvement
- sexual dysfunction
- fatigue
- cognitive impairment
150
What are the 2 named signs seen in MS?
- Lhermitte's sign - electric shock sensation down trunk and limbs when they flex their neck
- Uhtoff's phenomenon - symptoms worse in hot bath/hot environment
151
What is internuclear ophthalmoplegia?
decreased adduction of the ipsilateral eye
nystagmus on abduction of contralateral eye
lesion is in medial longitudinal geniculus
152
What will you see in the results of a lumbar puncture from someone with MS?
oligoclonal bands
153
How is MS treated?
methylprednisolone to tx relapses
interferons - IFN-1beta and IFN-1alpha - used to maintain remissions
monoclonal antibodies
154
What are some side effects of interferons?
- depression
- flu symptoms
- miscarriage
155
What is the definition of epilepsy?
continuing tendency to have seizures
156
How long does a seizure normally last?
30-120 seconds
157
What is the ddx for epileptic seizures?
```
postural syncope, cardiogenic syncope
TIA
Migraine
Hypoglycaemia
NEAD
Dystonia
Vertigo
```
158
How is status epilepticus managed?
Benzos followed by AEDs:
- 2 doses of benzo e.g. 2 x lorazepam
- then IV valproate or phenytoin or phenobarbitol
can be stopped with general anaesthesia if all else fails but high risk procedure
159
When should someone be seen by a specialist after a TIA?
Within 24 hrs
160
What are the features of a total anterior circulation infarct and which blood vessels does this involve?
problem with internal carotid artery and therefore MCA, ACA and PCA
causes cognitive imapirment, speech torubles, unilateral weakness/incooridnation, unilateral numbness or loss of sensation, dysarthra, unilateral visual loss or loss in 1 visual field.
161
What are the features of a lacunar stroke?
weakness or paralysis of the face, arm, leg, foot or toes - hemiplegia or hemiparesis
sudden numbness
difficulty walking
difficulty speaking
clumsiness of hand or arm
weakness or paralysis of eye muscles
162
What are the clinical features of a posterior circulation infarct and which vessels are involved?
vertigo
nausea and vomiting
```
imbalance
unilateral limb weakness
slurred speech
doubl vision
headache
gait ataxia, limb ataxia, nystagmus
```
basilar or vertebral arteries
163
What is the most important investigation to do if stroke is suspected?
CT head
164
What are some early CT signs of stroke?
hyperdense MCA
loss of grey white matter differentiation and sulcal effacement - cortical infarction
hypodense basal ganglia (deep vessel infarct)
165
What are some primary prevention methods for stroke?
smoking cessation
control hypertension
control hypercholesterolaemia
control diabetes
encourage active lifestyle and weight loss
reduce alcohol intake
in pts with AF - CHADSVASc to assess need for anticoagulation vs HASBLED for risk of bleeding.
166
What are some secondary prevention measures for stroke after TIA?
investigations - 72 hours ECG to look for paroxysmal AF, carotid doppler to look for carotid stenosis, BP, echo to look for endocarditis or patent foramen ovale, if neck pain investigate for dissection with CTA/MRA
drugs - aspirin, clopidogrel, antihypertensives, statins, diatary control and diabetes mgmt
physiotherapy and MDT approach to rehab
167
How is an acute ischaemic stroke managed?
- aspirin 300mg for 2 weeks
- potential for thrombolysis within 4.5 hours of onset
- seen by specialist within 24 hours
- control BP
- swallow assessment and supportive care
- FBC, LFT, U&E
168
What is the time cut-off for thrombolysis in ischaemic strokes?
4.5 hours from onset of symptoms
169
What medication is given for thrombolysis?
alteplase
streptokinase
170
What needs to be done within 24 hours of thrombolysis therapy?
second CT scan to check if they've had a bleed
171
What are some contraindications of thrombolysis?
- on anticoagulants (can if on warfarin and below 1.7)
- haemorrhagic stroke
- >6 hours after onset of symptoms
- recent surgery or GI bleed
- if active cancer
- hypertension cut off 185/110
172
What are some risks for thrombolysis?
haemorrhage - 1 in 20
| reaction to rTPA
173
What are some complications of stroke?
```
raised ICP - cerebral oedema, haemorrhage (signs = hypertension, new neuro signs, reduced GCS)
aspiration
pneumonia
VTE due to immobility
pressure sores
depression
cognitive impairment
long-term disability
```
174
What causes SAH?
rupture of berry aneurysms
AVM
unknown
175
What are some medical conditions associated with berry aneurysms?
polycystic kidneys
coarctation of the aorta
Ehlers-Danlos syndrome - hypermobile joints and skin elasticity
176
What are some risk factors for SAH?
```
smoking
alcohol misuse
hypertension
bleeding disorders
infected aneurysm
family hx
```
177
What are the symptoms of SAH?
```
sudden onset thunderclap headache
vomiting
collapse
seizures
coma
may have experienced a warning sentinel headache earlier on
```
178
What signs are associated with SAH?
neck stiffness
photophobia
Kernig's sign (takes 6 hrs to develop) - painful extension of knee on flexion of hip
retinal haemorrhages
179
What investigations should be done for suspected SAH?
CT head - white star shaped sign on CT - ventricles look white instead of black
LP if CT negative and no contraindications >12 hrs after headache onset
CT angiogram to locate aneurysms before surgical procedure
180
What would be seen on an LP for SAH?
Xanthochromia (yellow CSF) - confirms SAH
181
How is SAH treated?
immediate neurosurgery referral
nimodipine - calcium channel blocker - for 3 weeks to reduce vasospasm and cerebral ischaemia
endovascular coiling
surgical clipping
182
What are some complications of SAH?
re-bleeding
cerebral ischaemia due to vasospasm
hydrocephalus
hyponatraemia
183
How does an extradural bleed look on CT?
convex shape
lentiform lesion that does not cross the suture lines as the periosteum crosses through the suture continuous with the outer periosteal layer.
184
How does a subdural bleed look on CT?
concave shape - from front to back
crescentic lesion with internal margin paralleling the cortical margin of the adjacent brain.
185
What is a subdural haemorrhage?
bleed in between dura and arachnoid mater
venous bleeds from the dural venous sinuses
consider in all whose conscious level fluctuates
186
What is the most common cause of subdural haemorrhage?
truama - often forgotten/minor trauma from up to 9 months ago
result of acceleration-deceleration injury
particularly in elderly people, alcoholics and shaken babies due to smaller brain size
187
What are the symptoms of a subdural haemorrhage?
gradually reducing levels of consciousness
insidious onset of physical/intellectual slowing
sleepiness
headache
personality change
unsteadiness
188
What are some signs of subdural haemorrhage?
raised ICP
seizures
localising neuro symptoms - unequal pupils, hemiparesis
189
How is a subdural haemorrhage investigated?
CT scan showing concave bleed
| half-moon/crescent shape
190
How is a subdural haemorrhage treated?
evacuate the bleed - Burr holes, craniotomy
191
Where does an extra-dural haemorrhage occur?
happens in the space between the dura and the skull
usually arterial - middle meningeal artery
can be venous bleed if fractures disrupt venous sinuses
192
What is a big red-flag symptom of extradural haemorrhage?
lucid interval after head injury before becoming drowsy
| then conscious levels fall and are slow to improve
193
What is often the cause of an extradural haemorrhage?
fractured temporal or parietal bone at a place caled the pterion causing laceration of the middle meningeal artery and vein
typically after trauma to the temple
194
Where does an extra-dural haemorrhage occur?
happens in the space between the dura and the skull
usually arterial - middle meningeal artery
can be venous bleed if fractures disrupt venous sinuses
195
How do extradural haemorrhages present?
deteriorating consciousness after a head innjury that initially produced no LOC
lucid interval
headache, vomiting, confusion, fits, hemiparesis with brisk reflexes and up-going plantars
if bleeding continues - ipsilateral pupil dilates, coma, bilateral limb weakness, breathing becomes deep and irregular due to brainstem compression
196
What is the ddx for extradural haemorrhage?
epilepsy
carotid artery dissection
carbon monoxide poisoning
197
What investigations should be done for a suspected extradural haemorrhage?
CT head - convex shape
X-ray skull
LP CONTRAINDICATED
198
What are some signs seen in meningitis?
neck stiffness
photophobia
positive Kernig's sign
positive Brudzinski's sign
non-blanching petechial rash - later sign of meningococcal septicaemia
199
What investigations would you do for suspected meningitis?
blood cultures
blood glucose
LP - MC&S, glucose, virology
thorat swab looking for meningococcus
200
What LP findings would you see in someone with a bacterial meningitis?
LOW glucose
raised polymorphs - neutrophils, basophils and eosinophils
raised protein
cloudy appearance of fluid
201
What LP findings would you in a viral meningitis?
raised lymphocytes
normal/slightly low glucose
normal protein
clear appearance
202
How is meningitis treated?
as soon as suspected - stat dose of IM benzylpenicillin before admitting to hospital
in hospital - take cultures and treat BEFORE cultures come back with IV cefotaxime
if viral - IV acyclovir
adjust tx based on sensitivities from culture result
prophylaxis for close contacts with rifampicin
203
What type of presentation should always raise suspicion of encephalitis?
whenever odd behaviour, decreased consciousness, focal neurology or a seizure
is preceded by an infectious prodrome - pyrexia, rash, lymphadenopathy, sores, conjunctivitis, meningeal signs
204
What are the viral causes of encephalitis?
```
HSV
CMV
EBV
VZV
HIV
mealses
mumps
rabies
West Nile virus
Tick-borne encephalitis
```
205
How is suspected encephalitis investigated?
blood cultures ans serology for viral PCR
CT
LP
EEG
206
How should encephalitis be treated?
start acyclovir within 30 mins of pt arriving if suspected encephalitis
suppotive therapy in HDU or ICU if needed
symptomatic treatment - phenytoin for seizures
207
What is the most common cell type seen in primary brain tumours?
glial cell origin - 90% astrocytomas, 5% oligodendrocytoma
208
Which types of cancer can spread to the brain?
```
Lung
breast
melanoma
GI tract
kidney
```
209
What is the triad of presenting symptoms for brain tumours?
symptoms of raised ICP
loss of function/focal neurological deficit
seizures/epilepsy
210
What are the symptoms of raised ICP?
```
headache worse in the morning
worse when laying down/leaning forwards, coughing
drowsiness
confusion
comiting
papilloedema on fundoscopy
```
211
What is the gold standard investigation for a suspected brain tumour?
MRI brain
212
What is an important side effect of giving dexamethasone to reduce brain swelling in mgmt of tumours?
insomnia so give in the morning
213
What are some medications that can cause Parkinson's disease-like symptoms and how are they managed?
Haloperidol (dopamine blockade) and metoclopramide/domperidone (anti-emetics)
manged with anticholinergics like oxybutinin
214
How is PD managed?
education, exercise, physio
dopaine replacement - L-dopa (plus carbidopa - stops levodopa breaking down into dopamine outisde the brain to reduce side effects)
dopamine agonists - bromocriptine
MAO-B inhibitors - rasigiline, selegiline - inhibit dopamine breakdown
DBS
215
What are some side effects of L-dopa?
nausea
dyskinesia - chorea-like movements
effectiveness decreases over time
216
Which PD medication is most associated with gambling addiction?
dopamine agonists - cabergoline, bromocriptine
217
What are the main causes of GBS?
usually triggered by infection esp campylobacter jejuni, EBV and CMV
218
How does GBS present?
progressive onset of limb weakness/paralysis and peripheral neuropathy
stats distally and works its way up
usually symmetrical
reflexes lost early in illness
often sensory symptoms
can involve facial muscles --> bulbar palsy
respiratory failure
219
What is Miller-Fisher syndrome?
related varaint to GBS that affects CNS and eye muscles
characterised by ophthalmoplegia (eye muscle paralysis) and ataxia
220
Ddx for GBS?
other causes of neuromuscular paralysis - hypokalaemia, polymyositis, botulism, poliomyelitis
MRI spineto exclude transverse myelitis or cord compression
221
What investigations need to be done for GBS?
LP - CSF - protein elevated, normal glucose, normal cell count
NCS - show slowing of motor conduction
monitor serial FVC
222
How is GBS treated?
supportive therapy
IVIG (CI - IgA deficiency --> allergic reaction)
plasmapheresis
DVT prophylaxis
223
What are the 2 main complications of GBS?
resp failure
| prolonged disability
224
What is motor neurone disease?
UMN + LMN lesions
relentless and unexplained destruction of upper motor neurones and anterior horn cells in the brain and spinal cord
225
What are the symptoms of MND?
weakness - progressive
dysarthria
dysphagia
NO SENSORY SYMPTOMS
226
What are the signs associated with MND?
fasciculations (esp in tongue) - LMN
UMN - hypotonia, brisk reflexes
muscle wasting
227
Ddx for MND?
cervical spine lesion
228
What is the diagnostic tool used for MND?
El-Escorial diagnostic criteria
229
How is MND treated?
no curative tx
riluzole - neuroprotective glutamate antagonist
supportive - ventilation, NG feeding, antidepressants
palliative care
230
What are some complications of MND?
UTI
pneumonia
constipation
pressure sores
231
What type of dementia is associated with MND?
frontotemporal dementia - Pick's disease
232
What is myasthenia gravis?
autoimmune condition
anti-ACH receptor antibodies
associated with thymic hyperplasia
233
How does MG present?
```
Eye dropping (ptosis)
weakness and fatiguability - ocular, bulbar and proximal limb muslces
```
struggle with - hair, stairs chair, speech, face and neck weakness, mastication and swallowing, breathing
eventual muscle atrophy
234
How do you investigate MG?
anti-AChR antibodies
anti-MuSK antibodies
mediastinal CT or MRI to look for thymoma and lung cancer (Lambert-Eaton syndrome - small cell lung cancer)
repetitive nerve stimulation shows reduction
serial monitoring of resp function
235
What medications can worsen symptoms of MG?
```
antibiotics
CCBs
beta blockers
lithium
statins
```
236
How do you treat MG?
anti-cholinesterases - pyridostigmine or rivastigmine
immune suppress - steroids, azathioprine
thymectomy - in pts with thymoma or anti-AChR +ve disease
plasmapheresis for severe relapsing cases
237
What is a myasthenic criris and what can trigger it?
difficulty breathing or speaking, increased WOB with intercostal recessions, tiredness, trouble swallowing
triggers - infection, thyroid disease, monthyl periods/pregnancy, trauma/srugery, change in meds
tx - ventilation anticholinesterases, immunosuppressives, IVIG, IV fluids, plasmapheresis
238
What type of inheritance pattern is seen in Duchenne muscular dystrophy?
X-linked recessive
no male-to-male transmission
females are carriers but not affected
239
How does Duchenne's present?
proximal muscle weakness - Gower's sign
delayed milestones
suspect in all boys not walking by 18 months
240
Who is the typical patient you'd expect to see presenting with idiopathic intracranial hypertension?
obese woman
narrowed visual fields
blurred vision +/- diplopia
6th nerve palsy
enlarged blind spot if papilloedema present
consciousness and cognition preserved
241
What causes idiopathic intracranial hypertension?
unknown
secondary to venous sinus thrombosis
drugs - tetracycline, nitrofurantoin, vitamin A, isoreinoin (roccutane), danazol, somatropin
242
What investigations can be done for IIH?
CT head - shows no SOL
| LP - increased opening pressure
243
How can IIH be treated?
weight loss - firstline
acetozolamide (also used for glaucoma)
topiramate can als be used + helps with wt loss
therapeutic LP
surgery - optic nerve sheath decompression and fenestration to prevent optic nerve damage
lumboperitoneal or ventriculoperitoneal shunt
244
What is hydrocephalus and what are the different subtypes?
hydrocephalus - abnormal build-up of CSF around the brain
Subtypes -
1. congenital hydrocephalus - born with excess fluid on the brain
2. acquired - after birth, due to injury or illness
3. normla pressure hydrocephalus - develops in >60 yr olds
245
What causes congenital hydrocephalus?
spina bifida
| infection during pregnanct - mumps, rubella
246
What are the symptoms of normal pressure hydrocephalus?
abnormal gait
urinary incontinence
dementia
247
What are the general symptoms of hydrocephalus?
headache
vomiting
blurred vision
difficulty walking
248
How is hydrocephalus investigated?
CT and MRI scans to diagnose congenital and acquired hydrocephalus
NPH diagnostic criteria = walking, mental ability and bladder control
249
How is hydrocephalus managed?
ventriculoperitoneal shunt - sugically implanted into the brain to drain away excess fluid
endoscopic third ventriculotomy - alternative to shunt surgery - home made in the floor of the third ventricle to allow trapped CSF to escape to the surface where it can be re-absorbed
complications - shunt can become blocked or infected
