Neurology Flashcards
1
Q
What is a TIA
A
Transient neurological dysfunction secondary to ischaemia without infarction
2
Q
What is a crescendo TIA?
A
2 or more TIAs in one week
3
Q
List some stroke RF
A
CVD - angina, MI, PVD Previous stroke/TIA AF Carotid artery disease HTN DM Smoking Vasculitis Thrombophilia COCP
4
Q
Describe the management of stroke
A
Head CT to exclude haemorrhage
Exclude hypoglycaemia
Do not try to lower BP - risk of hypoperfusion
Aspirin 300mg STAT and continued for 2 weeks
Thrombolysis with Alteplase <4.5hrs symptom onset
Thrombectomy <24hrs
5
Q
Describe the secondary prevention of stroke
A
Treat modifiable RF
Clopidogrel 75mg OD (or dipyridamole 200mg BD)
Atorvastatin 80mg
Carotid endarterectomy fi stenosis
6
Q
What is a cerebral venous sinus thrombosis
A
Blood clot in veins that drain the brain resulting in venous congestion and tissue hypoxia
7
Q
What are the two types of haemorrhage
A
Intracerebral - bleeding into the brain secondary to ruptured vessel - interventricular or intraparenchymal
Subarachnoid haemorrhage - bleeding outside the brain between pia mater and arachnoid mater
8
Q
Describe a total anterior circulation stroke
A
All 3:
Unilateral weakness (and/or sensory deficit)
Homonymous hemianopia
Higher cerebral dysfunction
9
Q
Describe partial anterior circulation stroke
A
2 of the TACS
10
Q
Describe posterior circulation stroke
A
Cranial nerve palsy with a contralateral motor/sensory deficit
Bilateral motor or sensory deficit
Conjugate eye movement disorder
Isolated homonymous hemianopia
11
Q
Describe lacunar stroke
A
Subcortical stroke that occurs secondary to small vessel disease
No loss of higher cerebral function
Pure motor
Pure sensory
Sensori-motor
Ataxic hemiparesis
12
Q
List the layers under the skull
A
Dura mater
Arachnoid mater
Pia mater
13
Q
List some risk factors of intracranial bleeds
A
Head injury HTN Aneurysms Ischaemic stroke Brain tumour Anticoagulants
14
Q
Describe the presentation of an intracerebral bleed
A
Sudden onset headache Seizures Weakness Vomiting Reduced consciousness Sudden onset neurological symptoms
15
Q
Describe the glasgow coma scale
A
Max = 15 Min = 3
Eyes
- spontaenous = 4
- speech = 3
- Pain = 2
- None = 1
Verbal response
- Orientated = 5
- Confused conversation = 4
- Inappropriate words = 3
- Incomprehensible sounds = 2
- None = 1
Motor response
- obeys commands = 6
- Localises pain = 5
- Normal flexion = 4
- Abnormal flexion = 3
- Extends = 2
- None = 1
16
Q
Describe subdural haemorrhage
A
Rupture of bridging veins
Between dura and arachnoid mater
Crescent shape on CT
Not limited by cranial sutures - cross over suture lines
More frequent in eldely and alcoholic patients
17
Q
Describe extradural haemorrhage
A
Rupture of the middle meningeal artery in the temporo-parietal region - can be associated with fracture of the temporal bone
Between the skull and dura mater
Bi-convex shape and are limited by cranial sutures (do not cross the suture lines)
Young patient with traumatic head injury and ongoing headache
Brief period of improved neurological symptoms and consciousness followed by a rapid decline over hours as the haematoma gets large enough to compress the intracranial contents
18
Q
Describe intracerebral haemorrhage
A
Bleeding into brain tissue
Can occur spontaneously or as the result of bleeding into an ischaemic infarct or tumour or rupture of aneurysm
19
Q
Describe a subarachnoid haemorrhage
A
Bleeding into the subarachnoid space, where the CSF is located between the pia mater and arachnoid membrane - usually the result of cerebral aneurysm
Sudden onset occipital headache that occurs after strenuous activity such as weight lifting or sex. Thunderclap headache
Associated with cocaine and sickle cell disease
20
Q
Describe the management of intracerebral bleeds
A
Immediate head CT
Check FBC and clotting
Admit to specialist stroke unit
Discuss with neurosurgical centre to consider surgical treatment
Consider intubation, ventilation and ICU care if they have reduced consciousness
Correct severe hypertension but avoid hypotension
Correct any clotting abnormality
LP 12hrs after onset of symptoms in suspected subarachnoid haemorrhage and normal non contrast CT head
21
Q
What drug is given to people with subarachnoid haemorrhage which prevents vasospasm?
A
Nimodipine (21 day course - dihydropyridine calcium channel blocker)
22
Q
What is a common consequence of subarachnoid haemorrhage
A
Syndrome of inappropriate ADH (hyponatremia)
23
Q
List the risk factors of subarachnoid haemorrhage
A
HTN Smoking Excessive alcohol consumption Cocaine use FH Black and female patients Age 45-70 Sickle cell anaemia Connective tissue disorders - Marfans syndrome or Ehlers Danlos Neurofibromatosis Autosomal dominant polycystic kidney disease
24
Q
List the investigations in subarachnoid haemorrhage
A
CT head is first line - blood in subarachnoid space has hyper attenuation
LP is used to collect a sample of CSF if CT head negative - red cell count will be raised and xanthochromia (yellow colour of CSF caused by bilirubin)
Angiography - used to confirm the source of bleeding
25
Describe the management of subarachnoid haemorrhage
Specialist neurosurgical unit
Surgical intervention - coiling, clipping
Nimodipine - prevent vasospasm
Lumbar puncture or insertion of shunt if hydrocephalus
Antiepileptic medications - seziures
26
Describe the presentation of pituitary apoplexy
Sudden onset headache
Visual field defect
Evidence of pituitary insufficiency
27
What is multiple sclerosis
Chronic and progressive demyelination of myelinated neurones in the CNS - autoimmune condition
28
Who does MS affect
More commonly women <50 yo
29
How does pregnancy and post partum affect MS symptoms
Improves them
30
Name the two types of myelin
Schwann cells - PNS
| Oligodendrocytes - CNS
31
Which type of myelin does MS typically affect
CNS - oligodendrocytes
32
What is a characteristic feature of MS lesions?
Vary in their location over time - different nerves are affected at different points in time
Disseminated in time and space
33
What are the causes of MS
```
Combination of
Multiple genes
Epstein Barr virus
Low vit D
Smoking
Obesity
```
34
What causes MS symptoms to resolve in early disease?
Re-myelination
35
Describe symptom progression in the first presentation of MS
Progress over more than 24hrs and last days to weeks then improve
36
List some ways MS can present
Optic neuritis
| Eye movement abnormalities
37
What occurs in a 6th CN palsy
Internuclear ophthalmoplegia - eyes do not move together
Conjugate lateral gaze disorder - when gazing to the direction of the affected eye, the affected eye will not be able to abduct
Double vision
38
Describe optic neuritis
```
Loss of vision in one eye
Central scotoma - enlarged blind spot
Pain on eye movement
Impaired colour vision
Relative afferent pupillary defect
```
39
What is Lhermitte's sign
Electric shock sensation that travels down the spine and into the limbs when flexing the neck - indicates disease in the cervical spinal cord and dorsal column
Caused by stretching the demyelinated dorsal column
40
Give some examples of focal weakness in MS
Bells palsy
Horner's syndrome
Limb paralysis
Incontinence
41
List some focal sensory symptoms in MS
Trigeminal neuralgia
Numbness
Paraesthesia (pins and needles)
42
Describe ataxia in MS
Problem with coordination
Sensory ataxia - loss of proprioceptive sense
Cerebellar ataxia - problem coordinating movement
43
How do you test for sensory ataxia
Romberg's test
44
What is psuedoathetosis
Writhing movement due to loss of proprioception - sensory ataxia
45
What is meant by clinically isolated syndrome in MS
MS cannot be diagnosed on one episode as the lesions have not be disseminated in time and space
Patients may never have another episode or develop MS - of lesions are seen on MRI then more likely to progress to MS
46
Describe relapsing-remitting MS
Most common pattern at initial diagnosis
Characterised by episodes of disease and neurological symptoms followed by recovery
Active - new symptoms are developing or new lesions are on MRI
Worsening - overall worsening of disability over time
47
Describe secondary progressive MS
Where there was relapsing remitting at first but now there is progressive worsening of symptoms with incomplete remissions
Symptoms have become more and more permanent
Can be classified based on active and/or progressing
48
Describe primary progressive MS
Worsening of disease and symptoms from disease onset without remissions and relapses
49
How is MS diagnosed
MRI scan - typical lesion
| Lumbar puncture - oligoclonal bands in CSF
50
List some conditions which cause optic neuritis
```
Sarcoidosis
SLE
DM
Syphilis
Measles
Mumps
Lyme disease
```
51
How should optic neuritis be managed
Ophthalmology urgent review
2-6 weeks for recovery
Steroids
52
How is MS managed?
MDT
Disease modifying drugs
Biologic therapy
Relapses treated with Methylprednisolone - 500mg PO OD for 5 days, 1g IV daily for 3-5days when oral treatments failed previously or where relapses are severe
Exercise
Neuropathic pain - amitriptyline and gabapentin
Depression - SSRIs
Urge incontinence - anticholinergic medications - tolterodine and oxybutynin (worsen cognitive function)
Spasticity - baclofen, gabapentin and physio
53
What are motor neurone diseases
Umbrella term for number of diagnoses
Progressive and ultimately fatal condition where the motor neurones stop functioning
No effect on sensory symptoms and patients should not experience any sensory symptoms
54
What does progressive bulbar palsy affect
Affects the muscles of talking and swallowing
55
List some MNDs
Amyotrophic lateral sclerosis
Progressive bulbar palsy
Progressive muscular atrophy
Primary lateral sclerosis
56
Describe the neurological signs of MND
Bother upper and lower motor neurones affected
Sensory neurones are spared
57
What causes MND
Exact cause is unknown
Genetics (10%) - FH
Smoking
Exposure to heavy metals
Pesticides
58
List the signs of an upper motor neurone lesion
Increased tone
Upgoing plantars
Brisk reflexes
59
List the signs of a lower motor neurone lesion
Muscle wasting
Reduced tone
Fasciculation (twitches in the muscles)
Reduced reflexes
60
What drugs can be used to slow the progression of MND
Riluzole - extends survival by few months
Edaravone - used in US
NIV - support with breathing at night and improves QOL
61
Give the classic triad of Parkinson's disease
Resting tremor
Rigidity
Bradykinesia
62
Describe the physiology of Parkinson's disease
Basal ganglia in the middle of the brain are responsible for coordinating habitual movements such as walking, looking around, controlling voluntary movements and learning specific movement patterns
The substantia nigra (part of the basal ganglia) produces dopamine which is essential for the correct functioning of the basal ganglia but in PD there is a gradual and progressive fall in the production of dopamine
63
Describe the presentation of the tremor in Parkinson's disease
4-6Hz - meaning it occurs 4-6 times a second
Pill-rolling tremor - fingertips and thumb
More pronounced when resting and improves on voluntary
Tremor is worsened if the patient is distracted - ask them to mime painting the fence - exaggerate the tremor
64
Describe the rigidity in Parkinson's disease
Rigidity is a resistance to passive movement of a joint
If you take their hand and passively flex and extend their arm at the elbow, you will feel a tension in their arms that gives way to movement in small increments.
65
Describe signs of bradykinesia in Parkinson disease
Small handwriting
Shuffling gait
Difficulty initiating movement
Difficulty in turning around when standing, having to take lots of little steps
Reduced facial movements and facial expressions
66
What other features affect people with Parkinson's disease
```
Depression
Sleep disturbance and insomnia
Loss of sense of smell (anosmia)
Postural instability
Cognitive impairment and memory problems
```
67
Describe the difference between benign and essential tremor
Parkinson's tremor - Asymmetrical, 4-6 Hz, worse at rest, improves with intentional movement, other Parkinson's features, no change with alcohol
Benign essential tremor - Symmetrical, fine tremor, 5-8Hz, improves at rest, worse with intentional movement, worse with caffeine, tiredness and stress, no other Parkinson's features, improves with alcohol, not present at sleep
68
What is multiple system atrophy
Where neurones of multiple systems in brain degenerates.
Affects the basal ganglia as well as other areas - the degeneration in basal ganglia leads to Parkinson's presentation. Degeneration in other areas lead to autonomic dysfunction and cerebellar dysfunction (ataxia)
69
List some of the autonomic dsyfunction
Postural hypotension
Constipation
Abnormal sweating
Sexual dysfunction
70
Describe dementia with Lewy bodies
Dementia associated with features of Parkinsonism - visual hallucinations, delusions, disorders of REM sleep and fluctuating consciousness
71
How is Parkinson's disease diagnosed?
Clinical symptoms and examination
72
How is Parkinson's disease treated?
On - medication working
Off - medication wear off
Levodopa - synthetic dopamine give PO to boost their own dopamine levels.
Usually combined with a peripheral decarboxylase inhibitors (drug that stops levodopa being broken down in the body before it gets the chance to enter the brain)
Co-careldopa (levodopa and carbidopa)
Co-benyldopa (levodopa and benserazide)
COMT inhibitors - Entacapone - inhibits the COMT enzyme which usually metabolises levodopa in the body and brain. Taken with the levodopa to slow the breakdown of levodopa and extend its effective duration
Dopamine agonists (cabergoline, pergolide, bromocryptine) - mimic dopamine in the basal ganglia and stimulate the dopamine receptors. Less effective than levodopa but used to delay the use of levodopa and the dose of levodopa required.
Monoamine oxidase B inhibitors - enzymes break down neurotransmitters such as dopamine, serotonin and adrenaline. More specific to dopamine and does not act on serotonin or adrenalin. These medications block the enzyme and increase circulating dopamine Used to delay starting levodopa - selegilline and rasagiline
73
What happens to Levodopa effectiveness over time
Becomes less effective
74
What is the main side effect of levodopa
When dopamine levels are too high - this causes dyskinesia (excessive motor activity)
- dystonia - excessive muscle contraction leads to excessive movement and abnormal posture
- Chorea - abnormal involuntary movements that can be jerking and random
- Athetosis - involuntary twisting and writhing movements of the fingers, hands and feet
75
What is a side effect of the dopamine agonists
Pulmonary fibrosis with prolonged use
76
What is the differential diagnosis for a benign essential tremor
```
Parkinson's disease
MS
Huntington's chorea
Hyperthyroidism
Fever
Medication - antipsychotics and salbutamol overuse
```
77
Describe the management of benign essential tremor
No definitive treatment
| Medication can improve symptom - propranolol (non-selective beta blocker) and primidone (barbiturate anti-epileptic)
78
What is epilepsy
An umbrella term for a condition where there is a tendency to have seizures
79
What are seizures
Transient episodes of abnormal electrical activity in the brain
80
What investigations should be done for epilepsy
EEG
MRI brain
ECG
81
Describe a tonic-clonic seizure
Loss of consciousness
Tonic phase before clonic phase
Tonic phase is muscle stiffening
Clonic phase is muscle jerking
May be associated with incontinence, tongue biting, groans and irregular breathing
Post ictal period - confused, drowsy, irritable or depressed
82
Describe the management of tonic clonic seizures
1st line - sodium valproate
| 2nd line - lamotrigine or carbamazepine
83
Where do focal seizures originate?
Temporal lobe
84
Describe the presentation of focal seizures
```
These affect hearing, speech, memory and emotion Hallucinations
Memory flashbacks
Deja vu
Doing strange things on autopilot
Post-ictal weakness
```
85
How do you treat focal seizures
1st line - carbamazepine or lamotrigine
| 2nd line - sodium valproate or levetiracetam
86
Describe absence seizures
Happen in children
Patient becomes blank, stares into space and then abruptly returns to normal
These last 10-20seconds and the patient will not respond in this time
87
What is the management of absence seizures
Sodium valproate or ethosuximide
88
Describe atonic seizures
```
Drop attacks
Brief lapses in muscle tone
Don't usually last more than 3 mins
Begin in childhood
Indicative of lennox-gastaut syndrome
```
89
Describe the management of atonic seizures
1st line - sodium valproate
| 2nd line - lamotrigine
90
Describe myoclonic seizures
Sudden brief muscle contractions - sudden jump
Patient remains awake during the episode
Most often occur in juvenile myoclonic epilepsy
91
How are myoclonic seizures treated
1st line - sodium valproate
| 2nd line - lamotrigine, levetiracetam, topiramate
92
Describe infantile spasms (West syndrome)
Rare disorder - full body spasms
starting in infancy at around 6 months - poor prognosis where 1/3 die before 25 and the rest are seizure free
Treated with prednisolone and vigabatrin
93
How does sodium valproate work
Increases the activity of GABA
| Relaxing effect on the brain
94
List some side effects of sodium valproate
Teratogenic
Liver damage and hepatitis
Hair loss
Tremor
95
List some notable side effects of carbamazepine
Agranulocytosis
Aplastic anaemia
Induces P450 enzyme system - drug interactions
96
List some notable effects of phenytoin
Megaloblastic anaemia
Vit D and folate deficiency
Osteomalacia
97
List some notable side effects of ethosuximide
Night terrors
| Rashes
98
List some notable side effects of lamotrigine
Stevens - Johnson syndrome or DRESS syndrome
| Leukopenia
99
Define status epilepticus
A seizure lasting >5 mins or >3 seizures in 1 hour
100
How do you treat status epilepticus
```
ABCDE approach
Secure the airway - NP tube
Give high flow O2
Assess cardiac and respiratory function
Check blood glucose
Gain IV access - cannulate
IV lorazepam 4mg, repeat after 5 mins
If seizure persists try IV phenytoin and call anaesthetists with view to intubate and ventilate
```
101
What is the name of the post-ictal weakness focal epileptic patients get
Todd's paresis
102
What type of symptoms do frontal lobe seizures cause
Motor - jerking of the limb
| Jacksonian march
103
What types of symptoms would occur with an occipital lobe seizure
Visual hallucinations
104
What symptoms would a parietal lobe seizure present with
Paraesthesia
105
What symptoms do temporal lobe focal seizures present with
```
Hallucinations
Epigastric rising
Emotional
Automatisms - lip smacking, grabbing and plucking
Deja vu
Dysphasia
```
106
Describe a Jacksonian March
Seizure begins in one part of the body and then spreads over larger area of the brain causing a tonic clonic seizure
Secondary generalisation
107
When and how can anti-epileptic drugs be stopped
If seizure free for >2yrs and stopped over 2-3months
108
How can epileptic seizures be differentiated from vasovagal syncope
Vasovagal syncope - no post ictal period and rapid recovery
109
What is neuropathic pain, give examples of the causes
Abnormal functioning of the sensory nerves delivering abnormal and painful signals to the brain
Burning, tingling, pins and needles, electric shocks, loss of sensation to touch over the affected area
Post herpetic neuralgia from shingles in the distribution of a dermatome usually on the trunk
Nerve damage from surgery
MS
Diabetic neuralgia
Trigeminal neuralgia
Complex regional pain syndrome (CRPS)
110
What is the DN4 questionnaire
Used to assess the characteristics of neuropathic pain and the examination of the area
Scored out of 10 and a score >4 indicates neuropathic pain
111
Which drugs can be tried to relieve neuropathic pain
Amitriptyline
Duloxetine
Pregabalin
Gabapentin
Tramadol - short term flares
Capsaicin cream
112
Which drug is first line when treating trigeminal neuralgia
Carbamazepine
113
Describe complex regional pain syndrome
Abnormal nerve functioning - abnormal sensations and neuropathic pain
Often triggered by an injury to that area
Intermittent swelling, change in colour, temperature, flush and abnormal sweating
114
Describe the path of the facial nerve
Exits the brainstem at the cerebellopontine angle
Passes through the temporal bone and parotid gland
Divides into 5 branches that supply different areas of the face - temporal, zygomatic, buccal, marginal mandibular and cervical
115
Describe the functions of the facial nerve
Motor - facial expression, stapedius in inner ear and the posterior digastric, stylohyoid and platysma muscles of the neck
Sensory - taste of anterior 2/3 tongue
Parasympathetic - submandibular and sublingual salivary glands and the lacrimal gland
116
Describe the nerve innervation of the forehead
Each side of the forehead has upper motor neuron innervation by both sides of the brain
Each side of the forehead only has lower motor neuron innervation from one side of the brain
117
What can be seen in an UMN facial palsy
Forehead sparing
118
Describe the symptoms of Bells palsy
Forehead affected
Drooping of the eyelid
Exposure of the eye
Loss of the nasolabial fold
119
List some causes of an UMN facial palsy
Cerebrovascular accident
Tumour
Bilateral - pseudobulbar palsy and MND
120
List some causes of LMN facial palsy
```
Bells palsy
Ramsay Hunt syndrome
Otitis media
Malignant otitis externa
HIV
Lyme disease
Diabetes
Sarcoidosis
Leukaemia
MS
Guillian Barre syndrome
Acoustic neuroma
Parotid tumours
Cholesteatoma
Direct nerve injury
Injury during surgery
Base of skull fracture
```
121
How long does recovery from Bells palsy take?
several weeks - 12 months however some never recover completely (1/3 left with residual weakness)
122
How is Bells palsy treated
Prednisolone
50mg for 10days
60mg for 5 days followed by a 5 day reducing regime of 10mg a day
Lubricating eye drops
123
What may happen as a result of Bells palsy
Exposure keratopathy
124
Describe Ramsay Hunt syndrome
Varicella zoster virus (VZV)
Unilateral LMN facial palsy
Tender vesicular rash in ear canal, pinna, around ear on affected side
The rash can extend to the anterior 2/3 of the tongue and hard palate
125
How is Ramsay Hunt syndrome treated
Prednisolone
Aciclovir
Lubricating eye drops
126
What happens to perception of sound in an UMN facial nerve palsy
Hyperacusis - hypersensitivity to sound
| Paralysis of stapedius
127
When should facial nerve palsies be referred to ENT or neurology
Worsening or new neurological findings
Red flag features of cancer
No signs of improvement after 3 weeks of treatment
Symptoms of aberrant reinnervation 5 months or more after original onset
Unclear diagnosis
128
Describe the symptoms of a brain tumour
Focal neurological lesion
Signs of raised ICP
Often asymptomatic at the beginning
129
List some causes of raised intracranial pressure
Brain tumour
Intracranial haemorrhage
Idiopathic intracranial hypertension
Abscesses or infection
130
Describe the presentation of raised intracranial pressure
Headache - constant, nocturnal, worse on waking, coughing, straining or bending forward, vomiting
```
Altered mental state
Visual field defects
Seizures - focal
Unilateral ptosis
Third and sixth nerve palsy
Papilloedema
```
131
What is Papilloedema
Swelling of the optic disc secondary to raised intracranial pressure
The sheath around the optic nerve is connected with the subarachnoid space - possible for CSF under high pressure to flow into the optic nerve sheath and causes swelling
132
Describe the fundoscopic changes of Papilloedema
Blurring of the optic disc margin
Elevated optic disc - curve in retinal vessels
Loss of the venous pulsation
Engorged retinal veins
Haemorrhages around optic disc
Paton's lines - creases in the retina around the optic disc
133
Which cancers commonly metastasise to the brain
Breast
Lung
Renal cell carcinoma
Melanoma
134
Describe gliomas
Tumours of glial cells
- Astrocytoma - glioblastoma multiforme
- oligodendroglioma
- Ependymoma
Graded from 1-4 - grade 1 is most benign and grade 4 is most malignant
135
Describe a meningioma
Tumour of the meninges in the brain and lead to raised ICP and neurological symptoms
136
Describe pituitary tumours
Benign but if they become large can press on the optic chiasm causing bitemporal hemianopia (loss of the outer half of vision in both eyes
Hypo or hyperpituitarism
- Acromegaly
- Cushing's syndrome
- Hyperprolactinaemia
- Thyrotoxicosis
137
Describe acoustic neuroma
Tumour of Schwann cells surrounding the auditory nerve that innervates the inner ear
Occur at the cerebellopontine angle
Usually unilateral, bilateral ones associated with neurofibromatosis type 2
Slow growing but eventually grow large enough to cause symptoms and become dangerous
- Tinnitus
- Hearing loss
- Balance problems
Associated with facial nerve palsy
138
Describe the treatment of pituitary tumours
Trans-sphenoidal surgery
Radiotherapy
Bromocriptine - block prolactin secreting tumour
Somatostatin analogues - octreotide - block growth hormone secreting tumour
139
What is Huntington's chorea
Autosomal dominant progressive deterioration of nervous system
Asymptomatic until symptoms begin age 30-50
140
Describe the genetic abnormality in Huntington's chorea
Trinucleotide repeat disorder of HTT gene on chromosome 4
141
Describe what is meant by anticipation in Huntington's chorea
Each successive generation, more trinucleotide repeats resulting in earlier onset and increased severity of disease
142
Describe the presentation of Huntington's chorea
Begins with cognitive, psychiatric or mood problems
- Chorea - involuntary, abnormal movements
- Eye movement disorders
- Speech difficulties - dysarthria
- Swallowing difficulties
143
Describe the prognosis of Huntington's chorea
15-20yrs life expectancy following diagnosis
Death is often due to respiratory disease or suicide
144
Describe myasthenia gravis
Autoimmune condition
| Weakness gets progressively worse with activity and improves with rest
145
At what age does myasthenia gravis tend to present
<40yo women
| >60 men
146
What type of tumour is linked to myasthenia gravis
Thyoma (tumour of thymus gland)
147
Describe normal conduction across the neuromuscular junction
Pre-synaptic neurone secretes acetylcholine across the synapse which then binds to post synaptic receptors and stimulates a signal in the muscle and muscle contraction occurs
148
What happens to neuromuscular junction transmission in myasthenia gravis
Antibodies to the acetylcholine receptor are produced. These bind to the post synaptic membrane and block the receptor. Acetylcholine is prevented from stimulating the post synaptic membrane and prevent muscle contraction
As more receptors are used up during more muscle activity, more become blocked
There is more muscle weakness the more the muscles are used This improves with rest when more receptors are free for use again
Complement system is also activated and this damages the postsynaptic membrane
Antibodies against Muscle specific kinase (MuSK) and antibodies against low density lipoprotein receptor related protein 4 (LRP4) - used to create the ACH receptor. Destruction of these by antibodies leads to inadequate receptors
149
Describe the presentation of myasthenia
```
Proximal muscles
Diplopia - weak extraocular muscles
Ptosis
Weak facial muscles
Difficulty swallowing
Fatigue in the jaw when chewing
Slurred speech
Progressive weakness with repetitive movement
```
Symptoms are worse at night time
150
Describe the examination and investigations for myasthenia gravis
Upward gazing - diplopia on further eye movement test
Repeated blinking will exacerbate ptosis
Repeated abduction of one arm 20 times will result in unilateral weakness when comparing both sides
FVC
Check for thymectomy scar
151
How is a diagnosis of myasthenia gravis made
Test for antibodies - AchR, MuSK, LRP4
CT/MRI thymus
Edrophonium test - IV dose of edrophonium chloride (neostigmine) - block acetylcholinesterase enzyme in NMJ - relieves the weakness temporally
152
Describe the treatment of myasthenia gravis
Reversible anticholinesterase inhibitors - pyridostigmine and neostigmine
Immunosuppression - prednisolone and azathioprine
Thymectomy
Rituximab
Eculizumab
153
Describe a myasthenic crisis and its treatment
Severe complication of MG
Life threatening
Caused by other illness such as respiratory illness
Patients may require NIV or full intubation and ventilation
IVIG and plasma exchange may help
154
Which drug should be avoided in people with myasthenia
Beta blockers
155
Which anaesthetic drug are patients with myasthenia gravis often resistant to?
Suxamethonium
156
What is Lambert-Eaton Myasthenia
Slow progression
Proximal muscles more
Damage to the neuromuscular junction - antibodies produced by the immune system against voltage gated calcium channels in the presynaptic terminals of the NMJ
Tends to occur more in patients with small cell lung cancer
157
How is Lambert-eaton myasthenia treated?
Immunosuppressants - prednisolone or azathioprine
IV immunoglobulins
Plasmaphoresis
158
What is charcot marie tooth disease
Inherited disease that affects the peripheral motor and sensory nerves
Dysfunction in the myelin or the axons
Autosomal dominant pattern
Symptoms usually start to appear before the age of 10 but onset can be after 40
159
Name some classical features of Charcot Marie tooth disease
High foot arches
Distal muscle wasting causing inferted champagne bottle legs
Weakness in lower legs - ankle dorsiflexion
Weakness in the hands
Reduced tendon reflexes
Reduced muscle tone
Peripheral sensory loss
160
List the causes of peripheral neuropathy
```
Alcohol
B12 deficiency
Cancer and Chronic kidney disease
Diabetes
Drugs - isoniazid, amiodarone and cisplatin
Vasculitides
```
161
Describe Guilian-Barre syndrome
Acute paralytic polyneuropathy - affects the PNS
| Causes acute, symmetrical, ascending weakness and sensory symptoms
162
Which infections is Guillian Barre most associated with?
Campylobacter jejuni
Cytomegalovirus
Epstein Barr virus
163
Describe the pathophysiology of Guillian Barre syndrome
Molecular mimicry - B cells of the immune system create antibodies against antigens on the pathogen that causes the preceding infection
These antibodies also match the proteins on the nerve cells
They may have target proteins on the myelin sheath of the motor nerve cell or the nerve axon
164
Describe the presentation of Guillian Barre syndrome
Symmetrical ascending weakness
Reduced reflexes
Peripheral loss of sensation or neuropathic pain
Progress to affect the cranial nerves
165
Describe the clinical course of Guillian Barre syndrome
Symptoms start within 4 weeks of the preceding infection
Start in the feet and progress upwards
Symptoms peak within 2-4 weeks then a recover period can last moths to years
166
Describe how Guillian Barre is diagnosed
The Brighton criteria is used
Nerve conduction studies - reduced signal through nerves
LP for CSF - raised protein with a normal cell count and a normal glucose
167
How is Guillian Barre syndrome treated?
```
IV IG
Plasma exchange
Supportive care
VTE prophylaxis
Resp failure may require intubation, ventilation and admission
```
168
Describe the prognosis of Guillian Barre syndrome
80% fully recover
15% left with some neurological disability
5% will die
169
Describe neurofibromatosis
Nerve tumours throughout the nervous system
Benign - do cause neurological and structural problems
Two types - type 1 is more common than type 2
170
Describe neurofibromatosis type 1
NF1 gene mutations on chromosome 17 - neurofibromin (tumour suppressor gene). Inheritance is autosomal dominant
2 of the 7 features:
Cafe au lait spots
Relative with NF1
Axillary or inguinal freckles
Bony dysplasia - bowing of the long bone or sphenoid wing dysplasia
Iris haematomas - Lisch nodules (yellow brown spots on the iris)
Neurofibromas or plexiform neurofibroma
Glioma of the optic nerve
171
How do you investigate Neurofibromatosis
Genetic testing
X-ray - investigate bone pain and bone lesions #CT and MRI - investigate lesions in the brain, spinal cord and elsewhere in the body
172
Describe neurofibromatosis type 2
Chromosome 22 protein merlin (tumour suppressor protein) important in Schwann cells and mutations lead to development of schwannomas
173
What is neurofibromatosis most associated with?
Bilateral acoustic neuromas (hearing loss, balance problems, tinitus)
174
How is neurofibromatosis managed
Supportive - monitor the disease and treat any complications such as surgical resection
175
List some complications of neurofibromatosis type 1
Migraines
Epilepsy
Renal artery stenosis - causing hypertension
Learning and behavioural problems
Scoliosis of the spine
Vision loss
Malignant peripheral nerve sheath tumours
Gastrointestinal stromal tumour (sarcoma)
Brain tumours
Spinal cord tumour with associated neurology
Increased risk of breast cancer and leukaemia
176
List some red flags of headaches
Fever, photophobia, neck stiffness (meningitis, encephalitis)
New neurological symptoms (haemorrhage, malignancy, stroke)
Dizziness (stroke)
Visual disturbance (temporal arteritis or glaucoma)
Sudden onset occipital headache (SAH)
Worse on coughing or straining (raised ICP)
Postural, worse on standing, lying or bending over (raised ICP)
Severe enough to wake person up from sleep
Vomiting - raised ICP or CO poisoning
History of trauma (ICH)
Pregnancy (pre-eclampsia)
177
Describe a tension headache, the triggers and its management
Mild ache across the forehead in a band like pattern around the head
Due to muscle ache in frontalis, temporalis and occipitalis muscles
Come on and resolve gradually and do not produce any visual changes
Triggers - stress, depression, alcohol, skipping meals, dehydration
Treatment - reassurance, hot towels, relaxation techniques, basic analgesia
178
Describe what is meant by a secondary headache
Similar presentation to tension headache but with a clear cause
Non specific headache secondary to underlying medical conditions, alcohol, head injury and carbon monocide poisoning
179
Describe sinusitis and its course and treatment
Inflammation in the ethmoidal, maxillary, frontal or sphenoidal sinuses
Facial pain behind the nose, forehead, eyes
Tenderness over sinus
Resolves within 2-3 weeks
Most is viral
Nasal irrigation with saline can be helpful
Prolonged symptoms (>10 days) treated with steroid nasal spray and antibiotics are occasionally required
180
Describe what an analgesia overuse headache is and how it is treated
Long term analgesia use
Non specific features to a tension headache
Secondary to continous or excessive use of analgesia
Withdrawal of the analgesia is important in treating these headaches
181
Describe a hormonal headache
Related to low oestrogen
Non specific, generic, tension type headache
2 days before and first 3 days of period, around menopause and during pregnancy (2nd half pregnancy should raise suspicion of pre-eclampsia)
COCP can help treat this
182
Describe cervical spondylosis
Degenerative changes in the cervical spine
Causes neck pain made worse by movement
Presents with a headache
Exclude other causes of neck pain - inflammation, malignancy, infection
183
Describe trigeminal neuralgia
Trigeminal nerve has 3 branches - ophthalmic, maxillary, mandibular
Compression of the nerve
Occurs in patients with MS
Intense facial pain that comes on spontaneously and lasts for few seconds to hours
Often described as electrical shock pain
Attacks often worsen over time
Triggers - spicy food, cold weather, caffeine, citrus fruits
Carbamazepine or surgical decompression
184
List some types of migraine
Migraine without aura
Migraine with aura
Silent migraine
Hemiplegic migraine
185
Describe the headache in migraine
```
Lasts between 4 and 72hrs
Moderate to severe in frequency
Uusally unilateral but can be bilateral
Photophobia and phonophobia
With or without aura
Nausea and vomiting
```
186
Describe the aura in migraine
Visual changes associated with migraine
- sparks in vision
- Blurring vision
- Lines across vision
- Loss of different visual fields
187
Describe a hemiplegic migraine
```
Mimics a stroke
Typical migraine symptoms
Sudden or gradual onset
Hemiplegia - unilateral weakness of the limbs
Ataxia
Change in consciousness
```
188
List some triggers of migraines
```
Stress
Bright lights
Strong smells
Certain food
Dehydration
Menstruation
Abnormal sleep patterns
Trauma
```
189
List the 5 stages of migraine
```
Prodromal stage (3 days before)
Aura (60 mins)
Headache (4 to 72hrs)
Resolution stage - headache fades away or is relieved by vomiting or sleeping
Postdromal/recovery
```
190
Describe the acute management of migraine
Paracetamol
Triptans - 5HT receptor agonists - smooth muscle contraction in arteries to cause vasoconstriction, peripheral pain receptors inhibited activation of pain, reduce neuronal activity in the CNS
NSAIDs
Antiemetics
191
Describe migraine prophylaxis
```
Propranolol
Topiramate
Amitriptyline
Acupuncture
Supplementation with B2 (riboflavin)
Avoid triggers
```
192
What is a contraindication to topirmate
Pregnancy
193
Describe cluster headaches
Cluster of attacks and then disappear for a while
| Typical patient is 30-50yo, smoker, triggered by alcohol, strong smell and exercise
194
Describe the symptoms of a cluster headache
```
Red, swollen and watering eye
Pupil constriction (miosis)
Eyelid drooping (ptosis)
Nasal discharge
Facial sweating
```
195
What is the treatment of cluster headache
Triptans - Sumatriptan 6mg SC
| High flow O2 100% for 15-20mins
196
What prophylaxis of cluster headaches can be given
Verapamil
Lithium
Prednisolone - short course for 2-3 weeks to break the cycle
197
When is carotid endarterectomy performed
Symptomatic stenosis >75%
198
List some causes of myasthenic crisis
```
Pregnancy
Emotion
Exercise
Chest sepsis
Hypokalaemia
Beta blockers
Antibiotics -tetracyclines
Opiates
Acetylcholinesterase inhibitors
```
199
Problems with which structures can cause ptosis?
Midbrain
Cervical sympathetic chain
Oculomotor nerve
200
Describe pronator drift and what it signifies
Patient holds out arms with palms upwards
Observe for signs of pronation for 20-30 seconds
Ask patient to close eyes
Pronation with or without downward movement of the forearm suggests an UMN pyramidal tract lesion in the contralateral side as supinator's are weaker than pronator muscles in this context
201
Describe spasticity
Associated with pyramidal tract lesions
Velocity dependent - the faster you move the limb, the worse it is - typically increased tone in intial part of movement which then suddenly reduces past a certain point - clasp knife spasticity - also accompanied by weakness
202
Describe rigidity
Velocity independent - feels the same no matter how fast you move the limb
203
What are the two types of rigidity
Cogwheel rigidity - involves a tremor superimposed on hypertonia resulting in intermittent increases in tone during movement of the limb - Parkinson's disease
Lead pipe rigidity - uniformly increased tone throughout the movement of muscle - subtype of rigidity is typically associated with neuroleptic malignant syndrome
204
Describe the MRC power scale
0 - no contraction
1 - Flicker or trace of contraction
2- active movement, with gravity eliminated
3 - active movement against gravity
4 - active movement against gravity and resistance
5 - normal power
205
Which myotome and muscles are tested when assessing shoulder abduction
C5 (axillary nerve)
| Deltoids
206
Which myotome and muscles are tested when assessing shoulder adduction
C6/7 (thoracodorsal nerve)
| Latissimus dorsi, teres major, pectoralis major
207
Which myotome and muscles are tested when assessing elbow flexion
C5/6 (musculocutaneous and radial nerve)
| Coracobrachialis, biceps brachii and brachialis
208
Which myotome and muscles are tested when assessing elbow extension
C7 (radial nerve)
| Triceps brachii
209
Which myotome and muscles are tested when assessing the wrist extension
C6 (radial nerves)
| Extensors of the wrist
210
Which myotome and muscles are tested when assessing wrist flexion
C6/7 (median nerve)
| Flexors of the wrist
211
Which myotome and muscles are tested when assessing finger extension
C7 (radial nerve)
| Extensor digitorium
212
Which myotome and muscles are tested when assessing finger abduction
```
T 1 (ulnar nerve)
Abductor digiti minimi
First dorsal interosseous
```
213
Which myotome and muscles are tested when assessing thumb abduction
T1 (median nerve)
| Abductor pollicis brevis
214
Describe the pattern of weakness in an upper motor neuron lesion
Pyramidal weakness
Extensors in the upper limb are weaker than the flexors
Flexors in the lower limb are weaker than the extensors
215
Describe a reinforcement manoeuvre you can do to ensure the reflexes are absent
Ask patient to clench their teeth - relaxes the arms
216
Which myotome supplies the biceps reflex
C5/6
217
Where do you hit to elicit the biceps reflex
Medial aspect of the antecubital fossa
218
Which myotome is the supinator reflex
C5/6
219
Where do you hit to elicit the supinator (Brachioradialis) reflex
Brachioradialis tendon - 4 inches proximal to the base of the thumb on the posterolateral aspect of the wrist
220
Which myotome is the triceps reflex
C7
221
Where do you hit to elicit the triceps reflex
Triceps tendon - Superior to the olecranon process of the ulnar
222
Describe hyperreflexia
Associated with UMN lesion - loss of inhibition from higher brain centres which normally exert a degree of suppression over the lower motor neuron reflex arc
223
Describe hyporeflexia
LMN lesion - loss of efferent and afferent branches of the normal reflex arc
224
Describe the reflexes in cerebellar disease
Often normal
| Can be pendular - less brisk and slower in their rise and fall
225
Describe where you would test each of the dermatomes
C5 - lateral aspect of the lower edge of the deltoid muscle
C6 - palmar side of the thumb
C7- palmar side of middle finger
C8 - palmar side of little finger
T1 - medial aspect of antecubital fossa,, proximal to medial epicondyle of Humerus
226
What does light touch sensation involve
Dorsal columns
| Spinothalamic tract
227
What does pin-prick sensation involve
Spinothalamic tracts
228
What does vibration sense involve
Dorsal columns
229
What does proprioception involve
Dorsal columns
230
What causes peripheral neuropathy
Chronic alcohol excess
| Diabetes mellitus
231
What is a radiculopathy
Nerve root damage
232
What is mononeuropathy
Nerve damage
233
What type of sensory disturbance is caused by a thalamic lesion
Contralateral sesnory loss
234
What type of weakness results from myopathy
Symmetrical proximal muscle weakness
235
Describe an intention tremor
Broad, coarse, low frequency tremor that develops as a limb reaches the endpoint of a deliberate movement
236
What does dysmetria and intention tremor suggest
Ipsilateral cerebellar pathology
237
What does dysdiadochokinesia suggest
Inability to perform rapid, alternating rapid movement - suggest ipsilateral cerebellar pathology
238
What is a broad based ataxic gait associated with?
Midline cerebellar pathology - lesion in MS or degeneration of the cerebellar vermis secondary to chronic alcohol excess
239
In unilateral cerebellar disease in which direction will patients veer to?
The side of the pathology
240
What does a high stepping gait possibly indicate
Foot drop
241
What does tandem gait show
Dysfunction of cerebellar vermis (alcohol induced cerebellar degeneration)
Weak flexors or sensory ataxia
242
Describe hemiparetic gait
One leg held stiffly and swings round in an arc with each stride (circumduction) - associated with stroke
243
Describe spastic paraparesis gait
Bilateral hemiparetic gait with both legs stiff and circumduction
Inverted and scissor feet
Hereditary spastic paraplegia
244
Describe Romberg's test
Ask patient to put feet together and arms by their side
Close eyes - if patient has proprioceptive or vestibular dysfunction then will struggle to remain standing
245
What is a positive Romberg's and what does it suggest
Falling without correction
Sensory ataxia - proprioceptive dysfunction (Ehlers Danlos), B12 deficiency, Parkinson's disease and ageing
Vestibular dysfunction - vestibular neuronitis and Menieres disease
246
What does swaying with correction during rombergs test suggest
Rombergs negative
Cerebellar disease due to truncal ataxia
247
Describe what may be observed with increased tone in the leg
When lifting knee off the bed, the ankle will come up too
248
Describe ankle clonus
Involuntary rhythmic muscular contractions and relaxations that is associated with UMN lesions of the descending motor pathways (stroke, MS, cerebral palsy)
Position patients legs so the knee and ankle are slightly flexed, supporting the leg with your hand under their knee so they can relax
Rapidly dorsiflex and partially evert the foot to stretch gastrocnemius muscle
Keep the foot in this position and observe for clonus (>5 rhythmic beats of dorsiflexion and plantarflexion)
249
Which myotomes and muscles are assessed when testing hip flexion
L1/2 (iliofemoral nerve)
| Iliopsoas
250
Which myotomes and muscles are assessed when testing hip extension
L5/S1/S2
| Gluteus maximus
251
Which myotomes and muscles are assessed when testing knee flexion
S1 (Sciatic nerve)
| Hamstrings
252
Which myotomes and muscles are assessed when testing knee extension
L3/4 (femoral nerve
| Quadriceps
253
Which myotomes and muscles are assessed when testing ankle dorsiflexion
L4/5 (deep peroneal nerve)
| Tibialis anterior
254
Which myotomes and muscles are assessed when testing ankle plantarflexion
S1/2
Tibial nerve
Gastrocnemius and soleus
255
Which myotome and muscles are assessed when testing big toe extension
L5 (deep peroneal nerve)
| Extensor hallucis longus
256
Which myotome is associated with the patella reflex
L3/4
257
Which myotome is associated with the ankle jerk reflex
S1
258
Which myotome is associated with the plantar reflex
L5 and S1
259
Describe babinskis sign
Extension of the big toe and spreading of the other toes - UMN lesion
260
What is L1 dermatome
Inguinal region and very top of medial thigh
261
What is the L2 dermatome
Middle and lateral aspect of anterior thigh
262
What is the L3 dermatome
MEDIAL ASPECT OF KNEE
263
What is the L4 dermatome
Medial aspect of lower leg and ankle
264
What is the L5 dermatome
Dorsum and medial aspect of big toe
265
What is the S1 dermatome
Dorsum and lateral aspect of the little toe
266
What does inability to perform heel to shin test show
Incoordination - Dysmetria - ipsilateral cerebellar pathology
267
What is a relative afferent pupillary defect and what does it show
Constriction less so in the affected pupil
Swinging light test shows relative pupillary dilation when light shone from healthy eye to damaged eye
Retinal damage - central retinal artery occlusion, vitreous
detachment , compression due to tumour or abscess
Optic neuritis
268
What is an unilateral efferent defect and what does it show
Compression caused by the extrinsic pathway
Ipsilateral pupil dilates and is non-responsive but consensual response the unaffected pupil is normal
269
What is visual neglect and what does it show?
Deficiet in awareness of one side of the visual field
| Stroke in contralateral parietal region
270
What is the function of the inferior oblique
Elevates, abducts and laterally rotates the eyeball
271
What is the function of the superior oblique
Depresses, abducts and medially rotates the eyeballs
272
Describe the function of the medial rectus
Adducts the eyeball
273
Describe the function of the lateral rectus
Abduct the eyeball
274
Describe the function of the superior rectus
Elevation, adduction, medial rotation of the eyeball
275
Describe the function of the inferior rectus
Depression, adduction and lateral rotation of the eyeball
276
What are the features of an oculomotor nerve palsy
Down and out pupil
Ptosis
Mydriasis
277
What are the features of a trochlear nerve palsy
Vertical diplopia when looking inferiorly
278
Which muscle is supplied by the trochlea nerve
Superior oblique
279
Which muscle is supplied by the abducens nerve
Lateral rectus muscle
280
What does abducens nerve palsy cause
Convergent squint
| Horizontal diplopia
281
What is a normal rinnes test
Air conduction > Bone conduction
282
What would be the Rinnes test for sensorineural deafness
Air conduction >Bone conduction
| Rinnes positive
283
What would be the Rinnes test for conductive deafness
Bone conduction > Air conduction
| Rinnes negative
284
Describe normal Weber's
Sound heard equally in both ears
285
Describe Weber's in sensorineural deafness
Sound head louder in the normal ear
286
Describe Weber's in conductive deafness
Sound heard louder on the side of the affected ear
287
Describe the head thrust test/vestibular ocular reflex
Ask pt if they have any neck problems
Tell pt to focus on your nose at all times
Turn their head rapidly to left and then to right
Normal - eyes fix on your nose
Vesibular pathology - eyes move in direction of head movement before a corrective refixation saccade towards your nose
