Neurology Flashcards
1
Q
What are cluster headaches?
A
Unbearable unilateral headaches, centred around eye
Come in clusters and disappear for extended periods
2
Q
What are the symptoms of cluster headaches?
A
3-4 episodes/d for wks/mths, then pain-free period lasting several yrs
Attacks last between 15mins and 3hrs
Unilateral
Red, swollen and watering eye
Pupil constriction
Eyelid drooping
Nasal discharge
Facial sweating
3
Q
Outline acute management of cluster headaches
A
Triptans (eg: Sumitriptan)
High-flow 100% O2
4
Q
Outline prophylactic management of cluster headaches
A
1st line: Verapamil
Occipital nerve block
Prednisolone- Given short course to break the cycle
Lithium
5
Q
What are the 4 types of migraine?
A
Migraine w/o aura
Migraine with aura
Silent migraine (aura but no headache)
Hemiplegic migraine
6
Q
What are the 5 stages of migraine?
A
- Premonitory/prodromal- Several days before headache
- Aura (lasts up to 60mins)
- Headache- Lasts 4 to 72h
- Resolution- Headache fades/relieved abruptly by vomiting or sleeping
- Postdromal/recovery phase
7
Q
What are the typical features of a migraine headache?
A
Lasts between 4 and 72h
Usually unilateral
Moderate-severe intensity
Pounding or throbbing
Photophobia
Phonophobia
Osmophobia (discomfort with smells)
Aura (visual changes)
N&V
8
Q
What are the common features of an aura?
A
Affects vision/sensation/language
Sparks in vision
Blurred vision
Lines across vision
Loss of visual fields (eg: Scotoma)
Tingling/numbness
Dysphagia
9
Q
What is a hemiplegic migraine?
A
Unilateral limb weakness
Ataxia (loss of coordination)
Impaired consciousness
Familial hemiplegic migraine- Autosomal dominant
Can mimic stroke or TIA
10
Q
List some of the triggers of migraines
A
Stress
Bright lights
Strong smells
Certain foods (eg: Chocolate, cheese, caffeine)
Dehydration
Menstruation
Disrupted sleep
Trauma
11
Q
Outline acute management of migraines
A
NSAIDs
Paracetamol
Triptans (eg: Sumitriptan)
Antiemetics (eg: Metoclopramide or prochlorperazine)
Opiates can make migraine worse
12
Q
Outline triptans
A
5-HT receptor agonists (bind to serotonin receptors
MoA:
Cranial vasoconstriction
Inhibit transmission of pain signals
Inhibit release of inflammatory neuropeptides
13
Q
What are the CIs of triptans?
A
Associated with vasoconstriction:
HTN
Coronary artery disease
Previous stroke
TIA
MI
14
Q
Outline prophylaxis of migraines
A
Headache diary and avoid triggers
Propanolol
Amitriptyline
Topiramate (teratogenic)
Specialist:
Pizotifen
Candesartan
Sodium valproate
MA- Erenumab or fremanezumab
CBT
Mindfulness and meditation
Acupuncture
Vit B2 (riboflavin)
Menstrual migraines- Prophylactic triptans (eg: Frovatriptan or zolmitriptan)
15
Q
Which type of headache is the COCP CI in and why?
A
Migraine with aura
Associated with increased risk of stroke
16
Q
What are the red flags of headaches?
A
Fever, photophobia, neck stiffness- Meningitis, encephalitis, brain abscess
New neuro symptoms- Haemorrhage, tumours
Visual disturbance- Giant cell arteritis, glaucoma, tumours
Sub-onset occipital headache- SAH
Worse on coughing or straining- Raised ICP
Postural, worse on standing/lying/bending over- Raised ICP
Vomiting- Raised ICP or CO poisoning
History trauma- ICH
History of cancer- Brain metastasis
Pregnancy- Pre-eclampsia
17
Q
What is an important investigation in papilloedema?
A
Fundoscopy
Suggests raised ICP- Brain tumour, benign intracranial HTN, intracranial bleed
18
Q
Outline tension headaches
A
Common
Mild ache or pressure in band-like pattern around head
Develop and resolve gradually
No visual changes
19
Q
What are some of the associations of tension headaches?
A
Stress
Depression
Alcohol
Skipping meals
Dehydration
20
Q
Outline management of tension headaches
A
Reassurance
Ibuprofen or paracetamol
Amitriptyline for chronic/frequent tension headaches
21
Q
What are secondary headaches?
A
Similar presentation to tension headache, but with a clear cause
22
Q
List some of the causes of secondary headaches
A
Infections- Viral URTI
OSA
Pre-eclampsia
Head injury
CO poisoning
23
Q
Outline sinusitis
A
Inflammation of paranasal sinuses
Typically causes pain and pressure following recent viral URTI
Tenderness and swelling on palpation of affected areas
Resolve within 2-3wks
24
Q
What is the management of sinusitis?
A
Usually spontaneously resolves within 10d
Steroid nasal spray
Antibiotics- Pen V
25
What is a medication overuse headache?
Analgesic headache caused by frequent analgesia use
Similar non-specific features as tension headache
26
Outline hormonal headaches
Related to low oestrogen
Similar to migraine features
Occur:
2d before and 1st 3d menstruation
Perimenopause
Early pregnancy
27
Outline management of hormonal headaches
Triptans
NSAIDs- Mefenamic acid
28
Outline cervical spondylosis
Caused by degenerative changes in cervical spine
Causes neck pain, worse with movement
Often presents with headaches
29
Outline trigeminal neuralgia
Intense facial pain in distribution of trigeminal nerve
90% unilateral
More common in MS
30
Outline presentation of trigeminal neuralgia
Pain comes on suddenly and lasts secs to hrs
Electricity-like, shooting, stabbing or burning pain
May be triggered by touch, talking, eating, shaving or cold
Attacks may worsen over time
30
Outline management of trigeminal neuralgia
Carbamazepine
31
What is tuberous sclerosus?
Autosomal dominant genetic condition that affects multiple systems
Characteristic feature- Hamartomas (benign tissue growths)
32
What are the common locations of hamartomas in tuberous sclerosus?
Skin
Brain
Lungs
Heart
Kidneys
Eyes
32
What are the common mutations causing tuberous sclerosus?
TSC1 gene on Chr 9- Hamartin
TSC2 gene on Chr16- Tuberin
Hamartin and tuberin interact to control size and growth of cells- Abnormality leads to abnormal cell size and growth
33
Outline skin features of tuberous sclerosus
Ash leaf spots- Depigmented areas of skin shaped like ash leaf
Shagreen patches- Thickened, dimpled, pigmented patches of skin
Angiofibromas- Small skin-coloured/pigmented papules that occur over nose and cheeks
Ungual fibromas- Circular painless lumps that grow from nail bed and displace nail
Cafe-au-lait spots
Poliosis- Isolated patch of white hair on head/eyebrows/eyelashes/beard
34
List neurological features of tuberous sclerosis
Epilepsy
LD
Brain tumours
35
Outline other features of tuberous sclerosus
Rhabdomyolysis in heart
Angiomyolipoma in kidneys
Lymphangioleiomyomatosis in lungs
Subependymal giant cell astrocytoma in brain
Retinal hamartomas in eyes
36
Outline management of tuberous sclerosus
No treatment for underlying gene defect
Supportive- Monitor and treat complications
mTOR inhibitors may be used to suppress growth of brain/lung/kidney tumours
37
What is neurofibromatosis?
Genetic condition causing nerve tumours (neuromas) to develop throughout nervous system
Benign but can cause neuro and structural problems
38
Outline NFT1
More common than type 2
Chr 17- Codes for neurofibromin- Tumour suppressor protein
Autosomal dominant
39
Outline features of NFT1
C- Cafe au lait spots
R- Relative with NF1
A- Axillary or inguinal freckling
BB- Bony dysplasia, such as Bowing of long bone or sphenoid wing dysplasia
I- Iris hamartomas- Yellow-brown spots on iris
N- Neurofibromas
G- Glioma of optic pathway
40
What do neurofibromas look like on the skin?
Skin-coloured, raised nodules or papules with smooth, regular surface
41
Outline management of NF1
Diagnosis based on presentation
No treatment
Manage complications
42
List complications of NF1
Migraines
Epilepsy
Renal artery stenosis, causing HTN
LD
ADHD
Scoliosis of spine
Vision loss (2ndary to optic nerve glioma)
*Malignant peripheral nerve sheath tumours
*GI stromal tumour
Brain tumour
Spinal cord tumour
Increased risk cancer
43
Outline neurofibromatosis type 2
Chr 22- Codes for Merlin (tumour suppressor protein in Schwann cells (PNS))
Mutations lead to Schwannomas
Autosomal dominant
Acoustic neuromas (tumours of auditory nerve)
44
What is the key characteristic of NF2?
Bilateral acoustic neuromas
45
What is Guillain-Barre syndrome?
Acute paralytic polyneuropathy affecting PNS
Acute symmetrical ascending weakness and sensory symptoms
Triggered by infection- Campylobacter jejuni, CMV, EBV
46
Outline presentation of Guillain-Barre syndrome
Symptoms start within 4wks of triggering infection
Symmetrical ascending weakness
Reduced reflexes
Recovery period- Mths to yrs
May have peripheral loss sensation or neuropathic pain
May progress to CNs and cause facial weakness
Autonomic dysfunction- Urinary retention, ileus or heart arrhythmias
47
Outline diagnosis of Guillain-Barre syndrome
Clinically
Nerve conduction studies- Reduced signal through nerves
Lumbar puncture- Raised protein, normal cell count and glucose
48
Outline management of Guillain-Barre syndrome
Supportive care
VTE prophylaxis- PE leading cause of death
IVIG 1st line
Plasmapheresis- Alternative to IVIG
49
Outline prognosis of Guillain-Barre syndrome
Recovery mths-yrs
5% mortality due to respiratory or CV complications
50
What is Charcot-Marie-Tooth disease?
Inherited disease that affects peripheral motor and sensory neurones
Myelin or axon dysfunction
Autosomal dominant
Symptoms usually present <10y
51
Outline features of Charcot-Marie-Tooth disease
High foot arches (pes cavus)
Distal muscle wasting- Inverted champagne bottle legs
Lower leg weakness- Loss of ankle dorsiflexion (high stepping gait due to foot drop)
Weakness in hands
Reduced tendon reflexes
Reduced muscle tone
Peripheral sensory loss
52
List causes of peripheral neuropathy
Alcohol
B12 deficiency
Cancer and CKD
Diabetes and Drugs (isoniazid, amiodarone, leflunomide, cisplatin)
Every vasculitis
Charcot-Marie-Tooth disease
53
Outline management of Charcot-Marie-Tooth disease
Physio
Podiatrists
Analgesia (amitriptyline)
54
What is Myasthenia gravis?
AI condition affecting NMJ
Muscle weakness that progressively worsens with activity and improves with rest
55
Which condition has a strong link to myasthenia gravis?
Thymomas (thymus gland tumours)
56
Outline presentation of myasthenia gravis
Affects women <40y and men >60y
Associated with thymoma
Affects proximal muscles of limbs and small muscles of head and neck
Weakness worsens with muscle use and improves with rest
Difficulty climbing stairs, standing from a seat, raising hands above head
Extraocular muscle weakness- Diplopia
Eyelid weakness- Ptosis
Weak facial muscles
Difficulty swallowing
Fatigue in jaw when chewing
Slurred speech
57
How can you test muscles for fatiguability indicating myasthenia gravis?
Repeated blinking- Ptosis
Prolonged upward gazing- Diplopia
Repeated abduction of 1 arm 20 times- Unilateral weakness
58
Outline pathophysiology of myasthenia gravis
ACh receptor antibodies produced- Bind to postsynaptic ACh receptors- Blocked
More receptors used in activity= More blocked
Clear at rest
59
How do NMJ work?
Way for motor neurones to communicate with muscles
Axons release ACh from presynaptic membrane- Travels across synapse and attaches to receptors on postsynaptic membrane stimulating muscle contraction
60
Which antibodies can cause myasthenia gravis?
AChR antibodies
MuSK antibodies
LRP4 antibodies
61
Outline investigations in myasthenia gravis
Antibody tests
CT/MRI of thymus gland- Thymoma
Edrophonium test if doubt
62
Outline management of myasthenia gravis
Pyridostigmine- Cholinesterase inhibitor
Immunosuppression (eg: Prednisolone or azathioprine)
Thymectomy- Can improve symptoms even w/o thymoma
Rituximab
63
What is Myasthenic crisis?
Potentially life-threatening complication of myasthenia gravis
Acute worsening of symptoms- Often triggered by RTI
Respiratory muscles weaken- Can lead to respiratory failure
64
How is a myasthenic crisis managed?
NIV or mechanical ventilation
IVIGs
Plasmapheresis
65
What is Lambert-Eaton Myasthenic syndrome?
AI condition affecting NMJ
Symptoms less insidious/pronounced
Mostly paraneoplastic and occurs alongside SCLC
66
Outline pathophysiology of Lambert-Eaton Myasthenic syndrome
Antibodies against voltage-gated calcium channels in presynaptic membrane
Less ACh released- Weaker signal- Reduced muscle contraction
67
What is the role of voltage-gated calcium channels in muscle contraction?
Responsible for assisting release of ACh into synapse of NMJ
68
Outline presentation of Lambert-Eaton myasthenic syndrome
Proximal muscle weakness
Autonomic dysfunction- Dry mouth, blurred vision, impotence, dizziness
Reduced/absent tendon reflexes
*Signs and symptoms improve after periods of muscle contraction
69
Outline management of Lambert-Eaton Myasthenic syndrome
Exclude malignancy- SCLC
Amifampridine
Pyridostigmine
Immunosuppressants (eg: Prednisolone or azathioprine)
IVIGs
Plasmapheresis
70
What is Huntington's Chorea?
Autosomal dominant- Trinucleotide repeat disorder
Progressive neurological dysfunction
Has anticipation
71
Outline presentation of Huntington's Chorea
Symptoms begin 30-50y
Begins with cognitive/pschiatric/mood problems
Chorea
Dystonia
Rigidity
Eye movement disorders
Dysarthria
Dysphagia
72
What is chorea?
Involuntary, random, irregular and abnormal body movements
73
What is dystonia?
Abnormal muscle tone, leading to abnormal postures
74
What is rigidity?
Increased resistance to passive movement of joint
75
What is dysarthria?
Speech difficulties
76
What is dysphagia?
Swallowing difficulties
77
What is anticipation?
Huntington's chorea
Feature of trinucleotide repeat disorders- Successive generations have more repeats
Earlier age of onset
Increased severity of disease
78
Give examples of trinucleotide repeat disorders
Huntington's Chorea
Fragile X syndrome
79
Outline management of Huntington's chorea
Diagnosis- Genetic testing
Genetic counselling
Physiotherapy
Speech and language therapy
Tetrabenazine- Chorea symptoms
SSRIs
Advanced directives and end-of-life care
80
Outline prognosis of Huntington's disease
Progressive
Life expectancy- 10-20y after onset of symptoms
Death often due to aspiration pneumonia
Suicide also common
81
Outline presentation of brain tumours
As grow- Progressive focal neurological symptoms
Raised ICP symptoms:
Constant headache
Nocturnal
Worse on waking
Worse on coughing/straining
Vomiting
Papilloedema (fundoscopy)
Altered mental state
Visual field defects
Seizures (partial)
Unilateral ptosis
3rd and 6th nerve palsies
82
What are the causes of increased ICP?
Brain tumours
ICH
Idiopathic intracranial HTN
Abscess or infection
83
Outline papilloedema
Sign of raised ICP
Swelling of optic disc 2ndary to raised ICP
Paton's lines on fundoscopy
84
Outline gliomas
Tumours of glial cells in brain/SC
Glial cells surround and support neurones- Astrocytes, oligodendrocytes, ependymal cells
Most common and aggressive form- Glioblastoma
85
Outline meningiomas
Tumour growing from meninges
Usually benign
Take up space- Mass effect
86
What are the most common cancers to spread to the brain?
Lung
Breast
Renal cell carcinoma
Melanoma
87
Outline management of brain tumours
MRI 1st line
Biopsy during surgery
Options- Surgery/chemotherapy/radiotherapy/palliative care
88
Outline pituitary tumours
Tend to be benign
Press on optic chiasm- Bitemporal hemianopia
89
Outline presentation of pituitary tumour
Bitemporal hemianopia
Acromegaly (excessive GH)
Hyperprolactinaemia (excessive prolactin)
Cushing's disease (excessive ACTH and cortisol)
Thyrotoxicosis (excessive TSH and TH)
90
How can pituitary tumours be managed?
Trans-sphenoidal surgery
Radiotherapy
Bromocriptine- Blocks excess prolactin
Somatostatin analogues (eg: Octreotide)- Block excess GH
91
Outline acoustic neuromas
Benign tumours of Schwann cells that surround vestibulocochlear nerve
Occur at cerebellopontine angle
Bilateral= NF2
92
Outline presentation of acoustic neuroma
40-60y
Gradual onset:
Unilateral sensorineural hearing loss
Unilateral tinnitus
Dizziness or imbalance
Sensation of fullness in ear
Facial nerve palsy
93
Outline management of acoustic neuroma
Conservative- Monitor if no symptoms
Surgery
Radiotherapy
94
What are the infectious causes of LMN FN palsy?
Otitis media
Otitis externa
HIV
Lyme disease
95
What are the systemic disease causes of LMN FN palsy?
Diabetes
Sarcoidosis
Leukaemia
MS
Guillain-Barre
96
What are the tumour causes of LMN FN palsy?
Acoustic neuroma
Parotid tumour
Cholesteatoma
97
What are the trauma causes of LMN FN palsy?
Direct nerve trauma
Surgery
BoS fractures
98
What is the pathway of the facial nerve?
Exits brainstem at cerebellopontine angle
Passes through temporal bone and parotid gland
99
What are the 5 branches of the facial nerve?
Temporal
Zygomatic
Buccal
Marginal mandibular
Cervical
100
What is the motor function of the facial nerve?
Facial expression
Stapedius inner ear
Posterior digastric, stylohyoid and platysma muscles
101
What is the sensory function of the facial nerve?
Taste from ant. 2/3 of tongue
102
What is the parasympathetic supply of the facial nerve?
Submandibular and sublingual salivary glands
Lacrimal gland (tear production)
103
What are the features of UMN FN palsy?
Urgent
Forehead sparing on affected side- Can move forehead
Can be unilateral or bilateral
104
What are the features of LMN FN lesion?
Less urgent
Forehead not spared- Paralysis
105
Give examples of unilateral UMN lesions
Stroke
Tumours
106
Give examples of bilateral UMN lesions
Rare
Pseudobulbar palsies
MND
107
What is Bell's palsy?
Idiopathic
Unilateral LMN FN palsy
Recover over 12mths
108
Outline management of Bell's palsy
Prednisolone:
50mg for 10d
60mg for 5d, then 5d reducing regime
Lubricating eyedrops- Help prevent exposure keratopathy
109
Outline Ramsay-Hunt Syndrome
VZV
Unilateral LMN FN palsy
Painful and tender rash in ear canal, pinna and around ear on affected side- Can extend to anterior tongue and hard palate
110
Outline management of Ramsay-Hunt syndrome
Aciclovir and prednisolone
Lubricating eye drops
111
What are the SEs of NSAIDs?
Gastritis with dyspepsia
Stomach ulcers
Exacerbation of asthma
HTN
Renal impairment
Coronary artery disease, HF, stroke- Rare
112
What are the CIs of NSAIDs?
Asthma
Renal impairment
Heart disease
Uncontrolled HTN
Stomach ulcers
113
What is often prescribed alongside NSAIDs and why?
PPI- Omeprazole or lansoprazole
Reduce risk of GI SEs
114
What are the key SEs of opioids?
Constipation
Skin itching
Nausea
Altered mental state (sedation, cognitive impairment, confusion)
Respiratory depression
115
What is the antidote to opioids?
Naloxone
116
What are the options for opioid regimes in palliative care?
Background opiates- eg: 12hrly MR oral morphine or opioid patches
Rescue doses- For breakthrough pain, eg: Immediate-release oral morphine solution
Rescue dose is usually 1/6 of background 24h dose
117
Give examples of opioid patches
Buprenorphine patches- 5mcg/h patches
Fentanyl patches- 12mcg/h patches
118
What is complex regional pain syndrome?
Areas of abnormal nerve functioning, causing neuropathic pain, abnormal sensations and skin changes
Often triggered by an injury and isolated to one limb
119
What are the features of complex regional pain syndrome?
Hypersensitive
Allodynia- Pain associated with normal sensations
Intermittent swelling/colour changes/skin flushing/abnormal sweating
Abnormal hair growth
120
What are the 4 1st line treatments of neuropathic pain?
Amitriptyline- TCA
Duloxetine- SNRI
Gabapentin- Anticonvulsant
Pregabalin- Anticonvulsant
Tramadol- Only used as short-term rescue for flares
121
What is the 1st line medication for trigeminal neuralgia?
Carbamazepine
122
List common causes of neuropathic pain
Post-herpetic neuralgia from shingles in dermatome distribution on trunk
Nerve damage from surgery
MS
Diabetic neuralgia
Trigeminal neuralgia
Complex regional pain syndrome
123
What is the difference between chronic primary and chronic secondary pain?
Primary- No underlying condition
Secondary- As a result of an underlying condition
124
What are the options for managing chronic primary pain?
Supervised group exercises
Acceptance and commitment therapy
ABT
Acupuncture
Antidepressants (eg: Amitriptyline, duloxetine, SSRI)
125
What should patients with chronic primary pain not be started on?
Paracetamol
NSAIDs
Opiates
Anti-epileptics (eg: Pregabalin or gabapentin)
126
Outline a generalised tonic-clonic seizure
Tonic- Muscle tensing
Clonic- Muscle jerking
Associated with complete LOC
(also called Grand mal)
May have aura
Prolonged post-ictal phase
127
Outline partial seizures
(focal seizures)
Often in temporal lobe
Affect hearing, speech, memory and emotions
Patient awake
Simple partial seizures- Aware
Complex partial seizures- Lose awareness
128
What are some of the symptoms of partial seizures?
Deja vu
Strange smells, tastes, sight or sound
Unusual emotions
Abnormal behaviours
129
Outline myoclonic seizures
Sudden, brief muscle contractions- Abrupt jump/jolt
Remain awake
130
Outline tonic seizures
Sudden onset of increased muscle tone- Entire body stiffens
Usually results in fall if standing
Last seconds
131
Outline atonic seizures
Drop attacks
Sudden loss of muscle tone resulting in fall
Usually aware
May be indicative of Lennox-Gastaut syndrome
132
Outline absence seizures
Blank, stares into space, abruptly returns to normal
During episode- Unaware of surroundings, no response
Tend to stop as gets older
133
Outline infantile spasms
West syndrome
Rare
Starts at 6mths age
Clusters of full body spasms
Developmental regression and poor prognosis
134
What is a characteristic finding on EEG in West syndrome?
Hypsarrhythmia
135
Outline management of West syndrome
ACTH
Vigabatrin
136
Outline febrile convulsions
Tonic-clonic seizures occur during high fever
Not caused by epilepsy
Between 6mths and 5yrs
Don't usually cause lasting damage
1/3 have more than one
Slight increased risk of developing epilepsy
137
Outline investigations of seizures
EEG
MRI brain
ECG
Serum electrolytes
Blood glucose- Hypoglycaemia and diabetes
Blood cultures, urine cultures, lumbar puncture- If sepsis/encephalitis/meningitis suspected
138
What is the criteria for driving with seizures?
No driving if had seizures within past yr
139
What is the management of generalised tonic-clonic seizures?
1st line- Sodium valproate
2nd line- Lamotrigine or levetiracetam
140
What is the management of partial/focal seizures?
Lamotrigine or levetiracetam
141
Outline management of myoclonic seizures
1st line- Sodium valproate
2nd line- Levetiracetam
142
Outline management of tonic and atonic seizures
1st line- Sodium valproate
2nd line- Lamotrigine
143
Outline management of absence seizures
Ethosuximide
144
What are the SEs of sodium valproate?
Teratogenic
Liver damage and hepatitis
Hair loss
Tremor
Reduce fertility
145
What is the effect of using sodium valproate in pregnancy?
Neural tube defects
Developmental delay
146
What is status epilepticus?
Sezure lasting >5mins
Multiple seizures w/o regaining consciousness
147
Outline management of status epilepticus
Secure airway
High-conc. O2
Check blood glucose levels
IV access
148
Outline the medical management of status epilepticus
1st line- Benzo, repeat after 5-10mins if seizure continues
2nd line- IV levetiracetam, phenytoin or sodium valproate
3rd line- Phenobarbital or general anaesthesia
149
List options for use of benzos in status epilepticus
Buccal midazolam
Rectal diazepam
IV lorazepam
150
What are the acute complications of status epilepticus?
Hyperthermia
Pulmonary oedema
Cardiac arrhythmia
CV collapse
151
What are the long term complications of status epilepticus?
Epilepsy
Encephalopathy
Focal neurological defects
152
What is a benign essential tremor?
Fine tremor affecting all voluntary muscles
153
Outline features of benign essential tremor
Fine tremor (6-12Hz)
Symmetrical
More prominent with voluntary movement
Worse when tired/stressed/caffeine
Improved by alcohol
Absent during sleep
154
What are the differential diagnoses of tremor?
Parkinson's
MS
Huntington's chorea
Hyperthyroidism
Fever
Dopamine antagonists (eg: Antipsychotics)
155
Outline management of benign essential tremor
Propranolol
Primidone (barbiturate anti-epileptic)
156
Outline Parkinson's disease
Progressive reduction in dopamine in basal ganglia leading to disordered movement
157
What is the classic triad of features in Parkinson's disease?
Resting tremor
Rigidity
Bradykinesia
158
Outline features of Parkinson's disease
Symptoms asymmetrical (one side of body affected more)
Gradual onset
Tremor 4-6Hz (pill-rolling)
Resting tremor (improves with movement)- Gets worse when patient distracted
Rigidity- Resistance to passive movement of joint- Cogwheel rigidity
Bradykinesia:
Handwriting smaller
Shuffling gait
Difficulty initiating movement
Difficulty turning (take lots of little steps)
Hypomimia (reduced facial movement/expression)
Depression
Sleep disturbance and insomnia
Anosmia
Postural instability
Cognitive impairment and memory problems
159
What are Parkinson's-Plus syndromes?
Multiple system atrophy
Dementia with Lewy bodies
160
What is multiple system atrophy?
Parkinson's-Plus syndrome
Rare
Neurones of various systems in brain degenerate
Basal ganglia- Leads to Parkinson's presentation
Other areas- Autonomic dysfunction and cerebellar dysfunction
161
What are signs of autonomic dysfunction?
Postural hypotension
Constipation
Abnormal sweating
Sexual dysfunction
162
What are the signs of cerebellar dysfunction?
Ataxia
163
Outline dementia with Lewy bodies
Dementia with features of Parkinsonism
Progressive cognitive decline
Associated symptoms- Hallucinations, delusion, REM sleep disorders, fluctuating consciousness
164
Outline diagnosis of Parkinson's disease
Diagnosed clinically
165
Outline management of Parkinson's disease
Levodopa
COMT inhibitors
Dopamine agonists
MAOB inhibitors
166
Outline levodopa
Synthetic dopamine
Normally combined (eg: Co-careldopa or Co-beneldopa)- Stops it being metabolised in body before reaches brain
Most effective for treatment of Parkinson's but declines over time
167
What is the main SE of levodopa?
Dyskinesia- Abnormal movements associated with excessive motor activity:
Dystonia
Chorea
Athetosis
168
What is dystonia?
Excessive muscle contraction leads to abnormal postures or exaggerated movement
169
What is athetosis?
Involuntary twisting or writhing movements, usually in fingers/hands/feet
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What is an option for managing dyskinesia caused by levodopa?
Amantadine (glutamate antagonist)
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Outline COMT inhibitors
Eg: Entacapone
Slows breakdown of levodopa in body and brain
Extends effective duration of levodopa
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Outline Dopamine agonists
Mimic action of dopamine in basal ganglia, stimulate dopamine receptors
Less effective than levodopa in reducing symptoms
Eg: Bromocriptine, pergolide, cabergoline
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What is the most notable SE of dopamine agonists?
Pulmonary fibrosis
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Outline MAO-B inhibitors
Increase circulating dopamine
Eg: Selegiline, rasagiline
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What is motor neurone disease?
Variety of diseases affecting motor nerves
Progressive, eventually fatal condition where motor neurones stop functioning
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What are the types of MND?
Amyotrophic lateral sclerosis (ALS)- Most common
Progressive bulbar palsy- Affects muscles of talking and swallowing
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Outline pathophysiology of MND
Progressive degeneration of upper and lower motor neurones
Increased risk with smoking, FHx, exposure to heavy metals, and certain pesticides
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Outline presentation of MND
Late middle-aged man
Insidious, progressive weakness of muscles throughout body
Weakness first noticed in upper limbs
May be increased fatigue when exercising
May complain of clumsiness/dropping things more often
Can develop dysarthria (Slurred speech)
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What are the signs of lower MND?
Muscle wasting
Reduced tone
Fasciculations
Reduced reflexes
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What are the signs of upper MND?
Increased tone or spasticity
Brisk reflexes
Upgoing plantar reflex
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Outline diagnosis of MND
Clinical presentation
Diagnosis of exclusion
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Outline management of MND
Riluzole can slow progression and extend survival by several months in ALS
NIV- Support breathing when respiratory muscles weaken
Baclofen- For muscle spasticity
Benzos- Help breathlessness caused by anxiety
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Outline pathophysiology of Parkinson's disease
Basal ganglia- Coordinates habitual movement (walking/voluntary movement)
Dopamine- Essential for basal ganglia function
Slow progressive drop in dopamine production
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What is MS?
Chronic and progressive AI condition
Demyelination in CNS
Immune system attacks myelin sheath of myelinated neurones
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Outline pathophysiology of MS
Myelin covers axons of neurones to help electrical impulses travel faster
Ms affects CNS (oligodendrocytes)
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What are the different cells in myelin?
Oligodendrocytes- CNS
Schwann cells- PNS
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What is the characteristic feature of MS?
Disseminated in time and space:
Vary in location and symptoms change over time
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List causes of MS
Multiple genes
EBV
Low vit D
Smoking
Obesity
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Outline onset of MS
Symptoms progress over >24h and last days to weeks at 1st presentation then improve
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Outline optic neuritis and MS
Most common presentation of MS
Demyelination of optic nerve
Unilateral reduced vision
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What are the key features of optic neuritis?
Central scotoma (enlarged central blind spot)
Pain with eye movement
Impaired colour vision
RAPD
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What is RAPD?
Relative afferent pupillary defect
Pupil in affected eye constricts more when shining light in contralateral eye than when shining it in affected eye
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List causes of optic neuritis
MS
Sarcoidosis
SLE
Syphilis
Measles or mumps
Neuromyelitis optica
Lyme disease
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Outline symptomatic treatments of MS
Exercise
Fatigue- Amantadine, modafinil or SSRIs
Neuro pain- Amitriptyline or gabapentin
Depression- SSRIs
Urge incontinence- Solifenacin
Spasticity- Baclofen or gabapentin
Oscillopsia- Gabapentin or memantine
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How are MS relapses treated?
Steroids:
500mg orally daily for 5d
1g IV daily 3-5d if severe
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Outline diagnosis of MS
Clinical picture and symptoms
MRI scans
Lumbar puncture- Oligoclonal bands
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What is found on MS LP?
Oligoclonal bands
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Outline disease patterns of MS
Clinically isolated syndrome- 1st episode of demyelination and neuro signs/symptoms- Lesions on MRI suggest more likely to occur again
Relapsing-remitting- Most common
Secondary progressive- Was relapsing-remitting, now progressive worsening with incomplete remission
Primary progressive- Worsening disease from point of diagnosis, w/o relapses or remissions
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Outline ataxia and MS
Problem with coordinated movement
Sensory- Loss of proprioception- Positive Romberg's and pseudoathetocis (involuntary writhing movement)- Lesion in dorsal column
Cerebellar ataxia- Problems with cerebellum coordinating movement- Cerebellar lesion
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Outline focal weakness in MS
Incontinence
Horner syndrome
Facial nerve palsy
Limb paralysis
Transverse myelitis
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Outline focal sensory symptoms in MS
Trigeminal neuralgia
Numbness
Paraesthesia
Lhermitte's sign
Transverse myelitis
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What is Lhermitte's sign?
Electric shock sensation travelling down spine and into limbs when flexing neck
Indicates disease in cervical spinal cord in dorsal column
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Outline management of complications in SAH
Hydrocephalus:
LP
External ventricular drain
VP shunt
Seizures:
Anti-epileptics
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Which eye movement abnormalities can occur in MS?
Oscillopsia- Visual sensation of environment moving, can't create stable image
CN III/IV/VI- Diplopia and nystagmus
Internuclear ophthaloplegia- Lesion in medial longitudinal fasciculus- Impaired adduction in same eye as lesion, nystagmus in contralateral abducting eye
CN VI- Conjugate lateral gaze disorder- If lesion in L eye, look left, R eye adducts, L eye stays central
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What is a subarachnoid haemorrhage?
Bleeding in subarachnoid space
Between pia mater and arachnoid membrane
Cerebral aneurysms
High mortality- Approx. 30%
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List RFs for subarachnoid haemorrhage
45-70y
Women
Black ethnic origin
HTN
Smoking
Excessive alcohol intake
FHx
Cocaine use
SCA
Marfans or Ehlers-Danlos
Neurofibromatosis
ADPKD
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Outline presentation of subarachnoid haemorrhage
Sudden-onset occipital headache
During strenuous activity- Heavy lifting/sex
Thunderclap headache
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Outline investigations of subarachnoid haemorrhage
1st line- CT head- Hyper-attenuation in subarachnoid space
Lumbar puncture in normal CT head- Wait at least 12h after symptoms start (takes time for bilirubin to accumulate in CSF)
CT angiography- After confirmed diagnosis- Locates source of bleeding
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What will an LP of subarachnoid haemorrhage show?
Raised cell count
Xanthochromia- Yellow CSF due to bilirubin
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Outline management of SAH
Surgical intervention for aneurysms- Coils or clipping
Nimodipine- CCB- Prevents vasospasm (common complication following SAH- Causes brain ischaemia)
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What is an extradural haemorrhage?
Bleeding between skull and dura mater
Rupture of MMA in temporoparietal region
Bi-convex on CT, don't cross suture lines
Lucid period
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Outline subdural haemorrhage
Between dura mater and arachnoid mater
Rupture of bridging veins
Crescent shape on CT, cross suture lines
Occur in elderly and alcoholics
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Outline intracerebral haemorrhage
Bleeding in brain tissue
Presents similar to ischaemic stroke with sudden-onset focal neuro symptoms
Occur spontaneously or secondary to ischaemic stroke/tumour/aneurysm rupture
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Outline management of intracranial haemorrhage
Immediate CT head
FBC (platelets) and coagulation screen
Avoid hypotension
Small bleed:
Conservative management
Close monitoring, repeat imaging
Surgical (extradural or subdural):
Craniotomy
Burr holes
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What are the two types of cerebrovascular accident?
Ischaemia or infarction (ischaemic stroke)
Intracranial haemorrhage (haemorrhagic stroke)
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What is the difference between ischaemia and infarction?
Ischaemia- Inadequate blood supply
Infarction- Tissue death
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What is a TIA?
Temporary neuro dysfunction (lasts <24h) caused by ischaemia w/o infarction
Rapid onset and resolve quickly
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What are crescendo TIAs?
2+ TIAs within 1wk
Indicate high risk of stroke
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Outline presentation of stroke
Limb weakness
Facial weakness
Dysphasia (speech disturbance)
Visual field defects
Sensory loss
Ataxia and vertigo (post. circulation infarction)
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List RFs for stroke
Previous stroke/TIA
AF
Carotid artery stenosis
HTN
Diabetes
Raised cholesterol
FHx
Smoking
Obesity
Vasculitis
Thrombophilia
COCP
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Outline management of TIA
Aspirin 300mg daily
Refer within 24h for specialist assessment
Diffusion-weighted MRI scan
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Outline management of stroke
Exclude hypoglycaemia
Immediate CT brain
Aspirin 300mg daily for 2wks after scan
Thrombolysis with alteplase- Consider once haemorrhage excluded- Give within 4.5h symptoms onset
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What is the difference between management of BP in haemorrhagic stroke and ischaemic stroke?
Ischaemic- Low BP can worsen ischaemia
Haemorrhagic- Aggressively treated to lower and reduce ICP
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How are the underlying causes of stroke assessed?
Carotid imaging- Carotid artery stenosis
ECG- AF
Start anticoagulation following stroke due to AF after finishing course of aspirin
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What are the top risk factors for stroke?
AF
Carotid artery stenosis
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Outline secondary prevention of stroke
Clopidogrel- 75mg OD
Atorvastatin- 20-80mg- Delay at least 48h
BP and diabetes control
Stop smoking, lose weight, exercise as required
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