Neurology Flashcards
(205 cards)
1
Q
What causes a stroke?
A
- 85% due to ischaemia or infarction-> due to thrombus/emboli, atherosclerosis, shock or vasculitis
- 15% due to intracranial haemorrhage
2
Q
What is a transient ischaemic attack (TIA)?
A
Transient neurological dynsfunction secondary to ischaemia without infarction-> typically resolves within 24 hours and can precede stroke
3
Q
What is a crescendo TIA?
A
When a patient has 2+ TIAs in a week
4
Q
How can a stroke present (in general)?
A
- FAST-> face, arm, speech, time
- Weakess
- Dysphasia-> speech problems
- Visual or sensory loss
- Sudden onset
5
Q
What is the ROSIER tool used for?
A
Recognition of stroke in emergency room-> work out likelihood that clinical signs are due to a stroke
6
Q
What are the risk factors for a stroke/TIA?
A
CVD, previous TIA/stroke, AF, carotid artery disease, HTN, diabetes, smoking, thrombophilia, COCP
7
Q
Acute management of stroke?
A
- Admission
- Exclude hypoglycaemia (BMs)
- Exclude haemorrhage (CT head)
- Aspirin 300mg stat + continue for 2 weeks
- Thrombolysis-> with alteplase/streptokinase within 4.5 hours of symptom onset
- Thrombectomy-> mechanical removal within 6 hours (or 24 hours) of symptom onset
- Be careful with BP-> don’t try lower as can risk hypoperfusion to brain
- MDT + rehabilitation-> SALT, OT, physio etc
8
Q
Treatment for TIA?
A
Aspirin 300mg daily + CVD secondary prevention
9
Q
Investigations for stroke?
A
- Blood glucose-> rule out hypoglycaemia
- CT head-> rule out haemorrhage
- Bloods-> lipids, PTT
- ECG/CXR/TOE-> look for cardiac causes
- Diffusion weighted MRI-> assess which vascular territory affected
- Carotid US-> for stenosis + endartectomy/stent criteria
10
Q
Secondary prevention of stroke?
A
- Aspirin 300mg for 14 days + clopidogrel 75mg lifelong
- Can use dipyramidole if clopidogrel CI’d
- Consider atorvastatin 80mg OD + BP management
11
Q
How does an anterior cerebral artery stroke present?
A
Lower limb weakness, loss of spontaneous speech, drowsy
12
Q
How does a middle cerebral artery stroke present?
A
Upper limb weakness, face drop, aphasia, hemianopia
13
Q
How does a posterior cerebral artery stroke present?
A
Visual field defects, visual agnosia, prosopagnosia
14
Q
How does a brainstem stroke present?
A
Quadriplegia, locked in syndrome, retain awareness
15
Q
How does a lacunar stroke present?
A
Motor +/- sensory deficit, dysarthria etc
16
Q
What are the components of the Glasgow Coma Scale?
A
- Eyes-> spontaneously open (4), to speech (3), to pain (2), don’t open (1)
- Verbal response-> orientated (5), confused (4), inappropriate words (3), sounds (2), none (1)
- Motor-> obeys commands (6), localises to pain (5), normal flexion (3), abnormal flexion (3), extends to pain (2), none (1)
17
Q
What is an intracerebral bleed and where can this occur?
A
- Bleed into brain tissue
- Lobar, deep, intraventricular, basal ganglia, cerebellar
18
Q
What causes an intracranial bleed?
A
Spontaneous, ischaemia, tumours, aneurysm ruptures
19
Q
What causes a subarachnoid haemorrhage?
A
- Bleed between pia + arachnoid mater (where CSF located)
- Ruptured berry aneurysm
20
Q
How does a subarachnoid haemorrhage typically present?
A
- Thunderclap headache-> sudden, occipital, during strenuous activity
- Neck stiffness, photophobia, vision loss, speech/weakness, LOC, seizures
21
Q
What causes a subdural haemorrhage?
A
- Bleed between the arachnoid and dura mater
- Rupture of bridging veins in outer meningeal layer
22
Q
Who is most at risk of a subarachnoid haemorrhage?
A
- More common in black patients, females and aged 45-70
- Associated with smoking, alcohol, cocaine, sickle cell disease and connective tissue disorders
23
Q
Who is most at risk of subdural haemorrhage?
A
Often older and alcoholic-> more atrophy + more likely to rupture
24
Q
What does a subdural haemorrhage look like on a CT head?
A
- Crescent shape (thin line)
- Can cross cranial suture lines
25
What causes an extradural haemorrhage?
- Bleed between the dura and skull
- Middle meningeal artery rupture in temporo-parietal region
- Can be due to temporal bone fracture
26
What does an extradural haemorrhage look like on a CT head?
- Bi-convex (lemon) shape
| - Can't cross the cranial sutures
27
How does an extradural haemorrhage typically present?
- Often young patient with a head injury
- Ongoing headache
- Period of improved neuro function then severe decline over a few hours (begins to compress)-> lucid interval
28
Investigations for subarachnoid haemorrhage?
- CT head 1st line-> hyperattenuation
- LP-> raised RBCs or xanthochromia (yellow + due to bilirubin breakdown)
- Angiography (CT/MRI)-> locate source
29
Management of subarachnoid haemorrhage?
- Coiling or clipping surgically
- Nimodipine-> CCB used to prevent vasospasm (can cause ischaemia)
- LP or shunt for hydrocephalus
- Antiepiletics if seizures
30
How might a subdural haemorrhage present?
- Often after deceleration injury <9 months ago
- Latent period of 8-10 weeks
- Fluctuating consciousness, headaches, personaliry change
31
How is subdural haemorrhage treated?
Surgical removal of clot then address cause
32
How is extradural haemorrhage treated?
- Drainage + may ligate bleeding vessel
| - Mannitol IV
33
What is multiple sclerosis?
Chronic and progressive demyelination of neurones in CNS due to immune activation against myelin
34
Who does multiple sclerosis typically affect?
- Women under 50
| - Often due to genes, low vitamin D, smoking, obesity, EBV
35
When do the symptoms of MS typically improve?
-During pregnancy and post-partum
36
What is the pathophysiology of MS?
- Myelin covers axons in CNS-> from oligodendrocytes (CNS) + Schwann cells (PNS)
- MS affects CNS-> inflammation around myelin + immune cell infiltration
- Affects conduction of electrical signals of nerve
- Usually multiple areas of demyelination during attacks (eg optic neuritis) but not all cause symptoms at same time
- Re-myelinate in early disease but incomplete + permanent later
- Lesions vary in location-> 'disseminated in time and space'
37
How might MS present?
- Optic neuritis
- Eye movement abnormalities
- Focal weaknesses
- Focal sensory symptoms
- Ataxia
- Different disease patterns
38
What disease patterns of MS exist?
- Clinically isolated syndrome
- Relapsing-remitting
- Primary progressive
- Secondary progressive
39
How does optic neuritis typically present?
- Unilateral loss of vision over hours-days
- Central scotoma (enlarged blind spot)
- Pain on eye movement
- Impaired colour vision
- Relative afferent pupillary defect
40
What can cause optic neuritis?
MS, sarcoidosis, SLE, diabetes, syphilis, measles, mumps, Lyme disease
41
How is optic neuritis managed?
- Ophthalmologist assessment
- Steroids
- Recover in 2-6 weeks
- 50% get MS-> MRIs to predict
42
Other than optic neuritis, what eye symptoms can you get with MS?
Sixth cranial nerve palsy-> causes internuclear ophthalmoplegia + conjugate lateral gaze disorder
- IO-> nerve + muscle issue
- CLGD-> affected eye unable to abduct when looking laterally (ie stays in middle whilst other eye adducts) due to muscle dysfunction
43
What focal weaknesses can MS present with?
Bell's palsy, Horner's syndrome, limb paralysis, incontinence
44
What focal sensory symptoms can MS present with?
Trigeminal neuralgia, numbness, paraesthesia, Lhermitte's sign
45
What is Lhermitte's sign?
- Present in MS
- Electric shock down spine + limbs when flexing neck
- Indicates disease (demyelination) in cervical spinal cord's dorsal column
46
What types of ataxia can present in MS?
- Sensory-> lose proprioception, positive Romberg's test, psudoathetosis
- Cerebellar-> lesions
47
What does the 'clinically isolated syndrome' disease pattern present as in MS?
- The 1st episode of demyelination + neuro signs-> can't diagnose based on this
- If lesions are present on MRI are likely to develop MS
48
What does the 'relapse-remitting' disease pattern present as in MS?
- Disease + symptoms then recovery
- Active-> symptoms + lesions on MRI
- Not active-> no symptoms
- Can be worsening or not worsening over time
49
What does the 'primary progressive' disease pattern present as in MS?
Worsening disease from diagnosis, no relapse + remissions, active and/or progressive
50
What does the 'secondary progressive' disease pattern present as in MS?
- Relapsing-remitting at first then progressive worsening + incomplete remissions
- Symptoms become more permanent
- Active and/or progressive
51
What does 'active disease' mean in MS?
New symptoms or MRI lesions
52
What does 'progressing disease' mean in MS?
Worsening over time regardless of relapse
53
Investigations and diagnosis for MRI?
- Clinical
- Disseminated in space and time
- MRI lesions
- LP-> oligoclonal bands in CSF
54
How are relapses treated in MS?
- Methylprednisolone 500mg PO for 5 days
| - If oral previously failed-> methylprednisolone IV 1g for 3-5 days
55
How is MS managed long-term?
- Disease modifying drugs + biological therapies-> induce long term remission
- Symptom control-> exercise, neuropathic meds, anti-depressants, oxynutynin/tolterodine (for urge incontinence), baclofen (spasticity)
56
What is motor neurone disease?
An umbrella term for conditions causing progressive decline of motor neurone function (doesn't affect sensory nerves)
57
What are the different types of motor neurone disease?
- Amyotrophic lateral sclerosis-> most common
- Progressive bulbar palsy
- Progressive muscular atrophy
- Primary lateral sclerosis
58
What is the pathophysiology of MND?
- Degradation of upper + lower motor neurones
| - Risk factors-> genetics, smoking, heavy metals, pesticides etc
59
What is the presentation of MND?
- Insidious + progressive muscle weakness often starting in upper limbs-> progresses to trunk, face, speech, legs
- Initial-> clumsy + drop things, dysarthria
- LMN signs-> wasting, hypotonia, fasiculations, hyporeflexia
- UMN signs-> hypertonia, spasticity, hyperreflexia, upgoing plantar responses
60
How in MND diagnosed?
Clinically + after ruling out other causes
61
Treatment options for MND?
- None will stop/reverse disease
- Riluzole-> slows progression
- NIV-> for breathing support
- Advance directives + end of life care planning
62
What is Parkinson's disease?
Progressive reduction of dopamine in the basal ganglia (specifically the substantia nigra) causing movement disorders
63
What is the pathophysiology of Parkinson's disease?
- Basal ganglia structures help co-ordinate walking + voluntary movements + learning patterns
- Substantia nigra-> produces dopamine (needed for BG functioning)
- PD-> gradual fall in dopamine production
64
Presentation of Parkinson's disease?
Typically triad of resting tremor + rigidity + bradykinesia (often in older male)
- Resting tremor-> unilateral, pill rolling, improves with movement
- Cogwheel rigidity-> tension in arm + gives way to movement in small increments
- Bradykinesia-> small + slow movements eg small handwriting, shuffling gait, hard to initiate movements
- Hypomimia-> reduced facial expressions
- Depression + insomnia + cognitive impairment
- Postural instability
- Anosmia
65
What is benign essential tremor and how does it present?
- Symmetrical essential tremor that improves at rest + after alcohol
- Usually in hands, head, jaw
- Worsened by fatigue, stress + caffiene
66
What are the 'Parkinson's plus' syndromes?
- Progressive supranuclear palsy
- Corticobasal degeneration
- Multiple system atrophy
- Dementia with Lewy bodies
67
What is multiple system atrophy?
Neurones in multiple systems (including the basal ganglia) degenerate-> autonomic dysfunction, cerebellar and Parkinsonian symptoms
68
How does dementia with Lewy bodies typically present?
- Parkinson features + progressive cognitive decline
- Visual hallucinations (eg Lilliputian)
- Delusions
- REM sleep disorders
- Fluctuating consciousness
69
How is Parkinson's disease diagnosed?
Usually clinically-> can use the UK PD society brain bank clinical diagnostic criteria
70
Management options for Parkinson's disease?
- Co-beneldopa or co-careldopa (1st line)-> levodopa + peripheral decarboxylase inhibitors (to stop levodopa break down)
- Dopamine agonists-> cabergoline, bromocriptine, ropinerole
- Monoamine oxidase-B inhibitors-> selegiline or rasagiline
71
What are the potential side effects of dopaminergic drugs used in Parkinson's?
- Dystonias-> excess muscle contractions + abnormal movements
- Chorea-> involuntary movements
- Athetosis-> twisting/writhing of hands/feet
- Levodopa can wear off quite quickly + can become ineffective after using for an extended period
72
Management of benign essential tremor?
- Not usually needed
- Propranolol
- Primidone (anti-epileptic)
73
What is epilepsy?
An umbrella term for a tendency to have seizures
74
What is a seizure?
Transient episode of abnormal electrical activity in the brain
75
What are the investigations for a seizure/epilepsy?
- Electroencephalogram (EEG)-> patterns in different forms
- MRI brain-> structural problems
- ECG-> cardiac causes eg syncope
- BM-> rule out hypoglycaemia
76
How does a generalised tonic-clonic seizure typically present?
- Not always a prodrome
- LOC + tonic (tensing) then clonic (jerking)
- Tongue biting, incontinence, groaning, irregular breathing
- Post-ictal-> confused, drowsy, irritable, depression
77
How are generalised tonic-clonic seizures managed?
Sodium valproate or lamotrigine
78
How do focal seizures present?
- Usually start in temporal lobe causing hearing, speech, memory and emotional symptoms
- Hallucinations, deja vu, memory flashbacks, on auto-pilot etc
- May be aware but one area of body has seizure
79
How are focal seizures managed?
Carbamazepine or lamotrigine
80
How do absence seizures usually present?
- Usually in children
| - Blank, stare into space, non-responsive, 10-20 seconds, return to normal abruptly
81
How are absence seizures treated?
- Sodium valproate or ethosuximide
| - 90% stop as get older
82
What are atonic seizures?
- 'Drop attacks'-> brief lapse in muscle tone for <3 minutes
- Often start in kids
- May indicate Lennox-Gastaut syndrome
83
What might atonic seizures indicate?
Lennox-Gastaut syndrome
84
Treatment for atonic seizures?
Sodium valproate or lamotrigine
85
What are myoclonic seizures?
- Sudden brief muscle contractions + usually retain awareness
- Can indicate juvenile myoclonic epilepsy
86
What can myoclonic seizures in kids indicate?
Juvenile myoclonic epilepsy
87
What is West syndrome?
- Infantile spasms-> clusters of full body attacks
- Usually start aged 6 months
- Poor prognosis
88
How is West syndrome (infantile spasms) treated?
Vigabatrin + prednisolone
89
What is the prognosis of West syndrome (infantile spasms)?
- 1/3 can be seizure free
| - 1/3 die by age 25
90
What is the mechanism of action of sodium valproate?
Increases GABA activity and relaxes the brain
91
What are the side effects of sodium valproate?
- Teratogenic-> not given to women + girls unless meet pregnancy prevention programme criteria
- Liver damage, hepatitis, hair loss, tremor
92
What are the side effects of carbamazepine?
Agranulocytosis, aplastic anaemia, P450 inducer
93
What are the side effects of ethosuxamide?
Night terrors, rashes
94
What are the side effects of phenytoin?
Folate deficiency, megaloblastic anaemia, vitamin D deficiency, osteomalacia, P450 inducer
95
What are the side effects of lamotrigine?
- Steven Johnson syndrome
- Leukopaenia
- DRESS syndrome-> drug rash with eosinophilia + systemic symptoms
96
What is status epilepticus?
A seizure lasting >5 minutes (techically 30 minutes) or 3+ seizures in 1 hour
97
How is status epilepticus managed?
- ABCDE
- IV lorazepam-> 4mg and repeat in 10 minutes if need to
- IV phenytoin or phenobarbital
-If in the community-> buccal midazolam or rectal diazepam
98
What causes neuropathic pain?
Abnormal functioning of the sensory nerves-> painful signals to brain
99
What are some causes of neuropathic pain?
- Nerve damage-> surgery, MS, diabetic foot
- Trigeminal neuralgia
- Post-herpatic neuralgia
- Complex regional pain syndrome
100
What is postherpatic neuralgia?
Neuropathic pain due to shingles-> usually on trunk + one dermatome
101
What are the features of neuropathic pain?
- Burning, tingling, pins + needles, electric shocks, loss of sensation
- DN4 questionnaire-> score of 4+/10 may indicate
102
How is neuropathic pain managed?
Amitriptyline, duloxetine, gabapentin, pregabalin, tramadol (for short-term flare), capsaicin, physio, psych
103
How is trigeminal neuralgia treated?
- Carbamazepine
| - Decompression surgery
104
What is complex regional pain syndrome (CRPS)?
Abnormal nerve function causes neuropathic pain + sensory issues
105
How does complex regional pain syndrome (CRPS) present?
- Painful, hypersensitivity, swelling, colour change, temperature change, abnormal sweating, hair growth
- Usually in 1 limb after an injury
106
How is complex regional pain syndrome (CRPS) treated?
Under a specialist with neuropathic pain medications
107
What is the pathway of the facial nerve (CN VII)?
Exits brainstem at cerebellopontine angle-> through temporal bone-> through parotid gland-> splits to 5 branches (temporal, zygomatic, buccal, marginal manbibular, cervical)
108
What are the different functions of the facial nerve (CN VII)?
- Motor-> muscles of expression, stapedius (inner ear), some neck muscles
- Sensory-> anterior 2/3 of the tongue
- Parasympathetic-> submandibular, sublingual and lacrimal glands
109
How does an upper motor neurone lesion present in facial nerve (CN VII) palsy and what might this indicate?
- Forehead sparing + can move on affected side
- Each side of the forehead has UMN innervation from both sides of the brain-> lesion on one side means will still be innervated from the other side
- May indicate CVA or tumour-> more serious
110
What might bilateral facial palsy indicate?
Bilateral UMN lesions-> MND or pseudobulbar palsy
111
How does Bell's palsy present?
- Unilateral LMN palsy-> CN VII affected
| - Recovery after few weeks but may get residual weakness for 12+ months
112
What causes Bell's palsy?
Idiopathic-> CN VII problem
113
What is the treatment for Bell's palsy?
- Prednisolone 50mg for 10 days or reducing regime-> when present within 72 hours of symptoms
- Lubricating eye drops or taping eye-> prevent damage + drying (exposure keratopathy)
114
What is Ramsay-Hunt syndrome?
Facial nerve palsy due to varicella zoster virus (VZV)
115
How does Ramsay-Hunt syndrome present?
- Unilateral LMN palsy
- Painful vesicular rash in ear canal + pinna + around ear
- Can extend to tongue and hard palate
116
How is Ramsay-Hunt syndrome treated?
- Prednisolone + acyclovir
| - Lubricating eye drops
117
What can cause LMN facial nerve palsy?
- Bell's palsy
- Ramsay-Hunt syndrome
- Systemic-> DM, sarcoidosis, leukaemia, MS, GBS
- Tumours-> acoustic neuroma, parotid tumour, cholestatomas
- Trauma-> direct, damage from surgery, base of skull fracture
- Otitis media infection
- Malignant otitis externa
- HIV
- Lyme's disease
118
What are the symptoms and signs of raised intracranial pressure?
- Headache-> constant, nocturnal, worse on waking or cough or strain or bend forward
- Vomiting
- Altered mental state
- Visual field defect
- Seizures
- Unilateral pstosis
- CN III + VI palsy
- Papilloedema on fundoscopy
119
Causes of raised intracranial pressure?
Tumours, haemorrhage, idiopathic intracranial hypertension, abscess, infection
120
What are the red flag symptoms for a headache?
Constant, nocturnal, worse on waking or cough or strain or bend forward, weight loss, new neuro symptoms, vomiting
121
What is the pathophysiology of papilloedema?
- Swelling of optic disc secondary to raised ICP
| - Sheath around optic nerve connected to subarachnoid space-> CSF flows into sheath when high pressure-> swells
122
What does papilloedema look like on fundocscopy?
Blurred disc margin, curved retinal vessels over disc, loss of venous pulsation, engorged veins, haemorrhages, Paton's lines
123
What types of cancer commonly metastasise to the brain?
Lung, breast, renal cell, melanoma
124
What is a glioma?
A tumour of the glial cells in the brain + spinal cord-> graded from 1 (benign) to malignant
125
What are the different types of brain tumour?
- Gliomas-> Astrocytoma, Oligodendroglioma, Ependymoma
- Meningioma
- Pituitary tumours
- Acoustic neuroma
126
What are the different types of gliomas?
- Astrocytoma AKA glioblastoma multiforme-> most common + most malignant
- Oligodendroglioma
- Ependymoma (least malignant)
127
What is a meningioma?
A benign tumour of the meninges-> can lead to raised ICP symptoms due to mass effect
128
What is a meningioma?
A benign tumour of the meninges-> can lead to raised ICP symptoms
129
What do pituitary tumours typically cause and why?
- Bitemporal hemianopia (outer half of vision lost) due to pressing on the optic chiasm
- Hormone deficiencies (hypopituitarism)
- Hormone excess-> acromegaly, hyperprolactinaemia, Cushing's, thyrotoxicosis
130
What is an acoustic neuroma?
- AKA vestibular schwannoma-> tumour of Schwann cells around auditory nerve (innervates inner ear)
- Slow growing but can grow and produce symptoms
- Usually unilateral
131
In what condition can bilateral acoustic neuromas occur?
Neurofibromatosis type 2
132
What are the symptoms of an acoustic neuroma?
Hearing loss, tinnitus, balance problems, CN VII palsy
133
How are brain tumours treated?
Depends on type-> surgery, palliative care, chemo, radiotherapy
134
How are pituitary tumours managed?
- Transphenoidal surgery
- Radiotherapy
- Prolactin secreting-> bromocriptine
- GH-secreting-> somatostatin analogues eg octreotide
135
What is Huntington's Chorea?
Autosomal dominant genetic condition in which the nervous system progressively deteriorates causing movement disorders
136
What is the inheritance pattern of Huntington's chorea?
Autosomal dominant
137
What happens to the patient's genes in Huntington's chorea?
- Trinucleotide repeat of HTT gene on chromosome 4
| - Anticipation-> successive generations have more repeats so earlier onset + increased disease severity
138
How does Huntington's chorea present?
- Insidious + progressive onset
- Cognitive, behavioural + mood problems first
- Movement-> involuntary (chorea), eye disorders, dysarthria, dysphagia
139
How is Huntington's chorea managed?
- Genetic tests + counselling
- No treatment stops or slows progression
- Olanzapine or tetrabenzine can help with movement problems
140
What commonly causes death in Huntington's chorea?
- Respiratory disease (more susceptible + less able to fight infection)
- Suicide
141
What is myasthenia gravis?
An autoimmune condition in which antibodies are made against acetylcholine receptors in the neuromuscular junction causing muscle weakness
142
What is the pathophysiology of myasthenia gravis?
- NMJs between motor nerves + muscles
- Acetylcholine released by axons + bind to receptors on the posy-synaptic membrane to stimulate muscle contraction
- These receptors used up more during activity-> less effective stimulation when increased
- Antibodies in MG activate complement system in NMJ + damage cells at post-synaptic membrane
- Antibodies in MG also destroy MuSK + LRP4 proteins in receptors
143
How does myasthenia gravis present?
- Diplopia, ptosis, weak facial movements, poor swallowing, jaw fatigue when chewing, slurred speech
- Weakness worse with use, at the end of the day and when repeating movements
- Minimal symptoms in the morning
- Proximal + head/neck muscles
- 20-40% of thymoma patients get
144
Examination findings in myasthenia gravis?
- Ptosis exaggerated by repetitive blinking or prolonged upward gaze
- Repeated arm abduction causes weakness
- Thymoma or scar from surgery
145
How is myasthenia gravis diagnosed?
- Ach-receptor antibodies (85%)
- MuSK and LRP4 antibodies
- CT/MRI of thymus-> thymoma
- Edrophonium (neostigmine) test-> brief relief of symptoms when give to patient (stops Ach breakdown)
146
Long-term treatment of myasthenia gravis?
- Pyridostigmine or neostigmine-> reversible acetylcholinesterase inhibitors
- Prednisolone or azathioprine
- Thymectomy
- Monoclonal antibodies eg rituximab
147
What is myasthenia crisis?
Acute worsening of symptoms + respiratory failure-> often triggered by illness
148
How is myasthenia crisis treated?
- IV immunoglobiluns
- Plasma exchange
- May need BiPAP or intubation + ventilation
149
What is Lambert-Eaton myasthenic syndrome and what is the pathophysiology?
- Syndrome that occured in small cell lung cancer
- Antibodies produced against voltage-gated calcium channels on small cell lung cancer cells
- Antibodies damage calcium channels in NMJ presynaptic terminals-> less Ach released
150
How does Lambert-Eaton myasthenic syndrome present?
- Slower and less severe symptoms
- Proximal leg muscle weakness, diplopia, ptosis, dysphagia
- Autonomic dysfunction-> dry mouth, blurry vision, impotence, dizziness
- Post-tetanic potentiation-> reduced reflexes normally but normal after strong muscle contraction
151
How is Lambert-Eaton myasthenic syndrome managed
- Consider investigations for small cell lung cancer
- Amifampridine-> allows more Ach release in NMJ
- Immunosuppression, IV Igs and plasmapheresis
152
What is Charcot-Marie-Tooth syndrome?
An autosomal dominant inherited condition affecting peripheral motor and sensory nerves-> myelin or axonal dysfunction
153
Presentation of Charcot-Marie-Tooth?
- Reduced reflexes + tone
- Peripheral sensory loss
- Hand weakness
- Distal muscle wasting-> inverted champagne bottle legs
- Pes cavus-> high foot arch
154
Management of Charcot-Marie-Tooth?
Mainly supportive with neuro, physios, OT, ortho surgeons
155
Causes of peripheral neuropathy?
ABCDE
- Alcohol
- B12 deficiency
- Cancer or CKD
- Diabetes or Drugs (isonazid, amiodarone, cisplatin)
- Every vasculitis
156
What is Guillain-Barre syndrome?
An acute polyneuropathy than affects peripheral nerves usually triggered by an infection
157
What infections commonly trigger Guillain-Barre syndrome?
Campylobacter jejuni, CMV, EBV
158
How does Guillain-Barre syndrome present?
- Acute symmetrical ascending sensory weakness
- Reduced reflexes
- Neuropathic pain
- Can cause facial nerve weakness if progresses to cranial nerves
- Symptoms within 4 weeks of infection-> peak in 2-4 weeks-> recover in months-years
159
What is the pathophysiology of Guillain-Barre syndrome?
B-cells great antibodies against pathogen antigens but match proteins on nerve cells (myelin sheath or axon)
160
How is Guillain-Barre syndrome diagnosed?
- Clinically
- Brighton criteria
- Nerve conduction studies (reduced signal)
- LP (CSF proteins raised)
161
Treatment for Guillain-Barre syndrome?
- IV immunoglobulins or plasma exchange
- Supportive + VTE prophylaxis
- Intubation if respiratory failure
162
What is the prognosis of Guillain-Barre syndrome?
- 80% fully recover
- 15% get neuro disability
- 5% die
163
What is neurofibromatosis?
Genetic condition causing neuromas (benign nerve tumours) throughout the nervous system
164
What is the diagnostic criteria for neurofibromatosis type 1?
2+ of classic features ie CRABBING
- Cafe au lait spots-> 6+ measuring 5mm+ (kids) or 15mm+ (adults)
- Relative with NF1
- Axillary or inguinal freckles
- Bony dysplasia (long bone bowing etc)
- Iris hamartomas-> lisch nodules, 2+ yellow/brown spots on iris
- Neurofibromas-> 2+ or 1 plexiform neurofibroma
- Glioma of the optic nerve
165
Investigations for neurofibromatosis type 1?
- Clinical + CRABBING criteria
- Genetic testing
- X rays for bone lesions
- CT or MRI for brain lesions
166
Treatment for neurofibromatosis type 1?
None-> control symptoms + treat complications
167
What are the complications of neurofibromatosis type 1?
- Migraines
- Epilepsy
- LD + ADHD
- Scoliosis
- Vision loss due to optic nerve gliomas
- Renal artery stenosis + HTN
- Malignant peripheral nerve sheath tumours
- GI stromal tumour (sarcoma)
- Other cancers-> brain, spinal cord, leukaemia
168
What is the genetic mutation behind neurofibromatosis type 1?
Autosomal dominant-> NF1 gene on chromosome 17-> neurofibromin code (tumour suppressor gene)
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What is the genetic mutation behind neurofibromatosis type 2?
Autosomal dominant-> gene on chromosome 22-> for merlin (tumour suppressor gene)
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How does neurofibromatosis type 2 present?
Bilateral acoustic neuromas (vestibular schwannomas)-> hearing loss, tinnitis, balance problems
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How is neurofibromatosis type 2 treated?
Surgery to resect tumours but risk of nerve damage
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What is tuberous sclerosis?
A genetic condition causing hamartomas (benign neoplastic tissue growth) to grow throughout the body
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What is the pathophysiology of tuberous sclerosis?
TSC1 gene on chromosome 9 (hamartin) or TSC2 on chromosome 16 (tuberin) mutations-> abnormal control of/unregulated size of cell growth
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What are the signs of tuberous sclerosis?
-Hamartomas-> benign lesions all over body (mostly eyes)
-Ash leaf spots-> depigmented areas
-Cafe-au-lait spots
-Shagreen patched-> thick + dimpled
-Angiofibromas-> papules over nose + cheeks
-Subungual fibromata-> on nail bed
Poliosis-> Isolated white hair patch
-Rhabdomyomas
-Gliomas
-Polycystic kidneys
-Neuro signs-> epilepsy, LD, development delay
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What is the stereotypical history for someone with tuberous sclerosis?
Child with epilepsy + skin features (hamartomas, cafe-au-lait spots, ash leaf spots etc)
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How is tuberous sclerosis managed?
Supportive + treat epilepsy
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What are some red flag symptoms and signs for serious neurological conditions?
- Raised ICP signs (eg papilloedema, headache, vomiting)
- Fever, photophobia, neck stiffness-> meningitis + encephalitis
- New neurological symptoms-> haemorrhage, malignancy, stroke
- Visual disturbance-> glaucoma, temporal arteritis
- Dizziness-> stroke
- Symptoms in pregnancy-> pre-eclampsia
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How does tension headache present?
- Mild ache across the forehead, tight band, onset + resolves gradually
- Triggered by stress, depression, alcohol, skipping meals, dehydration
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How is tension headache treated?
Reassurance, analgesia, relaxation, hot towels
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What are some of the triggers for tension headache?
Triggered by stress, depression, alcohol, skipping meals, dehydration
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What is a secondary headache?
Similar presentation to tension headache but with a clear cause-> infection, alcohol, head injury etc
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What is sinusitis?
Inflammation of the ethmoidal, maxillary, frontal and/or sphenoidal sinuses-> mostly viral
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How does sinusitis present?
Facial pain behind the nose, forehead and/or eyes with tenderness over sinuses
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How is sinusitis treated?
- Usually resolves in 2-3 weeks
- Nasal irrigation with saline
- Steroid nasal spray if prolonged
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What is analgesia overuse headache?
Headache secondary to continuous use of analgesia-> presents similarly to tension
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How is analgesia overuse headache treated?
Withdrawal of analgesia
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What causes a hormonal headache?
Low oestrogen
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How does a hormonal headache present?
- Starts 2 days before period and continues for first 3 days
- Worsens around menopause and pregnancy (rule out pre-eclampsia)
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How is hormonal headache treated?
OCP
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What is cervical spondylosis?
Head and/or neck ache due to degenerative changes in the spine
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What are the symptoms of cervical spondylosis?
Neck pain worse on movement + headache
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How is cervical spondylosis treated?
-Exclude inflammation, malignancy, infection and nerve root/spinal cord lesions
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What is trigeminal neuralgia and what causes it?
- Symptoms caused by irritation of the trigeminal nerve (CN V)
- Can be any of the three branches (opthalmic, maxillary, mandibular)
- Compression or MS
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How does trigeminal neuralgia present?
- Intense facial pain
- Comes on spontaneously
- Lasts few seconds but can persist for hours
- Electric shock sensation
- Worsened over time
- Triggers-> cold, spicy food, caffiene
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What causes a migraine?
Structural lesions, functional, chemical, vascular problems, inflammatory factors
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What are the different types of migraine?
- With aura
- Without aura
- Silent-> aura + no headache
- Hemiplegic
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How does a migraine present?
- Headache-> moderate to severe, pounding/throbbing, unilateral normally, lasts 4-72 hours, comes in 'attacks'
- Aura-> visual changes eg sparks, blurring, lines
- Photo/phonophobia, nausea and vomiting
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How does a hemiplegic migraine present?
- Migraine type headache-> sudden or gradual onset
- Hemiplegia-> unlateral limb weakness
- Ataxia
- Change in consciousness
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What can trigger a migraine?
Chocolate, cheese, OCP, caffiene, alcohol, anxiety, travel, exercise, bright lights, strong smells, dehydration, menstruation, abnormal sleep, trauma
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What are the five stages of migraine?
May only get 1-2 or all 5
- Prodrome-> <3 days before, yawning, fatigue, mood changes
- Aura-> <60 minutes
- Headache-> 4-72 hours
- Resolution-> fades usually after vomiting or sleeping
- Postdromal/recovery
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Acute management of migraine?
- Paracetamol + NSAIDs
- Sumatriptan when starts
- Antiemetics eg metoclopramide
- Lying in dark room
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How does sumatriptan work in a migraine attack?
5HT receptor agonist-> acts on smooth muscle + vasoconstricts-> inhibits pain receptor activation + reduce CNS activity
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Prophylaxis of migraines?
- Propanolol
- Topiramate-> CI'd in pregnancy
- Amitriptyline
- Migraine diary + trigger avoidance
- Acupuncture
- Vitamin B2 (riboflavin) supplements
204
Who is a typical patient with cluster headaches?
30-50 year old male smoker + triggered by alcohol, strong smells or exercise
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How do cluster headaches present?
- Headache-> very severe, red/swollen/watery eye, 'pacing the room'
- Miosis, ptosis, nasal discharge, sweating
- Attacks-> 15 mins to 3 hours, 3-4 times a day, for a few weeks-months, then remission for few years
