Neurology Flashcards
1
Q
What is the aetiology of an ischaemic stroke?
A
- Small vessel occlusion/ thrombosis in situ
- Cardiac emboli from AF, MI or infective endocarditis
- Large artery stenosis
- Atherothromboembolism
- Hypoperfusion, vasculitis, hypervisocity
2
Q
What is the aetiology of a haemorrhagic stroke?
A
CNS bleeds due to:
- Trauma
- Aneurysm rupture
- Anticoagulation
- Thrombolysis
- Carotid artery dissection
- Subarachnoid haemorrhage
3
Q
What is the pathophysiology of an ischaemic stroke?
A
- Arterial disease and atherosclerosis
- Thrombosis occurs at the site of an atheromatous plaque in the carotid/ vertebral/ cerebral arteries
- Large artery stenosis acts as an embolism source rather than occluding the vessel
4
Q
What is the pathophysiology of a haemorrhagic stroke?
A
Hypertension resulting in micro aneurysm rupture
5
Q
What are the risk factors for a stroke?
A
- Male
- Black or Asian
- Hypertension
- Past TIA
- Smoking
- Diabetes mellitus
- Increasing age
- Heart disease
- Alcohol
- Polycythaemia
- AF
- High cholesterol
- Combined oral contraceptive pill
- Vasculitis
- Infective endocarditis
6
Q
What is the clinical presentation of an anterior cerebral artery stroke?
A
- Leg weakness more likely than arm weakness
- Sensory disturbances in legs
- Gait apraxia
- Truncal apraxia
- Incontinence
- Drowsiness
7
Q
What is the clinical presentation of a middle cerebral artery stroke?
A
- Contralateral arm and leg weakness
- Contralateral sensory loss
- Hemianopia
- Aphasia
- Dysphasia
- Facial droop
8
Q
What is the clinical presentation of a posterior cerebral artery stroke?
A
- Contralateral homonymous hemianopia
- Cortical blindness (eyes healthy but brain issues)
- Visual agnosia (can see but can’t interpret)
- Prosopagnosia (can’t see faces)
- Problems naming and distinguishing colours
- Unilateral headache
9
Q
What is the clinical presentation of a posterior circulation stroke?
A
- More catastrophic (wide region supplied)
- Motor deficits
- Dysarthria and speech impairment
- Vertigo, nausea, vomiting
- Visual disturbance
- Altered consciousness
10
Q
What are the differential diagnoses of a stroke?
A
- Always exclude hypoglycaemia
- Migraine aura
- Focal epilepsy
- Intracranial lesion
- Syncope due to arrhythmia
11
Q
How is a stroke diagnosed?
A
- Urgent CT/ MRI head before treatment to rule out haemorrhagic before starting thrombolysis
- Pulse, BP and ECG to look for AF
- Bloods – thrombocytopenia and polycythaemia on FBC; hypoglycaemia on blood glucose
12
Q
How is a stroke managed?
A
- Maximise reversible ischaemic tissue
- Thrombolysis up to 4.5h after onset of symptoms (give tissue plasminogen activator e.g. IV alteplase then antiplatelet therapy 24h later e.g. clopidogrel)
- If time of onset is unknown, then daily aspirin and lifelong clopidogrel
In haemorrhagic:
- No antiplatelets
- Control hypertension
- Reduced ICP (manually or with diuretics)
- May need surgery
Risk management for stroke prevention:
- Platelet treatment (e.g. aspirin with clopidogrel)
- Cholesterol treatment
- AF treatment (e.g. warfarin)
- BP treatment (e.g. Ramipril)
13
Q
What is the aetiology of a TIA?
A
- Small vessel occlusion
- Atherothromboembolism
- Cardioembolism
- Hyperviscosity
- Can result from hypoperfusion in younger people
14
Q
What is the pathophysiology of a TIA?
A
- Cerebral ischaemia resulting in a lack of O2 and nutrients to the brain causing cerebral dysfunction
- Period of ischaemic is short lived and symptoms rarely last more than 15m and resolve before irreversible cell death
- Gradually progressing symptoms suggests a different pathology e.g. demyelination, tumour or migraine
15
Q
What are the risk factors of a TIA?
A
- Increasing age
- Hypertension
- Smoking
- Diabetes
- Heart disease (valvular, ischaemic, AF)
- Past TIA
- Raised packed cell volume
- Peripheral arterial disease
- Combined oral contraceptive pill
- Hyperlipidaemia
- Excess alcohol
- Clotting disorder
- Vasculitis e.g. SLE, GCA
16
Q
What is the clinical presentation of an anterior circulation TIA?
A
- Weak, numb contralateral leg ± similar arm symptoms
- Hemiparesis (weakness on an entire side of the body)
- Hemi sensory disturbance
- Dysphagia
- Sudden transient loss of vision in one eye
17
Q
What is the clinical presentation of a posterior circulation TIA?
A
- Double vision
- Vertigo
- Vomiting
- Choking and dysarthria
- Ataxia
- Hemisensory loss
- Hemianopia vision loss
- Loss of consciousness (rare)
- Transient global amnesia (episode of confusion/ amnesia lasting several hours followed by complete recovery)
- Tetraparesis (muscle weakness in all 4 limbs)
18
Q
What are the differential diagnoses of a TIA?
A
- Impossible to differentiate from a stroke until a full recovery has been made
- Hypoglycaemia\migraine aura
- Focal epilepsy
- Intracranial lesion
- Syncope
- Todd’s paralysis
- Retinal or vitreous haemorrhage
- GCA
19
Q
How is a TIA diagnosed?
A
- Often based solely on description
- Bloods: FBC (for polycythaemia), ESR (for vasculitis), glucose (for hypoglycaemia), creatinine, electrolytes, cholesterol
- Carotid artery doppler ultrasound for stenosis/ atheroma
- CT/ MRI head
- ECG (look for AF or evidence of MI ischaemia)
- CT or diffusion weighted MRI
- Echocardiogram/ cardiac monitoring to assess for a cardiac cause
20
Q
How is a TIA managed?
A
- Immediate aspirin and dipyridamole for 2 weeks then a lower dose
- P2Y12 inhibitor long term
- Anticoagulant if they have AF, mitral stenosis or recent septal MI
- Statin long term
- Control cardiovascular risk factors
21
Q
What is the aetiology of a subarachnoid haemorrhage?
A
- Rupture of saccular aneurysms (e.g. Berry aneurysm) = rupture of the junction of the posterior communicating artery with the internal carotid or the anterior comm with the arterial cerebral
- Arteriovenous malformation = vascular development malformation
- Rare = bleeding disorder, mycotic aneurysms, acute bacterial meningitis, tumours
22
Q
What is the pathophysiology of a subarachnoid haemorrhage?
A
Most common cause is a ruptured aneurysm which leads to tissue ischaemia and rapidly raised ICP
23
Q
What are the risk factors of a subarachnoid haemorrhage?
A
- Hypertension
- Known aneurysm
- Family history
- Disease that predispose to aneurysm (polycyctic kidney disease, Ehler’s Danlos, coarctation of aorta)
- Smoking
- Bleeding disorders
- Post-menopausal decreased oestrogen
24
Q
What is the clinical presentation of a subarachnoid haemorrhage?
A
- Sudden onset severe occipital headache (incredibly painful)
- Vomiting, collapse, seizures, coma often follow
- Depressed level of consciousness
- Coma/drowsiness may last for days
- Neck stiffness
- Kernig’s signs (unable to extend leg at the knee when the thigh is flexed)
- Brudzinski’s sign (when neck is manually flexed, patient will flex hips and knees)
- Retinal and vitreous bleeds
- Papilloedema
- Vision loss or diplopia
- Focal neurology
- Marked increase in BP (reflex following haemorrhage)
- Sentinel headache (may present earlier, so ask about it in history)
25
What are the differential diagnoses of a subarachnoid haemorrhage?
- Must be differentiated from a migraine
- Meningitis
- Intracranial bleeds
- Cortical vein thrombosis
26
How is a subarachnoid haemorrhage diagnosed?
- ABG to exclude hypoxia
- Head CT = gold standard (seen as a ‘star shaped’ lesion)
- CT angiogram if aneurysm is confirmed to see extent
- Lumbar puncture
27
How is a subarachnoid haemorrhage managed?
| specific interventions
- Refer to neurosurgery immediately
- IV fluids to maintain cerebral perfusion
- Ca2+ blocker to reduce vasospasm and morbidity
- Endovascular coiling – FIRST LINE when angiography shows aneurysm
- Surgery – intracranial stents and balloon remodelling for wide-necked aneurysms
28
What is the aetiology of a subdural haemorrhage?
Trauma due to deceleration from violent injury or dural metastases results in bleeding from the bridging veins between cortex and venous sinuses
29
What is the pathophysiology of a subdural haemorrhage?
- Bridging veins bleed and form a haematoma between dura and arachnoid, reducing pressure and stopping the bleeding
- The haematoma starts to autolyse due to the massive increase in oncotic and osmotic pressure, so water is sucked into the haematoma, causing it to enlarge
- ICP rises gradually over many weeks
- Midline structures shift away from the side of the clot and if untreated can result in herniation and coning
30
What are the risk factors of a subdural haemorrhage?
- Traumatic head injury, cerebral atrophy and increasing age (all make bridging veins more vulnerable)
- Alcoholism, anticoagulation and physical abuse in infancy
31
What is the clinical presentation of a subdural haemorrhage?
- Interval between injury and symptoms can be days to weeks to months
- Fluctuating levels of consciousness ± insidious physical or intellectual slowing
- Sleepiness
- Headache
- Personality change
- Unsteadiness
- Signs of raised ICP e.g. headache, vomiting, nausea, seizures and raised BP
- Focal neurology e.g. hemiparesis or sensory loss
- Occasionally seizures
- Stupor, coma and coning may follow
- Symptoms will develop much slower in the elderly due to decrease in brain weight
32
What are the differential diagnoses of a subdural haemorrhage?
- Stroke
- Dementia
- CNS masses e.g. tumours or abscess
- Subarachnoid haemorrhage
- Extradural haemorrhage
33
How is a subdural haemorrhage diagnosed?
- CT head: diffuse spreading, hyperdense crescent shaped collection of blood over 1 hemisphere (sickle/crescent shaped differentiates subdural from extradural)
- MRI head for subacute haematomas and smaller haematomas
34
How is a subdural haemorrhage managed?
- Assess and manage ABCs
- Prioritise head CT
- Stabilise patient
- Refer to neurosurgeons
- Address cause of trauma
- IV mannitol to reduce ICP
35
What is the aetiology of an extradural haemorrhage?
Most commonly due to a traumatic head injury resulting in fracture of the temporal or parietal bone causing laceration of the middle meningeal artery, typically after trauma to the temple
36
What is the pathophysiology of an extradural haemorrhage?
Blood accumulates rapidly over minutes to hours between the bone and the dura
37
What is the clinical presentation of an extradural haemorrhage?
- Characteristic history: head injury, brief post-traumatic loss of consciousness or initial drowsiness, lucid interval while haematoma is still small
- Severe headache, nausea and vomiting, confusion and seizures (due to rising ICP) ± hemiparesis with brisk reflexes
- Ipsilateral pupil dilates, coma deepens, bilateral limb weakness and breathing becomes deep and irregular
- Decreased GCS and coning (brain herniates through foramen magnum)
- Death from respiratory arrest if surgical intervention not fast enough
- Bradycardia and raised BP are late signs
38
What are the differential diagnoses of an extradural haemorrhage?
- Epilepsy, carotid dissection and carbon monoxide poisoning
| - Subdural haematoma, subarachnoid haemorrhage, meningitis
39
How is an extradural haemorrhage diagnosed?
- CT head = gold standard (shows hyperdense haematoma)
| - Skull XR to see fracture lines (fracture would increase extradural haemorrhage risk)
40
How is an extradural haemorrhage managed?
- ABCDE emergency management (assess and stabilise patient)
- IV mannitol for increased ICP
- Refer to neurosurgery (clot evacuation ± litigation of bleeding vessel)
- Maintain airway via intubation and ventilation in unconscious patient
41
What is the aetiology of epilepsy?
- 2/3 idiopathic (often familial)
- Cortical scarring (head injury years before onset, cerebrovascular disease e.g. cerebral infraction, haemorrhage, CNS infection e.g. meningitis, encephalitis)
- Space-occupying lesion e.g. tumour
- Stroke
- Tuberous sclerosis
- AD or other dementia
- Alcohol withdrawal
42
What is the pathophysiology of primary generalised seizures?
- Simultaneous onset of electrical discharge throughout the whole cortex with no localising features referable to only one hemisphere
- Bilateral symmetrical and synchronous motor manifestations
- Always associated with a loss of consciousness
43
What is the pathophysiology of partial/ focal seizures?
- Focal onset with features referable to a part of one hemisphere e.g. temporal lobe
- Often seen with underlying structural disease
- Electrical discharge is restricted to a limited part of the cortex of one cerebral hemisphere
- These may later become generalised (e.g. secondary generalised tonic-clonic seizures)
44
What are the risk factors of epilepsy?
- Family history
- Premature baby small for age
- Abnormal blood vessels in the brain
- AD or other dementia
- Use of drugs e.g. cocaine
- Stroke/ brain tumour/ infection
45
What are the 5 types of primary generalised seizure?
- Generalised tonic-clonic
- Typical absence seizure
- Myoclonic seizure
- Tonic seizure
- Atonic seizure
46
What is the clinical presentation of the 5 types of primary generalised seizure?
(Generalised tonic-clonic, typical absence, myoclonic, tonic, atonic)
GENERALISED TONIC-CLONIC
- Often no aura
- Loss of consciousness
- Tonic phase = rigid stiff limbs (patient will fall to the floor if standing)
- Clonic phase = generalised bilateral, rhythmic muscle jerking lasting for seconds to minutes
- Eyes remain open and tongue often bitten
- May be incontinence of urine/faeces
- Followed by a period of drowsiness, confusion or coma for several hours
TYPICAL ABSENCE SEIZURE
- Usually in childhood
- Ceases activity, stares and pales for a few seconds (suddenly stops talking mid-sentence then carries on where they left off)
- Often don’t realise they’ve has an attack
MYOCLONIC SEIZURE
- Sudden isolated jerk of a limb, face or trunk
- Patient may be thrown suddenly to the ground or have a violently disobedient limb
TONIC SEIZURE
- Sudden sustained increased tone with a characteristic cry/grunt
- Intense stiffening of body
- Stiffening not followed by jerking
ATONIC SEIZURE
- Sudden loss of muscle tone and cessation of movement resulting in a fall
47
What are the 2 types of partial/ focal seizures?
- Simple partial seizure
| - Complex partial seizure
48
What is the clinical presentation of the 2 types of partial/ focal seizures?
(General symptoms for simple partial seizures and symptoms for each lobe for complex partial seizures)
SIMPLE PARTIAL SEIZURE
- Not affecting consciousness or memory
- Awareness is unimpaired with focal motor, sensory, autonomic or psychic symptoms
- No post-ictal symptoms
COMPLEX PARTIAL SEIZURE
- Affecting awareness or memory before, during, or immediately after the seizure
- Most commonly arise from the temporal lobe
- Temporal lobe → aura (deja-vu, auditory hallucinations, funny smells, fear), anxiety or out of body experience, automatisms (lip smacking, chewing, fiddling), post-ictal confusion
- Frontal lobe → motor features (posturing or peddling movements of the leg), Jacksonian march (seizure ‘marches’ up/down the motor homunculus starting in the face/thumb), post-ictal Todd’s palsy (paralysis of limbs involved in seizure for several hours)
- Parietal lobe → sensory disturbances (tingling/ numbness)
- Occipital lobe → visual phenomena (spots, lines or flashes)
49
What are the differential diagnoses of epilepsy?
- Postural syncope
- Cardiac arrhythmia
- TIA
- Migraine
- Hyperventilation
- Hypoglycaemia
- Panic attacks
- Non-epileptic seizures
50
How is epilepsy diagnosed?
- Need at least 2 unprovoked seizures occurring >24h apart to make a clinical diagnosis
- EEG – not diagnostic, but used to support a diagnosis, may determine seizure type
- MRI to view hippocampus
- CT head in emergencies to look for tumours and exclude structural abnormalities
- Bloods – FBC, electrolytes, Ca2+, renal function, liver function, urine biochemistry, blood glucose
- Genetic testing e.g. in juvenile myoclonic epilepsy
51
What are the emergency measures for managing epilepsy?
- ABCDE, glucose, prolonged/ repeated seizure treated with IV/rectal diazepam or lorazepam
- IV phenytoin loading
- Anaesthetic and ventilation if still fitting
52
How are primary generalised seizures managed?
| 3 medications for each of generalised tonic-clonic and absence seizures
GENERALISED TONIC-CLONIC
- Oral sodium valproate
- Oral lamotrigine
- Oral carbamazepine
ABSENCE SEIZURE
- Oral sodium valproate
- Oral lamotrigine
- Oral ethosuximide
53
What are the neurosurgical treatment options for epilepsy?
- Only if drugs not working
- If a single defined cause is found (e.g. hippocampal sclerosis or low-grade tumour) then surgical resection can stop seizures
- Vagal nerve stimulation can reduce seizure frequency and severity
54
What is the aetiology of Parkinson's disease?
- Idiopathic
- Drug induced
- Combination of environmental factors (pesticides, methyl-phenyl tetrahydropyridine), Parkinson genes, oxidative stress and mitochondrial dysfunction
55
What is the pathophysiology of Parkinson's disease?
- Parkinson’s results from mitochondrial dysfunction and oxidative stress causing progressive degeneration of dopaminergic neurons
- Therefore, reduced striatal dopamine levels due to loss of dopaminergic neurones
- Less dopamine → thalamus is inhibited resulting in a decrease in movement therefore symptoms of Parkinson’s
56
What is the clinical presentation of Parkinson's disease?
| General, before motor symptoms, classic triad symptoms
- Onset of symptoms is gradual and commonly presents with impaired dexterity or unilateral foot drop
- Onset is asymmetrical (one side is always worse than the other)
- Difficulty with fine movements e.g. doing up buttons
- Parkinsonian gait = stooped posture, small shuffling steps, reduced arm swing, narrow base, difficulty initiating movement and turning and poor balance
- Drooling of saliva and difficulty swallowing is a late feature (can lead to aspiration pneumonia as a terminal event)
- Depression, constipation and increased urinary frequency are all common
Before motor symptoms develop:
- Anosmia
- Depression/ anxiety
- Aches and pains
- REM sleep disorders
- Urinary urgency
- Hypotension and constipation
Classic triad:
1. TREMOR
- Worse at rest and often asymmetrical
- Usually most obvious in the hands
- Improved by voluntary movements and made worse by anxiety
- Rhythms gets worse the longer attempted (unique to PD)
2. RIGIDITY
- Increased tone in limbs and trunk
- Limbs resist passive extension through movement
- Can cause pain and problems turning in bed
3. BRADYKINESIA/HYPOKINESIA
- Slow to initiate movement and low, low-amplitude excursions in repetitive actions
- Reduced blink rate
- Monotonous hypophonic speech
- Micrographia (smaller writing)
57
What are the differential diagnoses of Parkinson's disease?
- Benign essential tremor (worse on movement and rare while at rest)
- Multiple cerebral infarcts, Lewy body dementia, drug-induced, Wilson’s disease, trauma, dopamine antagonists
58
How is Parkinson's disease diagnosed?
- Clinical based on history and examination
- Can confirm by response to levodopa
- MRI head (will show atrophy)
- Signs that it’s not PD if present early = dementia, incontinence, symmetry in symptoms and early falls
59
How is Parkinson's disease managed?
- Need to compensate for the loss of dopamine
- Physiotherapy for balance problems, speech and gait disturbances
- Oral levodopa with a decarboxylase inhibitor (e.g. co-careldopa) is gold standard of treatment
- Levodopa has a lot of complications though, so should only be used when other treatments are ineffective
- Dopamine agonists e.g. oral ropinirole
- Monoamine Oxidase B (MAO-B) inhibitors e.g. oral selegiline
- Catechol-O-methyl transferase inhibitors e.g. oral entacapone
- Deep brain stimulation may help those who are partly-dopamine responsive
60
What is the aetiology of Alzheimer's dementia?
| more than just atrophy
- Degeneration of cerebral cortex with cortical atrophy
- Accumulation of beta-amyloid peptide results in progressive neuronal damage, neurofibrillary tangles and increases in the number of amyloid plaques and the loss of ACh
61
What is the aetiology of vascular dementia?
Brain damage due to cerebrovascular disease (either major stroke, multiple smaller unrecognised strokes or chronic changes in smaller vessels)
62
What is the aetiology of dementia with Lewy bodies?
- Deposition of abnormal protein within neurons in the brain stem and neocortex
- Associated with Parkinson’s
63
What is the aetiology of fronto-temporal dementia?
- Specific degeneration/ atrophy of the frontal and temporal lobes
- Behavioural and personality change, early preservation of episodic memory and spatial orientation, emotional unconcern, lowers inhibitions
64
What are other aetiologies of dementia?
- Parkinson’s disease
- Mixed dementia
- Huntington’s
- Liver failure
- HIV
- Prion diseases
- Vitamin B12/ folate deficiency
65
What are the risk factors of dementia?
- Family history
- Age
- Down’s syndrome
- Alcohol use, obesity, high BP, high cholesterol, diabetes
- Atherosclerosis
- Depression
- High oestrogen
66
What is the clinical presentation of Alzheimer's?
- Insidious onset with steady progression over years
- Short-term memory loss is usually the most prominent early symptoms
- Slow disintegration of personality and intellect, eventually affecting all aspects of cortical function
- Decline in language (difficulty naming and in understanding what is being said), visuospatial skills, apraxia (difficulty carrying out skilled motor tasks) and agnosia (failure to recognise objects)
67
What is the clinical presentation of vascular dementia?
- Stepwise deterioration with declines followed by short periods of stability
- History of TIAs and strokes
- Evidence of artheropathy
- Signs of vascular pathology e.g. raised BP, past strokes and focal CNS signs
68
What is the clinical presentation of dementia with Lewy bodies?
- Fluctuating cognitive impairment with pronounced variation in attention and alertness
- Prominent or persistent memory loss may not occur in early stages
- Depression and sleep disorders occur
- Visual hallucinations
- Parkinson’s
- Loss of inhibitions
69
What are the differential diagnoses of dementia?
- Substance abuse
- Hypothyroidism
- Space-occupying intracranial lesions
- Huntington’s
70
How is dementia diagnosed?
- History – assess cognitive functions by asking various questions
- MMSE score/30 (>25 = normal; 18-25 = mild/moderate impairment; >17 = serious impairment)
- Blood tests (FBC, liver biochemistry, TFT, B12 and folate)
- Neuropsychology
- Brain CT in younger patients or those with atypical presentation
- MRI to see extent of atrophy
- Brain function assessment through PET/SPECT scanning and functional MRI
71
How is dementia managed?
| prevention, support, meditation
- No specific therapy
PREVENTION:
- Healthy behaviours
- Smoking cessation
- >6 leisure activities
- Education
- Occupation
SUPPORT:
- Socially active
- Cognitively active (cognitive stimulation programmes, board games etc.)
- Specialist memory service
- Carers may be required
MEDICATION:
- Blood pressure control to reduce further vascular damage e.g. ramipril
- Acetylcholinesterase inhibitors to increase ACh (especially in vascular dementia) e.g. oral donepezil or oral rivastigmine
72
What is the aetiology of migraines?
- No specific cause, but there are partial triggers: chocolate, hangovers, orgasms, cheese, oral contraception, lie-ins, alcohol, tumult (load noise), exercise
- Brain chemical imbalance may be a cause
- May be caused by changes in the brainstem and its interactions with the trigeminal nerve
73
What is the pathophysiology of migraines?
- Genetic and environmental factors play a role
- Genetic factors play a role in causing neuronal-hyperexcitability
- Changes in brainstem blood flow leads to an unstable trigeminal nerve nucleus and nuclei in the basal thalamus
- Cortical spreading depression – self-propagating wave of neuronal and glial depolarisation that spreads across the cerebral cortex is thought to cause the aura
→ Release of vasoactive neuropeptides results in the process of neurogenic inflammation leading to pain
74
What are the risk factors of migraines?
- Family history (has a strong genetic component)
- Female
- Age (majority start in adolescence)
75
What is the clinical presentation of a migraine prodrome?
- May precede the headache by hours/days
| - Yawning, cravings and mood/sleep changes
76
What is the clinical presentation of a migraine without aura?
- Attacks last 4-72h
- Two of the following: unilateral, pulsing, moderate/severe pain in head, aggravated by routine physical activity
- During headache at least one of: nausea and/or vomiting, photophobia and phonophobia (sound sensitive) during the headache
- Not attributable to another disorder
77
What is the clinical presentation of a migraine with aura?
- At least 2 attacks
- Aura precedes the attack by minutes and may persist during it
- VISUAL: chaotic cascading, jumbling, distorting lines, dots, zigzags, black hole in visual field (scotoma), hemianopia
- SOMATOSENSORY: paraesthesia spreading from fingers to face
- Unilateral, pulsatile headache
78
What is the general clinical presentation of a migraine?
At least 2 of:
- Unilateral pain
- Throbbing-type pain
- Moderate to severe intensity
- Motion sensitivity (made worse by head movement of physical activity)
At least 1 of:
- Nausea/vomiting
- Photophobia/phonophobia
- Normal examination with no other attributable cause
79
What are differential diagnoses of a migraine?
- Tension headache (bilateral, tight-band around head)
- Cluster headache (excruciating, autonomic symptoms)
- Medication over-use headache
- Sudden migraine may resemble meningitis or subarachnoid haemorrhage
- Thromboembolic TIA
- Brain tumour
- Temporal arteritis
80
How are migraines diagnosed?
| including red flag indications for neuroimaging and indications for lumbar puncture
- Mainly a clinical diagnosis
- Always examine eyes (papilloedema and other eye issues) and head & neck (scalp, neck muscles and temporal arteries)
- Lab tests – CRP, ESR
- Red flag indications for neuroimaging: worse/severe headache, change in migraine pattern, abnormal neuro exam, onset >50y, epilepsy, posteriorly located headache
- Indications for lumbar puncture: worst headache of life, severe rapid onset, progressive headaches, unresponsive headaches
- Neuroimaging should precede lumbar puncture to rule out mass/ lesion/ raised ICP
81
How are migraines managed?
| lifestyle, acute attack and prevention
- Reduce triggers e.g. avoid dietary factors
ACUTE:
- No ergots (e.g. opioids)
- Triptans e.g. sumatriptan
- NSAIDs (avoid paracetamol or ibuprofen)
- ± anti-emetic e.g. prochlorperazine
PREVENTION:
- If >2 attacks per month or if acute treatment is needed >2 times per week
- Beta-blocker
- Tricyclic anti-depressant
- Anti-convulsant
82
What is the aetiology of a tension headache?
- Stress
- Sleep deprivation
- Bad posture
- Hunger
- Eyestrain
- Anxiety
- Noise
83
What are the risk factors of a tension headache?
- Stress
- Sleep deprivation
- Bad posture
- Hunger
- Eyestrain
- Anxiety
- Noise
84
What is the clinical presentation of a tension headache?
- Episodic (<15d/month) or chronic (>15d/month for at least 3m)
- Usually has one of the following: bilateral, pressing/tight non-pulsatile, mild/moderate intensity ± scalp muscle tenderness
- Without vomiting or sensitivity to head movement, no aura
- Not aggravated by physical activity
- Tight band-like sensation
- Pressure behind eyes, mild-moderate pain
- Headaches can last from 30m to 7d
- Not attributed to any other disorder
85
What are the differential diagnoses of a tension headache?
- Migraine
- Cluster headache
- Giant cell arthritis
- Frug-induced headache
86
How is a tension headache diagnosed?
Clinical diagnosis from history
87
How is a tension headache managed?
- Reassurance and lifestyle advice e.g. regular exercise, avoidance of triggers
- Stress relief e.g. massage or acupuncture
- Symptomatic treatment for episodes occurring >2d/week (aspirin, paracetamol, NSAIDs, no opioids, limited analgesia, tricyclic antidepressants)
- Beware of medication over-use headaches
88
What is the aetiology of a cluster headache?
- Unknown
- May be due to superficial temporary artery smooth muscle hyper-reactivity to serotonin
- Hypothalamic grey matter abnormalities
- Autosomal dominant gene
89
What are the risk factors of cluster headaches?
- Smoker
- Male
- Autosomal dominant gene has a role
90
What is the clinical presentation of a cluster headache?
- Abrupt onset
- Rapid onset of excruciating pain around on eye, temple or forehead
- Ipsilateral cranial autonomic features: eye may become watery and bloodshot with lid swelling and lacrimation, facial flushing, rhinorrhoea (blocked nose), miosis (excessive pupil constriction) ± ptosis (drooping or falling of upper eyelid)
- Pain is strictly unilateral and almost always affects the same side
- Rises to crescendo over minutes and lasts 15-160m once or twice a day
- Often nocturnal/early morning (often wakes patient from sleep)
- ± vomiting
- Episodic = clusters last 4-12w followed by pain-free periods of months or even 1-2y
- Can be chronic (attacks from >1y without remission)
91
What is the differential diagnosis of a cluster headache?
Migraine
92
How is a cluster headache diagnosed?
- Clinical diagnosis
| - >5 headaches fulfilling the clinical presentation pattern
93
How is a cluster headache managed?
| acute attack and prevention
ACUTE ATTACK:
- Analgesics are unhelpful
- 100% 15L O2 for 15m via a non-rebreathable mask (not if COPD)
- Tripan e.g. sub-cut sumatriptan (selective serotonin agonist)
PREVENTION:
- Calcium channel blocker (FIRST LINE)
- Avoid alcohol during cluster period
- Corticosteroids may help during cluster
94
What is the aetiology of a drug overuse headache?
Mixed analgesics e.g. paracetamol + codeine/ opiates, ergotamine and triptans
95
What is the clinical presentation of a drug overuse headache?
Worsens while on regular analgesia (especially opioids)
96
What is the aetiology of motor neurone disease?
- Usually sporadic and of unknown cause
| - Small proportion of cases are familial
97
What is the pathophysiology of motor neurone disease?
- Characterised by selective loss of neurones in the motor cortex, cranial nerve nuclei and anterior horn cells
- Degenerative condition affecting motor neurons, mainly anterior horn cells
- Relentless and unexplained destruction of upper motor neurones and anterior horn cells in the brain and spinal cord, causing UMN and LMN disorders
- Caused by reactive oxygen species which damage DNA, lipids and proteins
- No sensory loss or sphincter disturbance (this distinguishes MND from MS and polyneuropathies)
- Never affects eye movements (distinguishes from MG)
98
What are the risk factors of motor neurone disease?
No established risk factors
99
What are the 4 clinical patterns of motor neurone disease?
- Amyotrophic lateral sclerosis
- Progressive muscular atrophy
- Progressive bulbar palsy
- Primary lateral sclerosis
100
What is the general clinical presentation of motor neurone disease?
- No sensory symptoms or rectal/bladder sphincter issues
- Think of MND in those >40y with stumbling spastic gait, foot drop ± proximal myopathy weak grip and shoulder abduction or aspiration pneumonia
- Frontotemporal dementia in 25%
- Weakness and atrophy spread to other parts of the body regardless of starting body part, with a varying amount of UMN symptoms until the muscles of all 4 extremities and the trunk + bulbar muscles are involved
101
What is the clinical presentation of amyotrophic lateral sclerosis in motor neurone disease?
- UMN + LMN involvement
- Most common type
- Loss of motor neurones in motor cortex and anterior horn of the cord
- Weakness with UMN signs and LMN wasting/ fasciculations (usually in one limb)
- Split hand sign – thumb side of the hand seems adrift due to excessive wasting around it (much less hypothenar wasting)
- Cramps are a common but non-specific symptom
- Examination shows UMN signs e.g. brisk reflexes, extensor plantar response and spasticity
- Wrist and foot drop
102
What is the clinical presentation of progressive muscular atrophy in motor neurone disease?
- LMN involvement only
- Presents with weakness, muscle wasting and fasciculations usually starting in one limb and gradually spreading to involve other adjacent spinal segments
- Affects distal muscle group before proximal
103
What is the clinical presentation of progressive bulbar palsy in motor neurone disease?
- Lower cranial nerves and nuclei initially only affected (CN 9,10,11,12)
- Dysarthria, dysphagia, nasal regurgitation of fluids and choking
- LMN lesion of the tongue and muscles of talking and swallowing
- Flaccid, fasciculating tongue
- Normal/absent jaw jerk
- Speech is quiet, horse or nasal
104
What is the clinical presentation of primary lateral sclerosis in motor neurone disease?
- Least common
- Loss of Betz cells in motor cortex
- Mainly UMN signs and marked spastic leg weakness with progressive tetraparesis and pseudobulbar palsy
- No cognitive decline
105
What are the differential diagnoses of motor neurone disease?
- MS or polyneuropathies
- Myasthenia gravis
- Diabetic amyotrophy
- Guillain-Barre syndrome
- Spinal cord tumours
106
How is motor neurone disease diagnosed?
Diagnosis based on clinical findings:
- Definite = LMN + UMN signs in 3 regions
- Probable = LMN + UMN signs in 2 regions
- Probable with lab support = LMN + UMN (or UMN in >1) + EMG showing acute denervation in >2 limbs
- Possible = LMN + UMN signs in 1 region
- Suspected = LMN or UMN only in 1+ regions
- Brain/ cord MRI excludes structural causes
- Lumbar puncture to exclude inflammatory causes
- Nerve conduction studies + electromyography to confirm denervation of muscles
107
How is motor neurone disease managed?
- Antiglutamatergic drugs e.g. oral riluzole (prolongs life by 3m)
- FOR DROOLING: oral propantheline or oral amitriptyline
- FOR DYSPHAGIA: blend food, nasogastric tube, percutaneous catheter gastrostomy
- FOR SPASMS: oral baclofen
- Non-invasive ventilation if respiratory failure
- Analgesia e.g. NSAIDs
- Specialist MDT support (neurologist, speech & language therapist, occupational therapist, specialist nurse, physiotherapist etc)
108
What is the aetiology of MS?
- Aetiology is not understood
| - Mix of genetic and environmental factors (EBV exposure in childhood may predispose to later MS development)
109
What is the pathophysiology of MS?
- Autoimmune demyelination at multiple CNS sites
- T-cell mediated (T cells activate B cells to produce autoantibodies against myelin)
- T lymphocytes cross the BBB and cause a cascade of destruction in the brain’s neuronal cells, resulting in demyelination and therefore conduction disruption
- Myelin sheath regenerates but is less efficient
- Repeated demyelination leads to axonal loss and incomplete recovery between attacks
- Plaques of demyelination are perivenular (occurs around veins) and can occur everywhere in the CNS, but particularly optic nerves, around ventricles, corpus callosum, brainstem, cerebellar connections and cervical cord
110
What are the risk factors of MS?
- EBV exposure
- Low levels of sunlight and vitamin D
- Female
- White
- Living far from the equator
111
What are the types of MS?
RELAPSING AND REMITTING:
- Most common pattern
- Symptoms occur in attacks (relapses) with onset over days
- Recovery (partial or complete) happens over weeks
- Periods of good health or remission are followed by sudden symptoms or relapses
- Patients may accumulate disability over time if they don’t fully recover after relapses
SECONDARY PROGRESSIVE:
- Follows on from relapsing and remitting MS (majority will evolve within 35y of onset)
- Late stage that consists of gradually worsening symptoms with fewer remissions
PRIMARY PROGRESSIVE:
- Gradually worsening disability without relapses or remission
- Typically presents later and is associated with fewer inflammatory changes on MRI
112
What is the clinical presentation of MS?
- Usually presents in young adults (20-40y)
- Initially monosymptomatic
- Symptoms may worsen with heat/ exercise (new myelin is inefficient and doesn’t work well in heat)
- Unilateral optic neuritis (pain in one eye on movement and reduced central vision)
- Numbness or tingling in the limbs
- Leg weakness
- Brainstem demyelination causes diplopia, vertigo, facial numbness/ weakness, dysarthria/ dysphagia and a clumsy/useless hand or limb due to loss of proprioception
- Cerebellar symptoms e.g. ataxia
- Trigeminal neuralgia
- Constipation
- Spasticity and weakness (can result in stiffness/ tightness of muscles that can interfere with normal movement, speech and gait)
- Intention tremor
- Bladder dysfunction e.g. urgency, frequency and incontinence
- Sexual dysfunction e.g. erectile dysfunction
- Cognitive decline
- Amnesia
113
What are the differential diagnoses of MS?
- Hereditary spastic paraplegia
- Cerebral variant of SLE
- Sarcoidosis
- HIV
114
How is MS diagnosed?
- Diagnosis requires 2 or more attacks affecting different parts of the CNS (i.e. 2 CNS lesions disseminated in time and space)
- Exclude differentials with FBC, inflammatory markers, U&E, LFTs, glucose, HIV serology, auto-antibodies, Ca2+ and vitamin B12
- MRI scan brain and cord: diagnostic if history matches
- Lumbar puncture shows oligoclonal IgG bands
- Electrophysiology (delayed nerve condition suggests demyelination)
115
How is MS managed?
| lifestyle, acute relapse, frequent relapse, symptomatic treatment
- Encourage reducing stress as much as possible to reduce number of new lesions
- Vitamin D supplement if poor diet and sun exposure
ACUTE RELAPSE:
- IV methylprednisolone for less than 3d to shorten relapse
FREQUENT RELAPSE:
- SC interferon 1B or 1A
- Disease modifying agents (monoclonal antibodies): IV alemtuzumab or IV natalizumab
- Dimethyl fumarate
SYMPTOMATIC TREATMENT :
- For spasticity: physiotherapy, Baclofen, Tizanidine, botox injection
- For urinary urgency/ frequency: intermittent self-catheterisation
- Incontinence: anticholinergic alpha-blocker drugs e.g. doxazosin
- Aggressive treatment is stem cell transplant
116
What is the aetiology of meningitis in adults and children?
- Neisseria meningitides (G-ve diplococci)
- Streptococcus pneumoniae/ pneumococcus
- Less common cause is haemophilus influenza
117
What is the aetiology of meningitis in pregnant women and older adults?
Listeria monocytogenes (found in cheese which is why it’s avoided in pregnancy)
118
What is the aetiology of meningitis in neonates?
- Escheria coli
| - Group B haemolytic streptococcus
119
What is the aetiology of meningitis in the immunocompromised?
- Cytomegalovirus
- Cryptococcus neoformans
- TB – mycobacterium tuberculosis
- HIV
- Herpes simplex virus
120
What is the pathophysiology of meningitis?
| how do the microorganisms reach the meninges?
Microorganisms reach the meninges either by direct extension from the ears, nasopharynx, cranial injury or congenital meningeal defect / by bloodstream spread
121
What is the pathophysiology of acute bacterial meningitis?
- Typically sudden
- Neisseria meningitides transmitted by droplet spread
- Can lead to meningococcal septicaemia when bacteria invade blood and the presence of endotoxin leads to an inflammatory response causing petechial rash and signs of sepsis
- Pia arachnoid is congested with polymorphs so a layer of pus forms which may organise to form adhesions causing cranial nerve palsies and hydrocephalus
122
What is the pathophysiology of viral meningitis?
- Predominantly lymphocytic inflammatory CSF reaction without pus formation
- Little or no cerebral oedema unless encephalitis develops
123
What are the risk factors of meningitis?
- Intrathecal drug administration (into spinal canal)
- Immunocompromised
- Elderly
- Pregnant
- Bacterial endocarditis
- Crowding (e.g. military base, university students)
- Diabetes
- Malignancy
- IV drug use
124
What is the classic triad of meningitis symptoms?
Headache, neck stiffness and fever
125
What is the clinical presentation of acute bacterial meningitis?
- Onset is typically sudden
- Papilloedema: swelling of optic disc on fundoscopy, usually bilateral, can occur over hours to weeks, caused by raised ICA
- Usually intense malaise, fever, rigors, severe headache, photophobia and vomiting within hours or minutes
- Patient is irritable and prefers to lie still
- Neck stiffness, positive Kernig’s and Brudzinski’s sign can appear within hours
- Meningococcal septicaemia is associated with a non-blanching and purpuric skin rash
- Altered mental state (reduced GCS) with high fever (often absent though)
- Seizures and focal CNS signs
- Progressive drowsiness, materialising signs and CN lesions indicate a complication (e.g. venous sinus thrombosis, severe cerebral oedema or cerebral abscess)
126
What is the clinical presentation of viral meningitis?
- Benign, self-limited condition lasting 4-10d
| - Headache may follow for some months
127
What is the clinical presentation of chronic meningitis?
- Mycobacterium tuberculosis
- Long history and vague symptoms of headache, anorexia and vomiting
- Signs of meningeal triad are often absent or late
128
What are the differential diagnoses of meningitis?
- Aseptic meningitis (due to tumour)
- Subarachnoid haemorrhage (headache is more sudden)
- Encephalitis (altered mental state is the predominant symptom)
129
How is meningitis diagnosed?
- Blood cultures before lumbar puncture
- Blood tests: FBC, U&E, CRP, serum glucose
- CT head to exclude lesions e.g. tumour
- CT before lumbar puncture if aged >60y, history of a CNS disease, papilloedema, immunocompromised, focal neurological signs, decreasing consciousness levels
- Throat swabs (bacterial and viral)
- Pneumococcal and meningococcal serum PCR
- If meningococcal septicaemia (non-blanching rash or purpuric rash) then immediate IV benzylpenicillin or IV cefotaxime
130
How is meningitis managed?
- Start antibiotics immediately
- IV cefotaxime or IV ceftriaxone (can give IV chloramphenicol if severe anaphylaxis)
- Add IV amoxicillin if >50y or immunocompromised
- Steroids e.g. oral dexamethasone to reduce cerebral oedema
- IV vancomycin for return travellers
- Prophylaxis for contacts: oral ciprofloxacin
131
What is the aetiology of encephalitis?
- Mainly viral (herpes simplex virus 1&2, varicella zoster, EBV, cytomegalovirus, HIV, mumps, measles)
- Non viral = bacterial meningitis, TB, malaria
132
What is the pathophysiology of encephalitis?
Disease which mostly affects the frontal and temporal lobes resulting in decreased consciousness, confusion and focal signs
133
What are the risk factors of encephalitis?
- Extremes of age
| - Immunocompromised
134
What us the clinical presentation of encephalitis?
- Whole brain affected – problems with consciousness compared to meningitis
- Onset can be insidious (over days) or abrupt
- CLASSIC TRIAD: fever + headache + altered mental state
- May have signs of meningitis ( = meningo-encephalitis and consists of inflammation of meninges and brain parenchyma)
- May have a history of travel and/or an animal bite
- Begins with features of a viral infection: fever, headaches, myalgia, fatigue and nausea
PROGRESSES:
- Personality and behavioural changes (common early manifestation)
- Decreased consciousness, confusion and drowsiness
- Focal neurological deficit (hemiparesis and dysphagia)
- Seizures
- Raised ICP and midline shift may occur, resulting in coning
- Coma
135
What are the differential diagnoses of encephalitis?
- Meningitis
- Stroke
- Brain tumour
136
How is encephalitis diagnosed?
- MRI shows area of inflammation and swelling and may have midline shifting
- Electroencephalography shows periodic sharp and slow wave complexes
- Lumbar puncture: CSF shows elevated lymphocyte count, also used to viral detection
- Blood and CSF serology is usually helpful
137
How is encephalitis managed?
- If viral, then immediate treatment with an anti-viral e.g. IV aciclovir (before test results are available)
- Anti-seizure medication e.g. primidone
- If meningitis is suspected, then emergency IM benzylpenicillin
138
What is the aetiology of herpes voster (shingles)?
Reactivation of varicella zoster virus, usually within the dorsal root ganglia
139
What is the pathophysiology of herpes voster (shingles)?
- Viral infection affecting peripheral nerves
- When latent virus is reactivated in the dorsal root ganglia it travels down the affected nerve via the sensory root in dermatomal distribution over a period of 3-4d, resulting in perineural and intramural inflammation
- In immunocompromised patients, the most frequent site of reactivation is the thoracic nerves followed by the ophthalmic division of the trigeminal nerve
- Can also affect the cervical, lumbar and sacral nerve roots
- Person with weeping shingles rash can cause chickenpox in non-immune person after close contact
140
What are the risk factors of herpes zoster (shingles)?
- Increasing age
- Immunocompromised
- HIV, Hodgkin’s lymphoma and bone marrow transplants
141
What is the clinical presentation of herpes zoster (shingles)?
- Pain and paraesthesia in dermatomal distribution priced rash for days
- Malaise, myalgia, headache and fever can be present
- Can be over a week before eruption appears
- Rash consists of papules and vesicles restricted to the same dermatome (neuritis pain, crust formation and drying occurs over the next week with resolution within 2-3w)
- Patients are infectious until lesions are dried
- Rash does not extent outside dermatome
142
What are the differential diagnoses of herpes zoster (shingles)?
- Cholecystitis or renal stones (before rash appears, the pain may come from the chest/ abdominal)
- Cluster headaches or migraine
- Atopic eczema, contact dermatitis or herpes simplex or impetigo
143
How is herpes zoster (shingles) diagnosed?
- Clinical diagnosis
| - Eruption of rash is virtually diagnostic
144
How is herpes zoster (shingles) managed?
- Oral antiviral therapy within 72h of rash onset
- Oral aciclovir 5x daily or oral valaciclovir/ famciclovir 2x daily
- Topical antibiotic treatment for secondary bacterial infection
- Analgesia e.g. ibuprofen for pain
