Neurology Flashcards
(222 cards)
1
Q
What is the function of the frontal lobe?
A
- Voluntary movement on contralateral side of body.
- Dominant hemisphere controls speech (Broca’s area) + writing.
- Intellectual functioning, thought processes, reasoning + memory.
2
Q
What is the function of the parietal lobe?
A
- Receive + interprets sensations including touch, pressure, size + shape.
- Body-part awareness (proprioception).
3
Q
What is the function of the temporal lobe?
A
- Understanding spoken word (Wernicke’s area), sounds, memory + emotion.
4
Q
What is the function of the occipital lobe?
A
- Understanding visual images + meaning of written words.
5
Q
What is the function of the cerebellum?
A
- Responsible for precise control, fine adjustment + co-ordination of motor activity based on continual sensory feedback.
- Computes motor error, adjusts commands + projects this information back to motor cortex.
- Decides HOW you do something.
6
Q
What is cerebellar dysfunction characterised by?
A
DANISH…
- Dysdiadochokinesia.
- Ataxia.
- Nystagmus.
- Intention tremor.
- Slurred speech.
- Hypotonia.
7
Q
How is ataxia severity shown? How does cerebellar dysfunction present on MRI brain?
A
- Mild = independent/1 walking aid, moderate = 2 aids, severe = wheelchair.
- Cerebellar atrophy (excludes tumour, hydrocephalus).
8
Q
What part of the brain does the…
i) anterior cerebral artery
ii) middle cerebral artery
iii) posterior cerebral artery
supply?
A
i) Antero-medial aspect.
ii) Lateral portions of cerebrum, basal ganglia.
iii) Occipital lobe, posteromedial parietal lobe.
9
Q
What is Duchenne muscular dystrophy?
A
- Dystrophin affected (out-of-frame mutation), scattered cell nuclei, muscle cells all have different morphologies.
- X-linked recessive.
10
Q
What is the clinical presentation + treatment for Duchenne muscular dystrophy?
A
- <5y/o, delayed milestones, wheelchair by teenager, arrhythmias/heart block.
- Supportive with PT, OT, scoliosis corrective surgery.
11
Q
What is the corticospinal tract?
A
Descending UMN…
- Motor.
- UMN originate in motor cortex.
- 75% decussate at medulla.
12
Q
What is the dorsal column medial lemniscus (DCML) tract?
A
Ascending sensory…
- Proprioception, vibration + 2-point discrimination.
- Fasciculus cuneatus (lateral, info from upper body to cuneate tubercle).
- Fasciculus gracilis (medial, info from lower body to gracile tubercle).
- Decussates at medulla, ascends to thalamus, then cortex.
13
Q
What is the spinothalamic tract?
A
Ascending sensory tract…
- Lateral = pain + temperature, medial = crude touch.
- Enters spinal cord, ascends 1–2 levels + then decussates.
14
Q
What is Brown-Sequard syndrome?
A
- Hemi-section of spinal cord.
- Ipsilateral loss of proprioception, motor + fine touch below lesion (DCML/corticospinal).
- Contralateral loss of pain, temperature + crude touch a few levels below lesion (spinothalamic).
15
Q
Describe the process of dopamine production. Where does the substantia nigra project to? What site is affected by brain stimulation?
A
- Tyrosine > L-dopa > Dopamine.
- Substantia nigra projects to striatum.
- Subthalamic nucleus.
16
Q
What neurotransmitters are excitatory/inhibitory? What site is affected by brain stimulation?
A
- Glutamate = excitatory.
- GABA = inhibitory.
- Dopamine D1 = excitatory.
- Dopamine D2 = inhibitory.
17
Q
CEREBROVASCULAR ACCIDENT
What is a CVA?
A
- A stroke is a rapid onset of neurological deficit which is the result of a vascular lesion + is associated with infarction of central nervous tissue.
18
Q
CEREBROVASCULAR ACCIDENT
What are the two types of CVA and how do they differ?
A
- Ischaemic = ischaemia leading to infarction + death of neural tissue leading to loss of functionality.
- Haemorrhagic = primarily intracerebral haemorrhage, risk factors lead to small vessel damage + aneurysms where a rupture may occur > haemorrhage.
19
Q
CEREBROVASCULAR ACCIDENT
What is the aetiology of CVA?
A
Cerebral infarction due to embolism/thrombosis (85%)
- Cardiac emboli (AF, endocarditis), atherothromboembolism.
Intracerebral/sub-arachnoid haemorrhage (15%)
- Primary = Hypertensive, lobar haemorrhages due to amyloid depositions.
- Secondary = anticoagulants, tumours (metastases).
20
Q
CEREBROVASCULAR ACCIDENT
What is Charcot-Bouchard aneurysms?
A
- Often found in basal ganglia due to chronic HTN.
21
Q
CEREBROVASCULAR ACCIDENT
What are the risk factors for CVA?
A
- HTN.
- DM.
- Smoking + alcohol.
- Hyperlipidaemia.
- Obesity.
22
Q
CEREBROVASCULAR ACCIDENT
How would an anterior cerebral artery CVA present?
A
- Lower limb weakness + loss of sensation (contralateral).
- Gait apraxia (unable to initiate walking).
- Incontinence.
- Drowsiness.
- Decrease in spontaneous speech.
23
Q
CEREBROVASCULAR ACCIDENT
How would a middle cerebral artery stroke present?
A
- Upper limb weakness + loss of sensation (contralateral).
- Hemianopia.
- Aphasia (inability to understand or produce speech = Broca’s area).
- Dysphasia (deficiency in speech generation = Wernicke’s area).
- Facial droop.
24
Q
CEREBROVASCULAR ACCIDENT
How would a posterior cerebral artery present?
A
- Visual field defects (contralateral homonymous hemianopia).
- Cortical blindness.
- Visual agnosia (Cannot interpret visual information but can see).
- Prosopagnosia (inability to recognise familiar face).
- Unilateral headache.
25
CEREBROVASCULAR ACCIDENT
| What are the investigations for CVA?
- Recognise – think F.A.S.T.
- CT head to identify if haemorrhagic/ischaemic BEFORE treatment.
- Bloods – FBC for thrombocytopenia + polycythaemia, blood glucose.
26
CEREBROVASCULAR ACCIDENT
| What is the treatment for an ischaemic stroke?
- Thrombolysis with IV alteplase (tissue plasminogen activator) within 4.5 hours.
- Alteplase converts plasminogen into plasmin + so promotes breakdown of fibrin clot.
- 300mg aspirin for 2 weeks post-stroke + lifelong clopidogrel.
27
CEREBROVASCULAR ACCIDENT
| What is the treatment for a haemorrhagic stroke?
- Stop anticoagulants, warfarin reversal with beriplex.
- Control BP with beta-blocker.
- Surgical = clipping or coiling.
28
CEREBROVASCULAR ACCIDENT
| What risk factor management is there for CVA + post-stroke what professionals work with the patient?
- Anti-hypertensives + statins.
- PT for physiotherapy
- OT for home modifications.
- SALT for swallowing + speech help.
29
CEREBROVASCULAR ACCIDENT
| What are some contraindications to thrombolysis?
- Haemorrhage.
- Active bleeding.
- Warfarin/heparin.
- Aneurysm.
- Pregnant.
- HTN.
30
TIA
| What is the pathophysiology of a transient ischaemic attack (TIA)?
- Acute loss of focal neurological deficit with symptoms lasting <24h + with a complete clinical recovery.
- Symptoms often most severe at start.
- Caused by inadequate cerebral blood supply > ischaemia + so oxygen deprivation of tissue + transient loss of function with resolution but possible remittance.
31
TIA
| What is the aetiology of TIA?
- Atherothromboembolism from carotid.
- Cardioembolism – mural thrombus post-MI, AF.
- Hyperviscosity – polycthaemia, sickle-cell anaemia.
32
TIA
| What are the risk factors for TIA?
- HTN.
- DM.
- Smoking + alcohol.
- Hyperlipidaemia.
- Obesity.
33
TIA
| What is the clinical presentation of a TIA in the carotid territory?
- Amaurosis fugax (sudden transient loss of vision in one eye).
- Aphasia.
- Hemiparesis.
- Hemisensory loss.
- Hemianopic visual loss.
34
TIA
| What is the clinical presentation of a TIA in the vertebrobasilar territory?
- Diplopia, vertigo, vomiting.
- Choking + dysarthria (unclear articulation of speech but understandable).
- Ataxia (no control of body movement).
- Hemisensory/hemianopic visual loss.
- Tetraparesis.
35
TIA
| What are the investigations for TIA?
- Bloods – FBC for polycythaemia, glucose for hypoglycaemia.
- Carotid doppler ± angiography.
- CT head.
36
TIA
| What score can be used to assess someone's 7-day stroke risk post-TIA?
```
ABCD2...
- Age >60 (1)
- BP >140/90mmHg (1)
- Clinical features (1)
- Duration – ≥60 (2), 10-59 (1)
- Diabetes (1)
Score >6 = specialist immediately, >4 specialist 24h, rest seen within 1w.
```
37
TIA
| What is the anti-thrombotic treatment given for TIA?
- Aspirin 300mg immediately then 75mg continued long-term.
| - Clopidogrel 75mg if intolerant.
38
TIA
| What is the secondary prevention for TIA? What other treatment can be given? Recommendations to patient who drives?
- Control HTN, treatment of statin for patients with high cholesterol.
- Carotid edarterectomy if ICA stenosis >70%.
- Do not drive for AT LEAST 1 MONTH following a TIA.
39
SAH
| What is the pathophysiology of a subarachnoid haemorrhage (SAH)?
- Spontaneous rupture causes a rapid release of arterial blood into subarachnoid space causing an increased intracranial pressure + possibly CVA.
40
SAH
| What is the aetiology of SAH?
Berry aneurysm rupture (80%)
- Common sites = bifurcations like anterior communicating + anterior cerebral, middle cerebral or posterior communicating + ICA.
Congenital arteriovenous malformations
Other – tumour, vasculitis.
41
SAH
| What are the risk factors of SAH?
- HTN.
| - Polycystic kidney disease, coarctation of aorta, Ehler's Danlos syndrome.
42
SAH
| What are the symptoms of SAH?
- Sudden-onset excruciating headache = thunderclap.
- Nausea, loss of consciousness, seizures.
- Preceding 'sentinel' headache = small warning leak from offending aneurysm.
43
SAH
| What are the signs of SAH?
- Neck stiffness.
- Kernig's (unable to extend patients leg at knee when thigh flexed).
- Retinal bleeds.
44
SAH
| What are complications of SAH?
- Rebleeding.
- Cerebral ischaemia due to vasospasm.
- Hydrocephalus due to blockage of arachnoid granulations.
- Hyponatraemia.
45
SAH
| What are the investigations for SAH?
CT head = gold standard...
- Star-shaped lesion due to blood filling in gyro patterns around brain + ventricles.
Lumbar puncture if CT-ve, wait 12 hours for Hb to break down, 3 samples = no bloody tap..
- Xanthochromic (yellow due to bilirubin) confirms.
CT angiography.
46
SAH
| What is the treatment for SAH?
- Neurosurgery immediately (endovascular coiling vs. surgical clipping).
- Maintain cerebral perfusion IV fluids but BP <160mmHg.
- Nimodipine (CCB) to reduce vasospasm.
47
EXTRA-DURAL HAEMATOMA
| What is the pathophysiology of extra-dural haematoma? What is the ventricles mechanism in this?
- Fractured temporal/parietal bone leads to rupture of the middle meningeal artery causing a bleed ABOVE the dura.
- Ventricles compensate by getting rid of their CSF to prevent rise in intracranial pressure.
48
EXTRA-DURAL HAEMATOMA
| What is the aetiology of extra-dural haematoma?
- Traumatic head injury or skull fracture.
49
EXTRA-DURAL HAEMATOMA
| What is the clinical presentation of extra-dural haematoma?
- Lucid interval pattern where progressive decrease (rapid) in GCS from rising intracranial pressure.
- Papilloedema (cardinal physical sign due to obstruction of venous return from retina).
- Increasingly severe headache, vomiting, confusion + seizures.
- Ipsilateral pupil dilation.
50
EXTRA-DURAL HAEMATOMA
| What are the complications of extra-dural haematoma?
- Brainstem compression causing breathing to become deep + irregular.
- Death may follow a period of coma due to respiratory arrest.
51
EXTRA-DURAL HAEMATOMA
| What are the investigations for extra-dural haematoma?
- CT head = biconvex/lens-shaped haematoma.
- Skull X-ray may show fracture lines crossing course of middle meningeal artery.
- Lumpar puncture C/I.
52
EXTRA-DURAL HAEMATOMA
| What is the treatment for extra-dural haematoma?
- IV mannitol for increased intracranial pressure.
| - Surgery for clot removal.
53
SUBDURAL HAEMATOMA
| What is the pathophysiology of subdural haematoma?
- Rupture of a vein running from the hemisphere to the sagittal sinus (bridging veins) that's beneath the dura.
- Often latent period after head injury + then clot starts to breakdown + massive increase in oncotic pressure so water is sucked up into haematoma causing symptoms > gradual rise in intracranial pressure.
54
SUBDURAL HAEMATOMA
| What is the aetiology of subdural haematoma? In what group of people are they most common in?
- Almost always head injury (often minor), can occur 9 months post-incident.
- Patients with small brains, at risk of falls + on anti-coagulation therapy (alcoholics, elderly with dementia).
55
SUBDURAL HAEMATOMA
| What are the symptoms of subdural haematoma?
- Fluctuating level of consciousness (GCS) ± insidious physical/intellectual slowing.
- Sleepiness.
- Headache.
56
SUBDURAL HAEMATOMA
| What are the signs of subdural haematoma?
- Increased intracranial pressure (headache, reduced GCS, papilloedema).
- Localising neurological symptoms (unequal pupils).
57
SUBDURAL HAEMATOMA
| What are the investigations + treatment of subdural haematoma?
- CT head = clot ± mid-line shift, crescent-shaped collection of blood.
- Surgical remove of clot – 1st line clot evacuation, 2nd line craniotomy, IV mannitol if increased intracranial pressure.
58
EPILEPSY
| What is the pathophysiology of epilepsy?
- Epilepsy is a recurrent tendency to spontaneous, intermittent, abnormal electrical activity in parts of the brain, manifesting as seizures where convulsions are the motor signs of electrical discharges.
- Innervation of muscle fibres cause physical movements (tonic clonic seizures) + sensory disturbance (partial seizures).
59
EPILEPSY
| What is the aetiology of epilepsy?
- 2/3rd idiopathic.
- Flashing lights (trigger).
- CNS infection like meningitis.
- Trauma.
60
EPILEPSY
| What are the two broad categories of seizures?
- Focal seizures where one hemisphere of the brain is affected.
- Generalised seizures where the whole brain is affected.
61
EPILEPSY
| What are the different types of focal seizures?
- Simple-partial = with counsciousness.
- Complex-partial = without consciousness.
- Secondary generalised seizures.
62
EPILEPSY
| What is the clinical presentation of a temporal lobe seizure?
- Aura.
- Deja-vu.
- Auditory hallucinations.
- Funny smells.
- Automatisms (chewing, picking at clothes).
63
EPILEPSY
| What is the clinical presentation of a frontal lobe seizure?
- Jacksonian march (starts in small area + spreads to larger).
- Post-ictal Todd's palsy (paralysis of limbs involved in seizure for several hours).
64
EPILEPSY
| What is the clinical presentation of a parietal lobe seizure?
- Tingling/numbness.
65
EPILEPSY
| What are the different types of generalised seizures?
Absence seizures (petite mals)...
- Brief (≤10s) pauses, presents in childhood.
Tonic-clonic seizures (grand mals)...
- Loss of consciousness, limbs stiffen (tonic) > jerk (clonic).
- Up to 120s, associated with tongue biting + incontinence.
Myoclonic seizures...
- Sudden jerk of a limb/face/trunk, patient may be thrown suddenly to ground.
Atonic (akinetic) seizures...
- Sudden loss of muscle tone causing full but conscious.
66
EPILEPSY
| How can you differentiate between epilepsy + syncope?
- Epilepsy aura, syncope light-headedness, faint.
- Epilepsy sudden, syncope avoidable with posture change.
- Epilepsy eyes open, convulsions, syncope eyes closed, falls forwad.
- Epilepsy recovery is confused + sleepy, syncope is pale, sweaty, cold.
- Epilepsy involves tongue biting, incontinence, rare in syncope.
67
EPILEPSY
| What is the major complication of epilepsy?
- Status epilepticus > medical emergency with continuous seizures >30m or ≥2 seizures without recovery over similar time period.
- Risk of death from cardiorespiratory failure – IV lorazepam.
68
EPILEPSY
| What are the investigations for epilepsy?
- Consider electroencephalogram (EEG).
- CT brain in emergencies.
- MRI brain.
69
EPILEPSY
| What is the treatment for epilepsy?
```
Focal seizures...
- 1st line = carbamazepine/lamotrigine.
- 2nd line = sodium valporate.
Generalised...
- 1st line = sodium valporate.
- 2nd line lamotrigine.
Seizure control...
- Diazepam (rectal), IV lorazepam.
```
70
PARKINSON'S DISEASE
| What is the pathophysiology of Parkinson's disease?
- There is progressive loss of dopamine secreting cells from the substantia nigra causing an alteration in neural circuits within the basal ganglia which regulates movement.
- Presence of Lewy bodies.
- Loss from non-striatal pathways for neuropsychiatric pathology.
71
PARKINSON'S DISEASE
| What is the aetiology of Parkinson's disease?
- Unknown, genetic link.
| - Parkinson's disease is less prevalent in tobacco smokers than in life long abstainers.
72
PARKINSON'S DISEASE
| What is the clinical presentation of Parkinson's disease? What are the typical Parkinsonism features?
- Asymmetrical, symptoms on one side always worse.
- Problems doing up buttons, writing smaller.
Parkinsonism...
- Bradykinesia.
- Rigidity (pain, problems turning in bed).
- Resting tremor ('pill-rolling' of thumbs over fingers).
- Postural changes (stooped).
73
PARKINSON'S DISEASE
| What are the gait issues in Parkinson's disease?
- Small steps/shuffling.
| - Walking slowly, reduce arm swing (asymmetrical).
74
PARKINSON'S DISEASE
| What are the pre-motor symptoms of Parkinson's disease?
- Anosmia (loss of sense of smell).
- Depression.
- REM sleep behaviour disorder.
- Autonomic features (urinary urgency, hypotension).
75
PARKINSON'S DISEASE
| What are the investigations for Parkinson's disease?
- Diagnosis = clinical.
| - Idiopathic Parkinson's disease shows a normal CT/MRI.
76
PARKINSON'S DISEASE
| What is the treatment in Parkinson's disease to increase the amount of dopamine in CNS?
- Levodopa (dopamine precursor) can cross BBB to be converted to dopamine by dopa-decarboxylase.
- Combined with peripheral dopa-decarboxylase inhibitor (carbidopa) so it crosses BBB first.
- Drug = co-careldopa (levodopa + carbidopa).
77
PARKINSON'S DISEASE
| What is the treatment in Parkinson's disease that mimics the action of dopamine? What is used for tremor management?
- Dopamine receptor antagonists like ropinirole.
| - Anticholinergic like amantadine.
78
PARKINSON'S DISEASE
| What is the treatment in Parkinson's disease that inhibits enzymatic breakdown of dopamine?
- COMT inhibitor (tolcapone).
| - MAO inhibitor selegiline.
79
HEADACHES
| What is the pathophysiology of headaches?
Primary...
- No underlying cause relevant to headache.
- Migraine, cluster + tension (most common).
Secondary...
- Underlying cause needs identifying.
- Meningitis, SAH, giant cell arteritis, medication overuse.
80
HEADACHES
| What are the red flags of a secondary headache?
- Thunderclap headache.
- Seizure/altered GCS.
- Papilloedema.
81
```
HEADACHES
What is the aetiology of...
i) cluster
ii) tension
iii) medication overuse
```
headaches?
i) More common in men + smokers.
ii) Commonest primary headache, precipitated by missed meals, stress, conflict, lack of sleep.
iii) Commonest secondary headache.
82
HEADACHES
| What is the clinical presentation of cluster headache?
15m–3h...
- Rapid onset excruciating pain around one eye, may be watery + bloodshot.
- Pain is unilateral, often nocturnally.
- Cranial autonomic features (lacrimation, lid swelling, facial flushing).
83
HEADACHES
| What is the clinical presentation of tension headache?
30m–7d...
- Bilateral.
- Pressing/tight band-like sensation.
- Mild-moderate intensity.
- Non-pulsatile.
84
```
HEADACHES
What are the investigations for...
i) cluster
ii) tension
iii) medication overuse
```
headaches?
i) ≥5 headaches fulfilling clinical presentation.
ii) Clinical diagnosis.
iii) Headache present for >15d/month
- Regular use for >3m of >1 symptomatic treatment drugs.
- Headache developed/markedly worsened during drug use.
85
HEADACHES
| What is the treatment + prevention for cluster headaches?
- Acute attack give 100% oxygen + sumatriptan s/c (serotonin receptor antagonist).
- Prevention use verapamil (CCB) as first line prophylaxis, avoid alcohol during cluster period.
86
HEADACHES
| What is the treatment for tension headaches?
- Reassurance + lifestyle advice (avoid triggers, exercise).
| - Symptomatic treatment for episodes like aspirin, paracetamol.
87
MIGRAINE
| What is the pathophysiology of migraines?
- Changes in brainstem blood flow lead to an unstable trigeminal nerve nucleus + nuclei in basal thalamus.
- Leads to release of vasoactive neuropeptides CGRP + substance P > neurogenic inflammation > vasodilation + plasma protein extravasation leading to pain propagating all over cerebral cortex.
88
MIGRAINE
| How long does a migraine tend to last? Who is more at risk?
4–72h, more common in females.
89
MIGRAINE
| What is the aetiology of migraines?
CHOCOLATE...
- Chocolate.
- Hangovers.
- Orgasms.
- Cheese/caffeine.
- Oral contraceptives.
- Lie-ins.
- Alcohol.
- Travel.
- Exercise.
90
MIGRAINE
| What are the three types of migraines?
- Episodic migraine without aura (80%).
- Episodic migraine with aura (20%).
- Migraine variant.
91
MIGRAINE
| What is the clinical presentation of episodic migraine without aura?
```
≥2 of these...
- Unilateral.
- Pulsatiles.
- Moderate/severe pain.
- Aggravated by physical activity.
≥1 of these...
- Photophobia + phonophobia,
- Nausea ± vomiting.
```
92
MIGRAINE
| What is the clinical presentation of episodic migraine with aura?
```
≥2 of these...
- ≥1 aura symptom is unilateral.
- Aura accompanied/followed within 60mins by headache.
≥1 of these...
- Visual disturbances like flashing lights, zig-zag lines.
- Paraesthesia.
- Aphasia.
Motor weakness (hemiplegic migraine).
```
93
MIGRAINE
| What is the clinical presentation of migraine variant?
- Characterised by unilateral motor/sensory symptoms resembling a stroke.
94
MIGRAINE
| What is the treatment for migraines?
```
Avoid triggers.
Prophylaxis...
- Propranolol (1st line).
- Acupuncture (2nd line).
- Amitryptyline (3rd line).
During an attack...
- Oral sumatriptan combined with NSAID/paracetamol.
```
95
TRIGEMINAL NEURALGIA
| What is the pathophysiology of trigeminal neuralgia? Which branch is most affected?
- Compression of the trigeminal nerve resulting in demyelination + excitation of the nerve resulting in erratic pain signalling.
- Mandibular branch.
96
TRIGEMINAL NEURALGIA
| What is the aetiology of trigeminal neuralgia?
- Can be idiopathic or secondary to tumour, MS.
| - Triggers = washing affected area, shaving, eating, talking + dental prostheses.
97
TRIGEMINAL NEURALGIA
| What is the clinical presentation of trigeminal neuralgia?
- Paroxysms of intense, stabbing pain, lasting seconds in the trigeminal distribution.
- Unilateral.
- Knife-like/shooting pain – no neurological deficit.
98
TRIGEMINAL NEURALGIA
| What are the investigations + treatment for trigeminal neuralgia?
- MRI head to exclude secondary, clinical diagnosis of ≥3 attacks, pain in ≥1 division + symptoms.
- Anti-convulsant carbamazepine, if drugs fail microvascular decompression or stereotactic radiation.
99
MULTIPLE SCLEROSIS
| What is the pathophysiology of MS?
- Chronic autoimmune inflammatory disorder of CNS due to T-cell mediated immune response.
- Demyelination heals poorly with thinner, inefficient myelin, eventually causing axonal loss of oligodendrocytes.
100
MULTIPLE SCLEROSIS
| Where do you find MS plaques? Are PNS myelinated nerves affected and why?
Plaques are perivenular + have predilection for distinct sites...
- Optic nerves.
- Around ventricles.
- Corpus callosum.
- Brainstem + Cerebellar connections.
- Cervical cord.
No as their myelin is Schwann cell based + have different antigens to that of CNS myelin from oligodendrocytes.
101
MULTIPLE SCLEROSIS
| What is the epidemiology of MS?
- Presents 20–40y/o.
| - Females > males.
102
MULTIPLE SCLEROSIS
| What is the aetiology of MS?
- Enviroment (EBV shown to be associated).
| - Genetic predisposition.
103
MULTIPLE SCLEROSIS
| What are the different types of MS?
Relapsing + remitting...
- Periods of remission followed by sudden relapses.
- Patients may accumulate disability over time if they don't fully recover from relapses.
Secondary progressive MS...
- Follows on from relapsing + remitting consisting of gradually worsening symptoms with fever remissions.
Primary progressive...
- Gradually worsening disability without relapses or remissions.
Progressive relapsing...
- Steady decline since onset with super-imposed attacks
104
MULTIPLE SCLEROSIS
| What is the clinical presnetation of MS?
DEMYELINATION, usually monosymptomatic...
- Diplopia.
- Eye movements painful (optic neuritis).
- Motor weakness.
- nYstagmus.
- Elevated temp worsens (Uhtoff's phenomenon.
- Lhermitte's sign (neck movement = shock).
- Intention tremor.
- Neuropathic pain.
- Ataxia.
- Talking slurred.
- Impotence.
- Overactive bladder.
- Numbness.
105
MULTIPLE SCLEROSIS
| What are the investigations for MS?
- MRI brain + spinal cord to see demyelination plaques.
- Lumbar puncture with CSF electrophoresis = oligoclonal bands of IgG on electrophoresis.
- Evoked potentials = delayed visual, brainstem, auditory + somatosensory potentials.
106
MULTIPLE SCLEROSIS
| What is the treatment for MS?
Lifestyle...
- Regular exercise, smoking cessation, avoid stress, CBT.
Mediciation...
- Relpases = steroids (methylprednisolone).
- Chronic = 1st, beta-interferon, 2nd, natalizumab (monoclonal antibody).
107
MULTIPLE SCLEROSIS
| What treatments are used for symptom control in MS?
- Baclofen = spasticity.
- Gabapentin = neuropathic pain.
- Beta-blocker = tremor.
108
MOTOR NEURONE DISEASE
| What is the pathophysiology of motor neurone disease?
- The relentless destruction/degeneration of motor neurones in...
- Motor cortex = UMN signs.
- Anterior horn cells = LMN signs.
- Cranial nerve nuclei = mixed.
109
MOTOR NEURONE DISEASE
| What are the different types of MND?
```
Amyotrophic lateral sclerosis (ALS)...
- Motor cortex + anterior horn so UMN + LMN.
Primary lateral sclerosis (PLS)...
- Motor cortex
Progressive bulbar palsy (PBP)
- Destruction of CN9–12 + so UMN + LMN of them.
Progressive muscular atrophy (PMA)
- Anterior horn cell lesion so LMN.
```
110
MOTOR NEURONE DISEASE
| What is the clinical presentation of ALS?
- Progressive focal wasting, weakness + fasciculation spreading to other limbs.
- Cramps.
- Spasticity + brisk reflexes (Babinski present).
111
MOTOR NEURONE DISEASE
| What is the clinical presentation of PLS?
- Slow progressive tetraparesis.
| - Pseudobulbar palsy (dysarthria, dysphagia, choking).
112
MOTOR NEURONE DISEASE
| What is the clinical presentation of PMA?
- Weakness + fasciculations starting in one limb + progressing to ajacent spinal segments.
113
MOTOR NEURONE DISEASE
| What is the clinical presentation of PBP?
- Dysarthria (slurred speech).
- Dysphagia.
- Choking.
114
MOTOR NEURONE DISEASE
| What are the investigations for motor neurone disease?
- EMG + nerve conductiion studies = muscle denervation.
- Increased creatinine kinase due to muscle breakdown.
- LP excludes inflammatory causes.
- Brain/cord MRI excludes structural causes.
115
MOTOR NEURONE DISEASE
| What features of motor neurone disease help distinguish it from MS/myasthenia gravis?
- No sensory loss.
- No disturbances in eye movements.
- No sphincter disturbances.
116
MOTOR NEURONE DISEASE
| What is the treatment for motor neurone disease?
- Riluzole = Na+ blocker inhibits glutamate release + slows disease progression.
- Symptomatic > dysphagia = NG/PEG tube, drooling = amitryptyline, pain = analgesics.
117
MENINGITIS
| What is the pathophysiology of meningitis? Who must all recorded cases of meningitis be reported to and why?
- Infection of the meninges leads to inflammation of the tissue.
- Microrganisms can reach the meninges either by direct extension from ears, nasopharynx or via bloodstream spread.
- Public Health England as it's a notifiable disease.
118
MENINGITIS
| What is the aetiology of bacterial meningitis?
- N. meningitidis, gram-ve diplococci (meningococcal meningitis).
- S. pneumoniae, gram+ve cocci chain (pneumococcal meningitis, most common).
- Listeria monocytogenes, gram+ve bacilli.
119
MENINGITIS
| What is the aetiology of viral meningitis?
- Enteroviruses (commonest), herpes simplex virus, TB.
120
MENINGITIS
| What is the clinical presentation of bacterial meningitis?
- Headache, papilloedema.
- Fever.
- Menigism = neck stiffness, photophobia, Kernig's sign.
- Non-blanching rash (+ signs of sepsis = meningococcal septicaemia).
121
MENINGITIS
| What is the clinical presentation of viral + TB meningitis?
- Blurred vision/headache, often benign + self-limited for about a week.
- Long history + vague symptoms (headache, vomiting) with meningism later on.
122
MENINGITIS
| What are the complications of meningitis infection?
- Cerebral oedema or abscess.
| - Later in life = recurring headaches, fatigue.
123
MENINGITIS
| What are the investigations for meningitis?
- Blood cultures before LP + Abx.
- Bloods = FBC, U+E, CRP, glucose.
- LP (L4/5 level) w/ CSF culture.
- CT head if other signs like papilloedema.
124
MENINGITIS
| In what situations is a lumbar puncture contraindicated in meningitis and why?
- Drowsy/seizures/signs of increased intracranial pressure + meningococcal septicaemia.
- Coning of cerebellar tonsils.
125
```
MENINGITIS
What would the lumbar puncture result look like for...
i) Bacterial.
ii) Viral.
iii) TB.
```
meningitis?
i) Turbid (cloudy), polymorphs, protein +, glucose –
ii) Clear, lymphocytes, protein, normal, glucose normal.
iii) Fibrin web, lymphocytes, protein +, glucose –/normal.
126
MENINGITIS
| What is the community treatment for someone suspected of meningitis?
- IM benzylpenicillin.
127
MENINGITIS
| What is the immediate hospital treatment for someone with bacterial meningitis?
- IV cefotaxime.
| - Add amoxicillin to cover listeria.
128
MENINGITIS
| What is the treatment for viral meningitis?
- Supportive therapy + aciclovir for herpetic infection.
129
MENINGITIS
| What is the prevention against meningitis?
- Children are vaccinated against meningitis B (8/16w), C (12w+12m) + ACWY (14y).
- Prophylactic rifampicin give to anyone in droplet range as effective against N. meningitidis.
130
ENCEPHALITIS
| What is the pathophysiology of encephalitis?
Infection + inflammation of the brain parenchyma (cortex/white matter/brainstem/basal ganglia).
131
ENCEPHALITIS
| What is the aetiology of encephalitis?
More common in immunocompromised.
- Mainly viral (Herpes simplex virus 1+2, EBV, HIV).
- Non viral = any bacterial meningitis, TB, malaria.
132
ENCEPHALITIS
| What is the clinical presentation of encephalitis?
- Fever, hedache + altered mental state (personality/behaviour changes).
- Reduced GCS compared to meningitis.
- Focal neurological deficit like hemiparesis, dysphasia.
133
ENCEPHALITIS
| What are the investigations for encephalitis?
- Viral PCR.
- Blood cultures.
- CT head.
- LP = increased CSF protein + lymphocytes, decreased glucose, send for CSF viral PCR.
134
ENCEPHALITIS
| What is the treatment for encephalitis?
- Immediate high dose IV aciclovir.
135
SHINGLES
| What is the pathophysiology of shingles?
- Caused by reactivation of varicella zoster virus (chickenpox), usually within dorsal root ganglia where it's been dormant.
- When it flares up, virus travels down affected nerve over 3–4 days, causing peri/intraneural inflammation.
136
SHINGLES
| What are the risk factors of reactivation in shingles?
- Old age.
| - Immunocompromised.
137
SHINGLES
| What is the clinical presentation for shingles?
Pre-eruptive...
- No skin lesions but burning itch in one dermatome.
Eruptive...
- Erythematous plaques that does not cross dermatomes, pain + paraesthesia.
138
SHINGLES
| What are the complications, investigations + treatment for shingles?
- Opthalmic branch of trigeminal nerve damaged it will affect sight.
- Clinical based on rash within dermatome.
- Antivirals like aciclovir, analgesia for pain.
139
DEMENTIA
| What is the epidemiology of dementia?
- Prevalence rises w/ age.
- Alzheimer's disease more common in females.
- Vascular + mixed more common in males.
140
DEMENTIA
| What are the 4 types of dementia?
- Alzheimer's disease (commonest).
- Vascular.
- Lewy-body.
- Fronto-temporal.
141
DEMENTIA
| What is the aetiology of Alzheimer's disease?
- Accumulation of beta-amyloid peptide resulting in degeneration of cerebral cortex with cortical atrophy, loss of acetylcholine.
- Temporal lobe affected.
- 25% develop Parkinsonism.
142
```
DEMENTIA
What is the aetiology of...
i) Vascular
ii) Lewy-body
iii) Fronto-temporal
```
dementia?
i) Cumulative effect of many small strokes.
ii) Characterised by Lewy-body deposition, associated with Parkinson's.
iii) Frontal + temporal lobe atrophy, associated with motor neurone disease.
143
DEMENTIA
| What is the clinical presentation of Alzheimer's disease?
- Insidious onset, short-term memory loss often first cognitive marker.
- Aphasia, agnosia (can't interpret sensory information), apraxia (difficult with motor planning).
144
DEMENTIA
| What is the clinical presentation of vascular dementia?
- Stepwise deterioration with short periods of stability, sudden onset.
145
DEMENTIA
| What is the clinical presentation of Lewy body dementia?
- Fluctuating congitive impairment.
- Detailed visual hallucinations.
- Parkinson's.
146
DEMENTIA
| What is the clinical presentation of fronto-temporal dementia?
- Behavioural/personality change.
| - Disinhibition.
147
DEMENTIA
| How can you differentiate between dementia and delirium?
- Dementia is insidious + progressive, delirium is acute + fluctuating.
- Dementa lasts months-years, delirium lasts hours-weeks.
- Dementia has normal consciousness, delirium doens't.
148
DEMENTIA
| What are the investigations for dementia?
- Clinical diagnosis.
- MMSE <17/30 = serious cognitive impairment.
- 6CIT = year? month? address? count 20-1? months in reverse? address?
- Alzheimer's shows plaques of amyloid + neuronal reduction.
149
DEMENTIA
| What is the treatment for dementia?
- Regular exercise, healthy diet, smoking/alcohol cessation.
- Acetylcholinesterase inhibitor (donepezil).
- BP control if vascular.
150
DEPRESSION
| What are the risk factors for depression?
- Genetic (family history).
- Death or loss.
- Conflict/abuse.
- Medical illness/substance abuse.
- Life events (positive or negative).
151
```
DEPRESSION
What are the...
i) Psychological
ii) Physical
iii) Social
```
symptoms of depression?
i) Continuous low mood/sadness, low self-esteem, feeling suicidal/tearful.
ii) Changes in appetite/weight, constipation, loss of libido.
iii) Not doing well at work, avoiding contact with friends.
152
DEPRESSION
| What are the investigations for depression?
```
PHQ-9, GAD-7 questionnaires.
DSM-IV criteria...
- Symptoms occurring ≥2w.
- Baseline mood change.
- Impaired function social/occupational/educational.
```
153
DEPRESSION
| What are the non-pharmacological treatments for depression?
- CBT (online).
- Mental health apps.
- Exercise.
- Counselling.
154
DEPRESSION
| What 2 drug classes are used for depression? Give examples.
- Selective serotonin reuptake inhibitors = citalopram.
| - Tricyclic antidepressants = amitriptyline.
155
DEPRESSION
| What is the mechanism of citalopram? What are the side effects?
- Preferentially inhibit neuronal reuptake of serotonin (5-HT) from synaptic cleft.
- GI disturbances, suicidal thoughts increased initially, prolong QT.
156
DEPRESSION
| What is the mechanism of amitriptyline? What are the side effects?
- Inhibit neuronal uptake of serotonin + noradrenaline from synaptic cleft + so increase availability for neurotransmission.
- QT prolongation, sexual dysfunction, sedation.
157
PRIMARY BRAIN TUMOURS
| What cell origin are the majority of primary brain tumours? What are benign brain tumours?
- Gliomas (glial cell in origin) like astrocytoma (most common) or oligodendroglioma.
- Benign tumours = meningiomas + neurofibromas (schwannomas).
158
PRIMARY BRAIN TUMOURS
| Describe the aetiology of malignant gliomas.
- Initial genetic error with mutation of IDH-1 resulting in genetic instability + inappropriate mitosis.
OR
- No IDH mutation, catastrophic genetic mutation.
159
PRIMARY BRAIN TUMOURS
| What is the WHO glioma grading?
- I = benign paediatric tumour (pilocytic astrocytoma).
- II = premalignant tumour (diffuse astrocytoma).
- III = anaplastic astrocytoma.
- IV = glioblastoma multiforme (GBE), all gliomas except grade I eventually lead to this very malignant cancer.
160
PRIMARY BRAIN TUMOURS
What is a medulloblastoma?
What is the advantage of the IDH-1 mutation?
- Cerebellum tumour.
| - Good prognostic factor, chemosensitive.
161
PRIMARY BRAIN TUMOURS
| What are the three cardinal signs of a primary brain tumour?
- Progressive focal neurological deficit.
- Raised intracranial pressure.
- Epilepsy (generalised or partial).
162
PRIMARY BRAIN TUMOURS
| Explain the mechanism behind progressive focal neurological deficit.
- Result of mass effect of tumour + surrounding cerebral oedema.
- Frontal = personality change, hemiparaesis, Broac's dysphasia.
- Temporal = dysphasia, amnesia.
- Parietal = hemisensory lsos, dysphasia.
- Occipital = contralateral visual defects.
- Cerebellum = DANISH.
163
PRIMARY BRAIN TUMOURS
| Explain the effects of raised intracranial pressure.
- Papilloedema.
- Headache (worst first thing in morning, coughing + bending forward).
- Nausea.
164
PRIMARY BRAIN TUMOURS
| What are the investigations for primary brain tumours?
- Histological.
- CT/MRI head.
- MR angiography.
165
PRIMARY BRAIN TUMOURS
| What is the treatment for primary brain tumours?
- Exploration, removal or biopsy.
- Meningiomas can be removed completely.
- Radiotherapy (all gliomas) + chemotherapy.
- Cerebral oedema reduced by corticosteroids.
166
SECONDARY BRAIN TUMOURS
| What's the pathophysiology of brain tumours?
Neoplasms which have metastasised to the CNS...
- Non-small cell carcinoma (most common).
- Breast.
- Malignant melanoma.
- RCC.
- GI.
167
SECONDARY BRAIN TUMOURS
| What's the clinical presentation of secondary brain tumours?
- Headache (often worse in morning, coughing, bending).
- Focal neurologcail signs.
- Ataxia.
- Fits, nausea, vomiting, papilloedema.
168
SECONDARY BRAIN TUMOURS
| What are the investigations + treatment for secondary brain tumours?
- CT/MRI chest, abdomen.
- Surgery + adjuvant radiotherapy, chemotherapy.
- Supportive care like dexamethasone to reduce cerebral oedema.
169
GIANT CELL ARTERITIS
| What is the pathophysiology of GCA?
- Chronic inflammation of the medium-large arteries, particularly the aorta + its extracranial branches.
170
GIANT CELL ARTERITIS
| What is the aetiology of GCA?
- Unknown but associated with polymyalgia rheumatica.
- Secondary to SLE, RA, HIV.
- Principally affects >50y/o, incidence increases with age.
171
GIANT CELL ARTERITIS
| What is the clinical presentation of GCA?
- Temporal artery + scalp tenderness.
- Tongue/jaw claudication.
- Headache.
- Superficial temporal artery firm + pulseless.
172
GIANT CELL ARTERITIS
| What are the complications with GCA?
- Blindness can occur due to inflammation + occlusion of the ciliary and/or central retinal artery = amaurosis fugax.
- Optic disc pale + swollen.
173
GIANT CELL ARTERITIS
| What are the investigations of GCA?
- Bloods, ESR/CRP raised (50/50 = over 50y/o, ESR over 50).
| - Temporal artery biopsy = diagnostic.
174
GIANT CELL ARTERITIS
| What is the treatment for GCA?
- Prompt corticosteroids + aspirin.
- PPI to prevent GI toxicity.
- Osteoporosis prophylaxis important (vitamin D, lifestyle advice).
175
SPINAL CORD COMPRESSION
| What is the pathophysiology of spinal cord compression?
- Myelopathy = compression of spinal cord resulting in UMN signs + specific symptoms based on compression.
176
SPINAL CORD COMPRESSION
| What is the aetiology of spinal cord compression?
- Osteophytes.
- Disc prolapse.
- Tumour.
177
SPINAL CORD COMPRESSION
| What is the clinical presentation of spinal cord compression?
- Progressive weakness of legs + UMN signs.
| - Sensory loss below level of lesion.
178
SPINAL CORD COMPRESSION
| What are the investigations + treatment for spinal cord compression?
- MRI spine instantly.
| - Surgical decompression + dexamethasone.
179
CAUDA EQUINA SYNDROME
| What is the pathophysiology + aetiology of cauda equina? What is radiculopathy?
- Nerve root compression caudal to termination of spinal cord at L1/2.
- Herniation of lumbar disc (commonly L4/5, L5/S1), tumour or trauma.
- Radiculopathy = spinal nerve root disease.
180
CAUDA EQUINA SYNDROME
| What is the clinical presentation of cauda equina?
- LMN SIGNS.
- Bilateral sciatica = sensory loss/pain in back of thigh/leg + lateral aspect of little toe.
- Bladder/bowel dysfunction.
- Saddle anaesthesia (bum).
- Erectile dysfunction.
181
CAUDA EQUINA SYNDROME
| What are the investigations + treatment for cauda equina?
- MRI spinal cord to localise lesion.
| - Surgery for emergency pressure relief, conservative management.
182
CARPAL TUNNEL SYNDROME
| What is the pathophysiology + aetiology of carpal tunnel syndrome?
- Inflammation of carpal tunnel leading to entrapment of median nerve + so pain + loss of sensation.
- Often idiopathic, associated with hypothyroidism, DM + RA.
183
CARPAL TUNNEL SYNDROME
| What is the clinical presentation of carpal tunnel syndrome?
- Aching pain in hand + arm (esp. night).
- Paraesthesia in thumb, index + middle fingers relieved by dangling hand over edge of bed + shaking (WAKE + SHAKE).
- ?wasting of thenar eminence.
184
CARPAL TUNNEL SYNDROME
| What are the investigations + treatment for carpal tunnel syndrome?
- Neurophysiological exam (nerve conduction studies).
- Phalen's test = maximally flex wrist for 1 min, Tinel's test = tapping on nerve at wrist induces tingling.
- Splinting, local steroid injection ± decompression surgery.
185
```
PERIPHERAL NEUROPATHIES
Define...
i) Mononeuropathy.
ii) Polyneuropathy.
iii) Demyelinating.
iv) Axonal.
```
i) 1 nerve involved.
ii) Multiple/systemic nerves involved, diabetes, MS, GB.
iii) Slow conduction velocities.
iv) Reduced amplitudes of potentials.
186
PERIPHERAL NEUROPATHIES
| What is the aetiology of peripheral neuropathies?
DAVID...
- Diabetes.
- Alcohol.
- Vitamin deficiency (B12).
- Infective.
- Drugs.
187
PERIPHERAL NEUROPATHIES
| Explain the pathophysiology of a median nerve mononeuropathy.
- Nerve of precision grip (LLOAF) = 2 lumbricals, opponens pollicis, abductor/flexor pollicis brevis.
- Sensory loss of radial 3.5 fingers + palm.
188
PERIPHERAL NEUROPATHIES
| Explain the pathophysiology of a ulnar nerve mononeuropathy.
- Elbow trauma.
- Weakness/wasting of interossei (good luck sign).
- Medial lumbricals (claw hand).
- Hypothenar eminence.
189
PERIPHERAL NEUROPATHIES
| Explain the pathophysiology of a radial nerve mononeuropathy.
- Compression against humerus, nerve opens fist.
| - Test for wrist + finger drop.
190
PERIPHERAL NEUROPATHIES
| Explain the pathophysiology of a brachial plexus mononeuropathy.
- Trauma/radiotherapy/heavy rucksack.
| - Pain/paraesthesia + weakness in affected arm.
191
PERIPHERAL NEUROPATHIES
| Explain the pathophysiology of a phrenic nerve mononeuropathy.
- Cancer/myeloma/thymoma.
| - Orthopnoea with raised hemidiaphragm on CXR.
192
PERIPHERAL NEUROPATHIES
| Explain the pathophysiology of a lateral cutaneous nerve of thigh mononeuropathy.
- Meralgia paraesthetica = Antero-lateral burning thigh pain from entrapment under inguinal ligament.
193
PERIPHERAL NEUROPATHIES
| Explain the pathophysiology of a scaitic nerve mononeuropathy.
- Pelvic tumour/fracture.
| - Sensory loss below knee laterally, foot drop.
194
PERIPHERAL NEUROPATHIES
| Explain the pathophysiology of a common peroneal nerve mononeuropathy.
- Cross-legged, trauma.
| - Foot drop, weak ankle dorsiflexion/eversion, sensory loss over dorsal foot.
195
PERIPHERAL NEUROPATHIES
| Explain the pathophysiology of a tibial nerve mononeuropathy.
- Inability to tiptoe (plantarflexion), invert foot, flex toes, sensory loss over sole.
196
PERIPHERAL NEUROPATHIES
| Explain the pathophysiology of an olfactory nerve mononeuropathy.
- Anosmia (lost sense of smell).
197
PERIPHERAL NEUROPATHIES
| Explain the pathophysiology of an optic nerve mononeuropathy.
- L optic nerve lesion = no vision through left eye.
- Optic chiasma lesion = bitemporal hemianopia.
- L optic tract lesion = contralateral (right) homonymous hemianopia.
- L Baum's (parietal) loop = inferior right homonymous quadrantanopia.
- L Meyer's (temporal) loop = superior right homonymous quadrantanopia.
198
PERIPHERAL NEUROPATHIES
| Explain the pathophysiology of an oculomotor nerve mononeuropathy.
- Tramps palsy = eye down + out.
- Ptsosis.
- Fixed dilated pupil = loss of parasymp outflow.
199
PERIPHERAL NEUROPATHIES
| Explain the pathophysiology of a facial nerve mononeuropathy.
- Post-viral.
Bell's palsy, muscles of facial expression...
- Diff from stroke as NO forehead sparring.
Tx = steroids.
200
PERIPHERAL NEUROPATHIES
| Explain the pathophysiology of a glossopharyngeal/vagus nerve mononeuropathy.
- Swallow, gag, cough issues.
| - Uvula deviated AWAY from side of lesion.
201
PERIPHERAL NEUROPATHIES
| Explain the pathophysiology of a hypoglassal nerve mononeuropathy.
- Tongue deviates TOWARDS side of lesion.
202
PERIPHERAL NEUROPATHIES
| What are the investigations for peripheral neuropathies?
- Clinical examination, nerve conduction studies, MRI.
| - Conservative management.
203
MYASTHENIA GRAVIS
| What is the pathophysiology of myasthenia gravis?
- Autoimmune disorder against nicotinic acetylcholine receptors (AChR) in the neuromuscular junction where anti-AChR antibodies (IgG) interfere with the neuromuscular junction via depletion of working post-synaptic receptor sites.
- This leads to fewer action potentials firing + so blocks the excitatory effect of ACh on nicotininc receptors – all or nothing principle.
- Both T + B cells implicated.
204
MYASTHENIA GRAVIS
| What is the aetiology of myasthenia gravis?
- Associated with autoimmune disease, esp. RA + SLE.
- If <50y/o commoner in females + associated w/ thymic hyperplasia.
- If >50y/o commoner in males + associated with thymic atrophy/tumour.
205
MYASTHENIA GRAVIS
| What muscle groups are affected in order?
- Extra-ocular > bulbar (swallowing, chewing) > face > neck > trunk.
206
MYASTHENIA GRAVIS
| What are the symptoms of myasthenia gravis?
- Slowly increasing muscle fatigue + weakness, improves after rest.
- Weakness exacerbated by pregnancy, infection, emotion + exercise.
207
MYASTHENIA GRAVIS
| What are the signs of myasthenia gravis?
- Ptosis, diplopia (extra-ocular).
| - Dysphasia, dysarthria (bulbar).
208
MYASTHENIA GRAVIS
| What are the investigations for myasthenia gravis?
Serum antibodies...
- Increased anti-AChR.
- Muscle-specific tyrosine kinase (MuSK), esp. males.
Electromyogram (EMG) shows decremental muscle response to repetitive nerve stimulation.
Count to 50 – voice fades.
CT chest to exclude thymoma.
209
MYASTHENIA GRAVIS
| What is the treatment for myasthenia gravis?
```
Symptom control...
- Anticholinesterase (pyridostigmine).
Immunosuppression...
- Treat relapses with prednisolone.
Thymectomy.
```
210
HUNTINGTON'S DISEASE
| What is the pathophysiology of HD?
- Presence of mutant Huntingtin protein which causes loss of neurones in the striatum (caudate nucleus + putamen) of basal ganglia causing depletion of GABA (inhibitory neurotransmitter) + acetylcholine.
- Dopamine is sparred.
211
HUNTINGTON'S DISEASE
| What is the impact of GABA depletion?
- Less regulation of dopamine to striatum causing increased movements.
212
HUNTINGTON'S DISEASE
| What is the aetiology of HD?
- Autosomal dominant inheritance.
- CAG repeart in Huntingtin protein gene on chromosome 4.
- >35 CAG repeats = HD.
- Number of repeats = indicative of age of onset.
- Presents middle age.
213
HUNTINGTON'S DISEASE
| What is the clinical presentation of HD?
```
Early signs...
- Irritability + depression.
- Personality change.
Later...
- Chorea (fidgety).
- Dementia.
- Psychiatric problems
```
214
HUNTINGTON'S DISEASE
| What are the investigations for HD?
- Genetic diagnosis.
| - MRI brain shows atrophy of striatum.
215
HUNTINGTON'S DISEASE
| What is the treatment for HD?
- Treat chorea w/ benzodiazepines, sodium valporate.
- Treat depression with SSRI (citalopram).
- Treat aggressive behaviour with antipsychotics like risperidone.
- Genetic counselling.
216
GUILLAIN-BARRÉ SYNDROME
| What is the pathophysiology of Guillain-Barré syndrome?
- Acute inflammatory demyelinating polyneuropathy, Schwann cells targeted.
- Demyelination + axonal degeneration due to a trigger causing antibodies to attack nerves.
- Causes ascending + progressive neuropathy.
217
GUILLAIN-BARRÉ SYNDROME
| What is the aetiology of Guillain-Barré syndrome?
Often post-infection...
- Campylobacter jejuni.
- CMV.
- EBV.
218
GUILLAIN-BARRÉ SYNDROME
| What is the clinical presentation of Guillain-Barré syndrome?
- Progressive symmetrical ascending muscle weakness a few weeks post-infection.
- Pain.
- Proximal muscles more affected like trunk, respiratory, cranial nerves.
- Sweating, tachycardia, BP changes.
219
GUILLAIN-BARRÉ SYNDROME
| What are the investigations for Guillain-Barré syndrome?
- Nerve conduction studies (slow).
- Lumbar puncture (raised protein, WCC normal).
- Monitor FVC for respiratory involvement.
220
GUILLAIN-BARRÉ SYNDROME
| What is the treatment for Guillain-Barré syndrome?
- IV immunoglobulin.
| - Ventilation if respiratory muscles involved.
221
ANTI-EPILEPTIC DRUGS
| Name 2 anti-epileptic drugs + explain their mechanisms.
- Carbamazepine – inhibits pre-synaptic Na+ channels + so prevents axonal firing.
- Sodium valporate – teratogenic.
222
ANTI-EPILEPTIC DRUGS
| What are the side effects of anti-epileptic drugs?
- Cognitive disturbances (blurred vision, diplopia).
- Drowsiness.
- Photosensitivity.
