What is the first-line treatment for trigeminal neuralgia?
Carbamazepine
Which drugs are used in the treatment of an acute migraine?
Paracetamol
NSAIDs e.g. ibuprofen or aspirin
Triptans e.g. sumatriptan
Which drugs are used in the prevention of migraine?
Propranolol Topiramate Acupuncture Gabapentin Riboflavin
What investigations should you do in suspected subarachnoid haemorrhage?
CT head
If CT head normal - do lumbar puncture 12 hours after onset of symptoms
Why should you wait at least 12 hours from the onset of symptoms in subarachnoid haemorrhage to do a lumbar puncture?
Takes this time for xanthechromia to develop
What is the cause of 80% of subarachnoid haemorrhages?
Ruptured Berry aneurysm
Which drugs are appropriate for treating generalised seizures?
Sodium valproate
Lamotrigine
Which drugs are appropriate for treating partial seizures?
Carbamazepine
Sodium valproate
Lamotrigine
What are the Parkinson’s Plus Syndromes?
Multisystem Atrophy
Progressive Supranuclear Palsy
Corticobasal Degeneration
Drug-Induced Parkinson’s
How would you test for progressive nuclear palsy in a Parkinson’s exam?
Do vertical gaze as patients with PSP have vertical gaze palsy
How might you test to see if a patient with Parkinsonism had Multi System Atrophy?
Lying / standing BP - Postural hypotension due to autonomic features
Urinary symptoms e.g. incontinence
Cerebellar exam may reveal features
What kind of drug is apomorphine?
Dopamine agonist
What kind of drug is Selegiline?
Monoamine oxidase inhibitor
What is the treatment for acute relapses of multiple sclerosis?
IV methylprednisolone
What is the chronic treatment of multiple sclerosis?
- Interferon beta
- Monoclonal antibodies e.g. Natalizumab
- Symptomatic relief e.g. Baclofen, gabapentin, oxybutinin
Which drugs can cause cerebellar syndrome?
Phenytoin
Carbamazepine
Lithium
What is myasthenia gravis?
Autoimmune condition - There are antibodies to the post-synaptic acetylcholine receptor so less receptors are available for the neurotransmitter. Causes problems with neuromuscular transmission.
What investigations would you do in myasthenia gravis?
Vital capacity
Anti-acetylcholine receptor antibodies (specific to myasthenia gravis)
Neurophysiology - Reduced muscle response on repetitive stimulation
CT thymus (70% associated with thymic hyperplasia)
What is the treatment for myasthenia gravis?
Anticholinesterase inhibitor e.g. Pyridostigmine
Immunosuppression - Prednisolone
Thymectomy
Expressive dysphasia is a problem with which part of the brain?
Broca’s area - Dominant frontal lobe
Receptive dysphasia is a problem with which part of the brain?
Wernicke’s area - Dominant temporal lobe
What is expressive dysphasia?
A problem with Broca’s area. The patient has normal understanding but reduced verbal fluency so they cannot express what they mean to say.
What is receptive dysphasia?
A problem with Wernicke’s area. Patient has fluent speech but it does not make sense as they frequently use the wrong word. Understanding is also impaired.
How might you test for expressive dysphasia?
Ask the patient to write a sentence. In expressive dysphasia, writing is also impaired.
How might you test for receptive dysphasia?
Ask the patient to follow a 3-stage command.
What connects the VIth and IIIrd nerve nuclei to allow conjugate gaze?
Medial longitudinal bundle
What would you observe if there is a lesion at the right longitudinal bundle in a patient when you ask them to look left?
- Failure of adduction on the right side
- Nystagmus on the left side, away from the lesion
What might cause a disruption of the medial longitudinal bundle, thus causing failure of conjugate gaze?
Demyelinating lesion e.g. in MS
Give some causes of proximal myopathy
Hypothyroid Metabolic abnormalities e.g. K+ low Inherited e.g. muscular dystrophy Myasthenia gravis Steroids Alcohol Myositis
When is the ABCD2 score used?
Calculated following a TIA to predict the patient’s risk of stroke
What is measured in the ABCD2 score?
Age over 60 = 1 point BP over 140/90 = 1 point Clinical features: - Unilateral weakness = 2 points - Speech disturbance, no weakness = 1 point Diabetes = 1 point Duration of symptoms - More than 1 hour = 2 points - 10 mins to 1 hour = 1 point
How is a patient with ABCD2 score 4 or more managed following a TIA?
300mg aspirin STAT
Refer to TIA clinic for assessment within 24 hours
Offer secondary prevention
How is a patient with an ABCD2 score less than 3 managed?
300mg aspirin STAT
Refer to TIA clinic for assessmeng within 1 week
What are the symptoms of a cerebellar lesion?
Remember DANISH - Dysdiadochokinesis - Ataxia - Nystagmus - Intention tremor - Slurred / staccato speech - Hypotonia (Also pendulum reflexes)
What are the causes of a cerebellar lesion?
Remember PASTRIES
- Posterior fossa tumour
- Alcohol
- Multiple Sclerosis
- Trauma
- Rare e.g. Friedrich’s ataxia
- Inherited
- Epilepsy medication e.g. carbamazepine, phenytoin, lithium
- Stroke
What is a crescendo TIA?
2 or more TIAs within 1 week
How is a crescendo TIA managed?
Same as for patients with ABCD2 score above 4…
- 300mg aspirin STAT
- Review in TIA clinic within 24 hours
- Secondary prevention
In a cerebellar lesion, does nystagmus go towards the lesion or away from the lesion?
Towards
When might carotid endarterectomy be considered following TIA?
If stenosis more than 70%
What is the antiplatelet of choice following TIA?
Clopidogrel following 2/52 of aspirin
Aspirin and dipyridamole if patient can’t tolerate clopidogrel
What is the further management of a TIA (after initial treatment)?
- Control lifestyle factors e.g. smoking, exercise
- Antiplatelets: Clopidogrel 1st line
- Antihypertensives
- Statin: Atorvastatin 20-80mg
- Anticoagulate if in AF
- Consider for carotid endarterectomy
What is the management of a haemorrhagic stroke?
- Aggressive control of BP with labetolol IV or GTN infusion
- Stop anticoagulation
- Discuss with neurosurgeons
What are the features of TACS (total anterior circulation syndrome)?
- Contralateral hemiparesis
- Contralateral homonymous hemianopia
- At least one additional feature of cortical disturbance: Dysphasia, inattention, dyspraxia
What are the features of PACS (partial anterior circulation syndrome)?
2 features of a TACI: Includes hemiparesis and one additional cortical feature e.g. sensory loss
Deficit is often incomplete e.g. affects arm and leg, or face and arm
What are the features of a lacunar stroke?
Pure motor or pure sensory changes
Might be pure ataxia
Which artery is affected in lateral medullary syndrome?
Posterior inferior cerebellar artery
What features might you expect in lateral medullary syndrome?
Infarct of posterior inferior cerebellar artery gives the following:
- Ipsilateral facial features: Nystagmus, Horner’s, facial numbness, cranial nerve palsies
- Ataxia, vertigo, dysphasia
- Contralateral body features: Sensory loss
What might be the appearance of an ischaemic stroke on a non-contrast CT scan?
- Loss of grey/white matter differentiation in the territory of the affected artery
- Might see ‘Dense MCA sign’ i.e. a linear opacity indicating a clot in the middle cerebral artery
Which brain imaging is the option of choice in suspected stroke?
CT angiogram
What is the time window for thrombolysis in ischaemic stroke?
Onset of symptoms within 4.5 hours and no other contraindications
How long is aspirin given for in acute stroke, and at what dose?
2 weeks of 300mg aspirin then switch to longer term anti-platelets
Which patients might be considered for thrombectomy in acute ischaemic stroke?
Onset of symptoms within 6.5 hours, and NIHSS (National Institute of Health Stroke Scale) score over 6
Give 3 conditions associated with Berry aneurysms
Ehler’s Danlos syndrome
Autosomal Dominant Polycystic Kidney Disease
Coarctation of the aorta
What is the management of subarachnoid headache?
DR ABCDE approach
- Monitor GCS
- Keep hydrated
- Nimodipine…reduces vasospasm and ischaemic complications
- Neurosurgical input e.g. endovascular coiling
Is a subdural haematoma formed of venous blood or arterial?
Venous
Is an extradural haematoma formed of venous blood or arterial?
Arterial
Which artery is ruptured in an extradural haematoma?
Middle meningeal artery
What is the management of an extradural haematoma?
Neurosurgical evacuation of the clot, and ligation of the bleeding vessel
What is Guillian Barre Syndrome?
An acute, inflammatory, demyelinating polyneuropathy, triggered by an infection. It is thought the infection stimulates production of antibodies which attach nerves, as the infection shares a similar antigen to the peripheral nerves.
Give 3 infections associated with Guillian Barre Syndrome
Campylobacter
EBV
CMV
Give some features of Guillian Barre Syndrome
- Progressive ascending symmetrical limb weakness…ascending (legs affected first) but proximal muscles tend to be affected first
- Back pain
- Sensory examination usually normal
- Autonomic features: Urinary retention
What is the treatment for Guillian Barre Syndrome?
IV immunoglobulin for 5 days - Reduces duration and severity of paralysis
Plasma exchange
Give 3 features of normal pressure hydrocephalus
Gait abnormality (similar to Parkinson’s)
Urinary incontinence
Dementia
What is the treatment for normal pressure hydrocephalus?
Ventriculoperitoneal shunt
List some causes of peripheral neuropathy
Remember ABCDDO:
- Alcohol
- B12 deficiency
- Charcot Marie Tooth, CKD
- Diabetes
- Drugs: Isoniazid, vincristine, cisplatin, nitrofurantoin
- Others: Hypothyroid, amyloid, Guillian Barre, sarcoidosis, vasculitides, Lyme disease, HIV
What is Charcot Marie Tooth also know as?
Hereditory sensory motor neuropathy
List some features of hereditory sensory motor neuropathy (Charcot Marie Tooth)
- Sensory loss in glove and stocking distribution
- Wasting of leg muscles: Inverted champagne bottle appearance
- Bilateral foot drop
- Pes cavus
- Ankle jerks absent
Which nerves are affected in a cerebellopontine angle syndrome?
Vth, VIIth, VIIIth
Also cerebellum
List some features of cerebellopontine angle syndrome
- Normal eye movements but nystagmus
- Loss of sensation on ipsilateral trigeminal distribution
- LMN facial weakness
- Sensorineural deafness
- Ataxia
List some causes of cerebellopontine angle syndrome
Acoustic neuroma
Schwannomas of Vth or VIth nerve
Meningioma
What is the inheritance pattern of neurofibromatosis?
Autosomal dominant
List some features of neurofibromatosis type 1
- Freckling in axilla or inguinal folds
- Cafe au lait macules
- Neurofibroma i.e. benign nodules which may be papillomatous and itchy
- Lisch nodules on iris
- Optic glioma
List some features of neurofibromatosis type 2
- Bilateral vestibular Schwannomas (acoustic neuroma)
- Juvenile posterior subcapsular lenticular opacity (a form of cataract)
- Other tumours e.g. meningioma, glioma
Which blood marker is raised in Duchenne muscular dystrophy?
CK
What is the inheritance pattern of Duchenne and Becker muscular dystrophies?
X-linked recessive
What is the inheritance pattern of myotonic dystrophy?
Autosomal dominant
Give some features of myotonic dystrophy
Weakness - Distal first then proximal Cataracts, ptosis Male baldness Cardiomyopathy Diabetes Testicular atrophy Dysphagia
True / False: Multiple sclerosis involves peripheral nerves
False
Which part of the CNS does multiple sclerosis affect?
Optic nerve
Periventricular white matter
Brainstem and cerebellar connections
Cervical spinal cord
A headache worst on lying down, in the morning, bending forward, coughing, is reflective of what?
Raised intracranial pressure
Painful Horner’s syndrome might be a sign of what underlying diagnosis?
Carotid artery dissection
Describe the features of trigeminal neuralgia
Severe, unilateral pain in the distribution of one or more branch of trigeminal nerve
Intermittent ‘shock-like’ pain
Triggered by touching e.g. brushing teeth
What is the treatment of trigeminal neuralgia?
1st line = Carbamazepine
Other agents = Lamotrigine, phenytoin, gabapentin
What is the acute management of a cluster headache?
100% oxygen
Subcutaneous or nasal sumatriptan
List some clinical features of motor neurone disease
[Depends what kind, but in general…]
Stumbling gait
Weak grip due to wasting of small muscles in hand
Fasciculation
Spasticity
Upgoing plantars, exaggerated reflexes
No cerebellar, no sensory, no eye signs, no sphincter involvement
What are the 4 types of motor neurone disease?
Progressive motor atrophy
Amyotrophic lateral sclerosis
Progressive bulbar / pseudobulbar palsy
Progressive lateral sclerosis
What is the most common type of motor neurone disease?
Amyotrohic lateral sclerosis
Which type of motor neurone disease affect predominantly cranial nerves?
Progressive bulbar / pseudobulbar palsy
Which type of motor neurone disease shows predominantly UMN features?
Progressive lateral sclerosis
Which typie of motor neurone disease shows predominantly LMN features?
Progressive motor atrophy
Which drug might prolong life in motor neurone disease?
Riluzole - An anti-glutamatergic drug
What is multiple sclerosis?
Chronic, autoimmune inflammatory disorder of the central nervous system, characterised by plaques of demyelination in the brain and spinal cord.
List some clinical features of multiple sclerosis at presentation
Optic neuritis
Spinal cord syndrome: Limb paraesthesia (with sensory level), sphincter disturbance, brisk reflexes, increased tone
Cerebellar signs e.g. ataxia
Brainstem signs e.g. diplopia, dysarthria
What criteria is used to diagnose MS?
McDonald criteria
What investigations might be used for diagnosing MS, and what might they show?
MRI: Hyperintense T2 lesions indicating demyelination
CSF: Oligoclonal bands of IgG on electrophoresis which are absent in the serum
Which nerve is damaged in winging of the scapula? What are the nerve roots?
Serratous anterior muscle supplied by the long thoracic nerve (C5, C6, C7)
What are the nerve roots of the median nerve?
C5, C6, C7, C8, T1
Which nerve is damaged in carpal tunnel syndrome?
Median nerve
List 2 ways in which the median nerve may become damaged
Compression i.e. in carpal tunnel syndrome
Supracondylar humeral fractures
What is the ‘Hand of Benediction’ and what is it a sign of?
Inability to flex the 1st and 2nd fingers due to damage to the median nerve
What is Tinel’s sign?
Tapping over carpal tunnel which might reproduce symptoms in carpal tunnel syndrome
What is Phalen’s sign?
Enforced wrist flexion for 1 minute which reproduces symptoms of carpal tunnel syndrome
What is the sensory supply of the median nerve?
Palmar aspect: Thumb and lateral 2 and a half fingers, lateral 2/3 of palm, thenar eminence
Dorsal aspect: Fingertips of 2nd and 3rd fingers
Which nerve supplies the LOAF muscles of the hand? Which muscles are these?
Median nerve
- Lateral 2 lumbricals
- Opponens pollicis
- Abductor pollicis brevis
- Flexor pollicis brevis
List some clinical features of a median nerve palsy
Wasting of the thenar eminence
Loss of sensation over the lateral 2/3 of the palm, the thenar eminence, the palmar aspects of the thumb and first 2 and a 1/2 fingers, and the dorsal aspect of the the first 2 and a 1/2 fingertips
‘Hand of Benediction’ i.e. unable to flex the 1st and 2nd fingers
What are the causes of median nerve entrapment?
Remember MEDIAN TRAPS
- Myxoedema
- Enforced flexion e.g. Colles splint
- Diabetic neuropathy
- Idiopathic
- Acromegaly
- Neoplasms
- Tumours (benign)
- Rheumatoid arthritis
- Amyloidosis
- Pregnancy
- Sarcoidosis
Which 2 nerves are supplied by all of the nerve roots of the brachial plexus?
Median Nerve
Radial Nerve
Which nerve roots supply the radial nerve?
C5, C6, C7, C8, T1
How do you test the radial nerve?
Wrist extension
Give some common mechanisms of injury to the radial nerve
Use of crutches
Fracture of proximal humerus or radius
Falling asleep with arm slung over chair
What is the sensory supply of the radial nerve?
Posterior arm and forearm
Lateral 2/3 of the dorsum of hand, including the thumb and first 2 and a half fingers (excluding fingertips)
List some features of a radial nerve palsy
Wasting of the posterior compartment of forearm
Wrist drop
Weak elbow extension
Sensory loss over lateral aspect of dorsum of hand
Absent triceps and supinator reflexes
What might be the cause of an ulnar nerve palsy?
Elbow injury e.g. supracondylar fracture
What nerve roots supply the ulnar nerve?
C8, T1
What is the sensory supply of the ulnar nerve?
Hypothenar eminence
Medial 1/3 hand - palmar and dorsal aspect
Medial 1 and a half fingers - palmar and dorsal aspect
How do you test ulnar nerve function?
Ask patient to grip a piece of paper between their thumb and 1st finger. Ulnar nerve palsy will cause weakness of the adductor pollicis muscle resulting in Froment’s sign (flexion of the IP joint of the thumb)
Give some features of an ulnar nerve palsy
Wasting of the hypothenar eminence
Loss of sensation over the medial 1/3 of the hand and the medial 1 1/2 fingers
Weak thumb adduction (weak pincer grip - Froment’s sign)
Partial claw hand - Flexion of the PIPJ of 4th and 5th fingers
What is epilepsy?
The recurrent tendency to spontaneous, intermittent, abnormal electrical activity in the brain, manifesting as seizures
What are the types of generalised seizure?
Tonic-clonic
Absence
Myoclonic
Atonic
List some causes of seizures
Primary epilepsy syndromes
Metabolic disturbances: Hypoglycaemia, hyponatraemia
Drugs: Alcohol, penicillins
Cerebrovascular disease
Space occupying lesion
Intracranial infection: Meningitis, encephalitis, syphilis
Hypoxia
Which drugs are used for generalised seizures?
Sodium valproate
Lamotrigine
Which drugs are used for partial seizures?
Carbamazepine
Sodium valproate
Lamotrigine
What are the side effects of sodium valproate?
Weight gain Liver dysfunction Pancreatitis Hair loss (reversible) Oedema Ataxia Teratogenicity Thrombocytopenia Encephalopathy Inhibitor of cytochrome p450 enzymesList
Which anti-convulsant medication might interact with warfarin and the COCP?
Carbamazepine - It is a cytochome p450 inducer
Which anti-convulsant is most safe for women of child bearing age?
Lamotrigine
What must pregnant women with epilepsy ensure they take?
High dose folic acid for first 12 weeks of pregnancy
List some key side effects of carbamazepine
Cytochrome p450 inducer Dizziness Leucopenia, agranulocytosis SIADH Teratogenicity Double vision
List some key side effects of lamotrigine
Steven Johnson’s Syndrome
What effect does phenytoin have on cytochrome p450 enzymes?
Phenytoin is a cytochrome p450 inducer
What is the initial drug given in a patient with status epilepticus?
IV lorazepam 4mg (assuming IV access obtained)
What should be given to a patient with status epilepticus who is still seizing after 2 doses of lorazepam?
Phenytoin infusion 15-20mg/kg at a rate of less than 50mg per minute
What are the features of Wernicke’s encephalopathy?
Ataxia
Ophthalmoplegia
Nystagmus
Confusion