Neurology Flashcards
Presentation of a tension headache
Mild ache / pressure in a band like pattern around the head
Management of tension headache
- Reassurance
- Simple analgesia (e.g., ibuprofen or paracetamol)
4 associations with tension headahces
Stress
Depression
Alcohol
Skipping meals
Dehydration
Presentation of sinusitis and it’s management
- Recent URTI
- Tenderness and swelling on palpation
- Prolonged cases (>10 days) = steroid nasal spray or Abx (phenoxymethylpenicillin)
Presentation of cervical spondylosis
- Neck pain, worse with movements
- Headahces
Presentation of trigeminal neuralgia and 1st line management ?
- Electric shock like pain
- Triggered by touch, talking, eating, shaving or cold
- First line = carbamazepine
Presentation of a cluster headache
- Unilateral severe pain
- Red, swollen and watering eye
- Miosis
- Ptosis
- Nasal discharge
- Facial sweating
Management of acute cluster headache attacke
-> Triptans (e.g., subcutaneous or intranasal sumatriptan)
-> High-flow 100% oxygen (may be kept at home)
1st line prophylaxis of cluster headaches
Verapamil
Typical symptoms of a migraine
Last between 4 and 72 hrs
- Usually unilateral
- Moderate-severe intensity
- Pounding or throbbing in nature
- Photophobia (discomfort with lights)
- Phonophobia (discomfort with loud noises)
- Osmophobia (discomfort with strong smells)
- Aura (visual changes)
- N&V
common migraine triggers
- Stress
- Bright lights
- Strong smells
- Certain foods (e.g., chocolate, cheese and caffeine)
- Dehydration
- Menstruation
- Disrupted sleep
- Trauma
Presentation of hemiplegic migraine
- Hemiplegia
- Ataxia
- Impaired consciousness
Acute management of migraine
- NSAIDs (e.g., ibuprofen or naproxen)
- Paracetamol
- Triptans (e.g., sumatriptan)
- Antiemetics if vomiting occurs (e.g., metoclopramide or prochlorperazine)
How do triptans work >
-> 5-HT receptor agonist, binding to serotonin receptors.
- > Cranial vasoconstriction, inhibit transmission of pain signals and inhibit release of inflammatory neuropeptides
3 prophylactic options for migraines
- Propranolol (a non-selective beta blocker)
- Amitriptyline (a tricyclic antidepressant)
- Topiramate (teratogenic and very effective contraception is needed)
Prophylaxis of menstrual migraines
Prophylactic triptans (e.g., frovatriptan or zolmitriptan)
definition of tuberous sclerosis and mutations involved
- AD condition causing development of hamartomas
- TSC1 gene on chromosome 9, which codes for hamartin
- TSC2 gene on chromosome 16, which codes for tuberin
- Leads to abnormal cell size and growth
skin features of tuberous sclerosis
-> Ash leaf spots (depigmented areas of skin shaped like an ash leaf)
-> Shagreen patches (thickened, dimpled, pigmented patches of skin)
-> Angiofibromas (small skin-coloured or pigmented papules that occur over the nose and cheeks)
-> Ungual fibromas (circular painless lumps that slowly grow from the nail bed and displace the nail)
-> Cafe-au-lait spots (light brown “coffee and milk” coloured flat pigmented lesions on the skin)
-> Poliosis (an isolated patch of white hair on the head, eyebrows, eyelashes or beard)
Neurological features of tuberous sclerosis
Epilepsy
Learning disability
Brain tumours - glioma
Define MS
- Autoimmune demyelination of the CNS
What cells produce myelin in the CNS and in the PNS ?
-CNS : oligodendrocytes
-PNS : schwann
How do the episodes of demyelination present in MS?
-Episodes disseminated in time and space
What is optic neuritis ?
-Unilateral painful reduced vision developing over hrs to days.
Give 4 key features of optic neuritis
-Central scotoma (blind spot)
-Pain on eye movement
-Impaired colour vision
-Relative afferent pupillary defect
Give 7 other causes of optic neuritis other than MS
-Sarcoidosis
-SLE
-DM
-Syphilis
-Measles
-Mumps
-Lyme disease
What investigations can support a diagnosis of MS
- MRI with contrast -> demyelinating lesions
- LP -> oligoclonal bans in CSF
- McDonald criteria for diagnosing
Give the 4 disease patterns of MS
-Clinically isolated syndrome
-Relapsing-Remitting
-Secondary progressive
-Primary progressive
Explain the relapsing-remitting pattern of MS
-Episodes of disease followed by recovery. Classified on whether active and/or worsening
-Active : new symptoms developing
-Not active : no new symptoms developing
-Worsening : overall worsening of disability over time
-Not worsening : no worsening of disability over time
Explain secondary progressive pattern of MS
-Was relapsing/remitting but now a progressive worsening of symptoms
-Can also be classified as active and/or worsening
Explain primary progressive pattern of MS
-Worsening of disease and neurological symptoms from the point of diagnosis without initial relapses and remissions
Give two common characteristics of MS
-Lhermitte’s sign : tingling electric shock shooting up the spine when flexing the neck
-Uhthoff’s phenomenon : symptoms are worse when hpt (e.g. in bath, hot weather, exercise).
What can be used to treat relapses in MS?
-Methylprednisolone
-500mg orally for 5 days or 1g IV for 3-5 days if treatment fails
What is the pathophysiology behind MND?
-Progressive degeneration of both upper and lower motor neurones
-Sensory neurones are spared
What can increase the risk of MND?
-Smoking
-Exposure to heavy metals
-Certain persticides
What is a typical presentation of MND
- Late middle aged man
- Insidious, progressive weakness of the muscles throughout the body affecting the limbs, trunk, face and speech
- Weakness often first noticed in upper limbs
- Fatigue when exercising
Give 4 signs of lower MND
-Muscle wasting
-Reduced tone
-Fasciculations
-Reduced reflexes
GIve 3 signs of upper MND
-Increased tone or spasticity
-Brisk reflexes
-Upgoing plantar responses
What is the most common presentation of MND ?
-Amyotrophic lateral sclerosis (ALS)
- Typically LMN signs in the arms and UMN signs in the legs
What can be used to slow the progression of MND ?
-Riluzole : prevents stimulation of glutamate receptors
Signs seen in primary lateral sclerosis (MND)
UMN signs only
Signs seen in progressive muscular atrophy (MND)
LMN signs only
affects distal muscles before proximal
carries best prognosis
Signs seen in progressive bulbar palsy
- Palsy of the tongue, muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei
- Carries worst prognosis
What is myasthenia Gravis ?
-Autoimmune condition that causes muscle weakness that gets progressively worse with activity and improves with rest
- Affects the NMJ
What is strongly associated with myasthenia Gravis?
-Thyoma : tumour of the thymus gland
Explain the pathophysiology behind myasthenia gravis
-In most cases, acetylcholine receptor antibodies are produced
-These bind to the postsynaptic receptors
-This prevents the stimulation of the receptor and so prevents muscle contraction
-The antibodies also activate the complement system, leading to damage to the cells of the postsynaptic membrane further worsening the symptoms
Why do the symptoms of myasthenia gravis get worse with exercise ?
-The receptors are used more during muscle activity and so more of them become blocked up.
-This leads to less effective stimulation of the muscle with increased activity
-There is more muscle weakness the more the muscles are used
-This improves with rest as more receptors are freed up again for use
What are 2 other antibodies involved in myasthenia gravis?
-Muscle-specific kinase (MuSK)
-Low density lipoprotein receptor-related protein (LRP4)
-These proteins are imprtant for the creation of acetylcholine receptor
-Destruction of these by the antibodies leads to inadequate acetylcholine receptors
What is a characteristic feature of myasthenia gravis
-Weakness that gets worse with muscle use and improves with rest
What muscles are most affected in myasthenia gravis ?
-Proximal muscles and small muscles of the head and neck
Give 8 common symptoms of myasthenia gravis
- Diplopia : extraocular muscle weakness
- Ptosis
- Weak upwards gaze
- Weakness in facial movements
- Difficulty with swallowing
- Fatigue in the jaw when chewing
- Slurred speech
- Progressive weakness with repetitive movements
Give 3 things to assess of examination for myasthenia gravis
-Thymectomy scar
-FVC
-Elicit fatiguability of muscles : repeated blinking will exacerbate ptosis ; prolonged upward gaze with exacerbate diplopia ; repeated abduction of one arm 20 times with result in unilateral weaknes when comparing both sides
Give 3 investigations for diagnosing myasthenia gravis
- Test for antibodies
- CT/MRI of the thymus to check for thymoma
- Edrophonium test if in doubt (tensilon)
What is the edrophonium test
-Used to aid diagnosis of myasthenia gravis
-IV edrophonium chloride is given which will prevent the breakdown of acetylcholine
-Acetycholine levels will rise giving temporary relief from the weakness
Give 3 treatment options for myasthenia gravis
-Pyridostigmine -> reversible acetylcholinesterase inhibitors -> increase the amount of acetylcholine at the junction (or rivastigmine)
-Immunosuppression with prednislone or azathioprine : suppress antibody production
-Thymectomy
What 2 monoclonal antibodies can be used in myasthenia gravis ?
-Rituximab -> targets b cells reducing antibody production
-Eculizumab -> targets complement protein C5.
What is a myasthenic crisis?
-Acute worsening of symptoms often triggered by a viral infection
-Can lead to resp failure due to weakness in the muscle of respiration
- FVC needs monitoring
What is used in the management of a myasthenic crisis?
- IV immunoglobulins
- Plasmapheresis
If needed :
- BiPAP
- Intubation and ventilation
what drugs can exacerbate myasthenia ?
- penicillamine
- quinidine, procainamide
- beta-blockers
- lithium
- phenytoin
- antibiotics: gentamicin, macrolides, quinolones, tetracyclines
What is Lambert-Eaton myasthenic syndrome ?
autoimmune condition affecting the neuromuscular junction
Who is usually affected by Lambert-Eaton myasthenic syndrome ?
-Patients with small cell lung cancer
Explain the pathophysiology behind Lambert-Eaton myasthenic syndrome
- Antibodies against voltage-gated calcium channels in the SCLC are produced.
- These target and damage the same channels in the presynaptic terminals of the neuromuscular junction
- This results in less acetylcholine being released into the synapse
How does lambert-eaton syndrome present ?
-Proximal muscles most affected -> leg muscle weakness (difficulty climbing stairs)
- Reduced or absent tendon reflexes
-Autonomic dysfunction : dry mouth, blurred vision, impotence and dizziness
-Double vision
-Ptosis
-Dysphagia
What is post-tetanic potentiation ?
-In lambert-eaton syndrome, tendon reflexes are normally reduced
-They can become temporarily normal for a short period following a period of strong muscle contraction
How is lambert-eaton syndrome managed?
-Treat underlying malignancy if present
-Amifampridine
-Immunosuppressants
-IV immunoglobulins
-Plasmaphersis
How does amifampridine work in the treatment of Lambert-Eaton?
-It blocks voltage-gated potassium channels in the presynaptic cells
-This prolongs the depolarisation of the cell membrane and assists calcium channels in carrying out their action
Define Gullian-Barre syndrome (GBS)
-Acute paralytic polyneuropathy affecting the peripheral nervous system
What usually triggers GBS ?
-Infection
-Campylobacter jejuni (food poisoning), cytomegalovirus, EBV
How does GBS present ?
- Back/leg pain in initial stages
- Symmetrical ascending weakness
- Reduced reflexes
-+/- peripheral loss of sensation or neuropathic pain - May progress to cranial nerves and cause diplopia, bilateral facial nerve palsy and oropharyngeal weakness
What is the clinical course of GBS?
-Preceding infection
-Symptoms begin 4 wks later
-Symptoms peak after 2-4 wks
-Recovery period lasting mnths to years
What investigations support the diagnosis of GBS?
-Once clinically diagnosed using Brighton criteria :
-Nerve conduction studies showing reduced signal through the nerves
-LP : raised protein with normal cell count and glucose
How is GBS managed ?
- IV immunoglobulins
- Plasmapheresis can be an alternative to IVIG
- VTE prophylaxis with LMWH
What are the features of a benign essential tremor ? (6)
-Fine tremor
-Symmetrical
- 6-12 Hz
-MORE prominent on VOLUNTARY movement
-Worse when tired, stressed or after caffeine
-Improved by alcohol
-Absent during sleep
Give 6 differentials of a tremor
-Parkinson’s
-MS
-Huntington’s chorea
-Hyperthyroidism
-Fever
-Medications (e.g. antipsychotics)
What 2 medications can improve the symptoms of a benign essential tremor ?
- First line = Propranolol
- Primidone
What is the cause of parkinson’s ?
- Progressive reduction of dopamine in the basal ganglia
- Leads to disorders of movement
- Symptoms are ASYMMETRICAL
What is the classic triad of parkinson’s
- Cogwheel rigidity
- Pill-rolling resting tremor (4-6Hz)
- Bradykinesia : smaller handwriting, shuffling gate, difficulty initiating movements, reduced facial expressions (hypomimia)
postural instability is an extra
Where is dopamine produced ?
-Substantia nigra of the basal ganglia
Give 5 other features of parkinson’s
-Depression
-Sleep disturbance and insomnia
-Anosmia
-Postural instability (caused by autonomic failure)
-Cognitive impairment and memory problems
Describe a parkinson’s tremor
-Asymmetrical / unilateral
-WORSE at REST
-IMPROVES with INTENTIONAL movement
-No change with alcohol
Give 4 treatment options for parkinson’s
- Co-benyldopa or co-careldopa
- COMT inhibitors
- Dopamine agonists
- Monoamine Oxidase-B Inhibitors
Explain how levodopa works
-Synthetic dopamine
-Most effective but becomes less effective over time
What is usually given with levodopa and why?
-Peripheral decarboxylase inhibitor
-Stops is being broken down before it reaches the brain
-Examples : carbidopa, benserazide
What is the main side effect of levodopa?
- Too high dopamine = dyskinesias = abnormal movements associated with excessive motor activity.
- E.g. dystonia, chorea, athetosis
How do COMT inhibitors work?
-Example : entacapone
-Inhibits catechol-o-methyltransferase which is responsible for metabolising levodopa
-Extends to duration of levodopa