Neurology Flashcards
What type of drugs are 5-HT3 antagonists?
Antiemetics
Where do 5-HT3 antagonists work?
Chemoreceptor trigger zone of the medulla oblongata
Examples of 5-HT3 antagonists
Ondanestron
Graniestron
What type of drug is ondansetron?
5-HT3 antagonist
What type of drug is graniestron?
5-HT3 antagonist
Side effects of 5-HT3 antagonists
Constipation
Prolonged QT interval
Drugs causing peripheral neuropathy
Amiodarone Isoniazid Vincristine Nitrofurantoin Metronidazole
What does the fourth cranial nerve supply?
What are its actions?
Superior oblique
Depresses eye, moves eye inwards
Features of fourth nerve palsy
Vertical diplopia
Subjective tilting of objects
Head tilt
Affected eye deviates upwards and is rotated out
Lamotrigine - indications
second line treatment for generalised and partial seizures
Lamotrigine - mechanism of action
Sodium channel blocker
Lamotrigine - side effects
Stevens-Johnson syndrome
Treatment for leg cramps
1) stretching calves
2) trial of quinine
Features of multiple system atrophy
Parkinsonism
Autonomic disturbance causing erectile dysfunction, postural hypotension, atonic bladder
Cerebellar signs
Causes of neonatal seizures
Vitamin B6 deficiency Hypoglycaemia Meningitis Head trauma Opiate withdrawal
Who is affected by neuroleptic malignant syndrome?
Antipsychotics - first few days
Parkinson medication - when reducing dose or suddenly stopped
Neuroleptic malignant syndrome - features
Pyrexia
Muscle rigidity
Autonomic lability = HTN, tachycardia, tachypnoea
Agitated delirium with confusion
Neuroleptic malignant syndrome - investigations
Raised CK
AKI secondary to rhabdomyolysis
Leukocytosis
Neuroleptic malignant syndrome - management
Stop antipsychotics
IV fluids
Dantrolene
Bromocriptine
What is neuroleptic malignant syndrome?
Rare but life threatening adverse effect to antipsychotics
Generalised tonic clonic seizures - 1st line
Sodium valproate
Generalised tonic clonic seizures - 2nd line
Lamotrigine or carbamazepine
Absence seizures - 1st line
Sodium valproate or ethosuximide
Myoclonic seizures - 1st line
Sodium valproate
Myoclonic seizures - 2nd line
Clonazepam or lamotrigine
Focal seizures - 1st line
Carbamazepine or lamotrigine
Focal seizures - 2nd line
Levetiracetam, oxcarbazepine or sodium valproate
Sodium valproate in pregnancy
Causes neurodevelopmental delay in children
In what circumstances would you start antiepileptics after one seizure?
Neurological deficit
Structural abnormality
EEG: unequivocal
Patient or family consider risk of another seizure unacceptable
What is Lennox-Gestaut Syndrome?
Childhood epilepsy syndrome
Lennox-Gestaut Syndrome - features
Atypical abscences, falls, jerks
Moderate to severe mental handicap
Lennox-Gestaut Syndrome - EEG
slow spike
Lennox-Gestaut Syndrome - onset
1-5 years
What is Infantile Spasms?
Childhood epilepsy syndrome
Infantile spasm - other name
West’s syndrome
Infantile spasms - features
Flexion of head, trunk, limbs then extension of arms - Salaam attack
Lasts 1-2 secs, repeated up to 50 times
Progressive mental handicap
Infantile spasms - EEG
Hypsarrhythmia
Infantile spasms - management
Vigabatrin, steroids
Poor prognosis
Infantile spasms - onset
4-6 months
Benign rolandic epilepsy - features
Paraesthesia (e.g. unilateral face), usually on waking
Juvenile myoclonic epilepsy syndrome - other name
Janz syndrome
Juvenile myoclonic epilepsy syndrome - onset
Teens
Juvenile myoclonic epilepsy syndrome - features
Infrequent generalised seizures, often in the morning/following sleep deprivation
Daytime absences
Sudden, shock-like myotonic seizure
Juvenile myoclonic epilepsy syndrome - management
sodium valproate
Neurofibromatosis - inheritence
Autosomal dominant
Neurofibromatosis type 1 - features
cafe au lait spots >6
Axillary/groin freckles
Irish hamatomas (Lisch nodules)
Pheochromocytomas
Neurofibromatosis type 2 - features
Bilateral vesticular schwannomas
Multiple intracranial schwannomas, meningiomas, ependymomas
Myotonic dystrophy - inheritence
Autosomal dominant
Trinucleotide repeat disorder
What is myotonic dystrophy?
Inherited myopathy
Affects skeletal, cardiac, and smooth muscle
Myotonic dystrophy type 1 - features
Distal weakness prominent
Myotonic dystrophy type 2 - features
Proximal weakness prominent
Severe congenital form not seen
Myotonic dystrophy - investigations
Genetic testing
CK may be raised
MRI - cerebellar degeneration
Myotonic dystrophy - features
Myotonic faces - long, haggared Frontal balding Bilateral ptosis Cataracts Weakness Diabetes Testicular atrophy Dysphagia Heart block, cardiomyopathy
What is myasthenia gravis?
Autoimmune disorder resulting in insufficient functioning acetylcholine receptors
What is the main antibody found in myasthenia gravis?
Antibodies to acetylcholine receptors
Myasthenia gravis - associations
Thymomas
Autoimmune disorders
Thymic hyperplasia
Myasthenia gravis - features
Muscle fatigability Extraocular muscles = diplopia Proximal muscle weakness = face, neck, limb girdle Ptosis Dysphagia
Myasthenia gravis - investigations
Single fibre EMG
CT thorax to exclude thyoma
CK normal
Antibodies to acetylcholine receptors
Myasthenia gravis - first line management
Pyridostigmine - long acting anticholinesterase inhibitor
Myasthenia gravis - second/third line management
Immunosuppression - steroids, azathioprine
Thymectomy
Myasthenia gravis - crisis management
Plasmaphoresis
IV Ig
Myasthenia gravis - exacerbating drugs
Penicillamine
Beta blockers
Lithium
Phenytoin
Gentamicin
Tetracyclines
What is multiple sclerosis?
Chronic cell mediated autoimmune disorder characterised by demyelination in the CNS
Multiple sclerosis - types
Relapsing remitting
Primary progressive
Secondary progressive
Multiple sclerosis - relapsing remitting
Acute attacks followed by periods of remission
85% cases
Multiple sclerosis - primary progressive
10% cases
Progressive deterioration from onset
More common in elderly
Multiple sclerosis - secondary progressive
Relapsing remitting patients who have deteriorated and have signs/symptoms between relapses
Primary gait and bladder disorders
65% patients with relapsing remitting
Multiple sclerosis - features
Lethargy
Optic neuritis
Paraesthesia, numbness, Lhermittes syndrome
Spastic weakness
Ataxia
Urinary incontinence, Sexual dysfunction
Intellectual deterioration
What is Lhermittes syndrome?
sudden brief shock that moves down the neck caused by bending the neck forward
associated with MS
Multiple sclerosis - acute relapse management
high dose steroids
Multiple sclerosis - chronic management
Treatment for fatigue?
Treatment for spasticity?
Treatment for bladder dysfunction?
Beta interferon reduces relapse rate by 30%
Fatigue - amantadine
Spasticity - baclofen, gabapentin
Bladder dysfunction - self catheterisation or anticholingerics
Multiple sclerosis - good prognostic factors
Female Young age of onset Relapsing-remitting type Sensory symptoms only Long interval between first two relapses Complete recovery between relapses
What is motor neuron disease?
Neurological condition presenting with upper and lower motor neuron signs
Motor neuron disease - features
Fasiculations
Mixture of LMN and UMN signs
Wasting of the small hand muscles
No sensory symptoms/signs
Motor neuron disease - Investigations
Clinical diagnosis
Nerve conduction studies - normal, exclude neuropathy
EMG - reduced action potentials with increased amplitude
MRI - to exclude cervical cord compression and myelopathy
Motor neuron disease - Management
Riluzole - prolongs life by 3 months
Respiratory care - BIPAP at night
Migraine - features
Severe, unilateral, throbbing headache Nausea Photophobia, phonophobia Lasts up to 72 hours Aura in 1/3rd
Migraine in children
Shorter
Bilateral
GI disturbance
Migraine - acute management first line
Oral triptans + NSAID/paracetamol
Nasal triptan if age 12-17
Migraine - acute management second line
Non-oral metoclopramide
Non-oral NSAIDs/triptans
Migraine - prophylaxis
Topiramate or propranolol
Acupuncture
Migraine management if menstrual symptoms
Frovatriptan or zolmitriptan
Mefanamic acid
Migrane management in pregnancy
Paracetamol
NSAIDs in 2rd or 3rd month
Topiramate in women of child bearing age
Teratogenic and reduces effectiveness of oral contraception
Medication overuse headache - features
15+ days per month
Develops/worsens whilst taking regular symptomatic medications
Medication overuse headache - which medication is highest risk?
Opioids and triptans
Idiopathic intracranial hypertension - features
Headache Blurred vision Papilloedema Enlarged blind spot 6th nerve palsy
Idiopathic intracranial hypertension - management
Weight loss
Diuretics - acetazolamide
Topiramate
Repeated LP
Idiopathic intracranial hypertension - risk factors
Obesity Female Pregnancy Combined oral contraceptive Steroids Tetracyclines Vitamin A Lithium
Huntington’s disease - inheritence
Autosomal dominant
Huntington’s disease - genetics
Trinucleotide repeat disorder
Genetic anticipation
Defect in huntington gene on chromosome 4
Huntington’s disease - features
Chorea Personality change - apathy, irritable, depression Intellectual impairment Dystonia Saccadic eye movements
Huntington’s disease - investigations
Genetic testing
MRI/CT
What is Guillain-Barre syndrome?
Immune mediated demyelination of the peripheral nervous system, often triggered by infection
Guillain-Barre Syndrome - classical trigger
Campylobacter jejuni
Guillain-Barre Syndrome - features
Early leg/back pain
Progressive, symmetrical weakness of all the limbs - ascending
Absent reflexes
Respiratory muscle weakness
Guillain-Barre Syndrome - Investigations
LP - rise in protein, normal WCC
Nerve conduction studies - reduced motor nerve conduction due to demyelination
Guillain-Barre Syndrome - management
IV immunoglobulin
Plasma exchange
Miller Fisher Syndrome - features
Descending paralaysis
Opthalmoplegia, areflexia, ataxia
Eye muscles affected first
What is Friedreich’s ataxia?
Early onset hereditary ataxia
Friedreich’s ataxia - inheritance
Autosomal recessive
Trinucleotide repeat disorder - NO genetic anticipation
Friedreich’s ataxia - onset
10-15 years
Presents with gait ataxia and kyphoscoliosis
Friedreich’s ataxia - features
Absent ankle jerk/ extensor plantars
Cerebellar ataxia
Optic atrophy
HOCM
Diabetes
High arched palate
Friedreich’s ataxia - prognosis
Wheelchair bound by 15 years after diagnosis
Life expectancy 40-50
Duchenne muscular dystrophy - inheritance
X linked recessive
Mutation in gene encoding dystrophin
Frame shift mutation = severe form
Becker muscular dystrophy - inheritance
X linked recessive
Mutation in gene encoding dystrophin
Non-frame shift mutation = less severe
Duchenne muscular dystrophy - features
Progressive muscle weakness from 5 years
Calf pseudohypertrophy
Gowers sign
30% have intellectual impairment
Becker muscular dystrophy - features
Develops after age 10
Normal intellect
What is degenerative cervical myelopathy?
Spinal cord dysfunction from compression in the neck
Degenerative cervical myelopathy - risk factors
Smoking
Genetics
Occupation with heavy axial loading
Degenerative cervical myelopathy - features
Pain in neck, upper and lower limbs
Loss of motor function - digital dexerity, arm/leg weakness
Loss of sensory function
Loss of autonomic function - incontinence, impotence
Hoffman’s sign
What is Hoffman’s sign?
involuntary flexion movement of the thumb and or index finger when the examiner flicks the fingernail of the middle finger down
Degenerative cervical myelopathy - investigations
MRI spine
Degenerative cervical myelopathy - management
Early referral to spinal services for decompression surgery
Cluster headaches - frequency of symptoms
Occur in clusters lasting 4-12 weeks
Generally a cluster every year
Cluster headaches - features
Intense sharp stabbing pain around the eye
Patient restless, agitated
Red eye, lacrimating, lid swelling
Nasal stuffiness
Cluster headaches - how long do symptoms last
15mins to 2 hours
Occur 1-2x per day
Cluster headaches - risk factors
Male
Smokers
Nocturnal sleep
Alcohol may trigger attack
Cluster headaches - acute management
100% oxygen
Sub cut triptan
Cluster headaches - prophylaxis
Verapamil
What is Charcot-Marie-Tooth?
The most common hereditary peripheral neuropathy
Results in predominantly motor loss
Charcot-Marie-Tooth - features
Foot drop High arched feet Hammer toes Distal muscle weakness and atrophy Hyporeflexia Stork leg deformity
Carbamazepine - uses
Epilepsy - partial seizures
Trigeminal neuralgia
Bipolar disorder
How does carbamazepine work?
Binds to sodium channels to increase their refractory period
Carbamazepine - side effects
P450 enzyme inducer
Dizzy
Drowsy
Diplopia
Headaches
Steven Johnson Syndrome
What is Bell’s palsy?
Acute, unilateral, idiopathic facial nerve paralysis
Bell’s palsy - features
LMN facial nerve palsy - forehead affected
How to differentiate between stroke and Bell’s palsy
Bell’s palsy - forehead affected
Bell’s palsy - management
Prednisolone within 72 hours
Antivirals in severe cases
Eyecare
Bell’s palsy - prognosis
Most make full recovery in 3-4 months
If untreated 15% have severe weakness permanently
Bell’s palsy - follow up
If no improvement after 3 weeks refer urgently to ENT
Absence seizures - features
Few seconds with quick recovery
Provoked by hyperventilation or stress
Children unaware
May occur multiple times a day
Absence seizures - EEG
Bilateral, symmetrical 3Hz spike and wave pattern
Absence seizures - management
Sodium valproate or ethosuximide
Wernicke’s aphasia - features
Sentences that make no sense but remain fluid “word salad”
Comprehension impaired
Conduction aphasia - features
Speech fluent but poor repetition
Aware they are making errors
Normal comprehension
Broca’s aphasia - features
Speech is non-fluent, laboured, halting
Repetition impaired
Normal comprehension
Global aphasia - features
Severe expressive and receptive aphasia
What location is affected in expressive dysphasia?
Broca’s
What location is affected in receptive dysphasia?
Wernicke’s
What is ataxia telangiectasia?
Inherited combined immunodeficiency disorder
Ataxia telangiectasia - inheritence
Autosomal recessive
Ataxia telangiectasia - features
Cerebral ataxia
Telangiectasia
Recurrent chest infections due to IgA deficiency
10% risk of malignancy, lymphoma or leukaemia
Onset of ataxia telangiectasia
Onset 1-5 years
Abnormal movements
Which antiepileptics should be prescribed by brand?
Carbamazepine
Phenytoin
Phenobarbital
Primodine
Drugs causing peripheral neuropathy
Amiodarone Isoniazid Vincristine Nitrofurantoin Metronidazole
Essential tremor - inheritance
Autosomal dominant
Essential tremor - features
Postural tremor - worse when arms outstretched
Improved by alcohol and rest
What is the most common cause of head tremor?
Essential tremor
Essential tremor -management
Propranolol
Primidone
In which conditions is cannabis licensed for use?
Chemo induced nausea and vomiting
Chronic pain
Spasticity in MS
Severe treatment resistance epilepsy
Who can prescribe cannabis?
Doctors on the specialist register
Lamotrigine - mechanism of action
Sodium channel blocker
Lamotrigine - adverse effects
Stevens Johnson syndrome
Which medication can be used to treat dementia symptoms in Parkinson’s disease?
Rivastigmine
When are antiepileptics stopped?
Over how long a time frame do you stop the drugs?
Seizure free >2 years
Drugs stopped over 2-3 months
What is cataplexy?
Sudden transient loss of muscular tone caused by strong emotion
Can HRT be given if migraine history?
Yes but may worsen migraine