Neurology Flashcards
Name some causes of headaches in children and what would you be looking for in each (in the history)?
Primary:
- Tension headache: band, stress/tiredness
- Migraine: aura, pulsatile, GI upset and photophobia
- Cluster headache: episodes of attacks
- Trigeminal neuralgia: dermatomal distribution
Secondary:
- Meningitis: fever, sepsis, rash, photophobia, stiffness
- Intracranial bleeds: trauma
- SOL: balance/movement, fits/faints, vomiting
- Hypertension: renal
A 14 year old girl has come to the GP complaining of headaches for the past 3 months. She says they come and go, and she feels the pain all over her head. What are your differentials so far and what do you want to know next?
- ?Tension headaches
- ?Migraines
- ?SOL
eg. non-acute headache.
Progression
Specific triggers eg. early morning/lying down, stress, foods
How long do they last? What does she try to get rid of them eg. lie down in dark room
Any other symptoms: vomiting, visual disturbance or smells or sensations, fits, weight loss, fever
Impact on life
PMH, Dhx, allergy
What are some red flags for headaches?
- Vomiting
- Rashes
- Fever
- Neck stiffness
- Seizures or any focal neuro
- History of trauma
- Sudden onset worst headache ever
- Waking at night with pain
Describe the management of migraines
Conservative: avoid triggers (consider using headache diary for 8 weeks), stress reduction, cold/heat pack
Medical:
-Acute: simple analgesia (paracetamol, NSAID) + nasal sumatriptan combo. Antiemetics eg. prochlorperazine.
-Prophylactic: topiramate or propranolol -> tricyclics.
Describe the treatment for tension headaches
- Conservative: reduce stress, hydration
- Medical: simple analgesia
Define seizures: both medical and layman
- An episode of signs/symptoms caused by transient abnormality of electrical functioning in the brain
- An episode of abnormal brain function
Define epilepsy
A condition predisposing a patient to recurrent unprovoked seizures
Name some causes of seizures
Primary: epilepsy (idiopathic, HIE, tumour) Secondary: -Febrile seizure -Infection: meningitis or encephalitis -Hypoxia -Brain injury -Drugs -Metabolic: hypoglycaemia, electrolyte abnormality
What is a febrile seizure? How does it present?
- A seizure that occurs in the presence of fever but the absence of intracranial infection eg. meningitis/encephalitis
- Common. Usually in infants/children 6mos-6years
- Early in an infection. Usually a focus of infection
- Brief, generalised tonic-clonic
What is the management for febrile seizures? What is the prognosis?
- Rule out red flags
- Education! Diagnosis, what to do when seizure occurs, when to seek help
- Prognosis: most will grow out, they may recur but there is no risk of brain damage and epilepsy risk is not increased
A child has just been diagnosed with a febrile seizure. Please explain the diagnosis to the parents
- Febrile seizure: type of seizure triggered by fever. Common in young children. The brain is just more sensitive to fever but as it matures this will stop.
- No management. Not at risk of any brain damage. Only tiny bit more risk of epilepsy than other children.
- Possibly recur in future illness. Can’t be prevented with meds to lower fever.
- If it happens: make sure child safe eg. no sharp objects near head. Start timer. When stops, put in recovery position.
- When to call for help: if >5 mins long, if severely unwell with fever eg drowsy, non-blanching rash.
The parent of a child calls the GP because their child has had a seizure. When should a child be sent to A&E?
- First episode of seizure
- Signs of meningitis/encephalitis
- <18 months
- Severely unwell
- Focal seizure
- New neuro signs
- Uncertainty over cause
What is a breath-holding attack?
Occurs in toddlers when upset. Crying -> hold breath -> go blue -> +/- LOC
What is a reflex anoxic seizure? What is the underlying cause?
Triggering event (eg fear, cold, pain) -> pale -> fall over +/- convulsions -Due to reflex cardiac asystole (vagal response)
Describe the classification of epileptic seizures and briefly how they present
Complex (LOC) or partial (no LOC)
Generalised: LOC, no warning. Tonic-clonic, atonic, myoclonic, absence, tonic.
Focal:
-Temporal (strange sensations/aura, lip-smacking, plucking at clothes, automatisms, deja vu)
-Frontal (behavioural change, motor dysfunction eg. Jacksonian march)
-Parietal (sensory dysfunction)
-Occipital (visual hallucinations)
How does an absence seizure present?
LOC, staring into space, +/- eyelids flickering
How does a tonic-clonic seizure present?
Fall to the ground (tonic), cyanosis
Jerking movements, salivation, tongue-biting, incontinence
Unconsciousness/drowsiness/sleeping
Name some types of epilepsy syndromes (7 types)
- Infantile spasms (West syndrome)
- Lennox-Gastaut syndrome
- Childhood absence epilepsy
- Benign rolandic epilepsy
- Panaylotopoulos syndrome
- Juvenile absence epilepsy
- Juvenile myoclonic epilepsy
Describe infantile spams/West syndrome (presentation, EEG findings, Mx)
Occurs in infants 3-12 months
Flexion -> extension
Multiple short bursts, lasting 1-2 seconds
Eventually regress + LDs
Hypsarrhythmia on EEG (chaotic slow waves, sharp multifocal areas)
Mx: vigabitrin
Describe Lennox-Gastaut syndrome (presentation, EEG findings, Mx)
1-3 years Multiple types eg. absence, atonic Developmental delay, behavioural issues EEG: slow generalised spike, 1-3 Hz Mx: lamo/valproate
Describe childhood absence epilepsy (presentation, EEG findings, Mx)
4-12 years
Sudden onset, brief absence seizures. Normal otherwise
EEG: generalised 3/s spike+wave (3-4 Hz)
Describe benign rolandic epilepsy (presentation, EEG findings, Mx)
4-10 years
Tonic-clonic seizures in sleep, or simple focal.
EEG: focal sharp waves in Rolandic area
Usually no need for Mx/carba or lamo
Describe Panayiotopoulos syndrome (presentation, EEG findings, Mx)
1-5 years
Autonomic features, staring
EEG: posterior sharp focal waves, occipital
Mx: carbamazepine or lamotrigine
Describe juvenile absence epilepsy (presentation, EEG findings, Mx)
10-20 years
Absence, generalised tonic-clonic
Normal otherwise
Describe juvenile myoclonic epilepsy (presentation, EEG findings, Mx)
10-20 years
Myoclonic, generalised tonic-clonic in the morning. Normal otherwise.
Describe the process of diagnosis of seizures in children
- History, collateral if teen, video
- Neuro exam, basic obs, BM
- Sepsis screen if relevant: eg urine dip and bloods, LP
- ECG
- Bloods: FBC, U+Es, bone profile, LFTs, VBG
- EEG if no clear cause
- Imaging if indicated: CT acute, MRI SOL or epilepsy
- Consider metabolic investigations for IMDs if also developmental issues/signs
The parents of a 4 year old boy have brought him to A&E after he had a seizure this morning. They described him becoming very stiff, then dropping to the floor and jerking continuously for about 4 minutes. He bit his tongue during the episode. In A&E, he looks very drowsy and has been asleep for most of the consultation. What is the likely differential and what are the next steps?
This sounds like a generalised tonic-clonic epileptic seizure.
Neuro exam, obs, BM, bloods, ECG, EEG.
Urgent referral to neurology
Explain that this may be the first presentation of epilepsy, but there may be other reasons. It isn’t possible to know now because we can’t usually diagnose epilepsy after only 1 episode.
If again: take video, protect child, call 999 if >5 mins.
When should anti-epileptic medication be considered?
- If the seizures are very frequent or prolonged
- Interfering with life/school performance
- Rescue therapy given if frequent prolonged seizures (>5 mins)
Name 4 commonly used AEDs. What are they used for and when are they avoided? What are their side effects?
- Valproate: commonly used for most types. Avoid in girls. SE: weight gain, hair loss
- Lamotrigine: commonly used for tonic-clonic (esp girls), absence. NOT myoclonic. SEs: rash
- Ethosuximide: absence in girls. SEs: N+V
- Carbamazepine: for focal, not absence. SEs: rash, neutropenia/agranulocytosis, hyponatraemia, ataxia, med interference
Which AEDs are used for the different seizure types (excluding syndromes)?
- GTC: lamotrigine or valproate
- Absence: ethosuximide or valproate. Not carbamazepine
- Myoclonic: valproate, levetiracetam, topirimate. Avoid lamotrigine
- Focal: carbamazepine or lamotrigine
- Alternatives: levetiracetam, topirimate, clobazam
Who is part of the MDT for epilepsy?
- Paediatrician: neuro
- Specialist nurse
- Physio/OT if also developmental issues
- School
- Psychologist
What is rescue therapy for epilepsy? How is it used?
Usually buccal midazolam, to be used at home if the child has prolonged seizures
- After 5 mins: administer
- If 10 mins after 1st dose the seizure is still going -> call 999
Describe the classification of movement disorders and how they present, name some causes.
Central
- Corticospinal tract- stroke, tumour, HIE: weak, hip + shoulder adduction, UMN signs
- Basal ganglia- Wilson’s, HIE: difficulty initiating movement, dystonia/dyskinesia, choreoathetosis
- Cerebellum- medulloblastoma, Friedrich ataxia: past-pointing, dysdiadochokinesia, wide-based gait, ataxia
Peripheral
- Anterior horn cells- spinal muscular atrophy, polio: weak, no reflex, wasting, fasciculations
- Nerve- Charcot-Marie-Tooth, GBS, Bell palsy: weakness, abnormal sensation
- NMJ- MG: weakness with fatiguability
- Muscle- dystrophies, myopathies: weakness, wasting
What is Friedrich ataxia?
AR inherited condition. Frataxin gene mutation.
-Ataxia, hearing loss, peripheral neuropathy, cardiomyopathy
What is spinal muscular atrophy?
AR degenerative condition affecting anterior horn cells. SMN1 mutation.
3 types
1: presents very early. Die before 1 yr
2+3 present later, more mild.
Describe Charcot-Marie-Tooth disease.
Hereditary motor/sensory neuropathy. Caused by myelin gene mutations esp CMT1A
- Symmetrical, slowly progressive muscle wasting, bilateral foot drop -> tripping, loss of reflexes, pes cavus
- Onion bulb appearance on biopsy
Describe the pathophysiology and presentation of Gullain-Barre syndrome
Weeks after URTI/Campylobacter -> ascending, progressive motor and sensory neuropathy
-Symmetrical weakness, loss of reflexes, reduced sensation, autonomic involvement
How is GBS diagnosed?
- Clinical
- MRI to exclude lesion
- LP: high CSF protein in 2nd week
Which test is used to diagnose myasthenia gravis? What is the treatment?
- Tensilon test: give edrophonium
- Antibodies: anti-AChR, anti-MuSK
- Treatment: cholinesterase inhibitors eg. pyridostigmine/neostigmine, immunosuppression
Define muscular dystrophy. Name some types.
Inherited conditions where there is progressive muscle degeneration
- Duchenne’s
- Becker
Describe the pathophysiology of Duchenne’s, the presentation and the prognosis.
- X-linked R condition, mutation in the dystrophin gene -> Ca influx and necrosis of the muscle fibres
- Presents around 5 years with waddling gait, language delay, climbing stairs 1 at a time, pseudohypertrophy of the calves, Gower’s sign
- Unable to walk by 10-14 years, death by 20-30
What is Gower’s sign? What does it mean?
Walking the hands up the thighs to stand up. Due to proximal muscle weakness. Sign of lower motor pathology, Cushings, Rickets.
What is the first test to order when suspecting muscular dystrophy? Which test will diagnose Duchenne’s?
Creatine kinase
Muscle biopsy showing absence of dystrophin, or genetic testing
Describe the management of muscular dystrophy.
- MDT approach at specialist centre
- Paediatrician, specialist nurses, physio, OT, psychology
How is Becker muscular dystrophy different to Duchennes?
- Caused by a different mutation in dystrophin -> some functional protein
- Presents later (11), has slower progression
What are the signs/symptoms of dermatomyositis? How is it diagnosed?
- Fever, symmetrical weakness, heliotrope rash, extensor rash
- Raised CRP, ESR, CK. Biopsy -> inflammatory infiltrate, atrophy
Name some causes of floppy newborns
- Hypoglycaemia, hypothyroidism
- Hypoxic-ischaemic encephalopathy
- Chromosomal abnormalities: Down’s
- Inherited conditions: muscular dystrophy, myopathy, myotonia
What are some types of intracranial bleed? How do they present and what are the risk factors?
- Extradural: following trauma. LOC -> lucid period -> declining consciousness and headache. Pupillary dilation, shock.
- Subdural: associated with NAI/shaking. Slowly progressive decreased consciousness.
- Subarachnoid: rare. Rapid onset thunderclap headache.
Strokes in children should make you think of……
Sickle cell. Also SLE, endocarditis etc.
What are the types of neural tube defects?
Anencephaly: no cranium. Stillborn/early death
Spina bifida occulta: failure of vertebral arch fusion. Dimple/tuft of hair/sinus. MRI
Meningocele: SBO and pouch of meninges protruding
Myelomeningocele: SBO and pouch of meninges and neural tissue protruding
What is hydrocephalus? What are the causes? How does it present and how is it treated?
An accumulation of CSF in the brain.
- Non-communicating: obstruction
- Communicating: failure to reabsorb CSF
- Increased head circumference, fixed downward gaze
- Treat cause, surgical insertion of shunt
Describe neurofibromatosis
Type 1: AD. NF1 gene mutation. Cafe au lait spots, neurofibroma, axillary freckles, optic glioma, Lisch nodule
Type 2: AD. NF2 gene mutation. Inherited schwannomas (bilateral acoustic neuroma), meningiomas, ependymomas
Describe the features of tuberous sclerosis
-AD condition caused by TSC1 and 2 mutations
Skin: Ash leaf patches appearing under Wood’s light, shagreen patches on spine, angiofibromata in butterfly shape
Neuro: infantile spasms, epilepsy, delay
Nodules and growths on many organs
What are the features of Sturge-Weber syndrome?
Port wine stain, epilepsy, learning disability
Which diseases cause neurodegeneration in children?
Lysosomal storage disorders: MPS, Tay-Sachs, Gauchers, Niemann-Pics
Peroxisomal enzyme defects
Wilson’s
What is the presentation of Tay-Sachs? Gauchers?
Tay-Sachs: developmental regression. Hypotonia, cherry red spot on macula
Gaucher: splenomegaly, BM suppression
Describe the management of status epilepticus
Start timer
A-E approach: secure airway, high flow O2. Get IV access, take bloods and glucose.
1. After 5 mins: buccal midaz (0.5mg/kg) or IV loraz (0.1mg/kg) if access
2. After 10 mins (at 15): IV loraz 2nd dose. Call for senior
3. After 10 mins (at 25): phenytoin 20mg/kg infusion over 20 mins or phenobarbital if already on phenytoin. Call anaesthetics
4. After 20 mins (45): rapid sequence induction w/ thiopental (4mg/kg).
Define status epilepticus
Continuous seizure activity or intermittent seizures with no recovery of consciousness for >30 mins
In practice, treat after 5 mins
