Paediatrics Neurology Flashcards
What is syncope?
Temporarly loss of consciousness due to a disruption of blood flow to the brain
What are syncopal episodes also known as?
Vasovagal episodes
Fainting
What is a vasovagal episode cause by?
Name some prodromes of fainting?
- Hot and clammy
- Sweaty
- Heavy
- Dizzy or lightheaded
- Vision going blurry / dark
- Headache
What is the difference between a postictal period following a seizure and period following a faint?
Postictal = longer periods of confusion, drowsiness, irritability, disorientation
(Incontinence may occur with both seizures and syncopal episodes)
What are some causes of primary syncope (simple fainting)?
Dehydration
Missed meals
Extended standing in a warm environment e.g. school assembly
Vasovagal response to stimuli e.g. sudden surprise or pain in the sight of blood
What are some secondary causes of syncope?
Hypoglycaemia
Dehydration
Anaemia
Infection
Anaphylaxis
Arrhythmias
Valvular heart disease
Hypertrophic obstructive cardiomyopathy
What questions to ask for a syncope history?
Syncope vs a seizure?
After exercise? (More like secondary cause)
Triggers?
Concurrent illness? Do they have a fever or signs of infection?
Injury? Do they have a head injury?
Associated cardiac symptoms, such as palpitations or chest pain?
Associated neurological symptoms?
Family history, particularly cardiac problems or sudden death?
Whats the difference between syncope or seizure?
What to look for on examination for syncopal episode?
Physical injuries?
Concurrent illness, for example an infection or gastroenteritis?
Neurological examination
Cardiac examination, specifically assessing pulses, heart rate, rhythm and murmurs
Lying and standing blood pressure
What investigations for syncopal episode?
ECG (arrhythmias and long QT)
24 hour ECG if paroxysmal arrhythmias are suspected
Echocardiogram if structural heart disease is suspected
Bloods, including a full blood count (anaemia), electrolytes (arrhythmias and seizures) and blood glucose (diabetes)
What is the management of a simple vasovagal episode?
- Avoid dehydration
- Avoid missing meals
- Avoid standing still for long periods
What is epilepsy?
Umbrella term where there is a tendency to have seizures
What are seizures?
Transient episodes of abnormal electrical activity in the brain
What is a generalised tonic-clonic seizure?
Loss of consciousness
Tonic (muscle tensing)
Clonic (muscle jerking) movements
Along with tongue biting, incontinence, groaning and irregular breathing
What is the post ictal period?
Time after seizure when the person is confused, drowsy and feels irritable or low
What is the management of tonic-clonic seizures?
First line: sodium valporate
Second line: lamotrigineorcarbamazepine
What are focal seizures?
Seizures which start in the temporal lobes affecting hearing, speech, memory and emotions
How do focal seizures present?
Hallucinations
Memory flashbacks
Déjà vu
Doing strange things on autopilot
What is the treatment for focal seizures?
First line: carbamazepine or lamotrigine
Second line: sodium valproate or levetiracetam
(opposite of generalised tonic-clonic seizures)
What are absence seizures?
Typically in children - patient is blank, stares into space and then abruptly returns to normal
Lasts 10 to 20 seconds (most stop having seizures as they get older)
What is the treatment for absence seizures?
First line: sodium valporate or ethosuximide
What are atonic seizures?
Also known as drop attacks characterised by brief lapses in muscle tone (don’t usually last more than 3 months) typically begin in childhood
What may atonic seizures be indicative of?
Lennox-Gastaut syndrome
What is the management of atonic seizures?
First line: sodium valproate
Second line: lamotrigine
What are myoclonic seizures?
Sudden brief muscle contractions like a jump - patient usually remains awake during the episode
What type of epilepsy do myoclonic seizures occur in?
Juvenile myoclonic epilepsy
What is the management of myoclonic seizures?
First line: sodium valproate
Other options: lamotrigine, levetiracetam or topiramate
What are infantile spasms also known as?
West syndrome (rare 1 in 4000)
What are the features of infantile spasms?
Begin at 6 months of age
Clusters of full body spasms
Poor prognosis 1/3 die by 25, 1/3 are seizure free
What are the treatments for infantile spasms?
Prednisolone
Vigabatrin
What are febrile convulsions?
Seizures which occur in children when they have a fever (not caused by epilepsy or meningitis or tumours - other underlying neurological pathology)
In what age children do febrile convulsions occur?
Between 6 months and 5 years (no lasting damage - slightly increases risk of developing epilepsy in the future)
What are the investigations for epilepsy?
Clear history (as opposed to febrile convulsion or vasovagal episode)
Electroencephalogram (EEG) show patterns in different forms of epilepsy - perform after second simple tonic-clonic seizure
MRI brain (tumours) - when first seizure is in children under 2, focal seizure, no response to first-line anri-epileptics
Which additional investigations considered to exclude pathology?
ECG to exclude problems in the heart
Blood electrolytes including sodium, potassium, calcium and magnesium
Blood glucose for hypoglycaemia and diabetes
Blood cultures, urine cultures and lumbar puncture where sepsis, encephalitis or meningitis is suspected
What is the general advice to patients with epilepsy?
- Showers rather than baths
- Cautious with swimming unless seizures are well controlled
- Cautious with heights/traffic/heavy, hot or electrical equipment
- Avoid driving unless they meet specific criteria
What is sodium valporate?
First line option for most forms of epilepsy
Increases activity of GABA which relaxes the brain
What are the side effects of sofium valporate?
Teratogenic so patients need careful advice about contraception
Liver damage and hepatitis
Hair loss
Tremor
What is first line for focal seizures?
Carbamazepine
What are some side effects of carbamazepine?
Agranulocytosis
Aplastic anaemia
Induces the P450 system so there are many drug interactions
What are the side effects of phenytoin?
Folate and vitamin D deficiency
Megaloblastic anaemia (folate deficiency)
Osteomalacia (vitamin D deficiency)
What are the side effects of ethosuximide?
Night terrors
Rashes
What are the notable side effects of lamotrigine?
Stevens-Johnson syndrome or DRESS syndrome. These are life threatening skin rashes.
Leukopenia
What is the management of seizures?
Put the patient in safe position (on a carpeted floor)
Put in recovery position
Put something under their head
Remove obstacles
Make a note of start and end time
Call an ambulance if more than 5 mins or if first seizure
What is status epilepticus?
Seizure lasting more than 5 mins or more than 3 seizures in one hour
What is the management of status epileptics?
ABCDE approach
Secure the airway
Give high-concentration oxygen
Assess cardiac and respiratory function
Check blood glucose levels
Gain intravenous access (insert a cannula)
IV lorazepam, repeated after 10 minutes if the seizure continues
What is the medication if seizures persist in status epileptics?
IV phenobarbital or phenytoin (consider intubation at this point and transfer to ICU)
What are the medical treatment options for status epileptics in the community?
Buccal midazolam
Rectal diazepam
What are simple febrile convulsions?
Generalised, tonic clonic seizures - last less than 15 minutes and only occur once during a single febrile illness
What are complex febrile convulsions?
Complex = partial / focal seizures
Last more than 15 minutes or multiple times during same febrile illness
What are the differentials diagnosis for febrile convulsion?
Epilepsy
Meningitis, encephalitis or another neurological infection e.g. cerebral malaria
Intracranial space occupying lesions e.g. brain tumours or intracranial haemorrhage
Syncopal episode
Electrolyte abnormalities
Trauma (think about non accidental injury)
What is the management of febrile convulsion?
Identify and manage the underlying source of infection
Control fever with simple analgesia e.g. paracetamol and ibuprofen
What is the general advice for managing a febrile convulsion?
Stay with child
Put child in safe place (carpeted floor)
Place in recovery position
Don’t put anything in their mouth
Call ambulance if lasts more than 5 minutes
What is the risk of developing epilepsy?
1.8% for gen population
2-7.5% for simple febrile convulsion
10-20% after complex febrile convulsion
What are breath holding spells?
Involuntary episodes where child holds breath (trigged by something upsetting/scary) between 6-18 months of age - not harmful in long term, don’t lead to epilepsy
Most outgrow by 4-5 years
What are the two types of breath holding spells?
Cyanotic breath holding spells
Pallid breath holding spells (also known as reflex anoxic seizures)
What are cyanotic breath holding spells?
When child is really upset, worked up and crying, after a long cry they some breathing, become cyanotic and lose consciousness - within a minute regain consciousness - may be tired and lethargic after episode
What are reflex anoxic seizures?
Occur when child is startled - vagus nerve sends strong signals to the heart to cause it to stop beating
Child suddenly goes pale, loses consciousness and may start to have some seizure-like muscle twitching
Within 30 seconds the heart restarts
What is the management of breath holding spells
Reassure after excluding other pathologies
Treat if iron deficiency anaemic (as this has been linked)
What are some causes of headaches in children?
Tension headaches
Migraines
Ear, nose and throat infection
Analgesic headache
Problems with vision
Raised intracranial pressure
Brain tumours
Meningitis
Encephalitis
Carbon monoxide poisoning
How do tension headaches present?
Mild ache across forehead
Band-like symmetrical pattern
Resolve gradually
No visual changes / pulsating sensation
What are the triggers for tension headaches in children?
Stress, fear or discomfort
Skipping meals
Dehydration
Infection
What is the management of stress headaches?
Reassurance
Analgesia
Regular meals
Avoiding dehydration
Reducing stress
What are the types of migraine?
Migraine without aura
Migraine with aura
Silent migraine (migraine with aura but without a headache)
Hemiplegic migraine
Abdominal migraine
No simple explanation for why these occur
What are the features of a headache caused by a migraine?
Unilateral
More severe than tension headaches
Throbbing in nature
Take longer to resolve
What are migraines associated with?
Visual aura
Photophobia and phonophobia
N&V
Abdo pain
What is the management of migraines in children?
Rest, fluids and low stimulus environment
Paracetamol
Ibuprofen
Sumatriptan
Antiemetics e.g. domperidone
What are some options for migraine prophylaxis?
Propranolol (avoid in asthma)
Pizotifen (often causes drowsiness)
Topiramate (girls with child bearing potential need highly effective contraception as it is very teratogenic).
What are the features of an abdominal migraine?
Central abdominal pain lasting more than 1 hour
Examination will be normal
May also have: N&V, anorexia, headache, pallor
What infections can cause headaches?
Viral upper respiratory tract infection
Otitis media
Sinusitis
Tonsillitis
What can be given for headaches caused by infection?
Paracetamol and ibuprofen
Where is the inflammation in sinusitis?
Ethmoidal
Maxillary
Frontal
Sphenoidal
What are the features of sinusitis?
Facial pain behind the nose, forehead and eyes
Tenderness over the affected sinuses - helps to diagnose
Resolves in 2-3 weeks
Mostly viral
What is cerebral palsy?
Name of the permanent neurological problems resulting from damage to the brain around time of birth - not a progressive condition
How does cerebral palsy vary?
Huge variation in symptoms from wheelchair bound to para-olympic athletes with only subtle problems with coordination / mobility
What are the causes of cerebral palsy?
Antenatal:
Maternal infections
Trauma during pregnancy
Perinatal:
Birth asphyxia
Pre-term birth
Postnatal:
Meningitis
Severe neonatal jaundice
Head injury
What are the types of cerebral palsy?
Spastic: hypertonia (increased tone) and reduced function resulting from damage to upper motor neurones
Dyskinetic: problems controlling muscle tone, with hypertonia and hypotonia causing athetoid movements and oro-motor problems - caused by problems with basal ganglia
Ataxic: problems with coordinated movement resulting from damage to the cerebellum
Mixed: spastic / dyskinetic / ataxic features
What is spastic CP also known as?
Pyramidal CP
Dyskinetic CP is also known as?
Athetoid CP and extrapyramidal CP
What limbs can be affected by cerebral palsy?
Monoplegia: one limb
Hemiplegia: one side of body
Diplegia: four limb are affected but mostly legs
Quadriplegia: four limb affected but more severely (seizures, speech distubance and other impairments)
What children are at risk of developing cerebral palsy?
Hypoxic-ischaemic encephalopathy
What are some signs or symptoms of cerebral palsy?
- Failure to meet milestones
- Increased / decreased tone, generally or specific limbs
- Hand preference below 18 months
- Proglems with coordination or speech
- Feeding or swallowing problems
- Learning difficulties
What information about the cerebral palsy do the following gaits give you?
Hemiplegic / diplegic: UMN lesion
Broad based gait / ataxic: cerebellar lesion
High stepping gait: foot drop / lower motor neurone lesion
Waddling gait: pelvic muslce weakness due to myopathy
Antalgic gait: limp = localised pain
What are the features of UMN lesions vs LMN lesions?
What is a hemiplegic / diplegic gait?
Increase in muscle tone and spasticity in legs
Leg is extended with plantar flexion of feet and toes - swing leg around in large semicircle due to lack of space
What is the differential diagnosis for an UMN lesion?
Acquired brain injury / tumour
What are some complications and associated conditionstocerebral palsy?
Learning disability
Epilepsy
Kyphoscoliosis
Muscle contractures
Hearning and visual impairment
GORD
What is the multi-disciplinary management of CP?
Physio to stretch and stregthen muscles, prevent contractures
OT help pts manage everyday activities - adaptations e.g. rails or hoist
Speech and language therapy for speech and swallowing (may require NG tube or PEG tube)
Dieticians to help ensure they meet nutritional requirements
Orthopaedic surgeons - profectures to lengthen tendons (tenotomy)
Paediatricians - regularly see child to optimise medications
Social workers for benefits and support
Charities and support groups to share info on condition
What medications are used in CP?
Muscle relaxants e.g. baclofen for muscle spasticity and contractures
Anti-epileptic drugs for seizures
Glycopyrronium bromide for excessive drooling
What is a squint?
Misalignment of the eyes also known as stabismus - images don’t match and person will experience double vision
How is a squint managed in childhood?
Brain copes by reducing signal from less dominant eye - so “lazy eye” becomes progressively more disconnected from the brain - can become worse called amblyopia
What are concomitant squints due to?
Differences in control of the extra ocular muscles - severity here can vary
What are paralytic squints due to?
Paralysis in one or more of the extra ocular muscles
Define the following terms:
Strabismus
Amblyopia
Esotropia
Exotropia
Hypertropia
Hypotropia
Strabismus: the eyes are misaligned
Amblyopia: the affected eye becomes passive and has reduced function compared to the other dominant eye
Esotropia: inward moving squint
Exotropia: outward moving squint
Hypertropia: upward moving affected eye
Hypotropia: downward moving affected eye
What are the causes of squints?
Idiopathic
Hydrocephalus
Cerebral palsy
Space occupying lesions, for example retinoblastoma
Trauma
What to examine for in a squint?
General inspection
Eye movements
Fundoscopy (or red reflex) to rule out retinoblastoma, cataracts and other retinal pathology
Visual acuity
What is Hirschberg’s test?
Shine a pen torch at the patient from 1 meter away when they look at the - look at reflection of light on cornea (should be central - deviation indicates squint) make a note of results
What is the cover test?
Cover one eye and ask patient to focus on object in front of them then switch and watch movement of previously covered eye - if this eye moves inwards then it had drifted outwards (exotropia) and if it moves outwards then it means it had drifted inwards when covered (esotropia)
When does treatment for a squint need to begin?
Before 8 years (as visual fields are developing)
What is the management of a squint?
Occlusive patch - cover the good eye and force the weaker to develop
Alternatively, atropine drops can be used in good eye, causing vision in that eye to be blurred
Management is coordinated by an ophthalmologist - important to treat any underlying pathology e.g. cataracts
Refractive errors can be corrected by corrective lenses
What is hydrocephalus?
Cerebrospinal fluid building up abnormally within the brain and spinal cord (due to over-production of a problem with draining or absorbing)
How many ventricles are there in the brain?
Four: 2 lateral, the third and fourth (these contain CSF)
What is the purpose of the CSF?
Cushion for brain tissue
Where is CSF created?
In the four choroid plexuses (one in each ventricle) by the walls of the ventricles
Where is CSF absorbed into the venous system?
By the arachnoid granulations
What is the most common cause of hydrocephalus?
Aqueductal stenosis leading to insufficient drainage of CSF - cerebral aqueduct that connects the third and fourth ventricle is narrowed (causing CSF to build up in the lateral and third ventricles)
What are some other causes of hydrocephalus?
Arachnoid cysts - these block the outflow of CSF if large enough
What is an Arnold-Chiari malformation?
Cerebellum herniates downwards through the foramen magnum blocking the outflow of CSF
What is a final cause of hydrocephalus?
Chromosomal abnormalities and congenital malformations can cause obstruction to CSF drainage
How does hydrocephalus present?
Large occipital-frontal circumference (as the cranial bones are not yet fused at the sutures)
Bulging anterior fontanelle
Poor feeding and vomiting
Poor tone
Sleepiness
What is the treatment for hydrocephalus?
Ventriculoperitoneal shunt which drains CSF from the ventricles into another body cavity (usaully teh peritoneal cavity)
Catheter is placed through a hole in the skull at the back of the neck and into one of the ventricles - this then runs under the skin into the peritoneal cavity
What are the complications of VP shunts?
Infection
Blockage
Excessive drainage
Intraventricular haemorrhage during shunt related surgery
Outgrowing them (replacement needed every 2 years)
What is craniosynostosis?
Skull sutures close prematurely (causing abnormal head shapes and restriction in growth of the brain)
What happens in untreated craniosynostosis?
Raised intracranial pressure - developmental delay, cognitive impairment, vomiting, irritability, visual impairment, neurological symptoms, seizures
What are the different types of synostosis and resulting head shapes?
What are some other presenting features of craniosynostosis?
Anterior fontanelle closure before 1 year of age
Small head in proportion to the body
What is the first line investigation for craniosynostosis?
What is used to confirm the diagnosis?
Skull x-ray
CT head with bone views to confirm the diagnosis or exclude if doubt on x-ray
What is the management of craniosynostosis?
Mild cases = monitored and followed up
Severe = surgical reconstruction of the skull
Lifelong scar on scalp where surgery was performed
What are plagiocephaly and brachycephaly?
Common conditions which cause abnormal head shapes in otherwise normal healthy babies
What is plagiocephaly?
Flattening of one area of baby’s head (plagio = oblique or slanted)
What is brachycephaly?
Flattening at the back of the head (brachy translates as short - short head back to front)
What is the cause of plagiocephaly or brachycephaly?
Baby has a tendency to rest head on a particular point - resulting in skull bones and sutures moulding with gravity (positional plagiocephaly)
Why is plagiocephaly more common?
Parents are advised to rest babies on their backs to reduce the risk of sudden infant death syndrome?
What is the typical presentation of plagiocephaly and brachycephaly?
Baby aged 3-6 months with abnormal shaped head (often with preferance to sleep on one side)
What is the management of plagio/brachycephaly?
Exclude craniosynotosis with thorough history and properly palpating sutures
Look for congenital muscular torticollis - a shortening of SCM muscle on one side (physio can help)
Reassurance - as vast majority of cases the head shapre returns to normal
What measures can be taken to encourage the baby to avoid resting on the flattened area?
Position on rounded side to sleep
Supervised tummy time
Using rolled towels or other props
Minimising time in pushchairs and car seats
What are plagiocephaly helmets?
Treatment option - some limitations in that they need to be used for the vast majority of the day and can lead to skin problems (contact dermatitis) and psychosocial problems - not routinely available on NHS
What is muscular dystrophy?
Genetic condition causing gradual weakening and wasting of muscles
What is the main muscular dystrophy to know for the purpose of exams?
Duchennes muscular dystrophy
What are the other types of muscular dystrophy?
Beckers muscular dystrophy
Myotonic dystrophy
Facioscapulohumeral muscular dystrophy
Oculopharyngeal muscular dystrophy
Limb-girdle muscular dystrophy
Emery-Dreifuss muscular dystrophy
What is Gower’s sign?
The
Who is involved in the management of muscular dystrophy?
OT
Physio
Medical appliances (wheelchairs and braces)
Surgial input for spinal scoliosis or heart failure
What causes Duchennes muscular dystrophy?
When do patients with Duchennes present? What is the prognosis?
3-5 years present (weakness around pelvis)
Eventually all muscles affected
Wheelchair bound by teenage
Life expectancy of 25-35 years with good management of cardiac and resp complications
What treatment can be given for Duchennes?
Oral steroids - slows progression of muscle weakness by 2 years
Creatine supplementation gives a slight improvement in muscle strength
What is Beckers muscular dystrophy?
Similar to Duchennes however dystrophin gene is less severely affected.
Clinical course is less predictable (symptoms appear at 8-12 years) some require wheelchairs in late 20s or 30s - others are able to walk with assistance into later adulthood
Management is similar to Duchennes
What are the typical features of myotonic dystrophy?
- Progressive muscle weakness
- Prolonged muscle contractions
- Cataracts
- Cardiac arrhythimas
What is facioscapulohumeral muscular dystrophy?
Muscular dystrophy which presents in childhood with weakness around the face progressing to the shoulders and arms
How does facioscapulohumeral muscular dystrophy present?
Sleeping with eyes slightly open and pursing their lips
Unable to blow their cheeks out without air leaking from their mouth
What is oculopharyngeal muscular dystrophy?
Presents in late adulthood with weakness of the ocular muscles (around the eyes) and pharynx (around the throat)
How does ocularpharyngeal muscular dystrophy present?
Bilateral ptosis
Restricted eye movement
Swallowing problems
Muscles around the limb girdles are also affected to varying degrees
What is limb-girdle muscular dystrophy?
Presents in teenage years with progressive weakness around the limb girdles (hips and shoulders)
What is Emery-Dreifuss muscular dystrophy?
Presents in childhood with contractures mostly in the elbows and ankles (shortening of muscles and tendons that restrict the range of movement in limbs)
Progressive weakening and wasting of muscles, starting with upper arms and lower legs
What is spinal muscular atrophy (SMA)?
Rare autosomal recessive condition which causes a progressive loss of motor neurones (causing progressive muscular weakness)
What are the features of SMA?
Affects lower motor neurones in the spinal cord
Lower motor neurone signs = fasciculations, reduced muscle bulk, reduced tone, reduced power, reduced / absent reflexes
What are the different categories of SMA?
SMA type 1 = onset in first few months of life, progess to death within 2 years
SMA type 2 = onset in first 18 months, never walk, survive into adulthood
SMA type 3 = onset after first year of life - walk without support - loose that ability. Resp muscles less affected and life expectancy is close to normal
SMA type 4 = onset in 20s - most retain ability to walk short distances but require wheelchair for mobility - everyday tasks can lead to significant fatigue. Resp muscles and life expectancy are not affected
What is the management of SMA?
No cure - management is supportive
Physio - to max the strength in the muscles and retain resp function (split, braces and wheelchairs used)
Resp support with non-invasive ventilation to prevent hypoventilation and resp failure (especially during sleep)
SMA type 1 may require tracheostomy with mechanical ventilation
PEG feeding when swallowing unsafe
