NEUROLOGY PAEDS Flashcards
Causes of syncope
Primary - dehydration - missed meals - extended standing in a warm environment - vasovagal response to a stimuli e.g. blood, pain, surprise Secondary - hypoglycaemia - dehydration - anaemia - infection - anaphylaxis - arrhythmias - valvular heart disease - hypertrophic obstructive cardiomyopathy
Key points in taking a syncope history
Features that distinguish between syncopal episode and seizure
After exercise? (More likely to be secondary cause)
Triggers?
Concurrent illness? Fever/signs of infection?
Injury secondary to the faint? Do they have a head injury?
Associated cardiac symptoms, such as palpitations or chest pain?
Associated neurological symptoms?
Seizure activity?
FH - cardiac problems? Sudden death?
Syncope: Examinations
- Any physical injuries as a result of the faint
- concurrent illness? Infection?
- Neurological examination
- Cardiac examination - pulse, HR, rhythm, heart sounds
- Lying and standing BP
Syncope: Investigations
ECG - arrhythmias, QT intervals, long QT syndrome
24 hour ECG
Echocardiogram (if structural heart disease suspected)
Bloods - incl. FBC, electrolytes, blood glucose
Generalised Tonic clonic seizures: characteristics and treatment
LOC, tonic (muscle tensing), clonic (muscle jerking) movements
May be associated: tongue biting, incontinence, groaning and irregular breathing
Prolonged post-ictal period
1st line: SODIUM VALPORATE
2nd line: LAMOTRIGINE, or CARBAMAZEPINE
Focal seizures: characteristics and treatment
Start in temporal lobes
Affect hearing, speech, memory, emotions
Various ways they can present: Hallucinations, memory flashbacks, Deja vu, doing strange things on autopilot
1st line: CARBAMAZEPINE or LAMOTRIGINE
2nd line: SODIUM VALPROATE or LEVETIRACETAM
!! The reverse of generalised tonic clonic medications !!
Absence seizures: characteristics and treatment
Typically happen in children. Pt becomes blank, stares into space, the abruptly returns
During episode unaware of surroundings and wont respond
Most pt stop having these seizures when older
1st line: SODIUM VALPROATE or ETHOSUXIMIDE
Atonic seizures: characteristics and treatment
Aka drop attacks
Brief lapse in muscle tone
Usually last less than 3 mins
Typically begin in childhood, may be indicative of LENNOX-GASTAUT SYNDROME
1st line: SODIUM VALPROATE
2nd line: LAMOTRIGINE
Myoclonic seizures: characteristics and treatment
Sudden brief muscle contractions, patient remains awake
Typically happen in children as part of juvenile myoclonic epilepsy
1st line: SODIUM VALPROATE
Other options: LAMOTRIGINE, LEVETIRACETAM, TOPIRAMATE
Infantile spasms: characteristics and treatment
aka WEST SYNDROME Rare, starts in infancy (around 6m) Clusters of full body spasms Poor prognosis - 1/3 die by 25, however 1/3 are seizure free Can be difficult to treat
1st line: PREDNISOLONE, VIGABATRIN
Febrile convulsions characteristics
Occur in children while they have a fever
Not caused by epilepsy or any underlying neurological pathology
Occur in children between the age of 6 months - 5 years
Epilepsy: Investigations
!! Good history is key !! To establish if actually seizures
N.B. Children are allowed one simple seizure before being investigated for epilepsy
EEG
MRI brain - check for any structural problems
ECG - to exclude problems with the heart
Blood electrolytes - Na, K, Ca, Mg
Blood glucose - hypoglycaemia and DM
Blood cultures, urine cultures, LP - where sepsis, encephalitis, or meningitis suspected
Epilepsy: General advice
Advise about safety precautions, managing and reporting further seizures
Avoid situations which may put the child in danger
- take showers rather than baths
- very careful with swimming unless seizures well controlled and closely supervised
- be cautious with heights, traffic, any heavy, hot or electrical equipment
- older teenagers should avoid driving
SODIUM VALPROATE: MOA and SE
Increases activity of GABA - which has a relaxing effect on the brain
SE: Teratogenic, liver damage and hepatitis, hair loss, tremor
- avoided in girls unless no other suitable alternative
CARBAMAZEPINE: MOA and SE
Blocks Na Channels and reduces cell excitability
SE: Agranulocytosis (drug induced blood disorder, reduction in no. Of white blood cells), aplastic anaemia, induces P450 system in the liver - so many drug interactions
PHENYTOIN: MOA and SE
Na channel blocker
SE: Folate and VitD deficiency, Megaloblastic anaemia (folate deficiency), osteomalacia (VitD deficiency)
ETHOSUXIMIDE MOA and SE
Antagonism of pot-synaptic VG Ca channels
SE: Night terrors, rashes
LAMOTRIGINE
SE: STEVENS-JOHNSON SYNDROME or DRESS syndrome (life threatening skin reaches)
Leukopenia
Acute management of seizures
Put patient in safe position
Place in recovery position if possible
Put something soft under head
Remove obstacles that could lead to injury
Make note of time at start and end of seizure
Call ambulance if lasting more than 5 mins or first seizure
STATUS EPILEPTICUS: definition
Seizures lasting > 5 mines
Or
> 3 seizures in 1 hour
STATUS EPILEPTICUS: Management in hospital
ABCDE approach
IV LORAZEPAM - repeated after 10 mins if seizure continues
When seizure persists the final step is infusion of IV PHENOBARBITAL or PHENYTOIN
STATUS EPILEPTICUS: medical options in the community
Buccal midazolam
Rectal diazepam
Simple and complex FEBRILE CONVULSIONS
Simple: generalised, tonic clonic seizures. Last <15 mins, only occur once during a febrile illness
Complex: consist of partial or focal seizures, last > 15 mins or occur multiple times during the same febrile illness
Febrile convulsions: Differential diagnoses
Epilepsy
Meningitis, encephalitis, or another neurological infection
Intracranial space occupying lesion (e.g. tumour, haemorrhage)
Syncopal episode
Electrolyte abnormalities
Trauma (always think about non-accidental injury)
Breath holding spells: Types
Cyanotic breath holding spells
Pallid breath holding spells (aka anoxic seizures)
Breath holding spells: what are they
Involuntary episodes during which a child holds their breath, usually triggered by something upsetting or scaring them
Typically occur between 6 and 18 months
Cyanotic breath holding spells
Occur when a child is really upset, worked up and crying
They become Cyanotic and lose consciousness
Within 1 min they regain consciousness and start breathing
They can be a bit tired and lethargic after an episode
Reflex anoxic seizures
Occur when a child is startled
Vagus nerve sends strong signals to the heart that causes it to stop beating
Child will suddenly go pale, lose consciousness and may start to have seizure like muscle twitching
Within 30 seconds the heart restarts and the child becomes conscious again
Breath holding spells: management
Exclude other pathology
Reassure parents
There has been a link to iron deficiency anaemia - treating this can help
Causes of headaches in children
Tension headaches Migraines ENT infections Analgesic headache Problems with vision RICP Brain tumours Meningitis Encephalitis CO poisoning
Management of Migraines in children
Rest, fluids, low stimulus environment Paracetamol Ibuprofen Sumatriptan Antiemetics (e.g. domperidone)
Migraine prophylaxis options
Propranolol (avoid in asthma)
Pizotifen (often causes drowsiness)
Topiramate (teratogenic)
Abdominal Migraine
More common in children than adults
Central abdominal pain lasting > 1 hour
May be associated: N&V, Anorexia, headaches, pallor
May predispose to head migraines
Causes of cerebral palsy
Antenatal - maternal infections, trauma during pregnancy
Perinatal - Birth asphyxia, pre-term birth
Post-natal - Meningitis, severe neonatal jaundice, head injury
Types of cerebral palsy
SPASTIC - hypertonia and reduced function from damage to UMN
DYSKINETIC - problems with controlling muscle tone, with hypertonia and hypotonia, causing athetoid movements and oro-motor problems. Result of damage to the basal ganglia
ATAXIC - problems with coordinated movement resulting from damage to the cerebellum
MIXED - mix of spastic, dyskinetic and/or ataxic features
Patterns of spastic cerebral palsy
Monoplegia - one limb affected
Hemiplegia - one side of body affected
Diplegia - four limbs are affected, but mostly the legs
Quadriplegia - four limbs affected more severely, often with seizures, speech disturbance and other impairments
What does child with cerebra palsy’s gate tell us?
Hemiplegic / diplegic gait: indicated UMN lesion
Broad based gait /ataxic gait: indicates cerebellar lesion
High stepping gait: indicates foot drop or LMN lesion
Waddling gait: Indicates pelvic muscle weakness due to myopathy
Antalgic gait: indicates localised pain
Cerebral palsy: Complications and associated conditions
Learning disability Epilepsy Kyphoscoliosis Muscle contractures Hearing and visual impairments Gastroenteritis-oesophageal reflux
Cerebral Palsy: Management
MDT APPROACH: Physio, OT, SALT, Dieticians, orthopaedic surgeon, Paediatricans, social workers, support groups
Surgery may be able to release contractures or lengthen tendons
Muscle relaxants (e.g baaclofen)
Anti-epileptics
Glycopyrronium bromide (for excessive drooling)
Causes of squints
Squint in otherwise healthy children are usually idiopathic
Other causes:
- hydrocephalus
- cerebral palsy
- space occupying lesions e.g. retinoblastoma
- trauma
What is ‘Hirschberg’s test’?
Shine pen torch at the patient from 1m away
When they look at it observe the reflection of the light source on their cornea
Reflection should be central and symmetrical
Deviation from the centre —> squint
What is the ‘cover test’?
Cover one eye and ask the patient to focus on an object in front of them
Move the cover across to the other eye and watch the movement of the previously covered eye
If this eye moves inwards (means it had drifted outwards when covered) = EXTROPIA
If it moves outwards (means it had drifted inwards when covered) = ESOTROPIA
Management of squints
Visual fields develop until the age of 8 years - so treatment needs to start before then
Delayed treatment increases risk of the squint becoming permanent
OCCLUSIVE PATCH. - can be used to cover the good eye and force the weaker eye to develop.
ATROPINE DROPS - in the good eye - making it blurry
Management coordinated by ophthalmologist
Treat any underlying pathology
Hydrocephalus: definition
CSF build up within brain and spinal cord
Either due to
- over production of CSF
- problem with draining or absorbing CSF
Normal CSF anatomy/physiology
4 ventricles
- 2 x lateral ventricles
- third ventricle
- fourth ventricle
CSF is created in the 4 x choroid plexuses (1 in each ventricle) and by the walls of the ventricles
CSF is absorbed into the venous system by the arachnoid granulations
Causes of HYDROCEPHALUS
CONGENITAL:
- aqueductal stenosis —> insufficient drainage of CSF (the cerebral aqueduct that connects the third and fourth ventricles is stenosed - blocking normal flow of CSF out the 3rd ventricle —> build up in the lateral and third ventricles) - most common
- Arachnoid cysts can block the flow of CSF if they area large enough
- Arnold-chiari malformation (cerebellum herniates downwards through the foremen magnum)
- chromosomal abnormalities and congenital malformations
Hydrocephalus: Presentation
Cranial bones don’t fuse at sutures until about 2 yrs
Babies with hydrocephalus before this age present with enlarging head and rapidly increasing head circumference (occipito-frontal circumference)
Other signs
- budging anterior fontanelle
- poor feeding and vomiting
- poor tone
- sleepiness
Ventriculoperitoneal shunt
VP shunt can be placed to drain CSF from the ventricles to another body cavity (usually perionteal cavity)
Valve helps to regulate the amount of CSF that drains from the ventricles
VP shunt complications
Infection
Blockage
XS drainage
Intraventricular haemorrhage (during shunt related surgery)
Outgrowing them (generally need replacing every 2 years as child grows)
Craniosynostosis: definition
When sutures close prematurely —> abnormal head shape and restriction to growth of brain
Craniosynostosis: if left untreated
Will lead to raised intracranial pressure Developmental delay Cognitive impairment Vomiting Irritability Visual impairment Neurological symptoms and seizures
Presentation of CRANIOSYNOSTOSIS
ABNORMAL HEAD SHAPE - Head shape depends on the affected cranial suture (Saggital synostosis / coronal synostosis / metopic synostosis / lambdoid synostosis )
Anterior frontanelle closure before 1 year of age
Small head in proportion to body
Craniosynostosis: investigation and management
1st line: skull X-ray
2nd line: CT head with bone views can be used to confirm diagnosis or exclude it if any double on X-ray
Management: mild cases monitored and followed up over time. More severe cases require surgical reconstruction
Prognosis usually good with proper management
Plagiocephaly and brachycephaly
Common conditions which cause abnormal head shapes in otherwise healthy babies
Plagio- = translates to oblique/slanted.
- Plagiocephaly refers to flattering of one area of baby’s head
Brachy- = translates to short
- Brachycephaly refers to flattering at the back of the head, resulting in a short head from front to back
Management of Plagiocephaly/brachycephaly
Exclude: Craniosynostosis (palpate sutures)
Check for congenital muscular torticollis (shortening of SCM muscle on one side) - may be the reason child always lies on one side of head
Usually resolves as child grows
Measures to avoid child lying on flatterened area
- position on rounded side for sleep
- supervised tummy time
- using rolled towels or other props
- minimising time in push chairs and car seats
PHAGIOCEPHALY HELMETS - although have limitations and can cause skin problems and psychosocial problems. Not provided on NHS
MUSCULAR DYSTROPHY: what is it
MS is an umbrella term for genetic conditions that cause a gradual weakening and wasting of muscles
Types of muscular dystrophy
Duchennes muscular dystrophy (the main one)
Others: beckers MD, myotonic MD, Facioscapulohumeral MD, oculopharyngeal MD, Limb-girdle MD, emery-Dreifuss MD
Gower’s sign
Children with proximal muscle weakness use a specific technique to stand up from a lying position = Gower’s sign
They get onto their hands and knees then push their hips up and backwards like the “downward dog” pose
They shift weight backwards and transfer their hands to their knees
Whilst keeping legs quite straight they walk hands up their legs
(They do this because the muscles around the penis are not strong enough to get their body erect without help of their arms)
Muscular dystrophy: Management
No curative treatment
MDT - OT, physio, medical appliances (e.g. wheelchairs)
Surgical and medical management of complications e.g. spinal scoliosis and HF
STEROIDS have been shown to slow progression of muscle weakness by as much as 2 years
CREATINE supplementation can give slight improvement
Genetics of duchennes muscular dystrophy
X linked recessive
Defective gene codes for dystrophin (a protein that holds muscles together at the cellular level)
Females usually not affected as they have a spare copy of the gene
Beckers Muscular dystrophy
Myotonic dystrophy
Fascioscapulohumeral MD
Oculopharyngeal MD
Limb-girdle MD
Emery-Dreifuss MD
Spinal muscular Atrophy (SMA): what is it
Rare autosomal recessive condition causing progressive loss of motor neurones, causing progressive muscular weakness
Affects LMN in the spinal cord —> LMN signs
LMN signs seen in SMA
Fasiculations, reduced muscle bulk, reduced tone, reduced power, reduced/absent reflexes
Spinal muscular atrophy: categories
SMA type 1: onset in first few months of life, usually progressing to death within 2 years
SMA type 2: onset within first 18 months. Most never walk but survive into adulthood
SMA type 3: onset AFTER the first year of life. Most walk without support, but subsequently lose that ability. Respiratory 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 a wheelchair for mobility. Everyday tasks cause severe fatigue. Respiratory muscles and life expectancy not affected.
Spinal muscular atrophy: management
No cure
Supportive and MDT approach
Physio, respiratory support (e.g. non-invasive ventilation, Type 1 SMA may require tracheostomy and mechanical ventillation)
PEG feeding if swallow unsafe
What is Benign Rolandic epilepsy?
seen between the ages of 4-12
focal seizure, involving their face, drooling, and one side or one limb twitching (that can sometimes progress to secondary generalised seizure)
Before or after bedtime. It is common for children to be sleep deprived.
Good prognosis - stop by adolescence
