Paediatric neurology Flashcards
What is a febrile seizure ?
A seizure that occurs in children with a high fever.
What age group suffer from febrile seizures?
6 mnths -> 5 years
What are simple febrile convulsions ?
- Generalised, tonic clonic seizures
- Last <15 mins
- Occur once during a single febrile illness
What are complex febrile convulsions ?
Any of the following :
- Partial or focal seizures
- Last >15 mins
- Multiple occur during the same febrile illness
-Febrile status epilepticus = >30 mins
What needs to be excluded before febrile convulsions are diagnosed ?
- Epilepsy
- Meningitis, encephalitis or other neurological infection
- Intracranial space occupying lesions
- Syncopal episode
- Electrolyte abnormality
- Traume
How are febrile convulsions managed ?
- Identify and treat underlying infection
- Fever : paracetamol and ibuprofen
- Period of observation
altered consciousness
altered cognition
unusual behaviour
acute onset of neurological symptoms
acute onset of focal seizures
fever
encephalitis
how is encephalitis investigated
LP for CSF with viral PCR testing
CT if LP is CI
how is encephalitis managed
Aciclovir for HSV and VZV
Ganciclovir for CMV
When would a child be admitted with a febrile seizure
-> First seizure or features of complex seizure
Benign Rolandic seizures (benign focal epilepsy) : presentation, EEG
- 3 to 10 yrs
- Boys and during sleep
- Tonic seizure : wake up on floor or with messy sheets
- EEG : centro-temporal spikes
Infatile spasms (west syndrome) : presentation, diagnosis
- Boys : 4-8 mnths
- Presentation : Salam attacks (flexion of head, trunk, arms, follwed by extension of arms).
- Lasts 1-2 seconds, repeated up to 50 times.
- EEG : hypsarrhymthmia
Inheritance of juvenile myoclonic epilepsy
AD
Management of a generalised Tonic-Clonic seizure
- First line: sodium valproate
- Second line: lamotrigine or carbamazepine
Management of focal seizures
- First line: carbamazepine or lamotrigine
- Second line: sodium valproate or levetiracetam
Management of absence seizures
First line: sodium valproate or ethosuximide
Management of atonic seizures (drop attacks
- First line: sodium valproate
- Second line: lamotrigine
Management of myoclonic seizures
- First line: sodium valproate
- Other options: lamotrigine, levetiracetam or topiramate
Management of west syndrome
- Prednisolone
- Vigabatrin
How does SV work and what are 4 notable SE
- Increasing activity of GABA
- Teratogenic (avoided in girls)
- Liver damage and hepatitis
- Hair loss
- Tremor
3 notable SE of carbamazepine
- Agranulocytosis
- Aplastic anaemia
- Induces the P450 system so many drug interactionms
3 notable SE of phenytoin
- Folate and vitamin D deficiency
- Megaloblastic anaemia (folate deficiency)
- Osteomalacia (vitamin D deficiency)
2 notable SE of ethosuximide
Night tremors
Rashes
2 notable SE of lamotrigine
- SJS or DRESS
- Leukopenia
How is status epilepticus defined
- > Seizure >5 minutes, 2 or more seizures without regaining consciousness
How is status epilepticus managed
- Secure the airway
- Give high-concentration oxygen
- Check blood glucose levels
- Gain IV access
- IV lorazepam, repeated after 10 minutes if the seizure continues
- If seizure persists : IV phenytoin or phenobarbital
Medical options for management of status epilepticus in community
Buccal midazolam
Rectal diazepam
What can cause CP
- Antenatal : maternal infection, trauma in pregnancy
- Perinatal : birth asphyxia, prematurity
- Postnatal : meningitis, severe neonatal jaundice, head injury.
What are the 4 types of CP ?
- Spastic: hypertonia 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. This is the result of damage to the basal ganglia.
- Ataxic: problems with coordinated movement resulting from damage to the cerebellum
- Mixed: a mix of spastic, dyskinetic and/or ataxic features
What are the 4 patterns of spastic CP
- Monoplegia: one limb affected
- Hemiplegia: one side of the body affected
- Diplegia: four limbs are affects, but mostly the legs
- Quadriplegia: four limbs are affected more severely, often with seizures, speech disturbance and other impairments
What can the gait tell you about a child with CP
- Hemiplegic / diplegic gait: indicates an upper motor neurone lesion
- Broad based gait / ataxic gait: indicates a cerebellar lesion
- High stepping gait: indicates foot drop or a lower motor neurone lesion
- Waddling gait: indicates pelvic muscle weakness due to myopathy
- Antalgic gait (limp): indicates localised pain
If not idiopathic, what are 4 underlying causes of strabismus ?
- Hydrocephalus
- Cerebral palsy
- Space occupying lesions, for example retinoblastoma
- Trauma
What can be done to manage strabismus ?
-> Start before 8 yrs of age.
-> Cover the good eye with an occlusive patch, forcing the weaker eye to develop.
What 6 definitions are used when described a strabismus ?
- Strabismus: the eyes are misaligned
- Amblyopia: the affected eye becomes passive and has reduced function compared to the other dominant eye
- Esotropia: inward positioned squint
- Exotropia: outward positioned squint
- Hypertropia: upward moving affected eye
- Hypotropia: downward moving affected eye
How can a cover test be used to define a strabismus ?
- Cover one eye and ask the pt to focus on an object in front of you
- Move the cover to the opposite eye
- Watch movement of previously covered eye
- If the eye moves in, it drifted outwards when covered = EXOTROPIA
- If the eye moves outward, it drifted inwards when covered = ESOTROPIA
What is the most common cause of hydrocephalus in children ?
- Aqueductal stenosis -> the cerebral aqueduct connecting third and fourth ventricle is stenosed blocking outflow of CSF
How will hydrocephalus present in children
- Rapidly increasing head circumference (sutures have not fused)
- Bulging anterior fontanelle
- Poor feeding and vomiting
- Poor tone
- Sleepiness
Wat the mainstay of treatment for hydrocephalus ?
- Ventriculoperitoneal (VP) shunt
- CSF is drained from the ventricles into the peritoneal cavity
Give 5 complications of a VP shunt
- Infection
- Blockage
- Excessive drainage
- Intraventricular haemorrhage during shunt related surgery
- Outgrowing them (they typically need replacing around every 2 years as the child grows)
What is craniosynostosis and its complication if left untreated
- > Premature closure of skull sutures
- > Complications : raised ICP = developmental delay, cognitive impairment, vomiting, irritability, visual impairment, neurological symptoms and seizures
what is the inheritance of Duchennes muscualr dystrophy ?
- X-linked recessive
- Due to defective gene for dystrophin on X chromosome required for normal muscular function
How does duchennes muscular dystrophy present, what is a positive finding and how is progression slowed ?
- Boys, aged 3-5 with weakness around the pelvis
- Gower’s sign = Using hands on legs to help them stand up due to proximal muscle weakness
- Oral steroids can help
what is the difference in presentation between Duchenne’s and Beckers muscular dystrophy
- Beckers presents later (around 8-12 yrs)
- Less severe
Give 4 features of myotonic dystrophy
- Progressive muscle weakness
- Prolonged muscle contractions = pt unable to let go after shaking hand etc
- Cataracts
- Cardiac arrhythmias
What is spinal muscular atrophy ?
- > Autosomal recessive condition causing progressive loss of motor neurones = progressive muscular weakness
- > Affects LMN !
Most common complication of meningitis
Sensorineural hearing loss
how will herpes simplex encephalitis present on CT and what will be present in the history?
- CT : bilateral hyperdensities of the middle temporal gyri
- History : confusion, fever, general malaise. + possible focal temporal seizures - rising feeling in stomach before generalised seizure
