Paediatric Neurology Flashcards
What are causes of cerebral palsy?
Antenatal:
Maternal infection
Trauma during pregnancy
Perinatal:
Birth asphyxia
Preterm birth
Postnatal:
Meningitis
Severe neonatal jaundice
Head injury
What are the types of cerebral palsy?
Spastic (hypotonia - LMN)
Dyskinetic (hyper and hypotonia (damage to basal ganglia)
Ataxic (problems with co-ordination - damage to cerebellum)
Mixed
How can cerebral palsy present?
failure to meet milestones
increased/decreased tone
hand preference <18m
problems with co-ordination/speech/walking
Learning difficulties
What is hydrocephalus?
Build up of CSF in the brain and spinal cord
What is the most common congenital cause of hydrocephalus? What are other causes?
Most common= Aqueductal stenosis
Other:
Arachnoid cysts
Arnold-Chiari Malformation
Chromosomal abnormalities
What is aqueductal stenosis?
Cerebral aqueduct that connects the 3rd and 4th ventricles = stenosed
How does hydrocephalus present?
Rapidly increasing head circumference Bulging fontanelle Poor feeding Vomiting Poor tone Sleepiness
How is hydrocephalus managed?
Ventriculoperitoneal shunt
What are complications of a ventriculoperitoneal shunt?
Infection Blockage Excessive drainage Intraventricular haemorrhage Outgrowing them
What is craniosynostosis?
Skull sutures close prematurely - results in a normal head shape
What is plagiocephaly and brachycephaly?
Plagiocephaly = flattening of one area of baby’s head
Brachycephaly = flattening of back of baby’s head
How is craniosynostosis investigated?
Skull XR
-CT head with bone views is used to confirm the diagnosis or exclude it if there is doubt on the xray.
How does Duchenne’s muscular dystrophy present?
Weakness in pelvic muscles
Gower’s sign +ve (use hands on legs to help them stand up)
-Children may have bulky-appearing muscles, as degenerated muscle is replaced by fat.
-Parents may notice that the child ‘slips through their hands’ when they pick them up (due to loose muscles in the shoulder)
-X-linked recessive
What is the most common ocular malignancy in children ad how does it present?
Retinoblastoma
Loss of red reflex
Strabismus
Vision problems
A 2-month-old girl is brought to the emergency department with her father. Her father reports a 2 hour history of a non-blanching rash over her body. He reports that in the past 24 hours she has seemed unsettled, refusing feeds and crying more often than usual.
Observations reveal a temperature of 38.6ºC and examination confirms the non-blanching petechial rash with generalised hypotonia. Examination also reveals a bulging anterior fontanelle.
Given the most likely diagnosis and following appropriate investigation, which of the following would be the most appropriate initial management option?
A. Intravenous (IV) cefotaxime
B. IV cefotaxime and IV amoxicillin
C. IV amoxicillin and IV dexamethasone
D. IV cefotaxime and IV dexamethasone
E. IV cefotaxime, IV amoxicillin and IV dexamethasone
B. IV cefotaxime and IV amoxicillin
Do not use corticosteroids in children younger than 3 months with suspected or confirmed bacterial meningitis
IV cefotaxime is useful to cover for pneumococcal and haemophilus influenza. However, children less than 3 months are at risk of listeria monocytogenes as a cause of bacterial meningitis. Therefore, without amoxicillin to cover for this, there would be inadequate cover.
When would you give steroids for suspected/confirmed meningitis?
-frankly purulent CSF
-CSF white blood cell count greater than 1000/microlitre
-raised CSF white blood cell count with protein concentration greater than 1 g/litre
-bacteria on Gram stain
what are the antibiotics given for meningitis (<3m and >3m)?
< 3 months: IV amoxicillin (or ampicillin) + IV cefotaxime
> 3 months: IV cefotaxime (or ceftriaxone)
A 4 year old boy is being brought to the Emergency Department by ambulance. He has been seizing for 5 minutes at home and was given a dose of buccal midazolam by the paramedics. He has been brought to the hospital and IV access has been established. He has been seizing for 15 minutes now.
What is the next most likely medication to be given?
A. PO Levetiracetam
B. IV Phenytoin
C. IV Lorazepam
D. Buccal Midazolam
E. Rapid sequence induction
C. IV Lorazepam: 0.1mg/kg is the next medication most likely to be started now that IV access has been established. The child is in status epilepticus.
-Not B: IV lorazepam should be trialled before phenytoin. It is important that senior support is sought before starting children on phenytoin.
A 7-year-old girl presents to the emergency department accompanied by her mother. While trying to wake her up this morning, her mother noticed her face twitching and mouth drooling. This episode lasted for 30 seconds and the girl was fully aware of what was happening. The girl was drowsy and confused for the subsequent 15 minutes.
She has been well in herself and has no conditions. The pregnancy and vaginal birth have been uncomplicated. Her mother is worried that she has been tired as she has been going to bed later than usual for the past couple of weeks.
What is the most likely diagnosis?
A. Absence seizure
B. Benign rolandic epilepsy
C. Infantile spasms
D. Juvenile myoclonic epilepsy
E. Reflex anoxic seizures
B. Benign rolandic epilepsy is characterised by partial seizures at night
Benign rolandic epilepsy is the correct answer. Benign rolandic epilepsy (BRE) is a syndrome seen in childhood, usually between the ages of 4-12. The children will usually have a focal seizure, involving their face, drooling, and one side or one limb twitching (that can sometimes progress to secondary generalised seizure) either before or after bedtime. It is common for children to be sleep deprived. The EEG classically shows centrotemporal spikes, as the seizure is initiated in the rolandic fissure (central sulcus), hence the name. It carried a really good prognosis, and might not even need treatment depending on the severity and frequency of the seizures.
Absence seizure is incorrect. This is a type of epilepsy in children (commonly misdiagnosed as attention deficit hyperactivity disorder) that is characterised by periods of absence and quick recovery. This is a generalised type of seizure, which means that the patient will not have awareness, unlike in this case. Moreover, there would not be any twitching of limbs, again suggesting this patient has a focal seizure.
Infantile spasms is incorrect. This is a condition that commonly occurs in infants (up to 1 year old) and involves ‘spasm’ like movements that are usually associated with developmental delay in the milestones. This is a very heavy diagnosis as it carries a poor prognosis, and is unlikely in this case.
Juvenile myoclonic epilepsy is incorrect. This is a relatively common syndrome and is focal and in fact associated with sleep deprivation. However, it would characteristically produce ‘myoclonic’ jerks, meaning fast and rhythmic movements of the limbs, commonly on a background of daytime absences. These have the potential to become secondarily generalised seizures, just like BRE.
Reflex anoxic seizures is incorrect. Also, known as a pallid breath-holding spell, this would commonly be seen in infants and children up to 2 years of age. They tend to be triggered by stress and pain eg a bump (it is presumed they occur due to very sensitive cardiac reflexes in babies). Once child falls to the ground there is a convulsive phase with tonic stiffening & some limb jerking or spasms. The baby will go pale and become unresponsive for a short period of time (vasovagal syncope) and then have a quick recovery (may have persistent pallor/drowsiness). These are benign in nature and have a good prognosis.
define epilepsy
tendency to have recurrent, unprovoked seizures
5 main types of epilepsy (mnemonic):
MTTAA
-myoclonic
-tonic-clonic
-tonic
-atonic
-absence
usual 1st line treatment for epilepsy/when to avoid?
sodium valproate (avoid in females of child-bearing age)
1st line for absence seizure:
ethosuxamide
2nd line: sodium valproate
1st line for focal seizures:
lamotrigine (or carbamezapine)
2 types of focal seizures:
- aware
- Impaired awareness
what does tonic clonic mean?
tonic (muscle tensing) and clonic (muscle jerking) movements.
Waking from sleep with pain of any origin should always be taken seriously. Fine motor skills may deteriorate with raised intracranial pressure. Many childhood tumours arise from the posterior fossa and may present with cerebellar signs.
Early morning vomiting and headache may indicate raised intracranial pressure although it is by no means always present. Weight loss should always arouse concern in children. A new squint may represent the development of cranial nerve palsy. Headaches of vague onset and resolution, without pattern or significant incapacity of normal activity, may represent a more benign cause – such as a presentation of stress and anxiety.
Poor school attendance is frequently seen in children who have headaches, with or without organic pathology. Long term and frequent use of simple analgesia (paracetamol and ibuprofen) may result in a ‘medication overuse/rebound headache.’ Headaches may worsen when analgesia is omitted and a programme of stepwise reduction in therapy may be needed. It is a diagnosis that needs to be made with caution, once other pathology has been ruled out.
Migraine often has a strong familial component. Migraine in children usually has a clear association with various food triggers (eg tyramine – cheese / chocolate, MSG – processed foods, nitrates – preserved meats).
Migraine is frequently associated with vomiting. It is important to manage the nausea and vomiting as part of the overall therapy. Visual disturbances occur in 20% of children with migraine. These include blurred vision, scotoma (field defects), black or coloured dots, or more unusual perceptive abnormalities.
Migraine is often unilateral and frontal in nature, but may become more generalised. Typically the same side of the head is affected in each attack. Migraines are usually of a periodic nature – e.g. several attacks per month, rather than a day to day phenomenon
-ask about pre-headache aura
meningitis management per age:
A 3-year-old girl presents to the Paediatric Emergency department after she fell off a trampoline.
She opens her eyes to voice and can speak words, although she is not putting sentences together as she usually does. She is not making spontaneous movements but withdraws from touch when you try to examine her.
What is her Glasgow Coma Score?
A. 10
B. 12
C. 11
D. 13
E. 14
B. 12
The Children’s Glasgow Coma Scale can be used to assess consciousness in the paediatric population. The scores differ depending on whether the child is 5 years or older or less than 5.
This child scores 3 out of 4 for eye-opening as she does not open her eyes spontaneously but responds to voice.
She scores 4 out of 5 for verbal communication as she is speaking, but less than her normal ability.
She scores 5 out of 6 for motor as she is not making normal spontaneous movements but is withdrawing from touch.
convulsing child algorithm
cerebral palsy types summary
Spastic (90% of patients with CP have some spastic features):
1. damage to pyramidal pathways ( pyramidal weakness)
2. General features: increased tone and reflexes, clasp knife, flexed hip and elbow
3. Scissor gait
4. May be monoplegic, diplegic, hemiplegic
Dyskinetic/ athetoid:
1. Damage to the basal ganglia pathways
2. Choreiform movements
3. Can exhibit signs of Parkinsonism
Ataxic:
1. Damage to cerebellar pathways
2. Uncoordinated movements
3. Signs of cerebellar lesions
