Paediatric Neurology

Written by Dr. Emily Chang (MBBS, FRACP) – Paediatrician

Question 1

A 20-month-old boy named Liam is brought to the Emergency Department by his parents after experiencing a seizure at home. The seizure lasted approximately 3 minutes and involved generalized tonic-clonic movements. He had a fever of 38.9°C earlier in the day due to a viral upper respiratory tract infection. He returned to his baseline within 10 minutes of the seizure and has been alert and playful since. There is no history of previous seizures, no focal neurological signs, and no family history of epilepsy.

Which one of the following is the most appropriate next step in his management?

A) Admit for overnight observation and start antiepileptic therapy
B) Perform urgent CT scan of the brain
C) Reassure the parents and provide safety advice
D) Arrange an EEG and paediatric neurology referral
E) Perform a lumbar puncture

Correct Answer: C) Reassure the parents and provide safety advice

This is a classic presentation of a simple febrile seizure — a generalized tonic-clonic seizure lasting less than 15 minutes, occurring in a febrile child aged 6 months to 5 years, with no prior neurological abnormality or recurrence within 24 hours.

Simple febrile seizures are benign and self-limiting, and they do not increase the risk of epilepsy significantly. The most important next step in management is reassurance and education of the caregivers. This includes advising on first aid during future seizures, managing fever appropriately, and monitoring for recurrence.

There is no need for neuroimaging (CT or MRI), antiepileptic medications, or EEG in simple febrile seizures. A lumbar puncture would only be indicated if there are clinical signs of CNS infection or the child appears unwell, which is not the case here.

Hospital admission is generally not required unless the child is persistently drowsy, has a complex seizure, or there are social concerns.

Question 2

A 3-year-old girl named Sophia is brought to a paediatric clinic by her mother due to concerns about her delayed motor development. Sophia started sitting without support at 10 months and is not yet walking independently. She uses her right hand more frequently and appears to drag her left leg when crawling. Her speech is limited to a few single words. On examination, she has increased tone and brisk reflexes in her left upper and lower limbs, with a clear hand preference.

Which of the following is the most likely diagnosis?

A) Global developmental delay
B) Spastic hemiplegic cerebral palsy
C) Autism spectrum disorder
D) Duchenne muscular dystrophy
E) Hypotonic cerebral palsy

Correct Answer: B) Spastic hemiplegic cerebral palsy

Sophia’s presentation is consistent with spastic hemiplegic cerebral palsy, a subtype of cerebral palsy that typically results from a unilateral brain insult, often perinatal in origin (e.g. periventricular leukomalacia, stroke). It presents with increased tone, hyperreflexia, and motor delay on one side of the body, usually more prominent in the upper limb.

An early hand preference before 18 months of age is a red flag and often indicates upper limb dysfunction, commonly seen in hemiplegic cerebral palsy. Children with this condition often have associated mild speech delay and learning difficulties, though intelligence can be normal.

Global developmental delay would involve delays across all domains without the focal motor findings seen here. Autism spectrum disorder primarily affects social and communication domains and does not explain unilateral motor signs. Duchenne muscular dystrophy causes progressive symmetrical proximal weakness, typically in boys, with no increased tone or brisk reflexes. Hypotonic cerebral palsy would present with decreased tone, not spasticity.

Question 3

A 6-month-old boy named Liam is brought to the emergency department by his parents after they noticed sudden, repeated jerking movements involving his arms and head. These movements typically occur in clusters, especially after waking up from sleep. Each episode lasts a few seconds. Developmentally, Liam was smiling and rolling over by 4 months but has recently stopped babbling and no longer reaches for toys.

An EEG is ordered and shows a pattern of hypsarrhythmia.

Which of the following is the most appropriate initial treatment?

A) Sodium valproate
B) Levetiracetam
C) Carbamazepine
D) Vigabatrin
E) Diazepam

Correct Answer: D) Vigabatrin

This clinical scenario describes infantile spasms, also known as West Syndrome, which is a severe epileptic encephalopathy of infancy. It typically presents between 3 and 12 months of age with brief symmetric spasms, often occurring in clusters after waking. The spasms may be flexor, extensor, or mixed.

Developmental regression, as seen in Liam’s case, is a hallmark feature and raises serious concern. The EEG finding of hypsarrhythmia — a chaotic, high-amplitude background with multifocal spikes — is characteristic of this condition.

Vigabatrin is the first-line treatment for infantile spasms associated with tuberous sclerosis complex and is also used more broadly depending on the underlying cause. In other cases, ACTH (adrenocorticotropic hormone) or high-dose oral corticosteroids are commonly used.

Sodium valproate and levetiracetam are used for other seizure types but are not first-line in infantile spasms. Carbamazepine is used for focal seizures and may worsen spasms. Diazepam is used for acute seizure termination but not for ongoing management of infantile spasms.

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15. Paediatrics Module

15.1 Neonatology

15.2 Growth and Development

15.3 Paediatric Neurology

15.4 Paediatric Cardiology

15.5 Paediatric Respiratory

15.6 Paediatric Gastroenterology

15.7 Paediatric Nephrology

15.8 Paediatric Infections

15.9 Other Paediatric Disorders