Common Paediatric Syndromes

Question 1

What is a syndrome?

Answer 1

A collection of signs and symptoms that suggest a particular known medical condition.

In epilepsy, signs and symptoms include age of onset, seizure types, EEG appearance, and presence or absence of learning difficulties.

Question 2

What types of epilepsy can occur in children?

Answer 2

Epilepsy in children can be:
* Generalised
* Focal

Question 3

What is Childhood Absence Epilepsy (CAE) and its age of onset?

Answer 3

A form of epilepsy with an age of onset between 4 to 8 years old.

Very rarely do absences start under the age of 4 years.

Question 4

What is the gender ratio for Childhood Absence Epilepsy (CAE)?

Answer 4

Occurs slightly more often in girls than boys (2:1).

Question 5

What is the prognosis for Childhood Absence Epilepsy (CAE)?

Answer 5

Prognosis is excellent and seizures are normally well controlled.

Question 6

What are common treatments for Childhood Absence Epilepsy (CAE)?

Answer 6

Treatments include:
* Sodium Valproate
* Ethosuximide
* Lamotrigine

Question 7

What is the typical duration of absence seizures in CAE?

Answer 7

Last between 4 and 20 seconds.

Question 8

What precipitates absence seizures in 80-90% of cases?

Answer 8

Hyperventilation.

Question 9

What EEG changes are observed during an absence seizure?

Answer 9

Ictal: Generalised rhythmical spike and wave activity at 3-3.5Hz, usually with frontal emphasis.

Question 10

What is Juvenile Absence Epilepsy (JAE) and its age of onset?

Answer 10

A form of epilepsy with an age of onset between 9-14 years.

Question 11

What is the gender ratio for Juvenile Absence Epilepsy (JAE)?

Answer 11

Female > Male.

Question 12

What is the seizure frequency in Juvenile Absence Epilepsy (JAE)?

Answer 12

Less frequent (1-9 per day) and longer in duration (up to 30 seconds).

Question 13

What are the common treatments for Juvenile Absence Epilepsy (JAE)?

Answer 13

Treatments include:
* Sodium Valproate
* Lamotrigine
* Ethosuximide (only if no generalised tonic clonic seizures have occurred).

Question 14

What is the prognosis for Juvenile Absence Epilepsy (JAE)?

Answer 14

70% of children will achieve seizure freedom with 1 or 2 antiepileptics.

Question 15

What triggers absence seizures?

Answer 15

Triggers include:
* Child being unwell
* Sleep deprivation or tiredness
* Child being bored

Question 16

What is Juvenile Myoclonic Epilepsy (JME) and its age of onset?

Answer 16

A form of epilepsy with an age of onset between 6 to 20 years, peaking at 12-16 years.

Question 17

What are the seizure types associated with Juvenile Myoclonic Epilepsy (JME)?

Answer 17

Seizure types include:
* Myoclonic
* Tonic-clonic
* Absence

Question 18

What is the treatment for Juvenile Myoclonic Epilepsy (JME)?

Answer 18

Treatments include:
* Sodium Valproate
* Levetiracetam
* Lamotrigine

Question 19

What are the characteristics of myoclonic seizures in JME?

Answer 19

Sudden jerks of the muscles, usually happening soon after waking or late evening.

Question 20

What is Childhood Epilepsy with Centro-Temporal Spikes (CECTS)?

Answer 20

A form of epilepsy with an age of onset between 3-10 years.

Question 21

What triggers seizures in Childhood Epilepsy with Centro-Temporal Spikes (CECTS)?

Answer 21

Sleep deprivation is a significant trigger.

Question 22

What is the semiology of seizures in Childhood Epilepsy with Centro-Temporal Spikes (CECTS)?

Answer 22

Symptoms include:
* Tingling feeling in the mouth
* Slurred speech
* Twitching or clonic movements of one side of the face

Question 23

What is Panayiotopoulos Syndrome and its age of onset?

Answer 23

A syndrome with an age of onset between 1-15 years, most common in ages 3-5 years.

Question 24

What are the features of seizures in Panayiotopoulos Syndrome?

Answer 24

Features include:
* Autonomic symptoms
* Jerking limb movements
* Head deviation

Question 25

What is West Syndrome (Infantile Spasms) and its age of onset?

Answer 25

A syndrome with an age of onset within the first year of life, commonly 3-8 months.

Question 26

What are the triad of signs and symptoms in West Syndrome?

Answer 26

Triad includes:
* Spasms
* Hypsarrhythmia on EEG
* Developmental regression

Question 27

What is the EEG characteristic of West Syndrome during a spasm?

Answer 27

High amplitude diffuse slow wave followed by electrodecrement with superimposed fast activity.

Question 28

What are common treatments for West Syndrome?

Answer 28

Treatments include:
* Corticosteroids
* Vigabatrin
* Sodium Valproate
* Nitrazepam
* Zonisamide

Question 29

What is the prognosis for children with West Syndrome?

Answer 29

Depends on the underlying cause, but 60-70% will have their spasms fully controlled.