Staring spells in children–planning the evaluation

Question 1

What is the differential for staring spells in children? (Broad categories)

Answer 1

Pseudoabsence (nonepileptic) or absence (epileptic).

Question 2

Pseudo absence events can occur in children with what underlying behavioral issues or disorders?

Answer 2

ADHD
Autism
Intellectual disabilities
And sometimes it can be normal

Question 3

In a child who has pseudo absence what can be common triggers?

Answer 3

Boredom and being inactive can trigger nonepileptic staring spells in children.

Question 4

What is a good physical finding that helps in distinguishing between pseudo absence and absence events?

Answer 4

The pseudo absence event can usually be interrupted with either a vocal or tactile stimulus. They rarely occur during physical activity and they never occur with motor signs, such as myoclonus or automatisms

Question 5

In a child with pseudo absence events what is the common EEG findings?

Answer 5

EEG will be normal at rest and may show generalized high voltage slow activity with hyperventilation.

Question 6

At what age do childhood absence seizures typically begin?

Answer 6

They generally begin around 4 to 10 years of age.

Question 7

How can an absence seizure in a primary care outpatient setting be triggered safely?

Answer 7

The patient is asked to hyperventilate for at least 3 minutes to provoke an episode (The child will stop hyperventilating and stare, usually with the eyes slowly looking upward).

Question 8

What other physical manifestations may occur in a child who has an absence seizure?

Answer 8

The trunk may begin to extend and cause the patient to slowly lean backward, the facial muscles, head and or arms could have myoclonic jerks with a low amplitude of 3/s. Automatisms are usually common.

Question 9

Following an absence seizure what is the patient’s recollection of the event?

Answer 9

zero recollection

Question 10

How long do absence seizures typically last

Answer 10

A few seconds to 20 seconds

Question 11

What are the classic EEG findings in a patient with absence seizures?

Answer 11

EEG findings show 3 Hz spike and wave pattern.

Question 12

In a healthy child with with non-absence seizures what affects can hyperventilation trigger? And what are the EEG findings?

Answer 12

Hyperventilating can cause altered consciousness and decreased cerebral blood flow and some healthy children. EEG only shows generalized high voltage slow activity

Question 13

What are 3 less common causes of absence staring spells?

Answer 13

Juvenile absence epilepsy (JAE).
Juvenile myoclonic epilepsy (JME)
GLUT 1 deficiency syndrome

Question 14

What distinguishes JAE from childhood absence seizures?

Answer 14

The onset of seizure activity typically is 10 to 11 years of age. And some patients may also have myoclonic seizures or generalized tonic clonic seizures which are not common with childhood absence seizures

Question 15

How are absence seizures and JME related?

Answer 15

JME can initially present with absence seizures several years prior to presenting with myoclonic or generalized tonic-clonic seizures.

Question 16

What is the difference between an EEG absence seizure and a EEG of a JME absence seizure?

Answer 16

The absence seizure phase will typically have a slightly higher frequencies such as 4 to 5 Hz instead of the 3 Hz seen in absence seizure

Question 17

In a patient with gluten 1 deficiency syndrome how can they initially present and at what age?

Answer 17

The patient can present with staring episodes prior to 4 years of age.

Question 18

In a patient with Glut1 deficiency syndrome what does CFS glucose levels reveal and what does genetic testing show?

Answer 18

CFS glucose levels can be low and genetic testing for SLC2A1 can confirm the diagnosis.

Question 19

What other features or co-morbidities may accompany a patient with Glut1 deficiency syndrome?

Answer 19

They may have developmental delays and generalized epilepsy.