Case: 6yo girl with staring spells

Case 6 year old girl with history of ADHD, otherwise healthy, presenting for evaluation of staring spells. She was born at full-term and met developmental milestones on time. There were no developmental concerns until the past year when her kindergarten teacher reported she did not pay attention in class. She was diagnosed with ADHD and started on a stimulant medication.

Her parents describe her spells as suddenly stopping what she is doing, staring for seconds, and then picking up where she left off. They do not cause her to fall or lose tone. If she is moving her arm it freezes in space. Sometimes her eyes flutter while staring. Clapping in her face does not cause any response. She is not sleepy afterward. She has no memory of the episode. Episodes occur 2-3 times per day.

General and neurological examinations are normal. DDx: ??

EEG: After blowing on a pinwheel for 30 seconds she had a spell consisting of activity arrest and blinking…

Gain is 50 uV (high amplitude)

Bifrontally predominant generalized 3 Hz spike and wave, evolves with time

No postictal period

Diagnosis: C.A.E. Childhood Absence Epilepsy Median age of onset 6yo, F>M Typical sz duration seconds Behavior arrest, staring, no loss of posture/tone, unresponsive to stimuli, +/- blinking, no awareness event occurred, no postictal period Typically can elicit by hyperventilation

Side note: other syndromes with typical absence seizures: Myoclonic Astatic Epilepsy (Doose syndrome, pronounced “doe-suh”) Epilepsy with Myoclonic Absences (E.M.A.) Juvenile Myoclonic Epilepsy (J.M.E.) Juvenile Absence Epilepsy (J.A.E.)

Back to the case The patient may or may not truly have difficulties with inattention. Children with C.A.E. are often misdiagnosed as having ADHD if the seizures are subtle/not detected. On the other hand, there may be a higher incidence of ADHD symptoms in children with C.A.E., even when seizures are well-controlled. First treat the seizures, then reassess.