Case history
Case history
A 6-year-old female without significant past medical history presents for evaluation of frequent unusual episodes for the past 3 months. The episodes consist of sudden activity arrest with staring and minimal eyelid flutter for 10 to 20 seconds occurring 5 to 10 times per day. The patient is unresponsive to voice or tactile stimulation during the episodes. She is able to immediately resume activities without any recollection of the event once the episode finishes. Her teachers have noted that she stares off in class repeatedly and does not seem to be remembering instructions and classroom material. The diagnosis of attention-deficit/hyperactivity disorder had been suggested. One such unusual episode is induced in front of medical staff with hyperventilation.
Other presentations
The most familiar absence epilepsy syndrome is childhood absence epilepsy (CAE), presenting with numerous typical absence seizures a day starting at early school age in children who are developing normally. Juvenile absence epilepsy (JAE) typically begins around puberty, absence seizures are considerably less frequent than in CAE, and many patients will have generalized tonic-clonic seizures. Juvenile myoclonic epilepsy (JME) also begins around puberty but with frequent myoclonic jerks, particularly around awakening. People with JME have generalized tonic-clonic seizures, and up to one third have rare typical absence seizures. Lennox-Gastaut syndrome begins in early childhood with multiple seizure types, including atypical absence seizures. Lennox-Gastaut syndrome patients almost universally are neurologically impaired. Patients with epilepsy with myoclonic absences also present in early childhood with atypical absence seizures. They rapidly develop other seizure types. Around 50% are neurologically impaired at baseline.[8]
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