Understanding Perioral Myoclonia with Absences (PMA)

Disclaimer: This guide is for educational purposes and cannot replace an epileptologist’s interpretation or a personalized seizure action plan.

Finding out your child has epilepsy is a life-altering moment. It is natural to feel overwhelmed, especially when the diagnosis involves a name you have likely never heard before: Perioral Myoclonia with Absences (PMA or POMA).

PMA is a rare way for epilepsy to show up in children. Because it is so uncommon, it is frequently misunderstood or even missed entirely during initial doctor visits ^[1]. This page is designed to help you understand what this condition is, why it might be unfamiliar to your local care team, and how it fits into the broader world of childhood epilepsy.

What is Perioral Myoclonia with Absences?

To understand PMA, it helps to break down the medical terms:

• Perioral: Referring to the area around the mouth.
• Myoclonia: Brief, involuntary, rhythmic muscle jerks or twitching.
• Absence: A type of seizure where a person briefly loses awareness—often called a “staring spell.”

In PMA, these two things happen at the same time. During a seizure, a child will typically experience a brief “blanking out” accompanied by rhythmic twitching of the mouth, chin, or lips ^[1][2].

PMA is considered a rare seizure presentation or phenotype within the Genetic Generalized Epilepsy (GGE) spectrum ^[3]. “Generalized” means the seizure activity reflects networks involving both sides of the brain at once, rather than starting in one specific “focal” spot. While “genetic” points to a presumed genetic susceptibility (often polygenic and complex), it does not necessarily mean it was inherited in a simple way; the EEG pattern supports the epilepsy classification but does not define the word genetic ^[2].

Who typically develops PMA?

PMA usually begins in early childhood. Research in small cohorts has shown:

• Age of Onset: The median age when seizures start is 5 years old, though they can begin as early as age 2 or as late as age 11 ^[2].
• Gender: In the small groups of patients studied so far, there appears to be a higher number of boys affected than girls. One referral series found that over 80% of their PMA patients were male, though this may not represent the whole population ^[2].

Why is this condition under-recognized?

It is very common for local neurologists to be unfamiliar with the specific name “Perioral Myoclonia with Absences.” There are a few reasons for this:

1. It is not yet a stand-alone syndrome: The International League Against Epilepsy (ILAE), which sets the official rules for naming epilepsy types, does not currently list PMA as its own stand-alone syndrome. Instead, it is viewed as a specific “phenotype”—a unique set of symptoms—within the larger family of generalized epilepsies ^[4][1].
2. It mimics other types of epilepsy: Because the twitching is limited to the mouth, doctors may mistakenly believe the seizure is focal (starting in one small part of the brain). In one small referral series, 4 out of 5 patients were initially misdiagnosed with focal epilepsy before further testing revealed the true nature of their condition ^[1].
3. The rarity factor: Because it is rare, a general neurologist may only see one or two cases in their entire career.

The Importance of the EEG

The most important tool for identifying PMA is the video-EEG (electroencephalogram), which records brain waves while a camera records your child’s physical movements.

In PMA, the EEG shows a specific pattern: a “3-Hz generalized spike-and-wave” discharge. This means the brain’s electrical activity spikes about three times per second ^[2]. Crucially, the mouth twitching happens consistently with these brain spikes—a phenomenon doctors call being time-locked ^[5].

Often, these seizures can be triggered during the EEG by having the child breathe deeply and quickly (hyperventilation), which helps the medical team capture an episode and confirm the diagnosis ^[2].

Moving Forward

Receiving this diagnosis is the first step toward getting your child the right support. Because PMA is often misidentified as focal epilepsy, the standard treatments for focal seizures (like certain medications) can sometimes make PMA seizures worse ^[1]. Identifying the “generalized” nature of PMA ensures that your child is considered for the most appropriate broad-spectrum therapy.

While the name is complex and the condition is rare, understanding the “how” and “why” of your child’s seizures will help you advocate for the specialized care they need.