What is Panayiotopoulos Syndrome?
Panayiotopoulos Syndrome (PS), also called early childhood occipital epilepsy, is a type of epilepsy characterized by prolonged seizures and an onset most often during early childhood. It is a common type of epilepsy, occurring in as many as 13 percent of children with epilepsy between the ages of three and six.
Features of PS Seizures
The distinctive features of PS seizures often include:
- Focal seizures that begin in one area of the brain, sometimes evolving into generalized seizures
- Seizures that start shortly after falling asleep (although they may occur at any time)
- Duration of 1-30 minutes
- Pale skin
- Nausea or vomiting
- Dilated pupils
- Vision disruption
- Eyes gazing to the side or wide open
- Brief convulsions on one or both sides of the body
What Causes Panayiotopoulos Syndrome?
The precise cause of PS is unknown. Most children with the disorder have no family history of epilepsy. Scientists believe that genetic factors may play a role in risk for the condition, but no specific genes have been definitively associated with PS.
Is Panayiotopoulos Syndrome Hereditary?
PS does not appear to be an inherited disorder. About 17 percent of children with PS have a family history of epilepsy, but most do not. Mutations (abnormal changes) in the SCN1A gene appeared in one case of three siblings with the disorder, but these mutations have not occurred in other cases of familial PS. Research has not identified any other potential genetic causes of this type of epilepsy.
How Is Panayiotopoulos Syndrome Detected?
The symptoms of PS often look like those of other non-epileptic disorders, leading to frequent misdiagnosis. Conditions often confused with PS seizures include:
- Motion sickness
- Cyclic vomiting syndrome
- Sleep disorders
How Is Panayiotopoulos Syndrome Diagnosed?
A doctor will most often diagnose PS when the description of seizure symptoms matches the distinctive characteristics of the disorder. Physical and neurological exam results will usually be normal. The only reliable diagnostic tool for PS is an electroencephalogram (EEG), which will typically show abnormal spikes in localized brain activity, often triggered by sleep.
PLEASE CONSULT A PHYSICIAN FOR MORE INFORMATION.
How Is Panayiotopoulos Syndrome Treated?
The seizures of PS are usually infrequent, and they typically resolve on their own by adolescence without treatment. However, in cases where seizures are more frequent and/or prolonged, treatment with anti-seizure medications may be recommended. Commonly used anti-seizure drugs include:
How Does Panayiotopoulos Syndrome Progress?
PS is a benign form of epilepsy, and it typically does not have long-term effects on children who experience it. Most children with the disorder have fewer than four seizures in total, and a third of them only have a single seizure. In addition, the seizures usually subside within two years after their first occurrence, and even in cases in which other types of seizures develop, all seizure activity usually stops by adolescence.
Although prolonged seizures carry the risk of cardiac arrest, this is very rare in PS. Furthermore, even prolonged seizures don’t seem to have any long-term neurological impacts.
How Is Panayiotopoulos Syndrome Prevented?
There is no known way to prevent PS. In cases where seizures are frequent or severe, doctors may recommend using anti-seizure medications to prevent seizures, but many patients will require no treatment at all.
In some cases, exposure to changing light conditions may trigger a seizure, so avoiding these situations may help prevent seizures.
Panayiotopoulos Syndrome Caregiver Tips
- Know the risks, but keep your anxiety in check. Consult your doctor about the necessity of an emergency rescue plan if your child has a prolonged, life-threatening seizure. Remember, however, that the risk of such a seizure in PS is low.
- Be part of a community. The Epilepsy Foundation provides links to support resources to help you remember that you and your child are not alone in living with epilepsy.
Panayiotopoulos Syndrome Brain Science
Children with PS typically show an abnormal electrical brain wave activity pattern, and this irregular pattern is usually the only finding that will aid in diagnosis. An EEG will typically show spikes of activity in localized parts of the brain. These spikes are often in the occipital area (in the back of the brain), but they can occur elsewhere, too. In about a quarter of cases, an EEG may show normal results.
The spikes characteristic of PS often occur during sleep, so doctors must take EEG readings during sleep to reliably diagnose the condition.
Panayiotopoulos Syndrome Research
Title: Making Mindfulness Matter© in Children With Epilepsy (M3Epilepsy)
Principal investigator: Kathy Nixon Speechley, PhD
Epilepsy is a debilitating condition characterized by spontaneous, unprovoked seizures. Up to 80% of children with epilepsy (CWE) may face cognitive, psychiatric, and/or behavioral comorbidities with significant unmet mental health needs. Mindfulness-based interventions may provide an ideal vector to target unmet mental healthcare needs in patients with epilepsy and their families. The investigators propose the Making Mindfulness Matter© (M3) program as an intervention to improve health-related quality of life and mental health for CWE and their parents. M3 is a live-online parent and child program incorporating mindful awareness, social-emotional learning skills, neuroscience, and positive psychology. This pilot RCT is needed to refine the implementation of the intervention to families with a child with epilepsy and collect information about the feasibility and effectiveness of the intervention in preparation for a subsequent multi-centered trial across Canada. Note: Due to COVID-19, the format has been modified for online delivery (from community-based), and the intervention has been restarted.
Mindfulness-based interventions have been increasingly utilized to recognize that medical management for chronic illnesses such as epilepsy does not address the stress and co-occurring psychological issues experienced by many patients. Mindfulness-based interventions are effective, well-validated interventions for several adult outcomes, including physical and mental health, social and emotional well-being, and cognition.
Title: Effect of Auditory Stimulation on Spike Waves in Sleep (ECLASS)
Principal investigator: Bernhard Schmitt, MD
University Children’s Hospital
Background: Close relationship exists between sleep slow-wave (SSW) and the generation of spike waves in NREM-sleep. SSW is cortically generated oscillations alternating between excitatory depolarization (“Up-phase” of the SSW) and inhibitory hyperpolarization (“Down-phase” of the SSW). It has been shown experimentally that with increasing synchrony of slow neuronal oscillations, SSW turns into spike waves. Acoustic pulses applied in correspondence to the SSW “Up-phase” enhance the amplitude of the subsequent SSW. Conversely, tones delivered at the SSW “Downphase” have a disruptive effect on the following SSW.
Participants: Patients with epilepsy and spike waves in NREM sleep.
Objective: Modification of spike-wave frequency, amplitude, and spreading during NREM sleep by acoustic pulses applied at the “Up-” or “Down-phase” of SSW.
Title: Safety and Efficacy of Topamax Versus Carbamazepine in Benign Rolandic Epilepsy
Janssen Korea, Ltd.
Benign rolandic epilepsy (BRE) is a common seizure disorder confined solely to children. The disorder is marked clinically by nocturnal generalized tonic-clonic seizures and diurnal seizures consisting of simple partial seizures consisting of brief unilateral facial clonic activity, dysphasia, and drooling. The EEG abnormalities are unique, consisting of generally high amplitude, centrotemporal spikes activated by sleep. The seizures typically begin in the first decade and almost always stop by age 16 years. The seizures are usually infrequent, although clusters of seizures do occur. When the physician elects to treat, the seizures are usually easily controlled. This is a randomized, open-label, active-controlled, multi-center based clinical trial to determine the efficacy and safety of Topiramate comparing with Carbamazepine in Benign rolandic epilepsy. The study hypothesis is that topiramate will be more effective in treating Benign rolandic epilepsy than Carbamazepine, as evaluated by the seizure-free rate at 24 weeks and Intellectual Functioning: KEDI-WISC (Korean Educational Development Institute Wechsler Intelligence Scale for Children-Revised) and is generally well-tolerated.