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Epileptic Syndromes in Children: Panayiotopoulos Syndrome


A little girl wakes up in the middle of the night, stumbles past her Frozen night light and out her bedroom door towards her parents room. She jumps in her parent’s bed complaining that her head hurts and she feels like she might get sick. The girl’s parents notice that she seems pale, and clammy. Their first thought is she must have the flu. When all of a sudden their daughter becomes unresponsive, her head tilts to one side as she gazes off into the distance and she begins to convulse. The girl’s parents rush her to the hospital, later they find out their little girl had a seizure.

Seizures can be terrifying to both the patient and the family when not educated or prepared. A way to prevent the stress and fear of a seizure is to be knowledgeable about different seizure disorders, especially now that multiple studies have been done so that specific seizure disorders can be recognized and diagnosed as quickly as possible. The little girl from the story above was experiencing a Panayiotopoulos form of epilepsy. Panayiotopoulos is a benign form of epilepsy that affects the central autonomic system in younger children. Panayiotopoulos is also known as PS for short. PS syndrome is multifocal and shows a lot of wave variability, mainly in the occipital region. There has been no proven reason behind why children have Panayiotopoulos syndrome. PS typically does not have any long term effects on children that have been diagnosed. The following will provide an understanding of how PS is identified, its symptoms, and how it can be managed.


Epidemiology/Incidences

Panayiotopoulos (PS) affects 13% of children age 3 to 6 years old who have had one or more afebrile seizures (Covanis, 2006). First seizures are typically seen between age 3 and 10 years (Hernandez, 2015). PS is not gender specific; therefore it can be seen in both males and female children. Research has shown that PS is not genetically determined; therefore it is neither predictable nor preventable form of epilepsy. No child is more likely to experience PS than any other. If a child were to have PS more than half the seizures would occur during sleep, however PS seizures can occur in awake patients (Hernandez, 2015).


Symptoms/Clinical Features

Panayiotopoulos is commonly mistaken for many other seizure disorders such as syncope, encephalitis, motions sickness, or even a sleep disorder (Hernandez, 2015).Autonomic seizures and Autonomic epilepticus are going to be the key players in PS. The seizures can be very prolonged, and can look very simple or sometimes life threatening (Koutroumanidis, 2002). However children will have normal physical and cognitive development (Hernandez, 2015). Patient will experience disturbed autonomic function with emesis as a predominant symptom (Covanis, 2006). Vomiting occurs in about three quarters of the seizures (Koutroumanidis, 2002). Focal to generalized seizures occur, showing tonic-clonic movement and eye deviation (Hernandez, 215). The seizure can last anywhere from 20 minutes to 60 minutes but about half of the seizures are typically under 30 minutes. While experiencing a seizure, if the child is initially conscious they will be confused and unresponsive, eyes may turn to one side and gaze off, and generalized convulsions would then occur (Koutroumanidis, 2002). Many times the child will feel sick, and need to vomit; they will also experience headaches with an overall feeling of being unwell (Ferrie, Guenwald, 2001). After severe seizures and status the child is normal after a few hours of sleep (Koutroumanidis, 2002). The only way to distinguish that the seizure is caused by PS is to have an electroencephalogram also known as an EEG.


Evaluation/Tests/Correlation with Neurodiagnostic Studies

When a patient is being tested for Panayiotopoulos it is common practice to have an electroencephalogram (EEG) completed and a magnetic resonance image (MRI) taken. If the patient has not been treated with any anticonvulsants the EEG will show abnormal spikes in one or both occipital lobes (Hernandez, 2015). Spikes will also be seen over the centro-temporal lobe in the motor area (Hernandez, 2015). Epileptiform or seizure discharges during the EEG will be seen while the patients eyes are closed and will attenuate when the eyes are opened (Hernandez, 2015). The same affect can be seen when the patient is unfocused and then brought back to being focused on something, meaning that the spikes will attenuate as the patient becomes focused again. When performing the EEG both awake and sleep test must be performed otherwise EEG can appear normal (Hernandez, 2015). Seizures have been proved to be benign in terms of frequency and evolution (Covanis, 2006). A magnetic resonance image (MRI) is basically a picture of the brain, so it is normal to conduct this test to rule out any cerebral lesions, or injuries that could be the leading cause of the seizures. Children diagnosed with PS still show normal physical and cognitive development, which is why PS is considered a benign seizure disorder (Hernandez, 2015).


Figure 1. wakefulness and spontaneous sleep EEG with sleep de spike-wave paroxysms in that predominate in occipital regions


Treatment/Management

PS usually goes into remission within two years after onset (Koutroumanidis, 2002). If the seizures are serious enough or often enough anti-seizure medication can be used to control the episodes. Such drugs as Oxcarbazepine, Carbamazepin, Levetiracetam, and Gabapentin would help with the seizures (Hernandez, 2015). Doctors do not usually like to use such medications because the seizures are typically benign, and there is a possibility that the medication administered may have negative effects in the future. Most doctors recommend emergency medical treatment or rescue therapy. Parents are encouraged to learn emergency techniques so the child does not have to take serious seizure medication. Emergency treatment could include applying diazepam rectal gel during a seizure, or benzodiazepine through the nose or under the tongue (Hernandez, 2015). But mostly parents in the case of PS seizures are taught to keep their child on their side and to remain calm. The seizure will eventually pass, and the child will recover in their sleep.


Conclusion/Summary

Panayiotopoulos syndrome may be a benign syndrome but it is not necessarily harmless. As a medical professional it is very important to know the correct way to recognize and diagnosis PS. PS is very commonly mistaken for sometimes worse epileptic syndromes and can cause more stress to the family then needed. It can be the difference of telling the girl’s parents of a disorder as serious as Gastaut or as benign as PS. It could also lead to improper treatment which could result in harm to the patient. The best way to diagnose PS is by performing an EEG, both when the patient is awake and asleep. Abnormal spikes should be seen over the occipital and centro-temporal regions of the brain. Even though there is clearly an effect on the brain when the seizures occur, bright side is PS has no long term effects on the youth. The child will eventually cease to have seizures as they get older. But in the meantime if the seizures are too intense or too frequent they can be treated with anti-convulsive medication. There is no way to prevent or officially cure Panayiotopoulos since there seems to be no underlying cause. However quick and correct diagnosis, plus education of PS makes this epileptic syndrome easier to manage. So next time the little girl has a PS related seizure it won’t be so terrifying, and the parents will understand and be better equipped to help their child through.


Written by: Caitlin Albers (2016)

References:

Caraballo R, Cersosimo R, Medina C, Fejerman N. (2000). Panayiotopoulos-type Benign Childhood Occipital Epilepsy: A Prospective Study. Neurology.55:1096–1100.


Covanis, A. (October 2006). Panayiotopoulos Syndrome: A Benign Childhood Autonomic Epilepsy Frequency Imitating Encephalitis, Syncope, Migraine, Sleep Disorder, or Gastroenteritis. Pediatrics, Volume 118, Issue 4.


Ferrie CD, Grunewald RA. (2001). Panayiotopoulos Syndrome: A Common and Benign Childhood Epilepsy. Lancet.357:821–823.


Hernandez, A. M.D. (February 2015). Panayiotopoulos. Epilepsy Foundation. Retrieved from http://www.epilepsey.com


Koutroumanidis, M. (2002). Panayiotopoulos syndrome : A common benign but underdiagnosed and unexplored early childhood seizure syndrome. BMJ : British Medical Journal, 324(7348), 1228–1229.


Figure 1. Published in European journal of paediatric neurology 2011

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