Introduction
Panayiotopoulos syndrome is an idiopathic benign childhood epilepsy and may mimic other common illnesses. The international league against epilepsy has recently recognized it. It affects approximately thirteen percent of children between three to six years of age with one or more non-febrile seizures. It may occur in six percent of children between one to fifteen years of age.
The seizures may happen any time during the day, and most commonly, they occur during sleep or just after falling asleep. The seizures can be prolonged and may mimic non-epileptic disorders. In addition, they may vary from person to person in severity, from being insignificant to life-threatening situations.
The longer the seizures last, the more chances to progress to generalized tonic-clonic movements. It is a self-limiting focal epilepsy that appears only in childhood and does not continue into adulthood. It can be best defined as idiopathic susceptibility to early onset benign childhood seizures with electroencephalographic occipital or extra occipital spikes and manifests mainly with autonomic seizure.
What Is the Pathophysiology of Panayiotopoulos Syndrome?
Panayiotopoulos syndrome is the early-onset, whereas rolandic epilepsy is the late-onset phenotype of a maturation-related benign childhood seizure. Ictal epileptic discharges activate autonomic disturbances and vomit, to which children are particularly vulnerable. The symptoms of autonomic seizures and autonomic status epilepticus are specific to childhood and do not occur in adults.
What Are the Clinical Features of Panayiotopoulos Syndrome?
The most characteristic manifestations of panayiotopoulos syndrome are autonomic epileptic seizures and autonomic status epilepticus. Disturbances in the autonomic nervous system characterize seizures. Because of this reason, they are also known as focal autonomic seizures. The autonomic nervous system is responsible for controlling functions without needing conscious control, like heart rate, breathing pattern, skin blood flow, temperature, the dilation or constriction of the pupil, and vomiting. Autonomic symptoms are the only features of this seizure. Autonomic seizure comprises episodes of disturbed autonomic function.
Other features include:
-
Pallor.
-
Flushing and cyanosis.
-
Mydriasis and sometimes miosis.
-
Cardiorespiratory and thermoregulatory alterations.
-
Urine and fecal incontinence.
-
Hypersalivation.
-
A cardiorespiratory arrest is rarely seen.
-
Vomiting.
The child may be initially fully conscious and able to respond to questions but suddenly becomes confused, unresponsive, and flaccid, like ictal syncope just before or often without convulsions. They typically present as eyes turned to one side or stare, and the episodes end with hemi-convulsions or generalized convulsions. Convulsive status epilepticus is rarely seen. Usually, the episodes last for more than thirty minutes, thus constituting autonomic status epilepticus, the more common non-convulsive status epilepticus in normal children. They usually occur during sleep.
What Are the Diagnostic Tests to Be Carried Out?
Panayiotopoulos syndrome may be misdiagnosed as it is mild and manifests as ictal autonomic symptoms. This would suggest insignificant non-epileptic conditions such as atypical migraine, gastroenteritis, or syncope. At the same time, prolonged and severe attacks may cause life-threatening insults such as encephalitis, for which many of these children are treated.
Even after a very severe seizure, the child becomes normal after a few hours of sleep; this is reassuring and plays a crucial role in diagnosis. The diagnosis is based on a well-detailed history by someone who has witnessed one or more such seizures.
Brain scans such as computed tomography (CT) and magnetic resonance imaging (MRI) are normal.
An electroencephalogram is an investigation of the brain's electrical activity. It shows multifocal spikes at various locations. Though occipital spikes predominate, they are non-specific. They are more common when the child closes their eyes or is not fixated on anything. Epileptiform abnormalities include posterior spike-wave discharges, occipital paroxysms, and cloned complexes.
What Is the Management of Panayiotopoulos Syndrome?
The seizures in case of generally respond to conventional antiepileptic drugs such as Carbamazepine, Sodium valproate, and Lamotrigine. But due to the potential adverse effects of these antiepileptic drugs and considering that these seizures are self-limiting, many children are managed without using conventional antiepileptic drugs. Autonomic status epilepticus in the acute stages requires a thorough evaluation; aggressive management may lead to iatrogenic or unknown life-threatening complications, including cardiorespiratory arrest.
In case of prolonged seizure may require emergency medical attention, a rescue treatment with either rectal Diazepam or oral Midazolam is recommended during the actual seizure attack. And the family may also be prescribed the same emergency medication. This drug helps reduce overexcited brain activity that occurs during seizure activity and thus terminates the seizure. It is remarkably benign in terms of seizure frequency and evolution.
The prognosis of the disease is fairly good. Approximately all the children cease to have seizures within two to three years after the first seizure, and it is unlikely to continue or to recur in adult life.
Conclusion
It is often known as benign focal epilepsy as they are not serious. The seizures themselves are quite frightening. However, there are no reports of any child suffering from permanent, irreversible neurological damage due to seizure. As it is a self-limiting disorder, it does not continue, and children grow out of it, usually within a year or so. The characteristic features of this seizure are not typical of those expected during an epileptic seizure.
And for this reason, epilepsy is not often suspected, especially with initial seizures. The rate of misdiagnosis is high as children with panayiotopoulos syndrome are thought to have motion sickness, acute encephalitis, syncope, abdominal migraine or cyclical vomiting syndrome, gastroenteritis, or faints. Many children may develop other types of childhood epilepsy, particularly rolandic epilepsy. While the seizure can be challenging and upsetting for the child and the caregiver, the prognosis for those with seizures of this nature is very good.
