Panayiotopoulos Syndrome
Definition
Panayiotopoulos syndrome (PS) is a common, benign childhood epilepsy syndrome. It primarily affects children between 3 and 10, with a peak around 5 years of age. PS is characterized by seizures with prominent autonomic symptoms, such as nausea, vomiting, pallor, and sometimes fainting, followed by altered consciousness or convulsions. The condition is self-limiting; children generally outgrow it without significant long-term consequences.
Epidemiology
Panayiotopoulos syndrome accounts for approximately 6% of all childhood epilepsy syndromes. It is more common in boys but can occur in both sexes. A genetic predisposition is often suspected, but specific genetic causes have not been identified.
Clinical Manifestations
Autonomic Symptoms: Nausea and vomiting (most common). Pallor, flushing, or cyanosis. Sweating or abnormal breathing patterns.
Altered Consciousness: Seizures may progress to impaired awareness or complete loss of consciousness.
Convulsions: Generalized convulsions can occur but are less common.
Seizure Duration: Seizures are often prolonged, lasting more than 5 minutes and sometimes up to 30 minutes or longer.
Triggers: Seizures may occur during sleep or relaxation and are typically infrequent.
Normal Development: Cognitive and neurological development is unaffected by the syndrome.
Diagnosis
Clinical History and Presentation: Autonomic symptoms preceding the seizure are a key feature of PS.
Electroencephalography (EEG): Interictal EEG shows multifocal spikes, most commonly in the occipital regions. However, findings may vary and are not exclusive to PS.
Neuroimaging (MRI): MRI is generally normal but may be done to exclude structural abnormalities if the presentation is atypical.
Treatment
Antiepileptic Drugs (AEDs): Treatment is often unnecessary due to the benign nature and infrequent seizures. If seizures are frequent or prolonged, AEDs like levetiracetam or carbamazepine may be considered.
Emergency Care for Prolonged Seizures: Rectal diazepam or intranasal midazolam can be used to stop prolonged seizures.
Lifestyle Adjustments: Maintaining good sleep hygiene and minimizing stress can help reduce seizure frequency.
Prognosis
Seizure Resolution: Most children outgrow Panayiotopoulos syndrome by age 10-12.
Long-Term Outcomes: Neurological development and cognitive abilities remain normal.
Complications: Seizures can be frightening for caregivers due to their dramatic autonomic symptoms and prolonged duration, but they rarely cause physical harm.
Quality of Life
With proper diagnosis and reassurance, Panayiotopoulos syndrome has minimal impact on a child’s life. Educating families and caregivers about the condition and seizure first aid is essential for managing episodes and reducing anxiety. Regular follow-ups with a neurologist can ensure the child’s continued health and development.
