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Panayiotopoulos syndrome: a clinical, EEG, and neuropsychological study of 93 consecutive patients
Nicola Specchio1, Marina Trivisano, Vincenzo Di Ciommo
1Division of Neurology, Bambino Gesù Children's Hospital IRCCS, Rome, Italy. nicola.specchio@opbg.net
Insights
Panayiotopoulos syndrome (PS) is a common childhood epilepsy characterized by autonomic seizures. This study found PS has an excellent prognosis for seizure remission and neuropsychological development in children.
Area of Science:
- Pediatric Neurology
- Epilepsy Syndromes
- Clinical Neurophysiology
Background:
- Panayiotopoulos syndrome (PS) is a distinct childhood epilepsy syndrome.
- Characterized by prominent autonomic features and often prolonged seizures.
- Understanding its clinical, EEG, and prognostic profile is crucial for diagnosis and management.
Purpose of the Study:
- To investigate the clinical, electroencephalography (EEG), neuropsychological features, and prognosis of Panayiotopoulos syndrome (PS).
Main Methods:
- Retrospective analysis of 1,794 children aged 1-14 years with first afebrile focal seizures.
- 93 children (5.2%) were diagnosed with PS based on clinical criteria.
- Included EEG, neuropsychological testing (WISC-R), and long-term follow-up.
Main Results:
- Age at onset varied (1.1-8.6 years), with earlier onset in cases with multiple seizures.
- Autonomic seizures often began with emesis, pallor, or flushing, frequently leading to vomiting.
- EEG showed variable spikes (79.5%), background abnormalities (16.1%), or normal studies (5.4%).
- Neuropsychological testing revealed generally normal IQ, with minor differences in specific subtests compared to controls.
- Cumulative recurrence probability decreased over time, and most children achieved remission.
Conclusions:
- Panayiotopoulos syndrome represents a childhood susceptibility to autonomic seizures with an excellent prognosis.
- Early age of development is associated with PS.
- Seizure remission and neuropsychological development are generally favorable in children with PS.
Purpose:
To explore the clinical, electroencephalography (EEG), neuropsychological features, and prognosis of Panayiotopoulos syndrome (PS).
Methods:
Of 1,794 children aged between 1 and 14 years referred for the first afebrile focal seizure, between January 1992 and December 2004, 93 (5.2%) had PS according to clinical criteria.
Results:
Age at onset ranged from 1.1 to 8.6 years, and was earlier in children with more than one seizure. Autonomic seizures followed a stereotypical onset and progression. Emesis, pallor, or flushing was almost always among the first symptoms that usually culminated in vomiting (77.4% of patients). More than half (55%) of seizures were longer than 30 min but these did not appear to affect remission and number of seizures. Interictal EEG showed great variability, with 79.5% of patients showing spikes of variable localizations and evolution over time; 16.1% had background abnormalities only, and 5.4% had consistently normal EEG studies. Onsets in five ictal EEGs were posterior or anterior-left or right. On neuropsychological testing, IQ and subtests of Wechsler Intelligence Scale for Children-Revised (WISC-R) were within normal limits, although some minor statistically significant differences were found in arithmetic, comprehension, and picture arrangement in comparison with controls. Cumulative probability of recurrence was 57.6%, 45.6%, 35.1%, and 11.7% at 6, 12, 24, and 36 months, respectively, after the first seizure. Thirty-four (58.6%) of 59 patients treated with antiepileptic drugs continued having seizures before ultimate remission.
Discussion:
PS is a uniform childhood susceptibility to autonomic seizures that is related to early age of development and with excellent prognosis with regard to seizure remission and neuropsychological development.
