Documentation of autonomic seizures and autonomic status epilepticus with ictal EEG in Panayiotopoulos syndrome
Nicola Specchio1, Marina Trivisano, Dianela Claps
1Division of Neurology, Bambino Gesù Children's Hospital IRCCS, Rome, Italy. nicola.specchio@opbg.net
Insights
Panayiotopoulos syndrome (PS) is a common childhood epilepsy. This study documents PS as a multifocal autonomic epilepsy, challenging previous views.
Area of Science:
- Neurology
- Epileptology
- Pediatric Neurology
Background:
- Panayiotopoulos syndrome (PS) is a prevalent childhood epilepsy syndrome characterized by autonomic seizures and status epilepticus.
- Despite its high prevalence, the underlying pathophysiology and EEG characteristics of PS have been debated.
- Understanding the electrophysiological basis of PS is crucial for accurate diagnosis and management.
Observation:
- This study presents ictal electroencephalogram (EEG) documentation of autonomic seizures and autonomic status epilepticus in six PS cases.
- A review of 14 previously reported cases was also conducted.
- Interictal EEGs revealed spikes with variable locations and temporal changes, while ictal EEG onsets were diverse, originating from anterior or posterior regions.
Findings:
- Ictal EEG onsets in PS were multifocal, often showing theta waves mixed with spikes and fast rhythms.
- Autonomic manifestations like vomiting and eye deviation did not correlate with specific EEG activation regions.
- The findings support PS as a multifocal autonomic epilepsy, with clinical symptoms arising from widespread epileptogenic foci.
Implications:
- These results challenge the notion of PS originating from a single focal point.
- The study supports the hypothesis that PS involves a hyperexcitable central autonomic network influenced by variable epileptogenic foci.
- This understanding may refine diagnostic criteria and treatment strategies for childhood epilepsy with autonomic features.
Abstract:
Panayiotopoulos syndrome (PS) is a common childhood susceptibility to autonomic seizures and status epilepticus. Despite its high prevalence, PS has been a source of significant debate. We present ictal EEG documentation of autonomic seizures and autonomic status epilepticus in six cases of PS and a review of 14 reported cases. Interictal EEGs showed spikes of variable locations that often changed with time. Ictal EEG onsets were also variable, starting from wide anterior or posterior regions usually with theta waves intermixed with small spikes and fast rhythms. Ictal vomiting and other autonomic manifestations, as well as deviation of the eyes, did not appear to relate to any specific region of EEG activation. These data document that PS is a multifocal autonomic epilepsy and support the view that the clinical manifestations are likely to be generated by variable and widely spread epileptogenic foci acting on a temporarily hyperexcitable central autonomic network.
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