Panayiotopoulos Syndrome: An Important Electroclinical Example of Benign Childhood System Epilepsy

• Published in: Epilepsia (Copenhagen) Vol. 48; no. 6; pp. 1044 - 1053
• Main Author: Koutroumanidis, Michael
• Format: Journal Article
• Language: English
• Published: Malden, USA Blackwell Publishing Inc 01.06.2007
• Subjects: Autonomic Nervous System Diseases - diagnosis; Autonomic Nervous System Diseases - epidemiology; Autonomic Nervous System Diseases - physiopathology; Autonomic seizures; Cerebral Cortex - physiopathology; Child; Comorbidity; Diagnosis, Differential; Electroencephalography - statistics & numerical data; Epilepsies, Partial - diagnosis; Epilepsies, Partial - epidemiology; Epilepsies, Partial - physiopathology; Epilepsy, Rolandic - diagnosis; Epilepsy, Rolandic - epidemiology; Epilepsy, Rolandic - physiopathology; Humans; Idiopathic epileptogenic focus; Models, Neurological; Occipital Lobe - physiopathology; Panayiotopoulos syndrome; Remission, Spontaneous; Status Epilepticus - diagnosis; Status Epilepticus - physiopathology; Syndrome; System epilepsies; Terminology as Topic; Videotape Recording
• ISSN: 0013-9580; 1528-1167
• DOI: 10.1111/j.1528-1167.2007.01096.x

As a result of the converging evidence from multiple large independent studies, Panayiotopoulos syndrome (PS) is now formally recognized as a distinct clinical entity within the spectrum of benign focal epilepsies of childhood. Clinically, PS is manifested by predominantly autonomic seizures and electrographically with multifocal interictal spikes, while the few published ictal recordings have documented onsets of variable lobar topography. These typical electroclinical features do not allow straightforward assignment to a distinctive cortical area, rendering the term “focal”—as we currently understand it—problematic. This is a critical review of the clinical and EEG features of PS, focusing on those characteristics that may shed some light on its so far elusive pathophysiology. We also explore its electroclinical similarities to other idiopathic “focal” epilepsies and its differences to symptomatic focal epilepsies that may also manifest with autonomic ictal symptoms and signs. This methodology allows the formation of a rational hypothesis on the pathophysiology of PS that seems to be emerging as a good model for the so‐called “system” (nonsymptomatic) epilepsies, with potentially important taxonomic implications.