Cardiovascular and neuroendocrine features of Panayiotopoulos syndrome

Abstract

Panayiotopoulos syndrome (PS) can be best understood as constitutional, age-related susceptibility to early-onset benign childhood seizures with mainly autonomic symptoms and signs (AS/S). Typical ictal symptoms include nausea, retching, and vomiting (the latter in up to 70–80% of seizures, frequently recurring several times during the same ictal event); pupillary changes; cardiorespiratory, gastrointestinal, and thermoregulatory alterations; incontinence; coughing; and hypersalivation [ 1 Koutroumanidis M. Panayiotopoulos syndrome: An important electroclinical example of benign childhood system epilepsy. Epilepsia. 2007; 48: 1044-1053 Crossref PubMed Scopus (86) Google Scholar , 2 Covanis A. Panayiotopoulos syndrome: a benign childhood autonomic epilepsy frequently imitating encephalitis, syncope, migraine, sleep disorder, or gastroenteritis. Pediatrics. 2006; 118: e1237-e1243 Crossref PubMed Scopus (116) Google Scholar , 3 Ferrie C.D. Caraballo R. Covanis A. et al. Autonomic status epilepticus in Panayiotopoulos syndrome and other childhood and adult epilepsies: a consensus view. Epilepsia. 2007; 48: 1165-1172 Crossref PubMed Scopus (91) Google Scholar , 4 Panayiotopoulos C.P. Michael M. Sanders S. Valeta T. Koutroumanidis M. Benign childhood focal epilepsies: assessment of established and newly recognized syndromes. Brain. 2008; 131: 2264-2286 Crossref PubMed Scopus (294) Google Scholar ]. As a rule, awareness is intact at seizure onset but gradually fades, although it may fluctuate for a while; “simple” autonomic seizures with apparently retained awareness may occur, but most likely may pass unnoticed. The term ictal syncope characterizes a distinct part of the seizure, in which the child becomes unresponsive and flaccid; it conveys the clinical resemblance to ordinary vasovagal syncope, but the mechanism here is epileptic, as clinically suggested by the fact that horizontal position does not result in quick recovery and as documented with ictal video/EEG recordings [ [5] Oguni H. Hayashi K. Imai K. Hirano Y. Mutoh A. Osawa M. Study on the early-onset variant of benign childhood epilepsy with occipital paroxysms otherwise described as early-onset benign occipital seizure susceptibility syndrome. Epilepsia. 1999; 40: 1020-1030 Crossref PubMed Scopus (94) Google Scholar ]. All major series agree that about half of the seizures last from more than 30 minutes to hours, amounting to autonomic status epilepticus and mimicking severe cerebral insults or gastroenteritis; convulsive status epilepticus is unusual [ 1 Koutroumanidis M. Panayiotopoulos syndrome: An important electroclinical example of benign childhood system epilepsy. Epilepsia. 2007; 48: 1044-1053 Crossref PubMed Scopus (86) Google Scholar , 2 Covanis A. Panayiotopoulos syndrome: a benign childhood autonomic epilepsy frequently imitating encephalitis, syncope, migraine, sleep disorder, or gastroenteritis. Pediatrics. 2006; 118: e1237-e1243 Crossref PubMed Scopus (116) Google Scholar , 3 Ferrie C.D. Caraballo R. Covanis A. et al. Autonomic status epilepticus in Panayiotopoulos syndrome and other childhood and adult epilepsies: a consensus view. Epilepsia. 2007; 48: 1165-1172 Crossref PubMed Scopus (91) Google Scholar , 4 Panayiotopoulos C.P. Michael M. Sanders S. Valeta T. Koutroumanidis M. Benign childhood focal epilepsies: assessment of established and newly recognized syndromes. Brain. 2008; 131: 2264-2286 Crossref PubMed Scopus (294) Google Scholar ]. The EEG shows interictal occipital and multifocal extra-occipital functional spikes and occipital, frontal, and frontotemporal seizure onsets.