Fever as a seizure precipitant factor in Panayiotopoulos syndrome: A clinical and genetic study

Child Neurology and Psychiatry Unit, University of Bologna, S. Orsola-Malpighi Hospital, via Massarenti 11, 40138 Bologna, Italy.
Seizure (Impact Factor: 1.82). 03/2012; 21(2):141-3. DOI: 10.1016/j.seizure.2011.09.013
Source: PubMed


To examine fever as a precipitating factor for focal seizures in patients with Panayiotopoulos syndrome (PS) and evaluate the role of SCN1A in PS patients with seizures triggered by fever.
From January 2000 to June 2008, we identified patients referred for seizures who fulfilled the criteria of PS. Patients were divided into two groups, according to the presence (group A) or the absence (group B) of seizures triggered by fever. Electroclinical features of the two groups were compared. In addition, an analysis of SCN1A in patients of group A was performed.
Thirty patients fulfilled the inclusion criteria. Eleven patients (36%) had at least one focal autonomic seizure triggered by fever (group A). In group A, 7/11 patients (63.5%) had the first focal autonomic seizure during a febrile illness. Two of these 7 patients were misdiagnosed at the onset of PS. The median age at the onset of PS was slightly lower in group A than in group B (p=.050). Moreover, patients in group A more frequently had a positive familial history of febrile seizures (FS) (p=.047). No mutations of SCN1A were found in any of the 10 patients screened.
Fever is a common trigger for focal autonomic seizures in PS. Knowing that an autonomic manifestation during fever can be an epileptic seizure could facilitate diagnosis and prevent unnecessary investigations and erroneous treatments. Moreover, our data show that SCN1A gene does not contribute significantly to susceptibility to autonomic seizures during fever in patients with PS.

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    • "In one of them, two seizures with fever occurred on the same day during perforated appendicitis treatment after a three-year seizure-free period following two autonomic seizures. This suggests that fever is a precipitating factor for seizures in PS [14]. The other patient had an autonomic seizure 6 months after discontinuation of medication at the age of 14 and after a 5-year seizure-free period. "
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    ABSTRACT: The aim of the study was to evaluate the demographic, clinical, and EEG characteristics of patients with Panayiotopoulos syndrome (PS) and the course of their illness. Thirty-eight patients followed up with a diagnosis of PS between January 2011 and December 2013 were evaluated. We found high rates of personal history of febrile convulsions, breath-holding spells, and family history of febrile convulsions, afebrile convulsion/epilepsy, migraine, and breath-holding spells. Seizures started before the age of eight in 87% of the patients, and the mean age at seizure onset was 4.6years. Seizures were sleep-related in 81.5%, and autonomic status was seen in a third of the patients. The number of seizures was between 2 and 10 in 66% of the patients. The most common symptoms were ictus emeticus, eye/head deviation, and altered consciousness. Rolandic features were seen in 26% of the patients, and visual symptoms in 5%. Multifocal epileptiform discharges on EEG were identified in 84% of the patients. Two or more antiepileptic drugs were required in only 13% of the patients. Evolution to electrical status epilepticus in sleep and Gastaut-type epilepsy were seen in patients with more than ten seizures. The high rates of febrile convulsions, afebrile convulsions/epilepsy, migraine, and breath-holding spells in the patients and families suggest the importance of genetic factors and, perhaps, a common pathogenesis. However, the high rates of febrile convulsions and breath-holding spells in patients can be related to a misdiagnosis because of the similar symptoms. Despite its disturbing symptoms, PS is a benign epileptic syndrome requiring multiple antiepileptic drug use only in a small proportion of patients.
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