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


METHODS
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.


RESULTS
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.


CONCLUSIONS
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.
Child Neurology and Psychiatry Unit, University of Bologna, S. Orsola-Malpighi Hospital, via Massarenti 11, 40138 Bologna, Italy.
Duccio Maria
DM
Cordelli
Anna
A
Aldrovandi
Valentina
V
Gentile
Caterina
C
Garone
Sara
S
Conti
Arianna
A
Aceti
Elena
E
Gennaro
Federico
F
Zara
Emilio
E
Franzoni
D017668:Age of Onset; D002648:Child; D002675:Child, Preschool; D004828:Epilepsies, Partial / Q000175:diagnosis* / Q000209:etiology / Q000235:genetics*; D005260:Female; D005334:Fever / Q000150:complications* / Q000175:diagnosis; D005838:Genotype; D006801:Humans; D007223:Infant; D008297:Male; D062550:NAV1.1 Voltage-Gated Sodium Channel; D009419:Nerve Tissue Proteins / Q000235:genetics*; D003294:Seizures, Febrile / Q000175:diagnosis* / Q000209:etiology / Q000235:genetics*; D015222:Sodium Channels / Q000235:genetics*; D013577:Syndrome
D062550:NAV1.1 Voltage-Gated Sodium Channel; D009419:Nerve Tissue Proteins; C568242:SCN1A protein, human; D015222:Sodium Channels