Panayiotopoulos syndrome: a clinical, EEG, and neuropsychological study of 93 consecutive patients.
OBJECTIVE
To explore the clinical, electroencephalography (EEG), neuropsychological features, and prognosis of Panayiotopoulos syndrome (PS).


METHODS
Of 1,794 children aged between 1 and 14 years referred for the first afebrile focal seizure, between January 1992 and December 2004, 93 (5.2%) had PS according to clinical criteria.


RESULTS
Age at onset ranged from 1.1 to 8.6 years, and was earlier in children with more than one seizure. Autonomic seizures followed a stereotypical onset and progression. Emesis, pallor, or flushing was almost always among the first symptoms that usually culminated in vomiting (77.4% of patients). More than half (55%) of seizures were longer than 30 min but these did not appear to affect remission and number of seizures. Interictal EEG showed great variability, with 79.5% of patients showing spikes of variable localizations and evolution over time; 16.1% had background abnormalities only, and 5.4% had consistently normal EEG studies. Onsets in five ictal EEGs were posterior or anterior-left or right. On neuropsychological testing, IQ and subtests of Wechsler Intelligence Scale for Children-Revised (WISC-R) were within normal limits, although some minor statistically significant differences were found in arithmetic, comprehension, and picture arrangement in comparison with controls. Cumulative probability of recurrence was 57.6%, 45.6%, 35.1%, and 11.7% at 6, 12, 24, and 36 months, respectively, after the first seizure. Thirty-four (58.6%) of 59 patients treated with antiepileptic drugs continued having seizures before ultimate remission.


CONCLUSIONS
PS is a uniform childhood susceptibility to autonomic seizures that is related to early age of development and with excellent prognosis with regard to seizure remission and neuropsychological development.
Specchio
Nicola
N
Division of Neurology, Bambino Gesù Children's Hospital IRCCS, Rome, Italy. nicola.specchio@opbg.net
Trivisano
Marina
M
Di Ciommo
Vincenzo
V
Cappelletti
Simona
S
Masciarelli
Giovanni
G
Volkov
Josiv
J
Fusco
Lucia
L
Vigevano
Federico
F
D000293:Adolescent; D017668:Age of Onset; D000927:Anticonvulsants / Q000627:therapeutic use; D001342:Autonomic Nervous System Diseases / Q000175:diagnosis / Q000188:drug therapy; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography / Q000379:methods / Q000706:statistics & numerical data*; D004828:Epilepsies, Partial / Q000175:diagnosis* / Q000188:drug therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D009483:Neuropsychological Tests / Q000706:statistics & numerical data*; D011379:Prognosis; D012189:Retrospective Studies; D013577:Syndrome
D000927:Anticonvulsants