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.
Division of Neurology, Bambino Gesù Children's Hospital IRCCS, Rome, Italy. nicola.specchio@opbg.net
Nicola
N
Specchio
Marina
M
Trivisano
Vincenzo
V
Di Ciommo
Simona
S
Cappelletti
Giovanni
G
Masciarelli
Josiv
J
Volkov
Lucia
L
Fusco
Federico
F
Vigevano
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