Clinicians from the Child Neurology Division, Sapienza University of Rome, and 7 other pediatric neurology centers in Italy report a series of 19 Sotos syndrome (SS) patients with febrile seizures (FS) and/or epilepsy during childhood and a long-term follow-up. Fifteen patients were male and 4 female. Mean age at first evaluation was 5 years 2 months (range 4 months–15 years). Minimum follow-up period was 5 years (range 5-19 years). FS were recorded in 11(58%) patients and afebrile seizures (AFS) in 15 (79%). FS were only simple in 6/11 (55%), only complex in 1 (9%), and both simple and complex in 4 (36%). AFS were temporal lobe type in 40%, and were responsive to AED therapy. All patients had the typical facial features of SS (high, broad forehead, down-slanting palpebral fissures, and pointed chin), tall stature and macrocephaly, and learning disability. Neurologic disorders associated with SS are hypotonia, incoordination, delayed language and motor development, behavioral abnormalities, and seizures. [1]
COMMENT. More than half of children with SS have FS and/or AFS, and more than half of those with FS develop epilepsy, temporal lobe type in 40%. Seizures in SS are usually responsive to AED monotherapy.