Fragile-X-syndrome is the most common cause of inherited intellectual disability.
Epilepsy is reported to occur in 10–20% of individuals with Fragile-X-syndrome. A
frequent seizure/electroencephalogram (EEG) pattern resembles that of benign
rolandic epilepsy.We describe the clinical features, EEG findings and evolution in three
patients affected by Fragile-X-syndrome and epilepsy mimicking Panayiotopoulos
syndrome. Age at seizure onset was between 4 and about 7 years. Seizures pattern
comprised a constellation of autonomic symptoms with unilateral deviation of the eyes
and ictal syncope. Duration of the seizures could be brief or lengthy. Interictal EEGs
revealed functional multifocal abnormalities. The evolution was benign in all patients
with seizures remission before the age of 14. This observation expands the spectrum of
benign epileptic phenotypes present in Fragile-X-syndrome and may be quite helpful in
guiding anticonvulsant management and counseling families as to expectations
regarding seizure remission.