The association between posterior fossa malformations and epilepsy is rarel
y reported in the literature. We describe 54 cases with posterior fossa mal
formations, according to embryogenesis classification, divided into two gro
ups on the basis of presence or absence of epilepsy. Epilepsy occurred in 2
2 cases (40.7%) and was not related to the type of posterior fossa malforma
tion or to supratentorial cerebral lesions associated with the malformation
. Familial antecedents for epilepsy and/or febrile convulsions influenced t
he presence of epilepsy in patients with posterior fossa malformations (P <
.01). Epilepsy was mainly partial (77.3%); benign partial/generalized epil
epsies and febrile convulsions occurred in 27.3% of cases. Seizures disappe
ared for 2 or more years at the end of follow-up in 36.4% of patients. Good
epilepsy prognosis was not related to the age at onset of seizures, famili
al antecedents for epilepsy and/or febrile convulsions, supratentorial asso
ciated lesions, or age of patients at the last observation. Profound mental
retardation prevailed in patients with epilepsy (P < .01), as did patholog
ic electroencephalograms (EEG) (P < .0001), with paroxysmal abnormalities (
P < .001) and asymmetry (P < .01). In our 54 cases of posterior fossa malfo
rmation, we identified two risk factors for epilepsy: familial antecedents
for epilepsy and/or febrile convulsions and the involvement of the cerebell
um in the malformation.