INTRODUCTION The corpus callosum is the major neopallial connection between the two cerebral hemispheres. The corpus callosum agenesis (CCA) is found in 14% of CNS malformations. The diagnosis is based on neuroimaging procedures (ultrasonography, CT, MRI). The CCA is usually associated with facial dysmorphia, developmental delay and epileptic seizures. Two casuistic are studied, one with necropsies material and another with CCA patients alive, on which CCA was frequently associated with other CNS malformations, in order to establish the circumstances in which the epileptic seizures have been observed. Development. It is commonly admitted that the presenting signs or symptoms in individuals with CCA are due to concurrent brain abnormalities and that isolated CCA is essentially asymptomatic. The CCA is a common component in some malformative syndromes, frequent in another, and occasional in many of them. The CCA has been reported in many chromosomal aberrations and less frequently in inborn errors of metabolism and neurocutaneous diseases. In the casuistic studies of 73 patients alive, 25 (39%) have presented epileptic fits: in 24 of them the CCA was associated to another brain abnormalities; in 17 cases, the first seizures was recorded during the first year, in 6 cases between 1 and 3 years and in cases after 2 years: the type of epileptic seizures is variable: neonatal convulsions in 4 cases, infantile spasms in 5, unilateral fits in 3, and partial seizures in 1 case. In the necropsies casuistic with 26 CCA cases, 6 (23%) suffered epileptic fits, all with another malformations of the CNS; in 3 the onset of the seizures was during the newborn period, 2 had infantile spasms during the first years and 1 case generalized seizures during the second year.
CONCLUSIONS 1. Epileptic seizures were observed in 23%-39% of the CCA cases studied. 2. All cases with CCA, except for one, have also another brain abnormality. 3. In 70% of the cases, the onset of the fits takes place during the first year. 4. The type of seizures is variable with predominance of infantile spasms and unilateral seizures.
KeywordsAgenesis corpus callosumBrain malformationsEpileptic seizuresInfantile spasmsCategoriesEpilepsias y síndromes epilépticos
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