Pattern of childhood epilepsies with neuronal migrational disorders in Oman.
Author: Koul R, Jain R, Chacko A.
Source:
Journal of child neurology, 21(11), 945-949.
Neuronal migrational disorders form a significant cause of psychomotor delay and
intractable epilepsy in children. Pediatric neurology services are available at
Sultan Qaboos University Hospital, Muscat, Oman, which is a tertiary care
hospital for the whole country. The children undergoing evaluation for
developmental delay and epilepsy formed the subjects of the study. Data were
analyzed from children found to have neuronal migrational disorders on imaging
(computed tomography [CT] or magnetic resonance imaging [MRI]). There were 40
cases of neuronal migrational disorders. Corpus callosum agenesis and
lissencephaly or pachygyria formed the major group. There were 22 cases of corpus
callosum agenesis, 12 of lissencephaly or pachygyria, 2 of schizencephaly, and 1
each of polymicrogyria, holoprosencephaly, hydranecephaly, and
hemimegalencephaly. Nineteen of these 40 (47.5%) cases of neuronal migrational
disorders had epilepsy. The break-down was 8 of 22 cases of corpus callosum
agenesis (36%), 7 of 12 (58.3%) cases of lissencephaly or pachygyria, and 1 each
of polymicrogyria, hydranencephaly, and hemimegalencephaly. The family history of
developmental delay, similar to the index case, was present in two children with
lissencephaly. However, the brain imaging did not reveal the abnormality. The
types of seizures were infantile spasms in five, tonic-clonic in nine, myoclonic
in two, partial in one, and mixed in five. Nineteen of 40 cases of neuronal
migrational disorders had epilepsy. Only 2 of 19 (10.5%) with epilepsy had good
seizure control. This raises the possibility of more and more surgical
intervention in the management of such cases. Neuronal migrational disorders are
related to exogenous and genetic factors from the 6th to 26th weeks of gestation.
Molecular and genetic research is defining the mechanism of these disorders. This
could help in early diagnosis, prevention, and eventual gene therapy in such
conditions.