Pattern of Childhood Epilepsies With Neuronal Migrational Disorders in Oman

Koul, Roshan; Jain, Rajeev; Chacko, Alexander
November 2006
Journal of Child Neurology;Nov2006, Vol. 21 Issue 11, p945
Academic Journal
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, tonicclonic 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.


Related Articles

  • A Review of 326 Children with Developmental and Physical Disabilities, Consecutively Taught at the Movement Development Clinic: Prevalence and Intervention Outcomes of Children with DCD. Miyahara, Motohide; Yamaguchi, Mami; Green, Clare // Journal of Developmental & Physical Disabilities;Aug2008, Vol. 20 Issue 4, p353 

    Data from 326 children taught consecutively in a physical education clinic were reviewed. Only 63 children (39 males, 24 females) (19%) met the diagnostic criteria for developmental coordination disorder (DCD) specified by the American Psychiatric Association (). The other children were...

  • Hemiconvulsion-Hemiplegia-Epilepsy Syndrome as a Presenting Feature of L-2-Hydroxyglutaric Aciduria. Lee, Céline; Born, Mark; Salomons, Gajja S.; Jakobs, Cornelis; Woelfle, Joachim // Journal of Child Neurology;Jun2006, Vol. 21 Issue 6, p538 

    L-2-hydroxyglutaric aciduria was diagnosed in a 9-month-old female infant after a complex febrile convulsion with subsequent transient left-sided hemiplegia. The symptoms were consistent with acute hemiconvulsion-hemiplegia-epilepsy syndrome. Magnetic resonance imaging (MRI) of the brain...

  • Parenting Stress in Parents of Children With Epilepsy and Asthma. Hua-Huei Chiou; Liang-Po Hsieh // Journal of Child Neurology;Mar2008, Vol. 23 Issue 3, p301 

    The purposes of this study were to identify differences in parenting stress between 2 samples and examine the factors associated with parenting stress. An asthma sample was selected to contrast with the epilepsy sample because both medical conditions are chronic episodic conditions that require...

  • Kidney Stones and the Ketogenic Diet: Risk Factors and Prevention. Sampath, Amitha; Kossoff, Eric H.; Furth, Susan L.; Pyzik, Paula L.; Vining, Eileen P. G. // Journal of Child Neurology;Apr2007, Vol. 22 Issue 4, p375 

    A cohort study was performed of children started on the ketogenic diet for intractable epilepsy from 2000 to 2005 (n = 195). Children who developed kidney stones were compared with those without in terms of demographics, urine laboratory markers, and intervention with urine alkalinization...

  • Systemic and Ocular Findings in 100 Patients With Optic Nerve Hypoplasia. Garcia, M. Lourdes; Ty, Edna B.; Taban, Mehryar; Rothner, A. David; Rogers, Douglas; Traboulsi, Elias I. // Journal of Child Neurology;Nov2006, Vol. 21 Issue 11, p949 

    To describe associated ocular, neurologic, and systemic findings in a population of children with optic nerve hypoplasia, a retrospective chart review of 100 patients with optic nerve hypoplasia for the presence of neurologic, radiologic, and endocrine abnormalities was performed. Neuroimaging...

  • Differentiating Between Benign and Less Benign: Epilepsy Surgery in Symptomatic Frontal Lobe Epilepsy Associated With Benign Focal Epileptiform Discharges of Childhood. Altenmüller, Dirk-Matthias; Schulze-Bonhage, Andreas // Journal of Child Neurology;Apr2007, Vol. 22 Issue 4, p456 

    Benign focal epileptiform discharges of childhood are a genetically determined electroencephalographic trait. Assessment of their clinical relevance in children with epilepsy may be difficult if imaging reveals a lesion congruent or incongruous with the focus of the benign focal epileptiform...

  • Natural History and Magnetic Resonance Imaging Follow-up in 9 Sturge-Weber Syndrome Patients and Clinical Correlation. Udani, Vrajesh; Pujar, Suresh; Munot, Pinki; Maheshwari, Shailendra; Mehta, Nirad // Journal of Child Neurology;Apr2007, Vol. 22 Issue 4, p479 

    The natural history of Sturge-Weber Syndrome is variable where some patients have refractory epilepsy and persistent neurologic deficits while others do well. Also, evolution of MRI abnormalities is largely unknown, This long-term follow-up study tries to address these two issues. This...

  • Megalencephaly, Polymicrogyria, and Hydrocephalus (MPPH) Syndrome: A New Case With Syndactyly. Pisano, Tiziana; Meloni, Marta; Cianchetti, Carlo; Falchi, Melania; Nucaro, Annalisa; Pruna, Dario // Journal of Child Neurology;Aug2008, Vol. 23 Issue 8, p916 

    Megalencephaly, polymicrogyria, and hydrocephalus (MPPH) syndrome is characterized by megalencephaly, perisylvian polymicrogyria, postaxial polydactyly, and hydrocephalus. Seven cases have been reported. This report presents a new sporadic patient with megalencephaly, polymicrogyria, and...

  • BEATING Bad Seizures. Mittan, Robert J. // Exceptional Parent;Jun2005, Vol. 35 Issue 6, p32 

    Discusses ways of dealing with bad seizures and epilepsy in children. Common types of seizures; Importance of monitoring seizures in order to determine more effective treatment. INSET: Left Side of the Brain..


Read the Article


Sorry, but this item is not currently available from your library.

Try another library?
Sign out of this library

Other Topics