Pattern of childhood neuronal migrational disorders in Oman
Roshan L Koul1, Amna M Alfuitasi, Dilip K Sankhla
1Department of Child Health, Sultan Qaboos University Hospital, PO Box 38, BW-1, Muscat 123, Sultanate of Oman. Fax. +968 24413128. E-mail: koulroshan@gmail.com / koul@squ.edu.om.
Insights
Neuronal migrational disorders (NMD) are common in pediatric neurology, often presenting with developmental delay and intractable epilepsy. Corpus callosum agenesis and lissencephaly are the most frequent NMD types, impacting early brain development.
Area of Science:
- Pediatric Neurology
- Neurodevelopmental Disorders
- Neuroimaging
Background:
- Neuronal migrational disorders (NMD) represent a spectrum of congenital brain abnormalities.
- These disorders arise from disruptions in neuronal development during embryonic and fetal stages.
- NMD are frequently associated with significant neurological deficits in children.
Purpose of the Study:
- To document the prevalence and patterns of various neuronal migrational disorders (NMD).
- To identify associated neurological conditions in children diagnosed with NMD.
- To analyze the clinical presentation and outcomes of NMD in a pediatric neurology cohort.
Main Methods:
- Retrospective analysis of pediatric patients with psychomotor delay and epilepsy.
- Brain imaging, primarily Magnetic Resonance Imaging (MRI), was used for NMD diagnosis.
- Data collected from a tertiary child neurology service over a 14-year period.
Main Results:
- Eighty-six cases of NMD were identified, with corpus callosum agenesis (48 cases) and lissencephaly/pachygyria (16 cases) being most prevalent.
- Developmental delay (93%) and motor deficits (77.9%) were common associated findings.
- Epilepsy affected 46.5% of cases, often proving intractable, with partial/complex partial seizures being most frequent.
Conclusions:
- NMD are a significant cause of psychomotor delay and refractory epilepsy in pediatric neurology.
- Disruptions in early embryonic/fetal development (6-26 weeks gestation) underlie NMD.
- Advances in genetic research are crucial for understanding mechanisms, enabling early diagnosis, and potential prevention of NMD.
Objective:
To record the pattern of different neuronal migrational disorders (NMD) and their associated neurological conditions.
Methods:
The data were collected at the Child Neurology Services of Sultan Qaboos University Hospital, Oman, from January 1993 to September 2006 from all children with psychomotor delay and epilepsy, who underwent brain imaging (mostly MRI). The MR imaging was used for the diagnosis of a neuronal migration anomaly.
Results:
There were 86 cases of NMD. Corpus callosum agenesis and lissencephaly/pachygyria formed the major group. There were 48 cases of corpus callosum agenesis, and 16 cases of lissencephaly/pachygyria. Other disorders were 10 cases of heterotopias, 5 schizencephaly, 3 holoprosencephaly, 2 polymicrogyria, and one each of hemimegalencephaly, and hydranencephaly. Developmental delay was the most common associated finding noted in 80 (93%) cases. Sixty-seven (77.9%) cases had motor deficit. Forty out of 86 (46.5%) cases had epilepsy. Partial/partial complex seizures were the most common at 13 out of 40 (32.5%). Syndromic seizures were seen in 11 out of 40 (27.5%) cases. The seizures were controlled in only 3/40 (7.5%) cases.
Conclusion:
The NMD constitute a significant number of child neurology patients with psychomotor delay and intractable epilepsy. Exogenic and genetic factors affecting the early embryonic and fetal development from sixth to twenty-sixth weeks of gestation result in NMD. Recent genetic studies are defining the underlying mechanism and these studies will help in early diagnosis and possible prevention of NMD.
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