Self Limited Familial and Non-Familial Neonatal Infantile Seizures
What is self-limited neonatal-infantile seizures?
The epilepsy syndrome of self-limited neonatal-infantile seizures used to be known as benign familial or non-familial neonatal-infantile seizures. Seizures begin in the first several months of life in an otherwise healthy infant. Typical seizure onset is between 2 days and 7 months of life. This type of epilepsy can affect infants in different ways. Some infants may have changes in thinking or cognitive problems as well as seizures. They may also have problems with balance and coordination.
There is no difference in the electrical patterns on EEG or the features of neonatal-infantile seizures that have a familial or non-familial pattern. The only difference is the presence of family history of seizures. If no family history is present, it is thought the genetic change causing this condition occurred for the first time in the child (termed de novo).
How often this syndrome occurs in not know. This may be due in part to the overlap between this condition and self-limited neonatal and infantile seizures.
Males and females are equally affected by this condition. Changes in several different genes has been seen in children with self-limited familial and non-familial neonatal-infantile seizures.
- An autosomal dominant expression pattern has been seen - this means that a change in only one copy of the gene is needed to cause the condition.
- The two genes most commonly identified include KCNQ2 and SCN2A.
- Children with an identified SCN2A mutation are more likely to have seizure begin after six months of age.
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Contact Our HelplineWhat type of seizures are seen?
The typical seizures have repetitive jerking on one side of the face or body, such as the arm, leg, or both, and often. The jerking often spreads to jerking movements on both sides of the body. The seizures sometimes cluster together when they occur.
How are self-limited neonatal-infantile seizures diagnosed?
Diagnosis is based on key clinical and EEG features. The required clinical features include seizures starting with the first several months of life in an otherwise healthy infant. This means that there were no health concerns during the pregnancy or delivery. Tests may include:
- EEG: The EEG will show abnormal electrical activity during a seizure. Between seizures, the EEG will typically be normal.
- MRI: This should be normal.
- Lumbar puncture (spinal tap): This is often done to evaluate for a serious bacterial infection, unless there is already a known family history of this condition and patient is healthy and does not have a fever.
- Genetic testing: Genetic testing is recommended in infants with a family history of similar seizures in the newborn and infant period.
How are self-limited neonatal-infantile seizures treated?
Typically, treatment is initiated with anti-seizure medications. Depending on the specific genetic mutation, sodium-channel blocking medications may be considered.
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Find Your Local Epilepsy FoundationWhat is the outlook?
Seizures typically stop by 1-2 years of age. They sometimes can last into early childhood. However, there can be an increased risk of febrile seizures and afebrile seizures later in life.
Historically, it is thought that development is normal and there is no long-term learning or cognitive effects. More recently, it has been suggested that mild learning difficulties may be present in some children.
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Subscribe to Our NewsletterReferences
- Wolff M, Johannesen KM, Hedrich UBS, et al. Genetic and phenotypic heterogeneity suggest therapeutic implications in SCN2A-related disorders. Brain. 2017: 140; 1316-1336.
- Grinton BE, Heron SE, Pelekanos JT. Familial neonatal seizures in 36 families: Clinic and genetic features correlate with outcome. Epilepsia. 2015; 56(7): 1071-1080.
- Learn more at http://www.epilepsydiagnosis.org.
- More from the National Institutes of Health.
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