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Study on early-onset benign occipital seizure susceptibility syndrome
H Oguni1, K Hayashi, M Funatsuka
1Department of Pediatrics, Tokyo Woman's Medical University, Tokyo, Japan.
Insights
Early-onset benign occipital seizure susceptibility syndrome in children typically shows a good prognosis. Most children achieve seizure freedom by age 12, even those with initially drug-resistant epilepsy.
Area of Science:
- Pediatric Neurology
- Epileptology
- Clinical Neuroscience
Background:
- Early-onset epilepsy requires accurate diagnosis to predict prognosis.
- Benign occipital seizure susceptibility syndrome is a recognized epilepsy syndrome in children.
- Confirming the benign nature of this syndrome is crucial for clinical management.
Purpose of the Study:
- To prospectively evaluate the long-term prognosis of early-onset benign occipital seizure susceptibility syndrome.
- To confirm the benign prognosis and identify factors influencing seizure course.
- To analyze clinical characteristics, treatment response, and EEG findings.
Main Methods:
- Prospective study of 37 children meeting specific diagnostic criteria for benign occipital seizure susceptibility syndrome.
- Inclusion criteria: normal development pre-epilepsy, onset 1-8 years, normal brain imaging, specific seizure semiology (ictal vomiting, tonic eye-deviations), and normal EEG background.
- Follow-up for over 2 years, analyzing seizure frequency, treatment response, and EEG data.
Main Results:
- Seizure frequency varied, with a median of five seizures per child.
- 15 children experienced recurrent prolonged seizures initially resistant to antiepileptic drugs.
- At final follow-up, 28 patients (76%) were seizure-free for at least 2 years, with remission by age 12.
- Occipital EEG foci were common, with a later shift in predominant foci observed in some cases.
Conclusions:
- Early-onset benign occipital seizure susceptibility syndrome generally has a benign prognosis.
- While some children present with severe, drug-resistant seizures, most achieve long-term remission.
- The syndrome's clinical spectrum ranges from mild to severe, but ultimate remission by adolescence is typical.
Abstract:
We prospectively studied the early-onset benign occipital seizure susceptibility syndrome to confirm the benign prognosis. The patients were 37 children followed for more than 2 years after meeting the following criteria on the first examination: (1) normal development before the onset of epilepsy, (2) onset between 1 and 8 years of age, (3) normal brain MRI and cranial CT findings, (4) partial seizures manifested both initial ictal vomiting and tonic eye-deviations, and (5) normal background activity with or without epileptic EEG foci regardless of location. The incidence and clinical characteristics of seizures, response to treatment, and EEG findings were analyzed. The total number of seizures ranged from one (n = 6) to 27 times, with a median of five times. Recurrent prolonged attacks resistant to antiepileptic drugs were recognized in 15 children, who had earlier onset of epilepsy and more frequent complications than the remaining 22 children. Interictal EEG revealed occipital foci in 26 children, 17 of whom later revealed a shift in predominant foci. At the final examinations, 28 patients had been seizure-free for at least 2 years. The clinical picture of this syndrome ranges from those with a few seizures to those with recurrent prolonged seizures initially resistant to antiepileptic drugs despite ultimate remission by 12 years of age.
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