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Benign partial epilepsy in infancy long-term outcome and marginal syndromes
Akihisa Okumura1, Kazuyoshi Watanabe, Tamiko Negoro
1Department of Pediatrics, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, Aichi 466-8550, Japan. okumura@med.nagoya-u.ac.jp
Insights
Benign partial epilepsy in infancy (BPEI) generally has an excellent long-term outcome. Most children diagnosed with BPEI do not experience recurrent seizures or cognitive issues past age eight.
Area of Science:
- Neurology
- Pediatrics
- Epilepsy Research
Background:
- Benign partial epilepsy in infancy (BPEI) is characterized by Watanabe et al. as an infantile epilepsy with favorable seizure and developmental prognosis.
- Previous studies suggested good outcomes, but long-term follow-up data beyond early childhood were limited.
Purpose of the Study:
- To evaluate the long-term seizure and developmental outcomes of patients initially diagnosed with possible Benign Partial Epilepsy in Infancy (BPEI) up to and beyond 8 years of age.
- To identify potential marginal syndromes or atypical presentations associated with BPEI.
Main Methods:
- A telephone interview survey was conducted with 39 patients previously diagnosed with possible BPEI.
- Data collected focused on seizure recurrence, cognitive development, and associated neurological conditions up to age 8 and beyond.
Main Results:
- The vast majority of patients diagnosed with BPEI maintained an excellent seizure and developmental outcome beyond 8 years of age.
- Six out of 39 patients did not meet BPEI criteria at follow-up: two had late unprovoked seizures, three had cognitive problems (mild mental retardation or Asperger syndrome), and one had both.
- Potential marginal syndromes were observed, including associations with paroxysmal kinesigenic choreoathetosis and seizures during mild gastroenteritis.
Conclusions:
- Benign partial epilepsy in infancy (BPEI) demonstrates a predominantly excellent long-term prognosis, with most patients remaining seizure-free and without significant cognitive impairment.
- The study identified potential marginal syndromes, suggesting a spectrum of BPEI that warrants further investigation.
- Early diagnosis of BPEI generally predicts a favorable long-term trajectory, though careful monitoring for atypical presentations is advised.
Abstract:
Benign partial epilepsy in infancy (BPEI) is an infantile epilepsy with excellent seizure and developmental outcome proposed by Watanabe et al. Our telephone interview survey revealed that the long-term outcome of patients with BPEI was also excellent over 8 years of age. Six of 39 patients did not fulfill the criteria of BPEI by the last follow-up. Two patients had a recurrence of unprovoked seizure beyond 2 years of age, three had cognitive problems (mild mental retardation in two and Asperger syndrome in one) and the other had both a recurrence of seizure and mild mental retardation. These results indicates that a large majority of patients diagnosed as possible BPEI at 2 years of age did not have a recurrence of unprovoked seizures and mental problems beyond 8 years of age. Our study also suggested a presence of some marginal syndromes of BPEI. An association of paroxysmal kinesigenic choreoathetosis was observed in three patients. Another three patients had experienced seizures with mild gastroenteritis. The seizure outcome of three patients with mild cognitive problems was quite excellent. These patients can be grouped as a marginal syndrome of BPEI.
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