Benign partial epilepsy with secondarily generalized seizures in infancy

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Overview

Benign partial epilepsy with secondarily generalized seizures in infancy (BPEI) is a rare, age-related epilepsy syndrome that typically presents during the first two years of life. It belongs to the group of benign infantile epilepsies and is characterized by focal (partial) seizures that may spread to become secondarily generalized tonic-clonic seizures. The seizures primarily affect the nervous system, with episodes that can include motor manifestations such as limb jerking, deviation of the eyes or head, and sometimes progression to bilateral convulsive activity. Between seizures, affected infants typically show normal neurological development and normal findings on brain imaging (MRI). Electroencephalography (EEG) may show focal epileptiform discharges during the ictal period but is often normal or near-normal interictally. The condition is considered benign because seizures generally respond well to standard antiepileptic medications and tend to remit spontaneously during early childhood, usually by age 2 to 3 years. Commonly used treatments include antiepileptic drugs such as carbamazepine or valproate, which are typically effective in controlling seizures. Long-term neurodevelopmental outcomes are generally favorable, with most children achieving normal cognitive and motor milestones. A family history of benign infantile seizures may be present in some cases, suggesting a genetic predisposition. This condition should be distinguished from other more severe infantile epilepsy syndromes that carry a less favorable prognosis, making accurate diagnosis important for appropriate counseling and management.

Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Infantile

Begins in infancy, roughly 1 month to 2 years old

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Benign partial epilepsy with secondarily generalized seizures in infancy.

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No specialists are currently listed for Benign partial epilepsy with secondarily generalized seizures in infancy.

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Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

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Common questions about Benign partial epilepsy with secondarily generalized seizures in infancy

What is Benign partial epilepsy with secondarily generalized seizures in infancy?

Benign partial epilepsy with secondarily generalized seizures in infancy (BPEI) is a rare, age-related epilepsy syndrome that typically presents during the first two years of life. It belongs to the group of benign infantile epilepsies and is characterized by focal (partial) seizures that may spread to become secondarily generalized tonic-clonic seizures. The seizures primarily affect the nervous system, with episodes that can include motor manifestations such as limb jerking, deviation of the eyes or head, and sometimes progression to bilateral convulsive activity. Between seizures, affected

At what age does Benign partial epilepsy with secondarily generalized seizures in infancy typically begin?

Typical onset of Benign partial epilepsy with secondarily generalized seizures in infancy is infantile. Age of onset can vary across affected individuals.