Overview
Progressive dementia with neuroserpin inclusion bodies, also known as familial encephalopathy with neuroserpin inclusion bodies (FENIB), is a very rare inherited brain disease. It is caused by a change (mutation) in the SERPINI1 gene, which provides instructions for making a protein called neuroserpin. When this protein is abnormal, it clumps together and forms deposits inside brain cells, especially in areas of the brain responsible for thinking, memory, and behavior. Over time, these clumps damage and destroy brain cells, leading to a slow but steady decline in mental function. The main symptoms include progressive memory loss, personality changes, difficulty thinking clearly, and seizures. The disease tends to get worse over time, eventually affecting a person's ability to carry out everyday tasks and live independently. Symptoms usually begin in adulthood, though the age of onset can vary depending on the specific gene mutation involved. There is currently no cure for FENIB. Treatment focuses on managing symptoms, such as using anti-seizure medications to control epilepsy and supportive care to maintain quality of life. Research into this condition is ongoing, but effective disease-modifying therapies are not yet available.
Key symptoms:
Progressive memory lossDifficulty thinking and concentratingPersonality and behavior changesSeizures (epilepsy)Confusion and disorientationDifficulty with language and finding wordsLoss of ability to perform daily tasksMood changes including depression or anxietyGradual loss of independence
Autosomal dominant
Passed on from just one parent; each child has about a 50% chance of inheriting it
Adult
Begins in adulthood (age 18 or older)
FDA & Trial Timeline
1 eventTarpeyo: FDA approved
to reduce proteinuria in adults with primary immunoglobulin A nephropathy (IgAN) at risk of rapid disease progression, generally a urine protein-to-creatinine ratio (UPCR) > or = 1.5 g/g
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
1 availableTarpeyo
to reduce proteinuria in adults with primary immunoglobulin A nephropathy (IgAN) at risk of rapid disease progression, generally a urine protein-to-creatinine ratio (UPCR) > or = 1.5 g/g
Clinical Trials
View all trials with filters →No actively recruiting trials found for Progressive dementia with neuroserpin inclusion bodies at this time.
New trials open frequently. Follow this disease to get notified.
Specialists
View all specialists →No specialists are currently listed for Progressive dementia with neuroserpin inclusion bodies.
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Financial Resources
1 resourcesTravel Grants
No travel grants are currently matched to Progressive dementia with neuroserpin inclusion bodies.
Community
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Start the conversation →Latest news about Progressive dementia with neuroserpin inclusion bodies
1 articlesCaregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.Which specific mutation in the SERPINI1 gene has been found, and what does that mean for how quickly the disease might progress?,Should other family members be tested for this gene mutation, and how should we approach that conversation?,What anti-seizure medications are most appropriate, and what are the signs that they are not working well enough?,Are there any clinical trials or research studies we should consider enrolling in?,What specialists should be part of our care team, and how often should we have follow-up appointments?,What safety measures should we put in place at home as the disease progresses?,What support services are available for both the patient and family caregivers?
Common questions about Progressive dementia with neuroserpin inclusion bodies
What is Progressive dementia with neuroserpin inclusion bodies?
Progressive dementia with neuroserpin inclusion bodies, also known as familial encephalopathy with neuroserpin inclusion bodies (FENIB), is a very rare inherited brain disease. It is caused by a change (mutation) in the SERPINI1 gene, which provides instructions for making a protein called neuroserpin. When this protein is abnormal, it clumps together and forms deposits inside brain cells, especially in areas of the brain responsible for thinking, memory, and behavior. Over time, these clumps damage and destroy brain cells, leading to a slow but steady decline in mental function. The main sym
How is Progressive dementia with neuroserpin inclusion bodies inherited?
Progressive dementia with neuroserpin inclusion bodies follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Progressive dementia with neuroserpin inclusion bodies typically begin?
Typical onset of Progressive dementia with neuroserpin inclusion bodies is adult. Age of onset can vary across affected individuals.