Overview
Frontotemporal dementia, right temporal atrophy variant (sometimes called right temporal variant FTD or semantic dementia, right-predominant type) is a rare form of frontotemporal dementia where the right side of the temporal lobe of the brain gradually shrinks and loses function. The temporal lobes sit on the sides of the brain and play important roles in recognizing faces, understanding emotions, and processing social cues. When the right temporal lobe is primarily affected, people often develop difficulty recognizing familiar faces (called prosopagnosia), trouble understanding emotions in others, and changes in personality and social behavior. They may become socially withdrawn, lose empathy, or develop unusual behaviors such as rigid routines, changes in food preferences, or compulsive habits. Unlike the left temporal variant of semantic dementia, where word-finding and language problems are the earliest signs, the right temporal variant tends to present first with behavioral and emotional changes, which can sometimes be mistaken for psychiatric conditions. Over time, language difficulties may also develop as the disease progresses to involve both temporal lobes. There is currently no cure for this condition. Treatment focuses on managing symptoms and supporting quality of life. Medications may help with behavioral symptoms such as agitation, depression, or compulsive behaviors. Speech therapy, occupational therapy, and structured daily routines can also be beneficial. Research into disease-modifying treatments is ongoing, but no therapies have yet been proven to slow or stop the underlying brain degeneration.
Key symptoms:
Difficulty recognizing familiar facesLoss of empathy or emotional understandingPersonality changesSocial withdrawal or inappropriate social behaviorCompulsive or ritualistic behaviorsChanges in eating habits or food preferencesLoss of interest in previously enjoyed activitiesDifficulty understanding emotions in others' facial expressionsGetting lost in familiar placesTrouble recognizing familiar objects or animalsRigid daily routinesWord-finding difficulties that develop over timeDepression or anxietyLack of awareness of their own symptoms
Variable
Can be inherited in different ways depending on the underlying gene
Adult
Begins in adulthood (age 18 or older)
FDA & Trial Timeline
3 eventsUniversità degli Studi di Brescia — NA
Nantes University Hospital — NA
University of Pennsylvania — PHASE3
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Frontotemporal dementia, right temporal atrophy variant.
3 clinical trialsare actively recruiting — trials can provide access to cutting-edge therapies.
View clinical trials →Specialists
View all specialists →No specialists are currently listed for Frontotemporal dementia, right temporal atrophy variant.
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
Travel Grants
No travel grants are currently matched to Frontotemporal dementia, right temporal atrophy variant.
Community
No community posts yet. Be the first to share your experience with Frontotemporal dementia, right temporal atrophy variant.
Start the conversation →Latest news about Frontotemporal dementia, right temporal atrophy variant
No recent news articles for Frontotemporal dementia, right temporal atrophy variant.
Follow this condition to be notified when news becomes available.
Caregiver 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.What specific type of frontotemporal dementia do I have, and how was this determined?,Should I or my family members undergo genetic testing?,What behavioral changes should I expect as the disease progresses?,Are there any clinical trials I might be eligible for?,What medications or therapies might help with my current symptoms?,When should I stop driving, and how will we know when it's no longer safe?,What resources are available for my family and caregivers?
Common questions about Frontotemporal dementia, right temporal atrophy variant
What is Frontotemporal dementia, right temporal atrophy variant?
Frontotemporal dementia, right temporal atrophy variant (sometimes called right temporal variant FTD or semantic dementia, right-predominant type) is a rare form of frontotemporal dementia where the right side of the temporal lobe of the brain gradually shrinks and loses function. The temporal lobes sit on the sides of the brain and play important roles in recognizing faces, understanding emotions, and processing social cues. When the right temporal lobe is primarily affected, people often develop difficulty recognizing familiar faces (called prosopagnosia), trouble understanding emotions in o
At what age does Frontotemporal dementia, right temporal atrophy variant typically begin?
Typical onset of Frontotemporal dementia, right temporal atrophy variant is adult. Age of onset can vary across affected individuals.
Are there clinical trials for Frontotemporal dementia, right temporal atrophy variant?
Yes — 3 recruiting clinical trials are currently listed for Frontotemporal dementia, right temporal atrophy variant on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.