Caring for someone with this diagnosis? Eleplan keeps diagnoses, medications, documents and every specialist in one plan.
Start free with EleplanBehavioral variant of frontotemporal dementia
ORPHA:275864Disease
Also called bv-FTD
What it is
Behavioral variant of frontotemporal dementia (bv-FTD) is a form of frontotemporal dementia (FTD), characterized by progressive behavioral impairment and a decline in executive function with frontal lobe-predominant atrophy.
Key facts
- Prevalence
- 1-9 / 100 000 (Netherlands)
- Age of onset
- Adult
- Inheritance
- Autosomal dominant
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Very common80–99%
25- Abnormal repetitive mannerisms
- Aggressive behavior
- Aphasia
- Atypical behavior
- Disinhibition
- Dyscalculia
- Dysgraphia
- Dyslexia
- Echolalia
- Emotional blunting
- Frontotemporal cerebral atrophy
- Frontotemporal dementia
- Hyperorality
- Inappropriate behavior
- Irritability
- Lack of insight
- Loss of speech
- Memory impairment
- Mental deterioration
- Perseveration
- Personality changes
- Poor speech
- Restlessness
- Restrictive behavior
- Thickened nuchal skin fold
Common30–79%
4Sometimes5–29%
11- Abnormality of extrapyramidal motor function
- Abulia
- Apathy
- Astrocytosis
- Bilateral tonic-clonic seizure
- Fasciculations
- Gait disturbance
- Hyperreflexia
and 3 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Genes
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
Powered by Eleplan
A rare diagnosis is just the start. Eleplan keeps the whole care plan in one place.
Diagnoses, medications, documents, appointments, and the whole care team — organized and always in sync, so you are not retelling the same story to every new specialist. With Ellie, your AI care assistant, on top of it. Free to start.