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Start free with EleplanFrontotemporal dementia with motor neuron disease
ORPHA:275872Disease
Also called FTD-ALS · FTD-MND · Frontotemporal dementia with amyotrophic lateral sclerosis
What it is
Frontotemporal dementia with motor neuron disease (FTD-MND) is a type of frontotemporal lobar degeneration characterized by the insidious onset (between the ages of 38-78 years) of dementia-associated psychiatric symptoms (e.g. personality changes, uninhibited behavior, irritability, aggressiveness), memory difficulties, global intellectual impairment, emotional disorders and transcortical motor aphasia that eventually leads to mutism, in addition to the manifestations of motor neuron disease such as neurogenic muscular wasting (similar to what is seen in amyotrophic lateral sclerosis). The disease is progressive, with death occurring 2-5 years after onset.
Key facts
- Age of onset
- Adult
- Inheritance
- Autosomal dominant
- Classified as
- Disease
Signs and symptoms
Very common80–99%
3Common30–79%
20- Abnormality of extrapyramidal motor function
- Apathy
- Apraxia
- Atypical behavior
- Degeneration of the lateral corticospinal tracts
- Depression
- Distal muscle weakness
- Dysarthria
- Dyscalculia
- Dysphagia
- Generalized amyotrophy
- Gliosis
- Hallucinations
- Neuronal loss in the cerebral cortex
- Paraparesis
- Parkinsonism
- Progressive cerebellar ataxia
- Proximal muscle weakness
- Tetraparesis
- Weakness due to upper motor neuron dysfunction
Sometimes5–29%
11- Abnormal mitochondrial morphology
- Babinski sign
- Bilateral sensorineural hearing impairment
- Bulbar palsy
- Disinhibition
- Fasciculations
- Global brain atrophy
- Hyporeflexia
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.
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