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Frontotemporal dementia with motor neuron disease
Disease definition
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; see this term). The disease is progressive, with death occurring 2-5 years after onset.
ORPHA:275872
Classification level: Disorder- Synonym(s):
- FTD-ALS
- FTD-MND
- Frontotemporal dementia with amyotrophic lateral sclerosis
- Prevalence: Unknown
- Inheritance: Autosomal dominant
- Age of onset: Adult
- ICD-10: G31.0
- ICD-11: 6D83
- OMIM: 105550 608030 612069 613954 615911 616437 616439 619133 619141
- UMLS: C3888102
- MeSH: C566288
- GARD: -
- MedDRA: -
A summary on this disease is available in Español (2015) Italiano (2015) Nederlands (2015)
Detailed information
Disease review articles
- Clinical genetics review
- English (2021) - GeneReviews
- English (2023) - GeneReviews
Genetic Testing
- Guidance for genetic testing
- Français (2018, pdf) - ANPGM
produced/endorsed by ERN(s) 
produced/endorsed by FSMR(s)Additional information
Further information on this disease
Patient-centred resources for this disease
Research activities on this disease
Specialised Social Services
The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.