MeSH Browser

MeSH Supplementary: Frontotemporal Dementia With Motor Neuron Disease
Unique ID: C566288
RDF Unique Identifier: http://id.nlm.nih.gov/mesh/C566288
Entry Term(s): ALSFTD; Amyotrophic Lateral Sclerosis And-Or Frontotemporal Dementia; FTDALS; FTDMND; Frontotemporal Dementia-Amyotrophic Lateral Sclerosis
Registry Numbers: 0
Heading Mapped to: *Amyotrophic Lateral Sclerosis; *Frontotemporal Dementia
Frequency: 138
Note: An autosomal dominant neurodegenerative disorder caused by hexanucleotide repeat expansion (GGGGCC) in a noncoding region of the C9ORF72 gene. It is characterized by adult onset of frontotemporal dementia or ALS in an affected individual, with significant intrafamilial variation. Patients tend to show a lower age of onset, shorter survival, bulbar symptom onset, increased incidence of neurodegenerative disease in relatives, and a propensity toward psychosis or hallucinations compared to patients with other forms of ALS and/or FTD. Psychiatric disturbances may also predate the onset of dementia. OMIM: 105550
Date of Entry: 2012/11/05
Revision Date: 2015/11/10

Frontotemporal Dementia With Motor Neuron Disease MeSH Supplementary Concept Data 2025