Frontotemporal Dementia With Motor Neuron Disease

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

Also Known As:

ALSFTD; Amyotrophic Lateral Sclerosis And-Or Frontotemporal Dementia; FTDALS; FTDMND; Frontotemporal Dementia-Amyotrophic Lateral Sclerosis

Nervous System Diseases: 14737
- Neurodegenerative Diseases: 44095
  - Motor Neuron Disease: 1851
    - Amyotrophic Lateral Sclerosis: 12080
      - Frontotemporal Dementia With Motor Neuron Disease: 20
  - TDP-43 Proteinopathies: 223
    - Amyotrophic Lateral Sclerosis: 12080
      - Frontotemporal Dementia With Motor Neuron Disease: 20
    - Frontotemporal Lobar Degeneration: 1712
      - Frontotemporal Dementia: 2813
        Frontotemporal Dementia With Motor Neuron Disease: 20
- Central Nervous System Diseases: 4371
  - Brain Diseases: 16141
    - Dementia: 39493
      - Frontotemporal Lobar Degeneration: 1712
        Frontotemporal Dementia: 2813
        Frontotemporal Dementia With Motor Neuron Disease: 20
  - Spinal Cord Diseases: 4420
    - Amyotrophic Lateral Sclerosis: 12080
      - Frontotemporal Dementia With Motor Neuron Disease: 20
- Neuromuscular Diseases: 2048
  - Motor Neuron Disease: 1851
    - Amyotrophic Lateral Sclerosis: 12080
      - Frontotemporal Dementia With Motor Neuron Disease: 20
Nutritional and Metabolic Diseases: 15
- Metabolic Diseases: 10634
  - Proteostasis Deficiencies: 1476
    - TDP-43 Proteinopathies: 223
      - Amyotrophic Lateral Sclerosis: 12080
        Frontotemporal Dementia With Motor Neuron Disease: 20
      - Frontotemporal Lobar Degeneration: 1712
        Frontotemporal Dementia: 2813
        Frontotemporal Dementia With Motor Neuron Disease: 20
Mental Disorders: 25040
- Neurocognitive Disorders: 1532
  - Dementia: 39493
    - Frontotemporal Lobar Degeneration: 1712
      - Frontotemporal Dementia: 2813
        Frontotemporal Dementia With Motor Neuron Disease: 20

Francisco Padron Selected Research

Francisco Padron Research Topics

Disease

Drug/Important Bio-Agent (IBA)