Frontotemporal Lobar Degeneration

Heterogeneous group of neurodegenerative disorders characterized by frontal and temporal lobe atrophy associated with neuronal loss, gliosis, and dementia. Patients exhibit progressive changes in social, behavioral, and/or language function. Multiple subtypes or forms are recognized based on presence or absence of TAU PROTEIN inclusions. FTLD includes three clinical syndromes: FRONTOTEMPORAL DEMENTIA, semantic dementia, and PRIMARY PROGRESSIVE NONFLUENT APHASIA.

Also Known As:

Degeneration, Frontotemporal Lobar; FTLD; Degenerations, Frontotemporal Lobar; FTLDs; Frontotemporal Lobar Degenerations; Lobar Degeneration, Frontotemporal; Lobar Degenerations, Frontotemporal

Nervous System Diseases: 14737
- Neurodegenerative Diseases: 44095
  - TDP-43 Proteinopathies: 223
    - Frontotemporal Lobar Degeneration: 1712
- Central Nervous System Diseases: 4371
  - Brain Diseases: 16141
    - Dementia: 39493
      - Frontotemporal Lobar Degeneration: 1712
Nutritional and Metabolic Diseases: 15
- Metabolic Diseases: 10634
  - Proteostasis Deficiencies: 1476
    - TDP-43 Proteinopathies: 223
      - Frontotemporal Lobar Degeneration: 1712
Mental Disorders: 25040
- Neurocognitive Disorders: 1532
  - Dementia: 39493
    - Frontotemporal Lobar Degeneration: 1712

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Disease

Drug/Important Bio-Agent (IBA)