disease 3,096 words KG: ent-dise-7738e353 2026-03-28
kind:diseasesection:diseases
Contents

FTD-ALS Spectrum

Disease Info
ALS incidence1-2 per 100,000 person-years globally
FTD incidence4-15 per 100,000 persons aged 45-64 years
FTD-ALS overlap5-15% of ALS patients have comorbid FTD
Gender distributionMale predominance (1.3-1.5:1 for ALS; 1:1 for FTD)
Age of onsetTypically 50-65 years for both conditions
Normal repeats<30
Pathological repeats>30 (typically 60-1000)
PenetranceAge-dependent, with most carriers developing symptoms by age 80
MechanismsThree proposed pathogenic mechanisms:
DatabasesOMIMOrphanetClinicalTrialsPubMed

Knowledge Graph

Related Hypotheses (30)

Microglial AIM2 Inflammasome as the Primary Driver of TDP-43
Score: 0.60
Multiplexed Base Editing for Simultaneous Neuroprotective Ge
Score: 0.53
Acid-Degradable LNP-Mediated Prenatal CRISPR Intervention fo
Score: 0.47
Cryptic Exon Silencing Restoration
Score: 0.46
Cross-Seeding Prevention Strategy
Score: 0.45

Related Analyses (23)

How do pathological stress granules in neurodegeneration esc
neurodegeneration · failed
RNA binding protein dysregulation across ALS FTD AD
neurodegeneration · failed
TDP-43 phase separation therapeutics for ALS-FTD
neurodegeneration · completed
RNA binding protein dysregulation across ALS FTD and AD
neurodegeneration · completed
TDP-43 phase separation therapeutics for ALS-FTD
neurodegeneration · archived

Related Experiments (30)

Autophagy Enhancement Drug Screening for Neurodegeneration
clinical · proposed · Score: 0.40
Proposed experiment from debate on TDP-43 undergoes liquid-l
falsification · proposed · Score: 0.40
Mechanism: C9orf72 Hexanucleotide Repeat Expansion in ALS/FT
validation · proposed · Score: 0.40
C9orf72 Phenotype Divergence: ALS vs FTD Mechanism Study
clinical · proposed · Score: 0.40
Frontal and Temporal Lobe Selective Vulnerability in FTD — M
validation · proposed · Score: 0.40