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Table 1.

C9orf72-FTD/ALS: Frequency of Diagnoses Based on Clinical Findings Alone

DiagnosisFrequencyComments (Frequency)
FTD34.8%
  • Behavioral variant FTD (31.4%)
  • Nonfluent/agrammatic variant PPA (1.8%)
  • Semantic variant PPA (0.9%)
  • Other tauopathy: corticobasal degeneration, progressive supranuclear palsy, other PPA (0.7%)
ALS19.3%
FTD-ALS11.0%
Atypical presentations mimicking other kinds of neurodegenerative brain diseases35.0%
  • Alzheimer disease, Parkinson disease, Huntington disease, & dementia w/Lewy bodies are common.
  • Also incl vascular dementia & dementia not otherwise specified
  • Atypical parkinsonian syndromes 1

ALS = amyotrophic lateral sclerosis; FTD = frontotemporal dementia; PPA = primary progressive aphasia

1.

From: C9orf72 Frontotemporal Dementia and/or Amyotrophic Lateral Sclerosis

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