C9+ ALS | C9− ALS | |
---|---|---|

n | 14 | 156 |

Male, n (%) | 10 (71) | 107 (69) |

Age at MRI, median (range) | 53.7 (40.6–67.0) | 61.8 (24.0–78.9) |

Right-handed, n (%)* | 11 (92) | 129 (91) |

ALSFRS-R, median (range) | 42 (33–47) | 42 (23–48) |

Age at onset, median (range) | 52.9 (39.1–66.4) | 60.9 (23.5–78.1) |

Bulbar onset, n (%) | 5 (36) | 41 (26) |

Disease duration in months, median (range) | 12.3 (3.3–31.8) | 12.0 (3.7–75.7) |

Rate of decline, median (range)† | 0.56 (0.15–1.14) | 0.46 (0.00–3.92) |

FAB score, median (range)‡ | 16 (13–18) | 17 (6–18) |

VFI score, median (range)§ | 12.9 (3.0–56.0)^{f} | 6.7 (3.2–24.9)^{f} |

ALS-FTD, n (%)¶ | 1 (7) | 1 (1) |

Familial ALS, n (%) | 7 (50)^{f} | 10 (6)^{f} |

Survival in months, median (95% CI) | 40.1 (28-NA) | 35.6 (31.6 to 44.2) |

*Handedness in a subset of patients; C9+ ALS: n=12, C9− ALS: n=141.

†Points decrease of ALSFRS-R per month.

‡FAB in a subset of patients (maximum score 18): C9+ ALS: n=9, C9− ALS: n=103.

§VFI in a subset of patients: C9+ ALS: n=8, C9− ALS: n=95.

¶ALS-FTD according to the Neary criteria.

^{f}p<0.01.ALSFRS-R, ALS-Functional Rating Scale Revised; ALS-FTD, amyotrophic lateral sclerosis with comorbid frontotemporal dementia; FAB, Frontal Assessment Battery; NA, not available; VFI, Verbal Fluency Index.