Highlights
- •Only few epidemiological studies on Lower Motor Neuron (LMN) syndromes survival are available.
- •We evaluated a cohort of LMN phenotype (LMNP) and assessed the contribute on survival or progression of the disease of the presence of subclinical Upper Motor Neuron (UMN) impairment at the diagnosis.
- •No difference was found in median survival times and 4 years survival rates according to the presence of Transcranial Magnetic Stimulation (TMS) impairment.
- •TMS abnormalities were associated with a 3.5 times higher risk for reaching King's 4 stage (Hazard Ratio: 3.5; 95% Confidence Interval: 1.1–10.9; p = 0.03).
- •Our data suggest a role of TMS abnormalities as potential indicator of disease progression and multidistrectual involvement in patients with pure clinical LMN phenotype at the diagnosis.
Abstract
Background
Only few epidemiological studies on survival of Lower Motor Neuron (LMN) phenotype
(LMNP) are available and with controversial results.
Aims
To prospectively evaluate a cohort of LMNP patients and assess the possible contribute
on survival or disease's progression according to the presence of subclinical Upper
Motor Neuron (UMN) impairment at the diagnosis.
Methods
Forty LMNP among 176 consecutive incident ALS cases observed in our tertiary center
from the ALS-Apulia Register were enrolled in the study. Each patient underwent to
a neurophysiological study with transcranial magnetic stimulation (TMS) at diagnosis.
The primary outcome was the impact of abnormalities at TMS on survival time (from
symptoms onset or diagnosis to death, tracheostomy or 30 June 2020, as censoring time).
Secondary outcome was time to reach the King's 4 stage.
Results
Approximately one half of LMNP reached the primary outcome during the study period.
No difference was found in median survival times and 4 years survival rates according
to the presence of TMS impairment. On the other hand, a shorter median time to reach
the King's 4 from onset was observed in the group of LMNP with TMS abnormalities (16 months
versus 50 months; p = 0.008). Consistently, TMS abnormalities were associated with a 3.5 times higher
risk for reaching King's 4 stage (Hazard Ratio: 3.5; 95% Confidence Interval: 1.1–10.9;
p = 0.03).
Conclusion
Our data suggest a role of TMS abnormalities as potential indicator of disease progression
and multidistrectual involvement in patients with pure clinical LMN phenotype at the
diagnosis.
Keywords
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Article info
Publication history
Published online: March 16, 2021
Accepted:
March 14,
2021
Received in revised form:
March 6,
2021
Received:
January 18,
2021
Identification
Copyright
© 2021 Published by Elsevier B.V.
