Literature DB >> 30684209

Tollgate-based progression pathways of ALS patients.

Özden O Dalgıç1,2, F Safa Erenay3, Kalyan S Pasupathy4,5, Osman Y Özaltın6, Brian A Crum7, Mustafa Y Sir8,9,10.   

Abstract

OBJECTIVE: To capture ALS progression in arm, leg, speech, swallowing, and breathing segments using a disease-specific staging system, namely tollgate-based ALS staging system (TASS), where tollgates refer to a set of critical clinical events including having slight weakness in arms, needing a wheelchair, needing a feeding tube, etc.
METHODS: We compiled a longitudinal dataset from medical records including free-text clinical notes of 514 ALS patients from Mayo Clinic, Rochester-MN. We derived tollgate-based progression pathways of patients up to a 1-year period starting from the first clinic visit. We conducted Kaplan-Meier analyses to estimate the probability of passing each tollgate over time for each functional segment.
RESULTS: At their first clinic visit, 93%, 77%, and 60% of patients displayed some level of limb, bulbar, and breathing weakness, respectively. The proportion of patients at milder tollgate levels (tollgate level < 2) was smaller for arm and leg segments (38% and 46%, respectively) compared to others (> 65%). Patients showed non-uniform TASS pathways, i.e., the likelihood of passing a tollgate differed based on the affected segments at the initial visit. For instance, stratified by impaired segments at the initial visit, patients with limb and breathing impairment were more likely (62%) to use bi-level positive airway pressure device in a year compared to those with bulbar and breathing impairment (26%).
CONCLUSION: Using TASS, clinicians can inform ALS patients about their individualized likelihood of having critical disabilities and assistive-device needs (e.g., being dependent on wheelchair/ventilation, needing walker/wheelchair or communication devices), and help them better prepare for future.

Entities:  

Keywords:  ALS progression; Kaplan–Meier analysis; Phenotypes; Tollgate-based staging system

Mesh:

Year:  2019        PMID: 30684209     DOI: 10.1007/s00415-019-09199-y

Source DB:  PubMed          Journal:  J Neurol        ISSN: 0340-5354            Impact factor:   4.849


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