Sai Anmisha Doddamreddy1, Jordan Killion1, Shafeeq Ladha1, Brad Racette1
1Barrow Neurological Institute
Objective:
To identify factors associated with specific diagnostic and surgical procedures often misdiagnosed in people with amyotrophic lateral sclerosis (ALS).
Background:
ALS is a fatal neurodegenerative disease with median survival of 2 to 5 years. Despite the need for early diagnosis, delays of 12-15 months are common due to poor recognition of the disease by non-neurologists, causing unnecessary testing.
Design/Methods:
We performed a population-based case-control study of incident ALS cases and controls (2016-2018) utilizing prodromal disease claims data (2014-18). Diagnostic and procedural codes prior to ALS diagnosis were analyzed using multivariable logistic regression to compare the occurrence of different diagnoses and procedures between ALS cases and controls, adjusting for demographics and healthcare use. Among ALS cases, we modeled factors associated with diagnostic testing for ALS mimics.
Results:
ALS cases were substantially more likely to be diagnosed with cervical and lumbar spine pathology, including spondylosis with myelopathy (OR: 8.3, 95% CI: 5.4–12.9) and lumbar radiculopathy (OR: 2.3, 95% CI: 1.8–2.8). ALS patients also had a greater risk of being diagnosed with carpal tunnel syndrome (OR: 5.0, 95% CI: 3.0–8.6) and stroke-related diagnoses such as dysarthria following cerebral infarction (OR: 7.2, 95% CI: 3.5–14.9). Cervical spine fusion surgery (OR: 6.2, 95% CI: 3.0–12.5) and diagnostic laryngoscopy (OR: 8.3, 95% CI: 5.0–13.7) were common among cases than controls. Among ALS patients, 40.4% had at least one potentially misattributed diagnosis and 36.7% underwent a related procedure. Younger patients (under 85) were more likely to receive testing or procedures compared to those 85 and older (OR: 0.43, 95% CI: 0.26–0.72), and White individuals were more likely than Black individuals (OR: 0.43, 95% CI: 0.22–0.87).
Conclusions:
ALS patients were more likely to have diagnostic testing and procedures for ALS mimics, many of which are likely unnecessary. These findings highlight the need for early and more accurate diagnosis to improve patient care.
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