The Incidence of Amyotrophic Lateral Sclerosis in Ohio 2016-2018: The Ohio Population-Based ALS Registry.

INTRODUCTION: Amyotrophic lateral sclerosis (ALS) is a fatal, neuromuscular disease with no cure. ALS incidence rates have not been assessed specifically in Ohio, yet the state contains both metropolitan and rural areas with a variety of environmental factors that could contribute to disease etiology. We report the incidence of ALS in Ohio residents diagnosed from October 2016 through September 2018. METHODS: We engaged practitioners from 9 Ohio sites to identify newly diagnosed ALS patients and to complete case report forms with demographic and clinical information. ALS was diagnosed according to the Awaji criteria and classified as either definite, probable, or possible. We developed a method to estimate missing cases using a Poisson regression model to impute cases in counties with evidence of undercounting. RESULTS: We identified 333 newly diagnosed ALS patients residing in Ohio during the 2-year index period and found incidence rates varied in the 88 state counties. After incorporating the estimated 27% of missing cases, the corrected crude annual incidence was 1.96/100,000 person-years, and the age- and gender-standardized incidence was 1.71/100,000 person-years (standardized to the 2010 US census). DISCUSSION/CONCLUSION: The estimated Ohio incidence of ALS is overall similar to that reported in other states in the USA. This study reveals a geospatial variation in incidence within the state, and areas with higher rates warrant future investigation.

Risk factors for amyotrophic lateral sclerosis: A regional United States case-control study.

Most amyotrophic lateral sclerosis (ALS) cases are considered sporadic, without a known genetic basis, and environmental exposures are thought to play a causal role. To learn more about sporadic ALS etiology, we recruited n = 188 ALS patients from northern New England and Ohio and matched controls 2:1 from the general population of the same regions. Questionnaires evaluated the association between a variety of lifestyle, behavioral (ie, hobbies and activities), and occupational factors and the risk of ALS, including the duration of time between exposure and ALS onset, and exposure frequency. Head trauma was associated with increased ALS risk (adjusted odds ratio [OR] 1.60 95% confidence interval [CI] 1.04-2.45), with significantly greater effects for injuries occurring 10 or more years prior to symptom onset (P = .037). ALS risk was increased for those reporting severe electrical burns (adjusted OR 2.86, 95% CI 1.37-6.03), with odds ratios highest for burns after age 30 (OR 3.14), and for burns 10 or more years prior to symptom onset (OR 3.09). Hobbies involving lead were the most strongly associated with ALS risk (adjusted OR 2.92, 95% CI 1.45-5.91). Exposures to lead 20 or more years prior to diagnosis had larger effect sizes compared to those occurring more recently. Holding a job in mechanics, painting, or construction was associated with ALS. The identification of these specific environmental factors associated with ALS highlight the need for future prospective and laboratory studies to assess causality, biological mechanisms, and find prevention or treatment opportunities.

The Oral Secretion Scale and Prognostic Factors for Survival in Subjects With Amyotrophic Lateral Sclerosis.

BACKGROUND: Noninvasive ventilation (NIV) can be tolerated in patients with amyotrophic lateral sclerosis (ALS), unless bulbar impairment becomes severe. Excessive oral secretions may result in NIV intolerance and insufficient ventilation. OBJECTIVE: To assess the reliability of the Oral Secretion Scale (OSS) for predicting the tolerance of NIV, when to initiate hospice or transition to tracheostomy, and prognostic factors for survival of users of NIV. METHODS: A validated OSS was developed to measure oral secretions in correlation with the ability to swallow saliva and clear the upper airway: OSS score of 4 = normal (automatic swallow); OSS score of 3 = infrequent secretions (automatic swallow decreased); OSS score of 2 = occasional drooling and/or pooling (conscious swallow required); OSS score of 1 = severe, frequent drooling and/or pooling (conscious swallow difficult); OSS score of 0 = most severe, constant drooling/pooling (conscious swallow impossible). A total of 137 subjects were followed up prospectively during ongoing patient visits from NIV initiation until death or tracheostomy. Survival was calculated by using Kaplan-Meier analysis. OSS scores when NIV became intolerable were determined. Uni- and/or multivariate Cox-regression analyses showed prognostic factors that affect survival. RESULTS: The median months of survival from NIV initiation were the following: 11 (95% CI 7.3-14.0), 5 (95% CI 3.1-6.1), and 1 (95% CI 1.1-1.5) stratified by OSS scores of 4, 3-2, and 1, respectively; and 21 (95% CI 8.6-33.2), 8 (95% CI 3.4-11.5), 6 (95% CI 4.2-8.2), and 2 (95% CI 1.5-2.7) stratified by 24, 17-23, 4-16, and <4 h/d of NIV use, respectively. Survival was significantly (P < .001) longer with an OSS score of 4 than an OSS score of 1 at NIV initiation; and significantly (P < .001) longer when NIV used 24 h/d than <24 h/d. In the subjects unable to tolerate NIV, ≥80% had OSS score of 1 or 0. Univariate and multivariate analyses hazard ratio 4.91, 95% CI 2.98-8.09, P < .001, and hazard ratio 4.60, 95% CI 2.66-7.96, P < .001), respectively, showed hours per day of NIV use was a significant factor associated with survival. CONCLUSIONS: The subjects with an OSS score of 4 tolerated NIV and survived significantly longer than subjects with an OSS score of <4. An OSS score of 1 signaled NIV intolerance and the need for hospice or transition to planned tracheostomy. Use of OSS can help guide NIV management decisions.

Validation of robust tools to measure sialorrhea in amyotrophic lateral sclerosis: a study in a large French cohort.

There is an unmet need for validated tools to measure sialorrhea in amyotrophic lateral sclerosis, especially to evaluate treatments. We assessed the inter-/intra-rate reviewer reliability of two scales: the Oral Secretion Scale (OSS), specifically developed for ALS patients, and the Sialorrhea Scoring Scale (SSS), initially developed for Parkinson's disease patients. Sialorrhea was rated in 69 ALS consecutive patients by four evaluators: two neurologists, one nurse and one speech therapist. Inter-rater reliability was evaluated by the light kappa coefficient and intra-rater reliability by the weighted kappa coefficient. We also compared patients' and caregivers' answers. Results demonstrated that the two scales present a high inter-/intra-rater reliability: weighted kappas were 0.85 for both scales and light kappas 0.89 for the OSS and 0.88 for the SSS. Both scales also showed a good intra-profession reliability (OSS kappa = 0.84; SSS kappa = 0.79) and agreement between patients' and caregivers' answers. The SSS showed a higher responsiveness compared to OSS. In conclusion, both Oral Secretion Scale and Sialorrhea Scoring Scale are reliable tools to measure sialorrhea in ALS patients. Because of the wide range of salivation degrees, SSS may be more sensitive as a tool to evaluate treatments in patients with severe hypersialorrhea.