The NEALS primary lateral sclerosis registry.

BACKGROUND AND OBJECTIVE: Primary lateral sclerosis (PLS) is a neurodegenerative disease characterized by progressive upper motor neuron dysfunction. Because PLS patients represent only 1 to 4% of patients with adult motor neuron diseases, there is limited information about the disease's natural history. The objective of this study was to establish a large multicenter retrospective longitudinal registry of PLS patients seen at Northeast ALS Consortium (NEALS) sites to better characterize the natural progression of PLS. Methods: Clinical characteristics, electrophysiological findings, laboratory values, disease-related symptoms, and medications for symptom management were collected from PLS patients seen between 2000 and 2015. Results: The NEALS registry included data from 250 PLS patients. Median follow-up time was 3 years. The mean rate of functional decline measured by ALSFRS-R total score was -1.6 points/year (SE:0.24, n = 124); the mean annual decline in vital capacity was -3%/year (SE:0.55, n = 126). During the observational period, 18 patients died, 17 patients had a feeding tube placed and 7 required permanent assistive ventilation. Conclusions: The NEALS PLS Registry represents the largest available aggregation of longitudinal clinical data from PLS patients and provides a description of expected natural disease progression. Data from the registry will be available to the PLS community and can be leveraged to plan future clinical trials in this rare disease.

Assessment of motor cortex excitability and inhibition during a cognitive task in individuals with concussion.

PRIMARY OBJECTIVE: To examine the function of the motor cortex during executive function tasks in individuals with concussion, relative to healthy controls. METHODS AND PROCEDURES: Transcranial magnetic stimulation (TMS) was used to assess motor cortex excitability and inhibition acutely, within 72 hours, and over two months, post-concussion in 23 participants, nine individuals with concussion and 14 controls. Participants performed a cognitive task during TMS to determine the impact of cognitive task on the motor cortex. MAIN OUTCOMES AND RESULTS: Resting motor threshold (p = 0.02) and motor-evoked potential (MEPRest) amplitude (p = 0.03) were different between groups, both suggesting greater corticospinal excitability in individuals with concussion. Cortical silent period (CSP) duration was greater at 72 hours (p = 0.03), one month (p = 0.003) and two months (p = 0.05) in individuals with concussion, suggesting increased intracortical inhibition. The performance of a cognitive task caused an increase in MEPRest (p = 0.006) and CSP (p = 0.04), compared to baseline in both groups, but no interaction of condition by group (p ≥ 0.91) for either measure. CONCLUSION: Simultaneously performing a cognitive task during motor cortex assessments increased corticospinal excitability and intracortical inhibition; however, the increase was not different between groups.