Publications by authors named "R Barohn"

Introduction: Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disease with no known cure, limited treatment options with minimal benefits, and significant unmet need for disease modifying therapies.

Aims: This study investigated memantine's impact on ALS progression, with an additional focus on the effects of memantine on cognitive and behavioral changes associated with the disease.

Methods: A randomized, double-blind, placebo-controlled clinical trial was conducted from December 2018 to September 2020.

View Article and Find Full Text PDF

Background: Non-dystrophic myotonias are skeletal muscle channelopathies caused by ion channel dysfunction. Symptom onset is frequently in the first decade of life, causing disability in a young cohort. Although there is no cure, symptomatic treatments exist.

View Article and Find Full Text PDF

Amyotrophic lateral sclerosis (ALS) patients lack effective treatments to maintain motor and neuromuscular function. This study aimed to evaluate the effect of a home-based exercise program on muscle strength, ALS scores, and transcriptome in ALS patients, Clinical Trials.gov #NCT03201991 (28/06/2017).

View Article and Find Full Text PDF
Article Synopsis
  • * Most MG patients have acetylcholine receptor antibodies, but other notable antibodies like MuSK and LRP4 have also been identified, with some being available for testing.
  • * The Myasthenia Gravis Foundation of America (MGFA) has established a disease classification system, and a range of therapies for managing MG are being researched and developed, guided by international recommendations.
View Article and Find Full Text PDF
Article Synopsis
  • The study assessed the effectiveness of the Inclusion Body Myositis Functional Rating Scale (IBMFRS) in measuring physical function in patients with IBM, focusing on its validity, reliability, responsiveness, and meaningful change threshold.
  • Data were collected from a 20-month multi-center clinical trial, employing various statistical methods to test the scale’s performance, revealing strong correlations with other health outcomes and reliability in its administration formats.
  • Results indicated that IBMFRS is a dependable tool for evaluating the impact of IBM on patients, with a score drop of 2 points considered a significant decline in function.
View Article and Find Full Text PDF