James D. Berry
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View article: Pridopidine treatment in ALS: subgroup analyses from the HEALEY ALS Platform trial
Pridopidine treatment in ALS: subgroup analyses from the HEALEY ALS Platform trial Open
Objectives: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease with limited treatment options. Pridopidine, a selective sigma-1 receptor agonist, was evaluated in Regimen D of the HEALEY ALS Platform Trial. Alth…
View article: Participation in U.S.‐Based <scp>ALS</scp> Clinical Trials by Sex and Race
Participation in U.S.‐Based <span>ALS</span> Clinical Trials by Sex and Race Open
Introduction/Aims In global amyotrophic lateral sclerosis (ALS) trials, women and men appear to be proportionately enrolled, but quantification of enrollment by sex and race in U.S.‐based ALS trials is limited. The objective of this study …
View article: Autoimmune response to C9orf72 protein in amyotrophic lateral sclerosis 9119
Autoimmune response to C9orf72 protein in amyotrophic lateral sclerosis 9119 Open
Description Amyotrophic lateral sclerosis (ALS), like many neurodegenerative disorders, features cytoplasmic inclusion of proteins, with the most prominent component identified being TDP-43. Neuroinflammation is also apparent in affected t…
View article: Autoimmune response to C9orf72 protein in amyotrophic lateral sclerosis
Autoimmune response to C9orf72 protein in amyotrophic lateral sclerosis Open
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by a progressive loss of motor neurons. Neuroinflammation is apparent in affected tissues, including increased T cell infiltration and activation of microglia…
View article: Real‐World Clinical Experience With Sodium Phenylbutyrate and Taurursodiol at a Single Amyotrophic Lateral Sclerosis Center in the United States
Real‐World Clinical Experience With Sodium Phenylbutyrate and Taurursodiol at a Single Amyotrophic Lateral Sclerosis Center in the United States Open
Introduction/Aims Sodium phenylbutyrate ( PB ) and taurursodiol ( TURSO )— PB and TURSO —was approved as a treatment for amyotrophic lateral sclerosis ( ALS ) in the United States in 2022 based on the results of a phase 2 trial, but was vo…
View article: Multicenter Expanded Access Protocol for Research Through Access to Trehalose in People With Amyotrophic Lateral Sclerosis
Multicenter Expanded Access Protocol for Research Through Access to Trehalose in People With Amyotrophic Lateral Sclerosis Open
Introduction/Aims Expanded access protocols (EAPs) allow individuals ineligible for clinical trials to receive investigational products. EAP data can be collected in parallel to randomized clinical trials (RCTs) and serve as a source of ev…
View article: Design considerations for <i>C9orf72</i> disease prevention trials
Design considerations for <i>C9orf72</i> disease prevention trials Open
The idea that it might be possible to prevent some forms of amyotrophic lateral sclerosis and frontotemporal dementia has finally come of age. The hexanucleotide repeat expansion in the C9orf72 gene accounts for ∼10% of all amyotrophic lat…
View article: Analytical and clinical validation of step counting method in people living with amyotrophic lateral sclerosis
Analytical and clinical validation of step counting method in people living with amyotrophic lateral sclerosis Open
Background Accelerometer-based digital measures offer a scalable and low-burden means of quantifying physical function, but existing processing algorithms may not quantify pathological gait correctly. In people living with amyotrophic late…
View article: Plasma tau and phosphorylated tau at T181 are altered in amyotrophic lateral sclerosis
Plasma tau and phosphorylated tau at T181 are altered in amyotrophic lateral sclerosis Open
There is an unmet need for reliable biomarkers for amyotrophic lateral sclerosis (ALS). Recent studies have demonstrated that the levels of the microtubule-associated protein tau, are altered in plasma and cerebrospinal fluid (CSF) from pe…
View article: Reliable monitoring of respiratory function with home spirometry in people living with amyotrophic lateral sclerosis
Reliable monitoring of respiratory function with home spirometry in people living with amyotrophic lateral sclerosis Open
Introduction Monitoring respiratory function is essential for assessing the progression of Amyotrophic Lateral Sclerosis (ALS) and planning interventions. Remote pulmonary function testing offers a promising alternative to in-clinic visits…
View article: Operational Development and Launch of an Adaptive Platform Trial in Amyotrophic Lateral Sclerosis: Processes and Learnings From the First Four Regimens of the <scp>HEALEY ALS</scp> Platform Trial
Operational Development and Launch of an Adaptive Platform Trial in Amyotrophic Lateral Sclerosis: Processes and Learnings From the First Four Regimens of the <span>HEALEY ALS</span> Platform Trial Open
Introduction/Aims Platform trials present several advantages over traditional interventional clinical trials. Here, we provide a detailed description of the operational framework of the HEALEY ALS Platform Trial. Methods Platform‐level pro…
View article: A Preliminary Investigation of Acoustic Features for Remote Monitoring of Respiration in <scp>ALS</scp>
A Preliminary Investigation of Acoustic Features for Remote Monitoring of Respiration in <span>ALS</span> Open
Introduction/Aims There is a substantial need to establish reliable approaches for low‐burden at‐home monitoring of respiratory function for people with amyotrophic lateral sclerosis (PALS). This preliminary study assessed the potential of…
View article: Clinical assessment and interpretation of dysarthria in ALS using attention based deep learning AI models
Clinical assessment and interpretation of dysarthria in ALS using attention based deep learning AI models Open
Speech dysarthria is a key symptom of neurological conditions like ALS, yet existing AI models designed to analyze it from audio signal rely on handcrafted features with limited inference performance. Deep learning approaches improve accur…
View article: Skeletal muscle biomarkers of amyotrophic lateral sclerosis: a large-scale, multi-cohort proteomic study
Skeletal muscle biomarkers of amyotrophic lateral sclerosis: a large-scale, multi-cohort proteomic study Open
Background Biomarkers with clear contexts-of-use are important tools for ALS therapy development. Understanding their longitudinal trajectory in the untreated state is key to their use as potential markers of pharmacodynamic response. To t…
View article: An Expanded Access Protocol of <scp>RNS60</scp> in Amyotrophic Lateral Sclerosis
An Expanded Access Protocol of <span>RNS60</span> in Amyotrophic Lateral Sclerosis Open
Aims RNS60 is an investigational product in clinical development for amyotrophic lateral sclerosis (ALS). RNS60 slowed disease progression in the ALS SOD1 G93A mouse model and was safe and well tolerated both in an open‐label pilot study a…
View article: Efficacy and Safety of Zilucoplan in Amyotrophic Lateral Sclerosis
Efficacy and Safety of Zilucoplan in Amyotrophic Lateral Sclerosis Open
Importance The etiology of amyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disease, is unknown. However, neuroinflammation and complement activation may play a role in disease progression. Objective To determine the effects …
View article: Reliable Monitoring of Respiratory Function with Home Spirometry in People Living with Amyotrophic Lateral Sclerosis
Reliable Monitoring of Respiratory Function with Home Spirometry in People Living with Amyotrophic Lateral Sclerosis Open
Monitoring respiratory function is essential for assessing the progression of Amyotrophic Lateral Sclerosis (ALS) and planning interventions. Using spirometry data from the Radcliff Study —a fully remote, longitudinal, exploratory study wi…
View article: Longer disease progression milestone-free time in people with amyotrophic lateral sclerosis treated versus not treated with intravenous edaravone: results from an administrative claims analysis
Longer disease progression milestone-free time in people with amyotrophic lateral sclerosis treated versus not treated with intravenous edaravone: results from an administrative claims analysis Open
Aim: To estimate time-to-progression milestones in people with amyotrophic lateral sclerosis (PALS) treated versus not treated with intravenous (IV) edaravone (Radicava ® IV, Mitsubishi Tanabe Pharma America [MTPA], hereafter “IV edaravone…
View article: Short prescribed exercises can quantify upper limb functioning in neurodegenerative disease
Short prescribed exercises can quantify upper limb functioning in neurodegenerative disease Open
Digital health technologies (DHTs) can quantify movements in daily routines but rely heavily on participant adherence over prolonged wear times. Here we analyze accelerometry data from wrist-worn devices during short episodes of prescribed…
View article: Listener effort measures clinically meaningful change of dysarthria in amyotrophic lateral sclerosis
Listener effort measures clinically meaningful change of dysarthria in amyotrophic lateral sclerosis Open
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative motor neuron disease that can cause progressive bulbar dysfunction and dysarthria, resulting in reduced quality of life. Quantitative motor speech analysis can identify features of…
View article: Multimodal Digital Phenotyping of Behavior in a Neurology Clinic: Development of the Neurobooth Platform and the First Two Years of Data Collection
Multimodal Digital Phenotyping of Behavior in a Neurology Clinic: Development of the Neurobooth Platform and the First Two Years of Data Collection Open
Quantitative analysis of human behavior is critical for objective characterization of neurological phenotypes, early detection of neurodegenerative diseases, and development of more sensitive measures of disease progression to support clin…
View article: Smartphone-based Step Counts as an IndependentDigital Biomarker of Survival in ALS Patients
Smartphone-based Step Counts as an IndependentDigital Biomarker of Survival in ALS Patients Open
Amyotrophic lateral sclerosis (ALS) is a progressive debilitating disease. Digital biomarkers obtained from smartphone data may allow for scalable, remote, unobtrusive, quantitative measurement of physical activity (PA) and could provide e…
View article: Access for ALL in ALS: A large‐scale, inclusive, collaborative consortium to unlock the molecular and genetic mechanisms of amyotrophic lateral sclerosis
Access for ALL in ALS: A large‐scale, inclusive, collaborative consortium to unlock the molecular and genetic mechanisms of amyotrophic lateral sclerosis Open
Recent progress in therapeutics for amyotrophic lateral sclerosis (ALS) has spurred development and imbued the field of ALS with hope for more breakthroughs, yet substantial scientific gaps persist. This unmet need remains a stark reminder…
View article: Effects of COVID-19 on motor neuron disease mortality in the United States: a population-based cross-sectional study
Effects of COVID-19 on motor neuron disease mortality in the United States: a population-based cross-sectional study Open
The total number of MND deaths was greater during COVID than in the preceding years. The analysis suggests there might have been a consequence of circumstances surrounding the global pandemic and the associated restrictions.