Scholars@Duke
Shruti Mukund Raja

Shruti Mukund Raja

Assistant Professor of Neurology
Neurology, Neuromuscular Disease
shruti.raja@duke.edu
DUMC 3403, Durham, NC 27710
DUMC 3403, Durham, NC 27710

Selected Publications

COVID-19 Vaccination and Infection in Myasthenia Gravis: A Longitudinal Patient Survey.

Journal Article Muscle Nerve · October 2025 INTRODUCTION/AIMS: Patients with myasthenia gravis (MG) often receive immunosuppressive treatments. While approved COVID-19 vaccines effectively prevent severe infections, the impact of vaccination among immunocompromised MG patients remains unclear. This ... Full text Link to item Cite

Sensitivity of Repetitive Nerve Stimulation Performed in Early and Later Stages of Myasthenia Gravis.

Journal Article Muscle Nerve · October 2025 INTRODUCTION/AIMS: Repetitive nerve stimulation (RNS) is a valuable tool in the diagnostic evaluation of myasthenia gravis (MG). However, there are conflicting reports of its sensitivity when performed ≤ 30 days from symptom onset. We aim to compare the se ... Full text Link to item Cite

A Phase 1 Assessment of the Safety, Tolerability, Pharmacokinetics and Pharmacodynamics of (2R,6R)-Hydroxynorketamine in Healthy Volunteers.

Journal Article Clin Pharmacol Ther · November 2024 (R,S)-Ketamine (ketamine) is a dissociative anesthetic that also possesses analgesic and antidepressant activity. Undesirable dissociative side effects and misuse potential limit expanded use of ketamine in several mental health disorders despite promising ... Full text Link to item Cite

Immune dysregulation in chronic inflammatory demyelinating polyneuropathy.

Journal Article J Neuroimmunol · June 15, 2024 OBJECTIVE: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune disorder of the peripheral nerves with an incompletely understood underlying pathophysiology. This investigation focused on defining B and T cell frequencies, T cell funct ... Full text Link to item Cite

Intravenous Ganaxolone: Pharmacokinetics, Pharmacodynamics, Safety, and Tolerability in Healthy Adults.

Journal Article Clin Pharmacol Drug Dev · March 2024 Ganaxolone, a neuroactive steroid anticonvulsant that modulates both synaptic and extrasynaptic γ-aminobutyric acid type A (GABAA ) receptors, is in development for treatment of status epilepticus (SE) and rare epileptic disorders, and has been approved in ... Full text Link to item Cite

Experience of Daily Life with Generalized Myasthenia Gravis: A Qualitative Investigation and Assessment of Instrument Content Validity

Journal Article Neurology and Therapy · December 1, 2023 Introduction: Generalized myasthenia gravis (gMG) is a rare autoimmune disease. Symptoms of gMG are diverse, and understanding of their impact on patients is limited. This qualitative study aimed to provide an in-depth exploration of patients’ daily experi ... Full text Cite

Pregnancy in MuSK-positive myasthenia gravis: A single-center case series.

Conference Muscle Nerve · July 2023 INTRODUCTION/AIMS: Myasthenia gravis (MG) with muscle-specific tyrosine kinase (MuSK) antibodies (MMG) is predominantly seen in women of childbearing age. Our objective in this study was to describe the course of MMG during pregnancy and within 6 months po ... Full text Link to item Cite

The Duke Myasthenia Gravis Clinic Registry: II. Analysis of outcomes.

Conference Muscle Nerve · April 2023 INTRODUCTION/AIMS: The Duke Myasthenia Gravis (MG) Clinic Registry contains comprehensive physician-derived data on patients with MG seen in the Duke MG Clinic since 1980. The aim of this study was to report outcomes in patients seen in the clinic and trea ... Full text Link to item Cite

Knowledge and perceptions of the COVID-19 pandemic among patients with myasthenia gravis: follow up survey

Conference RRNMF Neuromuscular Journal · February 7, 2023 Introduction  We previously conducted a survey study in April 2020 at the beginning of the SARS-CoV-2 (COVID-19) pandemic to understand how it affected patients with myasthenia gravis (MG). Since then, significant advances have occurred in th ... Full text Open Access Cite

Pharmacokinetics of Ceftazidime-Avibactam in Combination with Aztreonam (COMBINE) in a Phase 1, Open-Label Study of Healthy Adults.

Journal Article Antimicrob Agents Chemother · December 20, 2022 Scant pharmacokinetic (PK) data are available on ceftazidime-avibactam (CZA) and aztreonam (ATM) in combination, and it is unknown if CZA-ATM exacerbates alanine aminotransferase (ALT)/aspartate aminotransferase (AST) elevations relative to ATM alone. This ... Full text Link to item Cite

Safety of Ceftazidime-Avibactam in Combination with Aztreonam (COMBINE) in a Phase I, Open-Label Study in Healthy Adult Volunteers.

Journal Article Antimicrob Agents Chemother · December 20, 2022 This phase I study evaluated the safety of the optimal ceftazidime-avibactam (CZA) with aztreonam (ATM) regimens identified in hollow fiber infection models of MBL-producing Enterobacterales. Eligible healthy subjects aged 18 to 45 years were assigned to o ... Full text Link to item Cite

Lambert-Eaton Myasthenic Syndrome and Botulism.

Journal Article Continuum (Minneap Minn) · December 1, 2022 PURPOSE OF REVIEW: This article reviews the pathophysiology, epidemiology, clinical features, diagnosis, and treatment of Lambert-Eaton myasthenic syndrome (LEMS) and botulism, presynaptic disorders of neuromuscular transmission in which rapid diagnosis im ... Full text Link to item Cite

Classical Complement Pathway Inhibition in a "Human-On-A-Chip" Model of Autoimmune Demyelinating Neuropathies.

Journal Article Adv Ther (Weinh) · June 2022 Chronic autoimmune demyelinating neuropathies are a group of rare neuromuscular disorders with complex, poorly characterized etiology. Here we describe a phenotypic, human-on-a-chip (HoaC) electrical conduction model of two rare autoimmune demyelinating ne ... Full text Link to item Cite

First-in-Human Clinical Trial to Assess the Safety, Tolerability and Pharmacokinetics of Single Doses of NTM-1633, a Novel Mixture of Monoclonal Antibodies against Botulinum Toxin E.

Journal Article Antimicrob Agents Chemother · April 19, 2022 Botulism is a rare, life-threatening paralytic disease caused by botulinum neurotoxin (BoNT). Available treatments including an equine antitoxin and human immune globulin are given postexposure and challenging to produce and administer. NTM-1633 is an equi ... Full text Link to item Cite

Adverse Reactions in a Phase 1 Trial of the Anti-Malarial DM1157: An Example of Pharmacokinetic Modeling and Simulation Guiding Clinical Trial Decisions.

Journal Article Infect Dis Ther · April 2022 INTRODUCTION: There is an urgent need to develop new drugs to treat malaria due to increasing resistance to first-line therapeutics targeting the causative organism, Plasmodium falciparum (P. falciparum). One drug candidate is DM1157, a small molecule that ... Full text Link to item Cite

Perioperative Outcomes of Thymectomy in Myasthenia Gravis: A Thoracic Surgery Database Analysis.

Journal Article Ann Thorac Surg · March 2022 BACKGROUND: There is clinical equipoise regarding the perioperative and long-term outcomes of autoimmune myasthenia gravis (MG) patients undergoing open vs minimally invasive thymectomy, particularly for nonthymomatous MG. This analysis utilizes multicente ... Full text Link to item Cite

Evaluation of a Digital Handheld Hydrogen Breath Monitor to Diagnose Lactose Malabsorption: Interventional Crossover Study.

Journal Article JMIR Form Res · October 18, 2021 BACKGROUND: Lactose malabsorption is a common condition that affects a broad segment of the population. Clinical diagnosis based on symptom recall can be unreliable and conventional testing can be inconvenient, requiring expensive laboratory-based equipmen ... Full text Link to item Cite

Quantitative muscle ultrasound and electrical impedance myography in late onset Pompe disease: A pilot study of reliability, longitudinal change and correlation with function.

Journal Article Mol Genet Metab Rep · September 2021 BACKGROUND/OBJECTIVES: Late-onset Pompe disease (LOPD) is slowly progressive, making it difficult to assess clinical change and response to interventions. In this study, quantitative muscle ultrasonography (QMUS) and electrical impedance myography (EIM) we ... Full text Link to item Cite

Safety, Tolerability, and Pharmacokinetics of NTM-1632, a Novel Mixture of Three Monoclonal Antibodies against Botulinum Toxin B.

Journal Article Antimicrob Agents Chemother · June 17, 2021 Botulism is a rare, life-threatening paralytic disease caused by Clostridium botulinum neurotoxin (BoNT). Available treatments, including an equine antitoxin and human immune globulin, are given postexposure and challenging to produce and administer. NTM-1 ... Full text Link to item Cite

Reduced plasmablast frequency is associated with seronegative myasthenia gravis.

Journal Article Muscle Nerve · April 2021 BACKGROUND: The immunopathology of autoimmune seronegative myasthenia gravis (SN MG) is poorly understood. Our objective was to determine immune profiles associated with a diagnosis of SN MG. METHODS: We performed high-dimensional flow cytometry on blood s ... Full text Link to item Cite

Knowledge and perceptions of the COVID-19 pandemic among patients with myasthenia gravis.

Journal Article Muscle Nerve · March 2021 BACKGROUND: Patients with myasthenia gravis (MG) may be particularly vulnerable during the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic due to risk of worsening disease during infection, potential adverse impacts of coronavirus dis ... Full text Link to item Cite

First-in-Human Studies of MW01-6-189WH, a Brain-Penetrant, Antineuroinflammatory Small-Molecule Drug Candidate: Phase 1 Safety, Tolerability, Pharmacokinetic, and Pharmacodynamic Studies in Healthy Adult Volunteers.

Journal Article Clin Pharmacol Drug Dev · February 2021 MW01-6-189WH (MW189) is a novel central nervous system-penetrant small-molecule drug candidate that selectively attenuates stressor-induced proinflammatory cytokine overproduction and is efficacious in intracerebral hemorrhage and traumatic brain injury an ... Full text Link to item Cite

The Duke myasthenia gravis clinic registry: I. Description and demographics.

Journal Article Muscle Nerve · February 2021 INTRODUCTION: The Duke Myasthenia Gravis (MG) Clinic Registry is a disease-specific database containing physician-derived data from patients seen in the Duke MG Clinic since 1980. METHODS: Data from 1060 MG patients initially seen between 1980 and 2008 wer ... Full text Link to item Cite

Imbalance in T follicular helper cells producing IL-17 promotes pro-inflammatory responses in MuSK antibody positive myasthenia gravis.

Journal Article J Neuroimmunol · August 15, 2020 A detailed understanding of the role of Tfh cells in MuSK-antibody positive myasthenia gravis (MuSK-MG) is lacking. We characterized phenotype and function of Tfh cells in MuSK-MG patients and controls. We found similar overall Tfh and follicular regulator ... Full text Link to item Cite

Inhibition of the transcription factor ROR-γ reduces pathogenic Th17 cells in acetylcholine receptor antibody positive myasthenia gravis.

Journal Article Exp Neurol · March 2020 IL-17 producing CD4 T cells (Th17) cells increase significantly with disease severity in myasthenia gravis (MG) patients. To suppress the generation of Th17 cells, we examined the effect of inhibiting retinoic acid receptor-related-orphan-receptor-C (RORγ) ... Full text Link to item Cite

Clinical outcome measures following plasma exchange for MG exacerbation.

Journal Article Ann Clin Transl Neurol · October 2019 Our objective is to report longitudinal results of the MG-ADL, MG-Composite, MG-MMT, and MG-QoL15 in an open-label trial of therapeutic plasma exchange in myasthenia gravis. Ten MG patients experiencing exacerbation had assessments prior to, immediately fo ... Full text Link to item Cite

Validation of the triple timed up-and-go test in Lambert-Eaton myasthenia.

Journal Article Muscle Nerve · September 2019 INTRODUCTION: There are no validated, practical, and quantitative measures of disease severity in Lambert-Eaton myasthenia (LEM). METHODS: Data from the Effectiveness of 3,4-Diaminopyridine in Lambert-Eaton Myasthenic Syndrome (DAPPER) trial were analyzed ... Full text Link to item Cite

Validation of a simple disease-specific, quality-of-life measure for diabetic polyneuropathy: CAPPRI.

Journal Article Neurology · June 5, 2018 OBJECTIVE: We studied the performance of a 15-item, health-related quality-of-life polyneuropathy scale in the clinic setting in patients with diabetic distal sensorimotor polyneuropathy (DSPN). METHODS: Patients with DSPN from 11 academic sites completed ... Full text Link to item Cite

Comparative effectiveness clinical trials to advance treatment of myasthenia gravis.

Journal Article Ann N Y Acad Sci · February 2018 Myasthenia gravis (MG) presents many challenges for establishing treatment efficacy through clinical trials. Among these are the rarity and heterogeneity of the disease, spontaneous fluctuations, prolonged latency to effect for many immunosuppressive drugs ... Full text Link to item Cite

Reliability of the triple-timed up-and-go test.

Journal Article Muscle Nerve · January 2018 INTRODUCTION: We report the reliability of a new measure, the triple-timed up-and-go (3TUG) test, for assessing clinical function in patients with Lambert-Eaton myasthenia (LEM). METHODS: Intrarater reproducibility and interrater agreement of the 3TUG test ... Full text Link to item Cite

Phase 1 Randomized, Double-Blind, Placebo-Controlled Study to Determine the Safety, Tolerability, and Pharmacokinetics of a Single Escalating Dose and Repeated Doses of CN-105 in Healthy Adult Subjects.

Journal Article J Clin Pharmacol · June 2017 Spontaneous intracranial hemorrhage (ICH) remains a devastating stroke subtype, affecting as many as 80,000 people annually in the United States and associated with extremely high mortality. In the absence of any pharmacological interventions demonstrated ... Full text Link to item Cite

B10 Cell Frequencies and Suppressive Capacity in Myasthenia Gravis Are Associated with Disease Severity.

Journal Article Front Neurol · 2017 Myasthenia gravis (MG) is a T cell-dependent, B cell-mediated disease. The mechanisms for loss of self-tolerance in this disease are not well understood, and recently described regulatory B cell (Breg) subsets have not been thoroughly investigated. B10 cel ... Full text Open Access Link to item Cite

Dissecting the roles of DR4, DR5 and c-FLIP in the regulation of geranylgeranyltransferase I inhibition-mediated augmentation of TRAIL-induced apoptosis.

Journal Article Mol Cancer · January 29, 2010 BACKGROUND: Geranylgeranyltransferase I (GGTase I) has emerged as a cancer therapeutic target. Accordingly, small molecules that inhibit GGTase I have been developed and exhibit encouraging anticancer activity in preclinical studies. However, their underly ... Full text Link to item Cite

The natural product honokiol preferentially inhibits cellular FLICE-inhibitory protein and augments death receptor-induced apoptosis.

Journal Article Mol Cancer Ther · July 2008 Targeting death receptor-mediated apoptosis has emerged as an effective strategy for cancer therapy. However, certain types of cancer cells are intrinsically resistant to death receptor-mediated apoptosis. In an effort to identify agents that can sensitize ... Full text Link to item Cite