- |||||||||| Rituxan (rituximab) / Roche
Atypical Neurological Manifestations of Kikuchi-Fujimoto Disease: A Case Study (Colorado Convention Center | Exhibit Hall B-E) - Mar 8, 2024 - Abstract #AAN2024AAN_3763;
Since KFD is a rare entity with non-specific CSF and imaging findings, a thorough assessment to rule out alternative causes and histopathology are crucial for a definitive diagnosis. While KFD is generally self-resolving, clinicians must recognize its complications as severe cases may require aggressive immunosuppressive treatment.
- |||||||||| A Comprehensive Review of Novel FDA-approved Neurological Indications from 2018-2023 (Colorado Convention Center | Exhibit Hall B-E) - Mar 8, 2024 - Abstract #AAN2024AAN_3681;
In future studies, off-label utilization of neurological medications that have not yet been FDA-approved may be examined. These neurologic medications are expected to provide promising results that will lead to new patterns of research in the years to come.
- |||||||||| Estimates of Productivity Loss Due to Neurological Diseases in the United States (Colorado Convention Center | Exhibit Hall B-E) - Mar 8, 2024 - Abstract #AAN2024AAN_3679;
A total burden of disease estimate is fundamentally incomplete without consideration of patients' perspectives and experiences, including how these experiences shift as diseases progress. Our findings can support improved decision-making by incorporating productivity loss in how we value treatments and also highlight the need for disease-specific and patient-centered data collection.
- |||||||||| Clinical and Electrophysiological Evaluation of Patients with Thymic Pathology in Terms of Myasthenia Gravis (Colorado Convention Center | Exhibit Hall B-E) - Mar 8, 2024 - Abstract #AAN2024AAN_3329;
This study has shown that in patients consulted with thymic pathologies and neurological clinics, in the absence of clinical symptoms related to MG, serological and electrophysiological tests may increase the diagnostic burden. Furthermore, if there are clinical suspicions, relying solely on ASU evaluations is not sufficient; TLEMG test is necessary.
- |||||||||| Assessing the Impact of Immune Checkpoint Inhibitor Therapy on Clinical and MRI Activity in Patients with Multiple Sclerosis (Colorado Convention Center | Exhibit Hall B-E) - Mar 8, 2024 - Abstract #AAN2024AAN_3217;
ICIs included; pembrolizumab (15, [55%]), nivolumab (10, [37%]) and durvalumab (2, [7%])...Five patients experienced new disease activity; 1 (3.7%) had relapse with new MRI lesions (no DMT, fingolimod paused) and 4 (15%) developed new MRI lesions (no DMT, fingolimod paused [1], ocrelizumab [1], dimethyl fumarate [2])...In this PwMS cohort, clinical relapse after ICI was uncommon. However, asymptomatic new MRI activity was slightly more frequent suggesting a role for MRI monitoring in PwMS receiving ICI.
- |||||||||| Case Report of Autoimmune Encephalitis with Autoimmune Myocarditis (Colorado Convention Center | Exhibit Hall B-E) - Mar 8, 2024 - Abstract #AAN2024AAN_3153;
However, asymptomatic new MRI activity was slightly more frequent suggesting a role for MRI monitoring in PwMS receiving ICI. Our case indicates a potential coexistence of autoimmune encephalitis and autoimmune myocarditis.
- |||||||||| riluzole / Generic mfg., azathioprine / Generic mfg.
Coexistence of Amyotrophic Lateral and Myasthenia Gravis: A Question of Shared Pathophysiologies? (Colorado Convention Center | Exhibit Hall B-E) - Mar 8, 2024 - Abstract #AAN2024AAN_3136;
Although rare, the coexistence has been attributed to low-density lipoprotein receptor-related protein-4 and heat shock protein-70 antibodies disrupting normal neuronal protein conformation. Regulatory T cell deficiencies and neuronal nitric oxide synthase abnormalities found in both raise possibility of pathophysiological commonalities.
- |||||||||| The Impact of a Myasthenia Gravis Specialty Clinic in a Safety-net Hospital Setting (Colorado Convention Center | Exhibit Hall B-E) - Mar 8, 2024 - Abstract #AAN2024AAN_3059;
Our patient had MG, responsive to immunotherapy, which progressed to a phenotype more consistent with ALS. Establishment of an MG specialty clinic in a safety-net hospital can lead to improved show rates, reduced rates of hospitalizations, improved MG-ADL scores, and overall improvement in quality of life in patients with MG.
- |||||||||| Rituxan (rituximab) / Roche
Phenotypic Variability of Seronegative Myasthenia Gravis in Children (Colorado Convention Center | Exhibit Hall B-E) - Mar 8, 2024 - Abstract #AAN2024AAN_2879;
Children with seronegative myasthenia manifest with diverse phenotypes, and sometimes lack characteristic findings on repetitive nerve stimulation, making diagnosis more challenging. Nonetheless, they can suffer from severe disease and may respond well to immunotherapy, highlighting the need to correctly diagnose children with MG early in their disease course.
- |||||||||| Vyvgart Hytrulo (efgartigimod alfa and hyaluronidase-qvfc) / argenx
Characterization of the Earliest Adopters of Subcutaneous FcRn Blockers for gMG (Colorado Convention Center | Exhibit Hall B-E) - Mar 8, 2024 - Abstract #AAN2024AAN_2868;
Objective:To characterize early adopters of subcutaneous (SC) neonatal receptor (FcRn) blockers efgartigimod alfa and hyaluronidase (SC efgartigimod) and rozanolixizumab, approved for generalized myasthenia gravis (gMG).Background:Monthly survey fielded by an independent market intelligence agency specializing in tracking neurological therapeutic markets, including benchmarking new product launch metrics.Design/Online survey fielded in October 2023, approximately two months after SC efgartigimod and rozanolixumab availability, among 65 US neurologists and neuromuscular specialists.42% of surveyed neurologists reported prescribing either SC efgartigimod, rozanolixizumab, or both products within the first two months of commercial availability ("early adopters"). Prior use of IV efgartigmod may have increased comfort with SC FcRn products, leading to faster uptake and more positive perceptions of both brands across degree of advance, market access, and launch execution.
- |||||||||| Libtayo (cemiplimab-rwlc) / Regeneron, Rituxan (rituximab) / Roche
A Successful Treatment Story of a Patient with Immune Checkpoint Inhibitor Associated Myositis and Myasthenia Gravis (Colorado Convention Center | Exhibit Hall B-E) - Mar 8, 2024 - Abstract #AAN2024AAN_2867;
Cemiplimab is an ICI used for the treatment of squamous cell skin cancer and has been associated with neuromuscular complications...She underwent plasma exchange followed by a second round of IVIG and was started on prednisone with minimal improvement...This case, with an overlap of ICI-associated myositis and myasthenia gravis, presents a diagnostic and treatment challenge, but ultimately resulted in a favorable treatment outcome. It underscores the importance of early application of the correct treatment and the role of rituximab in such cases.
- |||||||||| Keytruda (pembrolizumab) / Merck (MSD)
A Rare Case of Checkpoint Inhibitor-induced Myasthenia Gravis (Colorado Convention Center | Exhibit Hall B-E) - Mar 8, 2024 - Abstract #AAN2024AAN_2866;
There are few case reports on checkpoint inhibitor related Myasthenia Gravis. This case aims to provide knowledge on diagnostic and management strategies with patients that present with similar clinical findings to improve future patient care.
- |||||||||| nipocalimab (M281) / J&J
Nipocalimab a High Affinity, Immunoselective Clinical FcRn Blocker with Unique Properties: Observations from Non-clinical and Clinical Studies (Colorado Convention Center | Exhibit Hall B-E) - Mar 8, 2024 - Abstract #AAN2024AAN_2864;
This case aims to provide knowledge on diagnostic and management strategies with patients that present with similar clinical findings to improve future patient care. These data demonstrate that nipocalimab is a high affinity, immuno-selective FcRn blocker that demonstrates a consistent concentration dependent receptor occupancy that results in rapid, deep, and sustained lowering of IgG, including IgG autoantibodies to a favorable clinical outcome in gMG.
- |||||||||| Successful Treatment of Concomitant Myasthenia Gravis and Lambert-Eaton Myasthenic Syndrome with Autologous CD19-targeted CAR-T Cells (Colorado Convention Center | Exhibit Hall B-E) - Mar 8, 2024 - Abstract #AAN2024AAN_2862;
These data demonstrate that nipocalimab is a high affinity, immuno-selective FcRn blocker that demonstrates a consistent concentration dependent receptor occupancy that results in rapid, deep, and sustained lowering of IgG, including IgG autoantibodies to a favorable clinical outcome in gMG. In conclusion, this report highlights the potential for an anti-CD19 CAR-T cell approach in achieving autoimmune resetting for the treatment of neuroimmunological diseases.
- |||||||||| Neuromuscular Immune-related Adverse Events of Checkpoint Inhibitors. A Case Series at a Third-level Hospital in Argentina (Colorado Convention Center | Exhibit Hall B-E) - Mar 8, 2024 - Abstract #AAN2024AAN_2861;
The ICI's received included pembrolizumab (n=6, 66%), nivolumab (n=2, 22%), and durvalumab (n=1, 12%)...Treatment included corticosteroids (n=9), intravenous immunoglobulin (n=8), plasma exchange (n=1), and Rituximab (n=1)...In this series, the most common PNS-irAEs were MG and MI, although rare, these events can be potentially fatal. Due to the widespread use of ICI's, physicians should remain aware to enable prompt recognition and management of PNS-irAEs to improve the prognosis.
- |||||||||| Rystiggo (rozanolixizumab-noli) / UCB
Interactions of Fc? Receptors with an IgG4 Format, Anti-FcRn Monoclonal Antibody, Rozanolixizumab (Colorado Convention Center | Exhibit Hall B-E) - Mar 8, 2024 - Abstract #AAN2024AAN_2858;
These data indicate that in the absence of exogenous IgG, IgG4 format therapeutic antibodies may down-regulate Fc?RI but, in the presence of physiological concentrations of IgG, the engagement of Fc?Rs by IgG4 format antibodies is expected to be rare. Thus the IgG4 format, frequently used for therapeutic mAbs, is likely to be relatively inert with respect to unwanted engagement of Fc receptors.
- |||||||||| etoposide IV / Generic mfg.
Diagnosis of Lambert-Eaton Myasthenic Syndrome (LEMS) May Be Missed Among Patients with Myasthenia Gravis and Autonomic Dysfunction: Results from a US Database Analysis (Colorado Convention Center | Exhibit Hall B-E) - Mar 8, 2024 - Abstract #AAN2024AAN_2854;
In patients with MG, subsequent diagnoses of LEMS, lung cancer, and the receipt of SCLC therapies (etoposide + platinum-based chemotherapy) were ascertained according to the presence of autonomic disorder claims.Among 130,762 eligible patients with MG, 400 (0.3%) also had LEMS diagnoses...LEMS should be suspected in patients diagnosed as MG with autonomic dysfunction, especially in those with SCLC. Based on our analyses, at least 2% of this MG population may actually have the diagnosis of LEMS and be appropriate for alternative treatments.
- |||||||||| Incidence and Prevalence of Myasthenia Gravis: Analysis of a US Commercial Insurance Claims Database (Colorado Convention Center | Exhibit Hall B-E) - Mar 8, 2024 - Abstract #AAN2024AAN_2852;
MG identification relied on billing diagnoses and was limited primarily to Medicaid and commercially insured patients, therefore older patients are likely underrepresented. However, the validity of the datasets used in this analysis give particular strength to the prevalence, incidence, and demographics observed in this study, which support the need for continued research in MG.
- |||||||||| Patient Preferences for Myasthenia Gravis Treatments: A Discrete-choice Experiment (Colorado Convention Center | Exhibit Hall B-E) - Mar 8, 2024 - Abstract #AAN2024AAN_2849;
Respondents preferred gMG treatments with lower ISR risk, less frequent administration, and those self-administered at home. However, respondents were willing to trade off among treatment attributes, suggesting an important role for patient preferences in treatment selection.
- |||||||||| Soliris (eculizumab) / AstraZeneca
Eculizumab Versus Rituxmab for Acetylcholine Receptor-positive Generalized Myasthenia Gravis: Single-center Experience (Colorado Convention Center | Exhibit Hall B-E) - Mar 8, 2024 - Abstract #AAN2024AAN_2848;
However, respondents were willing to trade off among treatment attributes, suggesting an important role for patient preferences in treatment selection. Out data provides real-world evidence supporting the use of eculizumab over rituximab for the treatment of refractory, anti-AChR-ab positive MG, as eculizumab led to rapid and sustained achievement of MM.
- |||||||||| Lack of Relationship Between Myasthenia Gravis and COVID-19 Severity (Colorado Convention Center | Exhibit Hall B-E) - Mar 8, 2024 - Abstract #AAN2024AAN_2846;
No association between MG severity and COVID-19 severity was found. However, we suspect the relationship between both diseases, if any, to be complex and multifactorial.
- |||||||||| Digital Conversations by Myasthenia Gravis Serostatus (Colorado Convention Center | Exhibit Hall B-E) - Mar 8, 2024 - Abstract #AAN2024AAN_2842;
Conversations reveal key differences by MG serotype that indicate the burden of misdiagnosis for seronegative MG patients and the difficulty of the diagnostic journey. This data supports some commonalities across MG serotypes and reveals key differences which can inform clinicians and decision-makers towards improving diagnosis and care.
- |||||||||| Rituxan (rituximab) / Roche
Trial Design and Impact on Study Outcome: Post-hoc Analysis of the BeatMG Study Based on RINOMAX Trial Criteria (Colorado Convention Center | Exhibit Hall B-E) - Mar 8, 2024 - Abstract #AAN2024AAN_2840;
P2, P3
Clinical trial design may impact the assessment of an intervention and should be considered when comparing studies. While this post-hoc analysis showed some trends of beneficial effect of rituximab in a subgroup of patients with AChR-Ab+ MG, it highlights the need to better understand the role of B cell depletion therapy in MG.
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