- |||||||||| Zilbrysq (zilucoplan) / UCB
Journal: Improvement of fatigue in generalised myasthenia gravis with zilucoplan. (Pubmed Central) - Feb 24, 2024
P3
Any ocular motor deficit, even if appearing to be centrally mediated or occurring without ptosis, may be caused by ocular MG. Treatment with zilucoplan demonstrated statistical and clinically meaningful improvements in fatigue scores and severity versus placebo during RAISE, which were sustained to Week 60 in RAISE-XT.
- |||||||||| Journal: How to assess blepharoptosis via telemedicine: method and its reliability. (Pubmed Central) - Feb 22, 2024
Video consultation is an efficient and reliable way to assess patients with ptosis, with 95% of reliability. Although a thorough ptosis assessment is advised, there was no difference between the accuracy of diagnosis on those who did not have the full suggested assessment.
- |||||||||| Journal: Plasma Exchange in Pediatric Neurology Patients: A Single-Center Experience. (Pubmed Central) - Feb 22, 2024
In conclusion, our study underscores the significance of TPE as a therapeutic modality, emphasizing the imperative for ongoing research to fully exploit its potential across diverse medical contexts for enhancing patient care. Our findings, consistent with prior research, reveal plasma exchange's (PLEX's) wide-ranging applications and complications in neurological disorders.
- |||||||||| tamoxifen / Generic mfg.
Preclinical, Journal, Gene therapy: AAV9-MEDIATED GENE THERAPY OF CHOLINE ACETYLTRANSFERASE DEFICIENT MICE. (Pubmed Central) - Feb 22, 2024
Injection of tamoxifen (Tx) at postnatal (P) day 11 in these mice induced downregulation of Chat, autonomic failure, weakness, and death...No significant histopathologic changes were observed in the brain, liver, and heart of AAV-injected mice after a follow-up of one year. Thus, AAV9-mediated gene therapy may provide an effective and safe treatment for patients severely affected with CHAT-CMS.
- |||||||||| Global prevalence of autoimmune diseases in patients with vitiligo: a systematic review (San Diego Convention Center (Upper Level, Sails Pavilion, Poster Center 2)) - Feb 20, 2024 - Abstract #AAD2024AAD_3060;
Unspecified hypothyroidism and hyperthyroidism were prevalent in 3.18% (342/10,763) and 0.99% (106/10,724) of vitiligo cases, respectively. Here, we provided weighted prevalences of multiple autoimmune diseases in a global vitiligo cohort.
- |||||||||| Vitiligo Vulgaris as a Significant Cutaneous Comorbidity in Myasthenia Gravis () - Feb 20, 2024 - Abstract #AAD2024AAD_3043;
Notably, an increased familial history and autoimmune comorbid rate for MGV compared to MG patients alone was found. Despite the higher likelihood of aggressive treatment, the non-significant difference in clinical endpoints indicate that vitiligo presents profound morbidity risks for MG and other autoimmune conditions.
- |||||||||| Lichen planus is associated with other autoimmune conditions: A retrospective population-level study (San Diego Convention Center (Upper Level, Sails Pavilion, Poster Center 1)) - Feb 20, 2024 - Abstract #AAD2024AAD_2199;
Overall, these findings suggest that clinicians should consider evaluating patients with LP for other autoimmune conditions. These results also lend further support to considering the use of systemic agents for management of LP in patients with other autoimmune conditions that may benefit from similarly targeted therapies, such as biologics, Janus kinase inhibitors, and other immunomodulators/immunosuppressants.
- |||||||||| My Asthenia to Breath Is Very Gravis (San Diego Convention Center, Room 31A-C (Upper Level)) - Feb 20, 2024 - Abstract #ATS2024ATS_8091;
It is fundamental to consider myasthenia gravis in the differential diagnosis of vocal cord palsy, as well as for recurrent respiratory distresses following stressors in a middle-aged woman. Prompt diagnosis and effective treatment should avoid the need for mechanical ventilation.
- |||||||||| famotidine / Generic mfg., diphenhydramine / Generic mfg.
Unraveling a Complex Case of Miller Fischer Syndrome (San Diego Convention Center, Room 1A-B (Upper Level)) - Feb 20, 2024 - Abstract #ATS2024ATS_7704;
Notably, 20-30% of patients require mechanical ventilation, with a large proportion requiring eventual tracheostomy. Early initiation of treatment is essential to prevent complications, notably respiratory muscle fatigue which carries a high mortality risk especially in those over 50-years-old.
- |||||||||| Pulmonary Metastasis From Type Ab Thymoma (San Diego Convention Center, Area J (Hall H, Ground Level)) - Feb 20, 2024 - Abstract #ATS2024ATS_6866;
It can be a challenging diagnosis to make, but with careful review of immunohistochemistry and clinical context, it is certainly possible. Tissue NGS continues to shed additional light in thymoma tumor biology.
- |||||||||| A Case of a Rare Middle Mediastinal Mass (San Diego Convention Center, Area J (Hall H, Ground Level)) - Feb 20, 2024 - Abstract #ATS2024ATS_6850;
However, most cases of paratracheal thymomas undergo simple tumor resection. Teaching Point: This case underscores a stepwise approach for mediastinum cysts with the identification of high-risk features (e. g. nearby organ compression, and mass component inside the cyst) to favor surgical excision.
- |||||||||| A Rare Case of Extra Pericardial Mass Causing Cardiac Tamponade Physiology (San Diego Convention Center, Area I (Hall H, Ground Level)) - Feb 20, 2024 - Abstract #ATS2024ATS_6400;
Anterior mediastinal masses such as thymic remnant cysts can occur after thymectomy and can lead to compression of the right side of the heart. Patients with cocaine abuse might not elicit profound hemodynamic compromise because of increased afterload but should still be treated promptly with the aspiration of cysts to prevent impending cardiovascular collapse.
- |||||||||| Cubicin (daptomycin) / Merck (MSD), Opdivo (nivolumab) / BMS, Yervoy (ipilimumab) / BMS
Immunotherapy Induced Encephalitis (San Diego Convention Center, Area K (Hall H, Ground Level)) - Feb 20, 2024 - Abstract #ATS2024ATS_6034;
Admitting physicians along with sub-specialists including infectious diseases, oncologists, and neurologists need to be aware of the clinical presentation of serious but uncommon neurologic adverse effects associated with checkpoint inhibitors. Prompt diagnosis and management are critical to minimize serious complications and patient outcomes.
- |||||||||| Keytruda (pembrolizumab) / Merck (MSD)
From Immune Checkpoint Inhibition to Intubation (San Diego Convention Center, Area K (Hall H, Ground Level)) - Feb 20, 2024 - Abstract #ATS2024ATS_6025;
Most patients develop symptoms of irMG within 8 weeks of treatment and the mortality rate is between 20-30% (2,3). Given the severity of irMG, clinicians prescribing Pembrolizumab should closely monitor for its symptoms and intervene as soon as those symptoms arise.
- |||||||||| prednisone / Generic mfg.
Take It Out Early - A Case of Myasthenia Gravis in a Young Patient With Invasive Malignant Thymoma (San Diego Convention Center, Area E (Hall A-B2, Ground Level)) - Feb 20, 2024 - Abstract #ATS2024ATS_5963;
Acetylcholine antibody, AchR-Ab, was positive and he was started on Pyridostigmine and Prednisone...Our patient had a rapidly growing locally invasive thymoma tumor associated with MG one year after initial presentation. This presentation offers a challenge to both diagnosis and management and we hypothesize that early resection with a multi-specialist approach is necessary for improved outcomes.
- |||||||||| Breathing for Two: A Myasthenic Twist in COVID-19 Pregnancy (San Diego Convention Center, Area E (Hall A-B2, Ground Level)) - Feb 20, 2024 - Abstract #ATS2024ATS_5961;
It emphasizes diagnostic challenges, the need for tailored treatment strategies, and the significance of comprehensive patient care. As we navigate the evolving landscape of COVID-19 and its diverse clinical presentations, adaptability and vigilance are essential when managing patients with complex cases like the one presented here.
- |||||||||| azathioprine / Generic mfg., apixaban / Generic mfg.
From Bulbar Weakness to PEA Arrest: A Unique Case of Lambert-Eaton Myasthenic Syndrome (San Diego Convention Center, Area E (Hall A-B2, Ground Level)) - Feb 20, 2024 - Abstract #ATS2024ATS_5957;
Diagnosis can be confirmed with serology or EMG studies; treatment options include 3,4 diaminopyridine, immunotherapy (prednisolone / azathioprine), IVIG and/or plasma exchange...Case: A 60-year-old lifetime non-smoker male with atrial fibrillation on Eliquis and pulmonary hypertension underwent maze procedure, AICD pacemaker placement, and mitral valve repair in July, after which he developed worsening muscle weakness, dysphagia and dysphonia over three months for which he presented to the hospital...However, this patient's poor functional status would preclude chemotherapy. Physicians should consider early EMG testing in patients with proximal muscle weakness, progressive dysphagia, and hyporeflexia as early identification of LEMS may allow the patient to start treatment while functional status would still tolerate chemotherapy.
- |||||||||| ceftriaxone / Generic mfg.
Acute Inflammatory Demyelinating Polyneuropathy Leading to Quadriplegia Mediated by H. Influenzae (San Diego Convention Center, Area E (Hall A-B2, Ground Level)) - Feb 20, 2024 - Abstract #ATS2024ATS_5953;
Sputum culture was positive for H influenzae and treated with ceftriaxone...In some cases, the manifestation of neurological symptoms could be delayed up to 90 days preceding URI symptoms to establish the diagnosis of GBS. An early CSF analysis, and nerve conduction test should be considered in clinical cases with high clinical suspicion but should not delay treatment.
- |||||||||| Rituxan (rituximab) / Roche
Musk I Wait in Myasthenia Gravis? (San Diego Convention Center, Area H (Hall H, Ground Level)) - Feb 20, 2024 - Abstract #ATS2024ATS_5664;
She was discharged home on VAPS therapy, steroids, and rituximab...MG associated with anti-MUSK antibodies may present with a prominent respiratory component, and less responsive to anticholinesterase medication. We suspect the diagnosis of OSA is false and her sleep disordered breathing was related to hypoventilation in the setting of this NMD rather than true obstruction, particularly given elevated bicarbonate noted years prior.
- |||||||||| cisplatin / Generic mfg., cyclophosphamide / Generic mfg.
Management of Stage IVa Thymoma With Lambert-Eaton Myasthenic Syndrome in a Post-Partum Female (San Diego Convention Center, Area J (Hall H, Ground Level)) - Feb 20, 2024 - Abstract #ATS2024ATS_4569;
The case is notable for necessary management of symptoms with use of acetylcholinesterase inhibitors, tumor shrinkage with neoadjuvant chemotherapy, and successful radical debulking surgery. Young, healthy patients may be considered for aggressive multimodal treatment for stage IVa thymoma.
- |||||||||| Superior Vena Cava Syndrome Due to Mediastinal Teratoma (San Diego Convention Center, Area J (Hall H, Ground Level)) - Feb 20, 2024 - Abstract #ATS2024ATS_4566;
Young, healthy patients may be considered for aggressive multimodal treatment for stage IVa thymoma. In patients with suspected SVCS, initial evaluation for cardiovascular and respiratory compromise, and prompt thoracic CT or MRI venography is critical to diagnose potentially life-threatening complications.
- |||||||||| prednisone / Generic mfg.
Pulmonary Sarcomatoid Carcinoma Presenting as a Myasthenia Gravis-like Syndrome (San Diego Convention Center, Area J (Hall H, Ground Level)) - Feb 20, 2024 - Abstract #ATS2024ATS_4564;
Past medical history includes treated prostate cancer and rheumatoid arthritis on chronic prednisone...Our case presents a rare occurrence of AChR-ab-positive myasthenia gravis as a presenting syndrome of underlying sarcomatoid carcinoma (SC). It is imperative that we continue to document and share unusual presentations so that they can potentially aid in detection of this rare, poor-survival disease.
- |||||||||| A Not So "Good" Syndrome: Thymoma and Hypogammaglobulinemia With Mystery Malignancy (San Diego Convention Center, Area J (Hall H, Ground Level)) - Feb 20, 2024 - Abstract #ATS2024ATS_4514;
While the exact hepatic malignancy could not be identified, prior reports of GS indicate associations with IBD and liver nodular hyperplasia, suggesting a possible increased risk of GI malignancy related to GS. Early assessment for hypogammaglobulinemia in patients with thymoma may thus provide significant benefit from autoimmune, GI, and infectious complications.
- |||||||||| pegylated liposomal doxorubicin / Generic mfg., trimethoprim/sulfamethoxazole / Generic mfg.
Pulmonary Kaposi Sarcoma Without Endobronchial Lesions in a 48-year-old Immunocompromised Male With Acute Respiratory Distress Syndrome (ARDS) (San Diego Convention Center, Area H (Hall H, Ground Level)) - Feb 20, 2024 - Abstract #ATS2024ATS_4069;
For those with symptomatic pulmonary disease, pegylated liposomal doxorubicin has a 45% response rate even without HAART. This case serves as an emphasis for KS to remain on the differential for patients with HIV who present with pulmonary concerns with or without typical cutaneous lesions of KS, particularly in those patients in who empiric steroids are started.
- |||||||||| prednisone / Generic mfg.
Crisis Averted: A Myasthenia Journey (San Diego Convention Center, Area K (Hall H, Ground Level)) - Feb 20, 2024 - Abstract #ATS2024ATS_3713;
Neurology recommended a 60 mg prednisone daily regimen, obviating the need for plasmapheresis...Early recognition and adherence to Neurology recommendations, alongside careful monitoring of respiratory muscle strength, were key factors in the patient's successful recovery and extubation. This case underscores the pivotal role of multidisciplinary care in the management of myasthenic crises, ensuring better outcomes for affected individuals.
- |||||||||| methimazole / Generic mfg., hydrocortisone / Generic mfg., methylprednisolone sodium succinate / Generic mfg.
Double Trouble: When Myasthenia Gravis Crisis and Thyroid Storm Collide (San Diego Convention Center, Area L (Hall H, Ground Level)) - Feb 20, 2024 - Abstract #ATS2024ATS_3360;
When physicians are faced with MGC, TS needs to be in the back of their minds with emphasis that laboratory markers of thyroid function are not always reflective of the clinical picture. Early diagnosis of thyroid storm is important to start aggressive treatment to reduce mortality.
- |||||||||| Keytruda (pembrolizumab) / Merck (MSD)
Diaphragmatic Involvement of Pembrolizumab - Induced Myopathy Leading to Type II Respiratory Failure (San Diego Convention Center, Area A (Hall A-B2, Ground Level)) - Feb 20, 2024 - Abstract #ATS2024ATS_1588;
Given the clinical improvement with steroids in our patient, a biopsy was not performed. Although pembrolizumab-induced myopathy is commonly linked with myasthenia gravis and myopathy as well, our patient's workup was negative for these conditions.
- |||||||||| Novel Use of Mini Convex EBUS Bronchoscope for Diagnosis and Staging of Thymic Neoplasms (San Diego Convention Center, Area C (Hall A-B2, Ground Level)) - Feb 20, 2024 - Abstract #ATS2024ATS_1469;
Levetiracetam was changed to lacosamide and clobazam for seizure prevention...Per oncology, the patient began chemotherapy with a 3-day course of cisplatin/doxorubicin/cyclophosphamide on day 16 with methylprednisolone, followed by IVIg...However, this comes with increased risk of complications given it is an open procedure. This case shows the possibility of using mini convex EBUS bronchoscopy as a less invasive option that could potentially reduce complications and improve patient outcomes.
- |||||||||| prednisone / Generic mfg.
Journal, Gold standard, Surgery: Are the minimally invasive techniques the new gold standard in thymus surgery for myasthenia gravis? Experience of a reference single-site in VATS thymectomy. (Pubmed Central) - Feb 16, 2024
Several publications raised doubts about the lack of evidence justifying thymectomy's role in MG until 2016 when a randomized study comparing thymectomy via sternotomy plus prednisone versus prednisone alone was published in the New England Journal of Medicine (NEJM)...The primary objective of this study is to validate the VATS technique as the preferred approach for thymectomy. Furthermore, we aim to analyze the impact of VATS thymectomy on symptoms and corticosteroid dosage in patients with MG, identifying factors that may predict a better response to surgery.
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