Autoimmune Neuromuscular Disorder Research Articles

Clinical remission of moderate generalized myasthenia gravis through exclusive use of Buzhong Yiqi decoction: A case report

BackgroundMyasthenia gravis (MG) is a rare autoimmune neuromuscular disorder with significant morbidity and mortality. Traditional Chinese medicine (TCM) offers an alternative approach to standard pharmacological and surgical interventions, which are often associated with adverse side effects. This case report details the clinical remission of a 50-year-old male with moderate generalized MG following exclusive treatment with a modified Buzhong Yiqi decoction (BYD), a TCM formula, without the use of immunosuppressive agents. Case summaryThe patient presented with diplopia, bilateral ptosis, weakness in chewing, limb weakness, and other symptoms indicative of spleen and stomach qi deficiency. Modified BYD was prescribed, focusing on strengthening the spleen, nourishing qi and blood, and enhancing immune response. The treatment included ingredients such as Radix Astragali, Angelica sinensis, Atractylodes macrocephala, and others, aiming to restore balance and improve the patient's condition. After two weeks of TCM treatment, the patient showed significant improvement in symptoms of myasthenia. By the second month, all clinical symptoms had disappeared. The patient continued to receive the TCM regimen until the thirtieth month of treatment. At the time of writing this report, the patient has no clinical symptoms and has experienced no relapse. Notably, no obvious adverse effects were reported throughout the treatment. ConclusionThe success of this case suggests that TCM may serve as an independent treatment option for moderate MG, offering a steroid-free alternative, which would be particularly valuable for patients who are intolerant of or refuse steroid therapy, potentially with significant clinical implications. However it needs a randomized clinical trial comparing TCM to conventional Western medicine treatment to validate it.

Myasthenic Crisis in a Child: Successful Recovery after Early Identification and Intervention

Background: Juvenile myasthenia gravis (MG) is a rare autoimmune neuromuscular disorder and has a varied presentation in childhood making it a diagnostic challenge. Clinical Description: An 8-year-old girl presented to the pediatric emergency with multiple episodes of falling, drooling of saliva, dysphagia, and bilateral ptosis. Within a few hours of admission, she had acute deterioration, developed respiratory failure, and was put on ventilatory support. Management and Outcome: A myasthenic crisis was suspected and a neostigmine challenge test was done. She was managed in the emergency with atropine, neostigmine, intravenous immunoglobulin, and supportive care. Subsequently, she achieved complete remission with steroids, physostigmine, and oral mycophenolate mofetil over the next 6 weeks. Conclusion: Juvenile MG may rarely be the cause of generalized muscular weakness. A strong suspicion of myasthenic crisis must be kept in case of sudden deterioration involving bulbar and respiratory muscles, in a patient with neurologic weakness. Early intervention in this rare condition can be lifesaving.

Identification of key genes as potential diagnostic and therapeutic targets for comorbidity of myasthenia gravis and COVID-19.

Myasthenia gravis (MG) is a chronic autoimmune neuromuscular disorder. Coronavirus disease 2019 (COVID-19) has a significant impact on the health and quality of life of MG patients and may even trigger the onset of MG in some cases. With the worldwide development of the COVID-19 vaccination, several new-onset MG cases and exacerbations following the COVID-19 vaccines have been acknowledged. The potential link between myasthenia gravis (MG) and COVID-19 has prompted the need for further investigation into the underlying molecular mechanism. The differential expression analysis identified six differentially expressed genes (DEGs) shared by myasthenia gravis (MG) and COVID-19, namely SAMD9, PLEK, GZMB, JUNB, NR4A1, and NR1D1. The relationship between the six common genes and immune cells was investigated in the COVID-19 dataset. The predictive value of the shared genes was assessed and a nomogram was constructed using machine learning algorithms. The regulatory miRNAs, transcription factors and small molecular drugs were predicted, and the molecular docking was carried out by AutoDock. We have identified six common DEGs of MG and COVID-19 and explored their immunological effects and regulatory mechanisms. The result may provide new insights for further mechanism research.

Masticatory Myositis in a Guinea Pig (Cavia porcellus): A Case Report

Masticatory myositis is an autoimmune neuromuscular disorder that affects the muscles of mastication. The affected individual has difficulties in opening or closing the mouth, pain, and swelling in the acute phase, and significant atrophy of the affected musculature in the chronic phase. A guinea pig ( Cavia porcellus) with a history of chronic hyporexia, recurrent cheek teeth overgrowth, and loss of facial silhouette, was suspected of having masticatory myositis. The disease was confirmed by computed tomography and histopathological examination of muscle fragments. The patient was treated with a protocol involving corticosteroids and gabapentin, and occlusal correction procedures.

Usefulness and Clinical Impact of Whole-Body MRI in Detecting Autoimmune Neuromuscular Disorders.

Autoimmune neuromuscular diseases are a group of heterogenous pathologies secondary to the activation of the immune system that damage the structures of the peripheric nerve, the neuromuscular junction, or the skeleton muscle. The diagnosis of autoimmune neuromuscular disorders comprises a combination of data from clinical, laboratory, electromyography, imaging exam, and biopsy. Particularly, the whole-body MRI examination in the last two decades has been of great use in the assessment of neuromuscular disorders. MRI provides information about the structures involved and the status of activity of the disease. It can also be used as a biomarker, detect the pattern of specific muscle involvement, and is a useful tool for targeting the optimal muscle site for biopsy. In this work, we summarized the most used technical protocol of whole-body MRI and the role of this imaging technique in autoimmune neuromuscular disorders.

Myasthenia gravis with myasthenic crisis: a case report

Objective: To present a favorable outcome in a Myasthenia gravis (MG) patient with myasthenic crisis and discuss its main diagnostic challenges. Case detail: We report the case of a previously healthy 80-year-old male who first presented with dysphagia that quickly progressed to a generalized myasthenic syndrome leading to ICU admission and intubation over the course of less than two months. He was investigated firstly for thymoma, followed by Parkinson’s disease and Lambert-Eaton syndrome before closing on MG. He was successfully stabilized after a month-long ICU stay intercurring with multiple infections and respiratory failure. Currently presenting no sequalae or recurrence. Final considerations: Myasthenia gravis is an important autoimmune neuromuscular disorder that affects the neuromuscular junction. It has many possible signs and symptoms, depending on the most affected muscle group. Although there is no cure for it, an early diagnosis allows an approach and treatment that provides a better quality of life, and the prevention of related complications. We discuss how to assess the main differential diagnoses, including Parkinson’s disease, Amyotrophic Lateral Sclerosis, Multiple Sclerosis, Lambert-Eaton Syndrome, and others, to help clinicians facing a similar patient and avoid a poor outcome on a fast-paced progression case such as the one we report.

Tekrarlayan başarısız weaning ve uzamış apne ile tanı konulan yaşlı myastenia gravis hastası

Myasthenia gravis (MG) is a chronic autoimmune neuromuscular disorder caused by the development of antibodies to nicotinic acetylcholine receptors. The disease characterized by variable muscle weakness worsening with exercise. Although most patients have neurological signs and symptoms, respiratory symptoms occur in the late stages of the disease. However, rarely, respiratory failure may be the first symptom in some undiagnosed patients. We report the case of an elderly patient who was admitted with respiratory failure and intubated and transferred to the internal medicine intensive care unit. Two days after intubation, when she was awake and met the extubation criteria, endotracheal tube was successfully removed. At this time, her vital signs were normal. However, next day, her arterial blood gas CO2 rose and he became drowsy, requiring re-intubation. This situation repeated on the fifth and 16th days of her hospitalization and prolonged apnea was observed after intubation with rocuronium. All other causes of failed extubation, such as sepsis and pneumonia, were ruled out. During follow-up, ptosis was detected in the right eye and acetylcholine receptor antibody was positive. On the 30th day of the follow-up, methylprednisolone 1mg/kg and pyridostigmine 240 mg/day was initiated. The patient, whose spontaneous respiration was sufficient, was extubated and taken to the neurology clinic for further examination and treatment.

Plasmapheresis Versus Intravenous Immunoglobulin in Patients With Autoimmune Neuromuscular and Neuro-immunological Conditions.

Plasmapheresis (PLEX) and intravenous immunoglobulin (IVIg) are commonly used to treat autoimmune neuromuscular disorders, including myasthenia gravis, acute inflammatory demyelinating polyradiculoneuropathy, chronic inflammatory demyelinating polyradiculoneuropathy, and other autoimmune neurological disorders. The side effect profiles of these therapies vary, and concern has been raised regarding the safety of PLEX in the elderly population. In this study, we have examined the pattern of PLEX and IVIg use for autoimmune neurological disorders at a single facility and in a national database, focusing on the complications in elderly patients. We performed a retrospective chart review of adult patients at our institution receiving PLEX or IVIg for any autoimmune neuromuscular or neuro-immunological disease. Next, we analyzed the National Inpatient Sample database to confirm the trend in IVIg and PLEX use from 2012 to 2018 for a set of neuromuscular and neuro-immunological primary diagnoses. IVIg was overall favored over PLEX. The adverse effects were similar among elderly patients (age ≥65 years) compared with younger patients (<65 years) in our institution, even after adequate matching of patients based on age, sex, and medical history. We examined the National Inpatient Sample dataset and noted increasingly higher frequency of IVIg use, consistent with the findings from our institution or facility. Both PLEX and IVIg are safe therapeutic choices in adult patients with autoimmune neuromuscular disorders and other neuro-immunological diseases and can be safely administered in the appropriate clinical setting.

Neuromuscular Disorders in Pregnancy

Women of childbearing age can develop autoimmune or hereditary neuromuscular disorders, which can exacerbate during pregnancy. Stabilizing disease activity during pregnancy has a positive impact on pregnancy and delivery outcomes. Selection of therapeutic agents during pregnancy should be based on the evaluation of the risks and benefits involved. Generally, spontaneous vaginal delivery is recommended; however, preterm and emergency cesarean deliveries may become necessary. Novel agents such as biologics and nucleic acid drugs have been introduced in clinical practice in recent years. These novel agents have provided significant benefit to patients with neuromuscular disorders, although verification of their safety profile in pregnant women is an important issue that should be addressed.

Management of Autoimmune urticaria in a patient with Myasthenia Gravis, a challenging case.

The pathophysiology of chronic spontaneous urticaria (CSU) is not yet fully understood; however, increasing evidence supports the association between CSU and autoimmunity. Myasthenia gravis (MG) is an autoimmune neuromuscular disorder. MG management relies on using immunosuppressants and avoiding certain medications that can precipitate an MG crisis. The coexistence of CSU and MG was described in the literature on elderly patients. Herein we present a challenging case regarding the management of CSU in a young female with MG. A 22-year-old lady known to have Myasthenia gravis post thymectomy, with a history of multiple MG crises, presented to the Allergy Clinic with recurrent itchy hives typical for urticaria without associated angioedema. Despite being on Azathioprine and low-dose steroids for MG treatment, she had an active CSU disease, UAS7:32, UCT: 5. The neurologist advised against the use of regular oral antihistamines because they might exacerbate MG. Although we do not have serum autologous skin testing, basophil activation test, or IgG autoantibodies for a definitive definition of autoimmune CSU (aiCSU), the patient has some features supporting the diagnosis of aiCSU (see Table 1). In addition, she has a normal total IgE level, normal C3, C4, negative RF, ANA, ANCA, anti-TPO, and anti-thyroglobulin antibodies. After the discussion with the neurologist, we started her on Omalizumab 300 mg every four weeks, which was increased to 450 mg every two weeks with partial control of CSU. She was started on rituximab to treat MG with improvement in CSU. To the best of our knowledge, limited data describing the association between MG and CSU in young patients. Moreover, there is insufficient data on the safety of antihistamines in patients with MG, which are the first line of treatment for CSU. There are clinical and laboratory biomarkers that help in identifying CSU endotypes. Recognition of aiCSU endotype is essential as it helps predict disease course and response to treatment. Moreover, careful therapeutic interventions in patients with CSU and coexistent autoimmune diseases are warranted to achieve efficacy and reduce drug interactions and adverse effects.

Use of Efgartigimod in Treatment of Myasthenia Gravis Relapse after Possible Recurrence of Invasive Thymoma: A Case Report. (P1-5.011)

<h3>Objective:</h3> NA <h3>Background:</h3> Myasthenia gravis is an autoimmune neuromuscular junction disorder with established association with thymoma. There is no consensus on optimal treatment of recurrent thymoma post-resection. Efgartigimod is a first-in-class FcRn antagonist approved by the FDA in December 2021 for treatment of AChR-antibody-positive adults with generalized MG. <h3>Design/Methods:</h3> NA <h3>Results:</h3> A 53-year-old right-handed man presented to us in September 2020 with a 10-month history of recurrent diplopia, ptosis, dysarthria, dysphagia, and bilateral hand weakness. Workup for MG revealed blocking, binding, and modulating AChR antibodies. CT scan of his chest demonstrated an invasive anterior mediastinal mass. He started pyridostigmine, mycophenolate and prednisone, and underwent a sternotomy with thymectomy with sparing of the directly adjacent right phrenic nerve in October 2020. Histopathology revealed a locally invasive Masaoka stage IIIA, pT1bN0M0, WHO type B2 thymoma. He received adjuvant radiotherapy until January 2021. He was weaned off prednisone and pyridostigmine by May 2021. He remained asymptomatic for about 18 months post-thymectomy except for transient dysarthria in September 2021 attributed to missed mycophenolate doses. Patient noticed recrudescence of bulbar and respiratory symptoms around April 2022. Tests showed elevated AChR antibodies but surveillance chest CT scans did not demonstrate tumor recurrence or metastases. His cardiothoracic surgeon remained suspicious of residual thymoma. He started efgartigimod in July 2022 which he tolerated well, resulting in symptom resolution and objective improvement on MG-specific outcome measures (MG-ADL, MG-QoL15r and MGC). His symptoms recurred during a COVID-19 infection a month later, but he rapidly became asymptomatic after completing the second efgartigimod cycle in September 2022. <h3>Conclusions:</h3> Our patient with AChR antibody-positive, thymoma-associated, generalized MG with recurrent symptoms despite mycophenolate treatment, thymectomy and radiotherapy showed a dramatic response to initial cycles of efgartigimod therapy without significant adverse effects. We propose that efgartigimod be considered early in the management of MG patients with recurrent symptoms after thymectomy. <b>Disclosure:</b> Dr. Akemokwe has stock in CVS HEALTH CORP. Dr. Akemokwe has stock in LABORATORY CORP AMERICAN HOLDINGS. Dr. Coskun has nothing to disclose. Dr. Meyerson has nothing to disclose. Dr. Ebong has nothing to disclose.

Effect of COVID-19 on Myasthenia Gravis (P2-8.006)

<h3>Objective:</h3> To describe the effects of COVID-19 on comorbid myasthenia gravis (MG) regarding common symptoms such as limb weakness, oculobulbar weakness, and respiratory failure by evaluating worsening MGFA scores or therapy escalation following COVID-19 infection. <h3>Background:</h3> Myasthenia gravis (MG) is an autoimmune neuromuscular disorder characterized by fluctuating weakness that affects the limbs, oculobulbar muscles, and diaphragm. Therapies include steroids and immunosuppressants, placing patients at high risk of opportunistic infections. Certain triggers can worsen MG, such as medications and infections. We describe the outcomes of 36 patients with confirmed MG and COVID-19, with particular focus on changes in Myasthenia Gravis Foundation of America (MGFA) scores and treatment escalation following infection. <h3>Design/Methods:</h3> Single center retrospective chart review of 36 patients from March 2020-January 2022 with clinically or serologically confirmed MG and COVID-19 <h3>Results:</h3> Of these 36 patients, 20 (55.6%) were not vaccinated against COVID. Twelve patients (33%) had MGFA scores of 3 or 4. Nineteen patients (53%) were prescribed steroids and 18 patients (50%) were on alternate immunotherapy prior to diagnosis of COVID. A total of 5 patients (14%) had a change in baseline MGFA score. One patient’s MGFA score worsened from 1 to 2 and 4 patients required MG treatment escalation. <h3>Conclusions:</h3> Myasthenia gravis patients with higher premorbid MGFA scores, steroid use, or other immunosuppressive therapy would be expected to have worse clinical outcomes in their MG course following COVID infection. Our results indicate 14% of MG patients with COVID had a change from their baseline MGFA score. Our study results revealed higher premorbid MGFA scores or use of immunosuppressant therapy did not alter a patient’s MGFA score following COVID infection. These results are consistent with current literature that COVID infection has little impact on these patients. <b>Disclosure:</b> Dr. Sandhu has nothing to disclose. Dr. Quezada has nothing to disclose. Dr. Rana has received personal compensation in the range of $500-$4,999 for serving as a Consultant for CSI. Dr. Rana has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Pharmawrite. Dr. Rana has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biohaven. Dr. Rana has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Argenx. Dr. Rana has received personal compensation in the range of $500-$4,999 for serving as a Consultant for amylyx. Dr. Rana has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Alexion. Dr. Small has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Alexion.

Rare case of double seronegative ocular myasthenia gravis and non-functional pituitary macroadenoma

Chronic autoimmune neuromuscular disorder known as Myasthenia Gravis (MG). The relationship of myasthenia gravis with pituitary adenomas is relatively uncommon, despite the fact that it is widely known that people with the condition have a higher prevalence of other autoimmune diseases. Here, we describe the case of a 45-year-old Indian man who had complained of severe cephalgia, associated with ocular pain, dizziness, diplopia, ptosis of the unilateral right eye. Although he has a history of severe headaches that started prior to 3 months, his complaints became more obvious at the end of the day. An MRI of the brain revealed an increased pituitary gland that measured 13.7 × 15.5 x 16. 9 mm. Furthermore, post admission histological examinations supported the diagnosis of a double seronegative ocular myasthenia gravis and non-functional pituitary macroadenoma. Following treatment, it was discovered that Pyridostigmin and Prednisolone considerably reduced his myasthenia symptoms. Finally, it should be noted that pituitary tumours are one potential underlying cause of headache in myasthenic patients.

Myasthenia gravis with achalasia secondary to thymoma: a case report and literature review

BackgroundMyasthenia gravis is an autoimmune neuromuscular junction disorder characterized by fatigable muscle weakness and autoantibodies. Frequent associations exist between myasthenia gravis and thymic abnormalities, including hyperplasia and thymoma. Several autoimmune illnesses have been identified to be associated with thymoma; however, a few case reports have linked thymoma and achalasia, and the underlying mechanism is unknown.Case reportA 43-year-old man with thymoma-associated myasthenia gravis presented with dysphagia that was refractory to conventional treatment of myasthenia gravis. This dysphagia was challenging to diagnose even after multiple gastroenterology consults and upper endoscopy. The diagnosis of achalasia type II was established after a comprehensive evaluation, including upper endoscopy, barium swallow, and high-resolution esophageal manometry. The patient underwent elective pneumatic balloon dilatation, which successfully alleviated his dysphagia.ConclusionThis case confirmed the association between myasthenia gravis secondary to thymoma and achalasia and showed how the diagnosis of achalasia was challenging. Awareness of this association is crucial for early diagnosis and treatment, improving affected patients’ quality of life.

Objectivity, practicality, and significance of practice guidelines for the practicing neurologists: What we learnt from consensus criteria in CIDP, Myasthenia Gravis and Inflammatory Myopathies.

The value of practice guidelines in the three most common autoimmune neuromuscular disorders, namely Chronic Inflammatory Demyelinating Polyneuropathy (CIDP), Myasthenia Gravis (MG) and Autoimmune Inflammatory Myopathies (AIM), has been extensively debated regarding their usefulness in clinical practice, objectivity and universal value considering that guidelines are also established regionally in certain countries. This commentary highlights common concerns on how guidelines are presently generated, pointing out: (a) non-sufficient diversity among Task-Force members to identify and address not only routine clinical and electrophysiology issues but also immunology, imaging, pathology, biomarkers, epidemiology or treatment economics; (b) Task-Force being often comprised by the same or seemingly like-minded members conveying the erroneous impression that experts with opposing views might have been excluded, even if this is clearly not the case; and (c) relying on web-based registries or retrospective data collections from heterogeneous sources. As a result, the existing practice guidelines in CIDP, MG and AIM remain an unfinished business but an excellent base for further enhancement. Guidelines can be extremely helpful not only for clinical trials but also in clinical practice if viewed as a living document with continuously updated versions by experts even with opposing views with precise information on diagnostics, pathomechanisms, therapeutic schemes, evolving biomarkers and economics of new therapies with validation of the post-guidelines criteria. Geographic diversity should be taken into consideration because the availability of biomarker testing, and therapies differ among countries. Patient preferences need to be also considered in therapeutic guidelines because newly marketed drugs offer more options steadily changing the therapeutic algorithms in autoimmune neuromuscular diseases generating also questions as to whether they also influence decisions on insurance coverage. Collectively, these startup considerations are aimed to make practice guidelines more objective, widely acceptable worldwide and more practical or easier to follow in clinical practice.

Testing for N-type Voltage Gated Calcium Channel Antibody has Limited Utility in Evaluating Patients With Suspected Lambert-Eaton Myasthenic Syndrome

Objective To determine if testing for N-type voltage-gated calcium channel antibody improves diagnostic performance of serological testing for Lambert-Eaton Myasthenic Syndrome. Background Patients with suspected autoimmune neuromuscular junction transmission disorders are commonly tested for both P/Q-type (VGCC-P/Q) and N-type (VGCC-N) voltage-gated calcium channel antibodies to aid the diagnosis of Lambert-Eaton Myasthenic Syndrome (LEMS). Design/Methods This retrospective cross-sectional study included 93 patients diagnosed at Mayo Clinic with LEMS based on electrodiagnostic findings, clinical presentation and positive serology for VGCC-P/Q and/or VGCC-N. Results Forty-five patients (48.4%) were female, of median age 61 years (range 11- 99). Twenty-five (26.9%) were positive for both VGCC antibody types (reference interval &lt;0.04 nmol/L [VGCC-N] and &lt;0.03 nmol/L [VGCC-P/Q]); 67 (72.0%) were positive for VGCC-P/Q only and one (1.1%) was positive for VGCC-N only. The single VGCC-N positive only result [VGCC-N = 0.10 nmol/L; VGCC-PQ = 0.02 nmol/L] was from a patient with classic LEMS presentation and no evidence of malignancy by Chest CT. The prevalence of VGCC-N only positivity was lower than in the healthy population or other disease control populations. VGCC-P/Q antibody titers were higher in patients who had detectable co-existing VGCC-N antibody (median titer 1.58 versus 0.39 nmol/L, P = 0.02) and there was a positive correlation (r = 0.762, P &lt; 0.001) between VGCC-N and VGCC-P/Q titers in double positive cases. Dual positivity did not significantly increase the likelihood of an underlying cancer (40.9% versus 30.1%, P = 0.33). Conclusions Testing for VGCC-P/Q antibodies alone is sufficient in the serological evaluation of suspected LEMS cases. Inclusion of VGCC-N antibody testing does not improve diagnostic performance. A positive VGCC-N antibody did not significantly increase the risk of paraneoplastic LEMS.

Real-World Utilization Patterns of Intravenous Immunoglobulin in Adults With Generalized Myasthenia Gravis in the United States

Objective To evaluate real-world utilization patterns of intravenous immunoglobulin (IVIg) among patients with generalized myasthenia gravis (gMG) over 3 years post-IVIg initiation. Background gMG is a rare autoimmune neuromuscular disorder with no known cure. Although IVIg is the most commonly used add-on therapy after standard of care treatments in gMG, it is currently unclear whether it is more commonly used as a “one-off” treatment to manage exacerbations, or as maintenance therapy aimed at reducing or replacing steroid use. Design/Methods Patients with gMG who initiated IVIg treatment were identified from a US claims database (Symphony Health, an ICON plc Company, Integrated Dataverse [IDV]®, January 1, 2014-December 31, 2019). The frequency of subsequent IVIg treatment and associated cost during the first 12 months post-IVIg initiation were analyzed. Usage patterns of concomitant gMG treatments during the year preceding and 3 years post-IVIg initiation were compared. Results Among 1225 patients with gMG who initiated IVIg treatment, 706 patients (57.6%) received 1 to 5 IVIg treatment courses (intermittent IVIg users), and 519 patients (42.4%) received = 6 IVIg treatment courses (chronic IVIg users) within the subsequent year. Mean annual medical cost per patient was nearly 2.5-fold higher for chronic vs intermittent IVIg users ($161,478 vs $64,888, p &lt; 0.001). Usage frequency of other immunotherapy treatments did not decrease over the follow-up period, even for patients who continued annual chronic IVIg for 3 consecutive years post-initiation. Conclusions Nearly half of patients with gMG received chronic and multiple IVIg treatment courses within the first year once initiating IVIg treatment, indicating higher usage than expected. For all IVIg initiators, usage frequency of other immunotherapy treatments did not decrease over 3 years despite IVIg initiation.

A Symptoms and Signs Approach to the Patient With Neuromuscular Weakness

Muscle weakness is a common feature of many neuromuscular disorders. This article outlines a symptoms and signs approach to the patient presenting with neuromuscular weakness, highlighting key aspects of the clinical history and examination. The past several years have seen a dramatic increase in the ability to test for many inherited and autoimmune neuromuscular disorders more reliably and accurately. Similarly, numerous targeted therapies have been recently approved to treat previously untreatable disorders. Therefore, timely and accurate diagnosis is essential so that patients can receive appropriate therapy, ultimately leading to better clinical outcomes. Muscle weakness is a common symptom resulting from dysfunction that can occur at any level of the neuraxis and is a cardinal feature of many neuromuscular disorders. An accurate and meticulous history and a thorough neurologic examination are paramount in localizing the lesion in order to generate a differential diagnosis and guide appropriate ancillary testing. The patient's age at symptom onset, any identified inciting factors, tempo of symptom progression, pattern of weakness, and associated symptoms and signs are all important diagnostic clues in the evaluation of a patient presenting with muscle weakness.

WEST NILE-INDUCED MYASTHENIA GRAVIS CRISIS: AN UNEXPECTED CASE OF RESPIRATORY FAILURE

WEST NILE-INDUCED MYASTHENIA GRAVIS CRISIS: AN UNEXPECTED CASE OF RESPIRATORY FAILURE

Ocular myasthenia

Myasthenia gravis is a chronic autoimmune neuromuscular disorder typically presenting with variable skeletal muscle fatigue. The most prevalent initial manifestation of myasthenia is ocular myasthenia. Approximately 50% of all myasthenia cases begin with the weakening of the eye muscles that generally presents as diplopia and/or ptosis. However, about 60% of these cases progress to a generalized type within the first 2 years. The diagnosis of ocular myasthenia is often difficult but is collectively confirmed by clinical evaluation and laboratory results.Clinical manifestations, diagnostic approach, relevance, and therapeutic options are discussed in this article.