Myasthenia gravis: understanding treatment patterns and direct medical costs in the Czech Republic.

Background: Myasthenia gravis (MG) is a rare autoimmune disorder with significant clinical implications, including life-threatening myasthenic crises and exacerbations. Understanding real-world treatment patterns, especially associated direct medical costs, is essential for the effective management of healthcare delivery.

Methods: We conducted a descriptive cohort study using health administrative claims data from the Czech Republic covering more than 1,500 prevalent MG patients.

Immunochemotherapy for small cell lung cancer with paraneoplastic Cushing syndrome: A case report and literature review.

Rationale: Paraneoplastic Cushing syndrome (PCS) is an adverse prognostic factor for small cell lung cancer (SCLC) patients. Retrospective studies have shown that the median survival of SCLC complicated with PCS was <7 months. No immunochemotherapy has been recorded in the treatment of SCLC with PCS.

The limbic and extra-limbic encephalitis associated with glutamic acid decarboxylase (GAD)-65 antibodies: an observational study.

We aimed to define the clinical features and outcomes of encephalitis associated with anti-GAD65 Abs. In addition, we reviewed cases published in the literature with GAD65 encephalitis. We retrospectively studied 482 consecutive patients attending a tertiary care center for evaluation of an autoimmune neurological disorder.

A clinical perspective on muscle specific kinase antibody positive myasthenia gravis.

The discovery of autoantibodies directed against muscle-specific kinase (MuSK) in "seronegative" myasthenia gravis (MG) patients marked a milestone in MG research. In healthy muscle, MuSK regulates a phosphorylation pathway, which is essential for the development and maintenance of acetylcholine receptor (AChR) clusters at the neuromuscular junction. Autoantibodies directed against MuSK are predominantly of the IgG4 subclass, but there is increasing evidence that IgG1-3 could also contribute to the pathology underlying MuSK-MG.

Zika virus infection and acute transverse myelitis: a comprehensive systematic review.

The Zika virus (ZIKV) has been associated with several complications, including acute transverse myelitis (ATM), an acute inflammation of the spinal cord, with rapid development of motor, sensory and dysautonomic symptoms. It is a rare disease, and its clinical features, as well as differences in relation to idiopathic ATMs, are still not completely known. The objective of this paper is to review the literature in search of clinical features and complementary exams of ATM post-ZIKV infection, alone or in association with other neurological conditions (mixed diseases), as well as its treatments and prognoses.

Seizures in a healthy 20-year-old woman.

Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is the most common type of autoimmune encephalitis. The initial presentation can vary from subtle mental status changes to more severe symptoms such as seizures, coma, or death. This case report outlines the interdisciplinary and collaborative approach to diagnosing and treating a patient with anti-NMDAR autoimmune encephalitis.

[Successful treatment of rheumatoid arthritis complicated with myasthenia gravis with low-dose rituximab: A case report].

Rheumatoid arthritis (RA) and myasthenia gravis (MG) are two distinct autoimmune diseases. Compared with the general population, the incidence of RA is notably higher among patients with MG. Similarly, the rate of MG in patients diagnosed with RA is also significantly increased.

[Clinical and epidemiological characteristics of myasthenia in the Altai region].

Objective: To analyze the epidemiological and clinical characteristics of myasthenia gravis (MG) in the Altai region (AR).

Material And Methods: One hundred and twenty-two patients with a verified diagnosis of MG, living in the AR, took part in the study. To collect data, a questionnaire was developed, the data of which formed the basis for the study.

Trousseau syndrome-induced cerebral infarction: Two case reports.

Rationale: As a paraneoplastic syndrome, Trousseau syndrome (TS) is a collective term for various thromboembolic events caused by clotting and fibrinolytic abnormalities in patients with tumors, clinically manifesting as venous and arterial thromboembolism, as well as disseminated intravascular coagulation (DIC). The incidence rate of arterial thrombosis in patients with TS is 2% to 5%.

Patient Concerns: This article reports 2 patients with TS-induced cerebral infarction.

Unveiling the Enigma: A Peculiar Encounter of Concurrent Anti--methyl-D-aspartate Receptor and Anti-Hu Antibody Positive Paraneoplastic Syndrome in Small Cell Lung Cancer.

Paraneoplastic neurological syndromes (PNS) are mostly immune-mediated, tumor-associated disorders. Earlier the 2004 PNS criteria were used which are now partially outdated due to advances in PNS research and also identification of new phenotypes and antibodies that have transformed the diagnostic approach to PNS; hence, a new criterion was proposed in 2016. They can have multifarious presentations, ranging from behavioral abnormalities to altered sensorium and coma.

Regional ischaemic immune myopathy: unraveling a rare subtype of dermatomyopathy.

Regional ischaemic immune myopathy (RIIM) is a subtype of dermatomyopathy which has distinct histopathology features of regional muscle fibre necrosis and regeneration. This case report aims to provide an in-depth exploration of RIIM, emphasising its clinical features and the crucial role of muscle biopsy as a diagnostic investigation, as well as the potential role of immunomodulatory therapies.

Multicenter Validation of the Ocular Myasthenia Gravis Rating Scale Questionnaire.

Background And Objectives: Ocular myasthenia gravis (OMG) causes disabling ocular symptoms of ptosis and diplopia, but a validated disease-specific patient-reported outcome measure (PROM) has not been reported. We sought to validate a novel PROM for OMG, OMG Rating Scale Questionnaire (OMGRate-q), as a measure of visual functioning to support patient-centered care.

Methods: This was a prospective study of patients aged 18 years and older with OMG receiving care at 3 medical centers (January 2022-October 2023).

Small Ovarian Teratoma Causes Anti-N-methyl- D-aspartate Encephalitis.

This is the case of a 22-year-old female who arrived at our institution after experiencing refractory insomnia, disorganized behavior, inappropriate laughter, and anorexia. Upon admission, a physical examination revealed mutism, irritability, and visual hallucinations. Infectious, metabolic, and other, alternative, causes for the presenting symptoms were excluded.

Patient-Reported Outcome Measures in NMDA Receptor Encephalitis.

Background And Objectives: The characteristics of persistent long-term symptoms and their contribution to subjective quality of life remain unclear in patients with NMDAR encephalitis. In this study, we aimed to evaluate postacute neuropsychiatric symptoms, subjective cognitive complaints, and disease coping mechanisms and identify predictors of health-related quality of life (HRQoL) after N-methyl-D-aspartate receptor (NMDAR) encephalitis.

Methods: This cross-sectional observational study investigated patients with NMDAR encephalitis in the postacute phase.

Changes in Myasthenia Gravis Incidence in Denmark From 1985 to 2021: A Nationwide Population-Based Study.

Background And Objectives: Previous studies on incident myasthenia gravis (MG) have shown inconsistent results. In this study, we determined the MG incidence rates (IRs) over a 36-year period to detect potential trends in MG incidence over time and for different age groups.

Methods: In this nationwide population-based study from 1985 to 2021, we identified patients aged 18 and older with a primary MG diagnosis in the Danish National Patient Registry.

A nationwide epidemiological study of myasthenia gravis in Turkey.

Background: Variable incidence and prevalence rates of myasthenia gravis (MG) have been reported over time from different geographical regions. We aimed to determine incidence and prevalence of MG in Turkey and contribute to the development of proper national health policies.

Methods: Patients with G70.

Medical costs of treating myasthenia gravis in patients who need intravenous immunoglobulin (IVIg) - a register-based study.

Background: Several innovative treatments are expected for myasthenia gravis (MG) in the coming years. Healthcare payers usually require cost-effectiveness analyses before reimbursement. We aimed to investigate resource utilization and direct medical costs for patients with MG treated with intravenous immunoglobulin (IVIg) to inform such analyses.

Factors affecting the diagnostic delay of myasthenia gravis.

Background: Early detection and diagnosis of myasthenia gravis (MG) is important to improve the chance of remission and overall prognosis. This study aims to investigate the factors affecting the diagnostic delay of MG thereby highlighting the challenges in the diagnostic process.

Methods: We conducted a retrospective study examining characteristics and factors involved in the diagnostic process of MG.

[Anti-Hu syndrome - an unusual cause of vertigo].

On the palliative care ward, we treated a man with a small cell lung cancer who was suffering from vertigo for six years, however the vertigo got stronger the last six months with pronounced coordination disorders. After several examinations, the cause was a paraneoplastic neurologic syndrome (PNS) with Hu-antibodies. PNS are various neurological malfunctions, which occur mostly in certain patterns.

Ectopic olfactory neuroblastoma is associated with increased frequency of syndrome of inappropriate antidiuretic hormone secretion and reduced disease control: Case series with systematic review and pooled analysis.

Introduction: Olfactory neuroblastoma (ONB) is a rare malignant tumor originating from the olfactory neuroepithelium, typically within the sinonasal cavity. Cases of ONB originating outside of the olfactory cleft area are extremely rare and are referred to as "ectopic" (eONB), in contrast to "orthotopic" tumors (oONB). ONB has been associated with paraneoplastic syndromes (PNSs), including the syndrome of inappropriate antidiuretic hormone secretion (SIADH).

Occult ovarian adenocarcinoma manifesting as dermatomyositis.

A woman in her 60s presented with erythematous lesions predominantly over the joints. After evaluation by dermatology and rheumatology, she was diagnosed with dermatomyositis and initiated on oral steroids and immunosuppressants. She was subsequently referred to gynaecology services for further evaluation of possible malignancies.

Anti-NMDAR encephalitis presenting with persistent fever and meningitis and responding well to distinctive individualized first-line treatment: A case report.

Rationale: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune encephalitis characterized by diverse neurological and psychiatric symptoms. It predominantly affects young women, particularly those with ovarian teratomas. However, cases without teratomas are also commonly reported, often exhibiting poorer treatment responses and higher relapse rates.

A Paradigm Shift in Renal Cell Carcinoma: Recognizing Hypoglycaemia as a Significant Paraneoplastic Syndrome.

Recurrent hypoglycaemia is a rare paraneoplastic syndrome associated with several cancers, including renal cell carcinoma (RCC). Here we present a curious case of a patient with type 2 diabetes mellitus (T2DM) who had numerous hospitalisations due to recurrent hypoglycaemia. A well-defined lesion at the lower pole of the right kidney and bilateral pyelonephritis were found during imaging investigations.