ABSTRACT
Background@#and Purpose Advances in serological tests are transforming the classification of idiopathic inflammatory myopathy (IIM). The new criteria suggested by the 119th European Neuromuscular Center international workshop divide IIM cases into four main diseases according to clinical and pathological findings, adding immune-mediated necrotizing myositis and nonspecific myositis to the classic categories of polymyositis and dermatomyositis. @*Methods@#Seventy one cases of IIM with sufficient available clinical and pathological data were reviewed to be reclassified according to the new criteria. @*Results@#Most of the cases previously classified as polymyositis (77.8%, 35/45) were reclassified as immune-mediated necrotizing myopathy. The results of myositis-specific antibodies matched well with the new clinicopathological classification. @*Conclusions@#This new clinicopathological classification for IIM in combination with serological test results could be applied to our previous case series. Adoption of the new criteria will lead to a better understanding of the disease and hence new therapeutic insights.
ABSTRACT
Background@#During the coronavirus disease 2019 (COVID-19) pandemic, patients with myasthenia gravis (MG) were more susceptible to poor outcomes owing to respiratory muscle weakness and immunotherapy. Several studies conducted in the early stages of the COVID-19 pandemic reported higher mortality in patients with MG compared to the general population. This study aimed to investigate the clinical course and prognosis of COVID-19 in patients with MG and to compare these parameters between vaccinated and unvaccinated patients in South Korea. @*Methods@#This multicenter, retrospective study, which was conducted at 14 tertiary hospitals in South Korea, reviewed the medical records and identified MG patients who contracted COVID-19 between February 2022 and April 2022. The demographic and clinical characteristics associated with MG and vaccination status were collected. The clinical outcomes of COVID-19 infection and MG were investigated and compared between the vaccinated and unvaccinated patients. @*Results@#Ninety-two patients with MG contracted COVID-19 during the study. Nine (9.8%) patients required hospitalization, 4 (4.3%) of whom were admitted to the intensive care unit. Seventy-five of 92 patients were vaccinated before contracting COVID-19 infection, and 17 were not. During the COVID-19 infection, 6 of 17 (35.3%) unvaccinated patients were hospitalized, whereas 3 of 75 (4.0%) vaccinated patients were hospitalized (P < 0.001). The frequencies of ICU admission and mechanical ventilation were significantly lower in the vaccinated patients than in the unvaccinated patients (P = 0.019 and P = 0.032, respectively). The rate of MG deterioration was significantly lower in the vaccinated patients than in the unvaccinated patients (P = 0.041). Logistic regression after weighting revealed that the risk of hospitalization and MG deterioration after COVID-19 infection was significantly lower in the vaccinated patients than in the unvaccinated patients. @*Conclusion@#This study suggests that the clinical course and prognosis of patients with MG who contracted COVID-19 during the dominance of the omicron variant of COVID-19 may be milder than those at the early phase of the COVID-19 pandemic when vaccination was unavailable. Vaccination may reduce the morbidity of COVID-19 in patients with MG and effectively prevent MG deterioration induced by COVID-19 infection.
ABSTRACT
Methylene-tetrahydrofolate reductase (MTHFR) is a homocysteine metabolism-related enzyme and defects of MTHFR is a risk factor for hyperhomocysteinemia and related various neurological disease. Among them, 665C>T polymorphism is the most common form. We report a 48 years old man presenting with progressive psychiatric problems along with severe demyelinating polyneuropathy due to homozygous c.665C>T homozygote polymorphism superimposed by compound heterozygous mutation (c.1417C>T, p.Arg473Trp) in the MTHFR gene, without thromboembolic changes.
ABSTRACT
Inclusion body myositis is a rare condition of idiopathic inflammatory myopathy. Prior criteria for the diagnosis of inclusion body myositis essentially required pathological features of rimmed vacuoles, tubulofilamentous inclusions, and amyloid deposits. However, recently developed new diagnostic criteria emphasize clinical characteristics including weakness of finger flexors and knee extensors. In addition, a serological evaluation of anti-cN1A antibody is helpful for the diagnosis. We report a case of inclusion body myositis with clinical, pathological, and serological consideration.
ABSTRACT
Coronavirus disease 2019 (COVID-19) is an infectious disease caused by severe acute respiratory syndrome coronavirus 2 that may trigger Guillain-Barre syndrome (GBS) in selected patients. We describe a case of GBS presenting as marked finger extensor weakness in a 73-year-old woman with COVID-19. Her clinical and electrophysiological findings were consistent with a diagnosis of acute motor axonal neuropathy subtype of GBS with prominent finger dropping. Treatment with intravenous immunoglobulin for 5 days completely resolved her finger extension weakness after 19 months, although other involved extremities recovered earlier at 3 months. This study highlights that COVID-19-associated GBS can present in various forms aside from the classic variant, even in patients without any COVID-19 symptoms. Therefore, it is important to always consider the diagnosis of GBS in patients with COVID-19.