Internal medicine
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We herein report a Japanese patient with myotonic dystrophy type 2 (DM2), which is rare in Japan. A 64-year-oldman had proximal muscle weakness and grip myotonia. ⋯ A muscle biopsy revealed increased central nuclei, pyknotic nuclear clumps and muscle fiber atrophy, mainly in type 2 fibers, raising the possibility of DM2. The diagnosis was genetically confirmed by the abnormal CCTG repeat size in cellular nucleic acid-binding protein (CNBP) on repeat-primed polymerase chain reaction, which was estimated to be around 4,500 repeats by Southern blotting.
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Case Reports
Nonepisodic Angioedema with Eosinophilia Following Receipt of the BNT162b2 mRNA COVID-19 Vaccine.
Angioedema with eosinophilia (AE) is a rare disease of unknown etiology characterized by episodic (EAE) or nonepisodic AE (NEAE). Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) mRNA-based vaccines function as immunogens and intrinsic adjuvants and have been shown to be safe in large-scale trials. ⋯ We herein report a case of self-limited but severe NEAE that developed in a young woman one week after receiving the second BNT162b2 mRNA vaccine. The symptoms that impaired her activities of daily living, such as edema, gradually resolved with supportive care over 10 weeks without corticosteroid treatment.
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A 33-year-old woman developed paresthesia in her right thumb approximately 30 minutes after receiving the BNT162b2 (Pfizer-BioNTech) severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccine. The paresthesia gradually spread to her right-side limbs and trunk, and cervical magnetic resonance imaging (MRI) revealed a localized lesion in the right dorsal column. After glucocorticoid therapy, her symptoms and MRI findings improved. Although disease developing less than 24 hours after vaccination is considered an unlikely cause of immuno-associated adverse events following vaccination, we discuss the possible mechanisms involved in early-onset central nervous system inflammation after vaccination in view of preexisting immunopathological susceptibility.
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Objective Venous thromboembolism (VTE) is a common complication of severe coronavirus disease 2019 (COVID-19) and is associated with its prognosis. The fibrin monomer complex (FMC), a marker of thrombin generation, is reportedly useful in diagnosing acute thrombosis. To date, there has been only one report on FMC in COVID-19, and the usefulness of FMC in COVID-19 is unknown. ⋯ A multivariate analysis showed that severe progression was associated with elevated FMC levels (odds ratio, 7.40; 95% confidence interval, 2.63-22.98; p<0.001). Conclusion FMC can be useful for diagnosing VTE in the acute phase of COVID-19. Elevated FMC was found to be associated with severity on admission and severe progression.
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Objective Steroid pulse therapy is a regimen involving the intravenous administration of supra-pharmacological doses of corticosteroids in the short term. It is used to treat various inflammatory and autoimmune conditions. However, the strengths and limitations of steroid pulse therapy for induction of remission in type 1 autoimmune pancreatitis (AIP) are unknown. ⋯ The log-rank test revealed that the relapse-free survival in the Pulse-alone group was significantly shorter than that in the PSL (p=0.024) and Pulse+PSL groups (p=0.014). The exacerbation of glucose tolerance after steroid therapy was less frequently observed in the Pulse-alone group (0%) than in the PSL group (17%, p=0.050) and Pulse+PSL groups (26%, p=0.011). Conclusion Although treatment with IVMP pulse alone resulted in unsatisfactory relapse prevention outcomes compared with conventional steroid therapy, the IVMP pulse-alone regimen might be an alternative treatment strategy for type 1 AIP from the perspective of avoiding adverse events from steroids.