Internal medicine
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The coexistence of leucine-rich glioma-inactivated 1 (LGI1) and contactin-associated protein-like 2 (CASPR2) autoantibodies in the same individual is surprisingly often observed. We herein report the first case of LGI1 encephalitis followed by Isaacs syndrome in which LGI1 and CASPR2 antibodies in the serum and cerebrospinal fluid (CSF) were measured during the entire disease course. After the resolution of limbic encephalitis, LGI1 antibodies disappeared from the CSF simultaneously with the appearance of CASPR2 antibodies in the serum. The alternating presence of these pathogenic autoantibodies along with the clinical and phenotypic alternations suggested that LGI1 encephalitis was associated with CASPR2 autoantibody production in the peripheral tissue, leading to CASPR2-associated Isaacs syndrome.
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We herein report a 76-year-old woman who developed lower cranial dystonia with a peculiar appearance of cheek inflation. The patient showed strong contraction of the orbicularis oris muscles. Consequently, her cheeks were passively inflated by expiration without exit. ⋯ We tentatively named this characteristic dystonia "lower cranial dystonia with inflated cheeks" because of its peculiar appearance of inflated cheeks. This dystonia can cause respiratory failure. Therefore, neurologists should recognize such dystonia as a movement disorder emergency.
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Objective Pleural infection is a significant disease that continues to pose severe problems for respiratory physicians. However, prognostic factors of pleural infection remain poorly understood. The controlling nutritional status (CONUT) score represents the immune-nutrition status of patients with chronic infectious diseases. ⋯ The incidence of 90-day mortality was higher in patients with higher CONUT scores than in those with lower scores [25.3% (21/84) vs. 2.8% (7/251), p<0.001]. In addition, after adjusting for confounders, a high CONUT score was found to be an independent prognostic factor for 90-day mortality (hazard ratio, 9.30; 95% confidence interval, 3.96-21.87; p<0.001). Conclusion Our study indicated that a high CONUT score was associated with an increased risk of 90-day mortality in patients with pleural infection and can be considered for clinical evaluations in practice.
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Objective Hereditary ATTR (ATTRv) amyloidosis was once an incurable disease; however, in recent years, disease-modifying therapies, such as tafamidis and patisiran, have become available. We herein report the medical care situation in an ATTRv amyloidosis non-endemic area of Japan. Methods We confirmed the information in the medical records of our department and analyzed the data retrospectively. ⋯ Although it took a long time to start treatment among our experienced cases, there were some cases in which treatment could be introduced relatively early. Conclusion ATTRv amyloidosis is treatable and should be included in the differential diagnosis of neuropathy so that it can be diagnosed early and introduced into treatment. In the near future, the presymptomatic diagnosis of ATTRv amyloidosis and genetic counseling will become more important.
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Case Reports
A Hepatitis B Virus Reactivation Case Potentially Triggered by the Onset of Diffuse Large B Cell Lymphoma.
An 81-year-old man underwent rituximab-containing chemotherapy for chronic lymphocytic leukemia (CLL). Thirteen years after his last chemotherapy, he was diagnosed with hepatitis B virus (HBV) reactivation. He was then treated with entecavir, and improvement was seen in his liver injury. ⋯ Despite chemotherapy, he contracted the coronavirus disease 2019 (COVID-19) and died of COVID-19. We suspect that HBV reactivation was triggered by DLBCL. When HBV reactivation occurs a long time after chemotherapy has concluded, the onset of DLBCL should be considered.