Recent reports have shown that patients with Lambert-Eaton myasthenic syndrome (LEMS) improve transiently after high-dose intravenous immunoglobulin (IVIG) administration. Information about the usefulness of IVIG for long-term treatment is rather scanty. Our findings demonstrate the efficacy of monthly IVIG courses at a dose of 0.4 g/kg/day for 5 days, in a 41-year-old patient with LEMS without detectable malignancy. Improvement in limb strength, peak expiratory flow rate, and electrophysiological parameters, as well as clinical signs following IVIG, was evident as early as 7 days after the first course and is still maintained at 24-months follow-up.
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http://dx.doi.org/10.1002/(sici)1097-4598(199706)20:6<674::aid-mus3>3.0.co;2-5 | DOI Listing |
BMC Neurol
January 2025
Department of Neurology, Liuzhou People's Hospital affiliated to Guangxi Medical University, No.8 Rd.wenchang Liuzhou, Liuzhou, 545000, Guangxi Province, China.
Background: Anti-NMDA receptor encephalitis is an autoimmune, antibody-mediated inflammatory disease of the brain characterized by the presence of IgG antibodies targeting the excitatory N-methyl-D-aspartate receptor (NMDAR). Previous research has established that the neonatal Fc receptor (FcRn) regulates the transport and circulation of immunoglobulins (IgG). Efgartigimod, an FcRn antagonist, has been shown to enhance patient outcomes by promoting IgG clearance, and it has exhibited substantial clinical efficacy and tolerability in the treatment of myasthenia gravis.
View Article and Find Full Text PDFRheumatol Adv Pract
January 2025
Department of Pediatrics, Dokkyo Medical University, Tochigi, Japan.
Graphical Abstract.
View Article and Find Full Text PDFProg Rehabil Med
January 2025
Division of Rehabilitation Medicine, Gunma University Hospital, Maebashi, Japan.
Background: Immune-mediated necrotizing myopathy (IMNM) is a type of autoimmune myositis. Anti-signal recognition particle (SRP) antibodies are highly specific to this disease.
Case: A 76-year-old woman presented with a 4-month history of acute progressive limb muscle weakness and dysphagia.
Front Med (Lausanne)
January 2025
Department of Hematology, The Second Affiliated Hospital of Nanchang University, Nanchang, China.
Congenital thrombotic thrombocytopenic purpura (cTTP) is a thrombotic microangiopathy (TMA) characterized by severe hereditary ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 motifs 13) deficiency caused by mutations. This rare autosomal recessive genetic disorder is often misdiagnosed as immune thrombocytopenia (ITP) or hemolytic uremic syndrome (HUS). Here, we report a 21-year-old male cTTP patient with a compound heterozygous mutation.
View Article and Find Full Text PDFZhonghua Er Ke Za Zhi
January 2025
Department of Neonatal Intensive Care Unit, Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, National Children's Regional Medical Center, Hangzhou310052, China.
To assess the changes in epidemiologic and clinical characteristics of respiratory syncytial virus (RSV) infection among inpatient under 3 months of age with lower respiratory tract infection (LRTI) in different stages of the SARS-CoV-2 pandemic. A retrospective cohort study was conducted. Clinical data on general condition and laboratory tests were collected from 3 144 RSV-positive LRTI infants less than 3 months of age hospitalized at Children's Hospital, Zhejiang University School of Medicine from January 2017 to January 2024.
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