Objective: To summarize the characteristics of muscle-specific receptor tyrosine kinase antibody-positive myasthenia gravis (MuSK-MG) in Northeast China. Methods: We retrospectively collected 183 confirmed MG patients and divided them into three groups based on the type of serum antibodies: MuSK-MG (14 cases), acetylcholine receptor (AChR)-MG (130 cases), and double-seronegative (DSN)-MG (39 cases). The clinical, diagnostic, therapeutic, and prognosis data were analyzed. Results: MuSK antibody was detected in 26.7% of seronegative MG. The mean age of onset in MuSK-MG was 53.2 ± 13.6 years. Fifty percent of MuSK-MG patients with an onset symptom of pure ocular muscle weakness. The time from onset to other muscle groups' involvement and the time from onset to myasthenic crisis had no significant difference among the three groups ( P > 0.05). The proportion of Osserman classification I in MuSK-MG group was lower than that in DSN-MG group. The proportion of Osserman classification IV in MuSK-MG group was higher than that in the other two groups. The incidences of other coexisting autoimmune diseases in MuSK-MG group were higher. Prognosis after the treatment of steroid combined with tacrolimus for MuSK-MG was similar to AChR-MG treated with steroid combined with an immunosuppressant agent ( P > 0.05). Conclusion: Patients with MuSK-MG in Northeast China have a modestly later onset age and a proportion of patients may have a mild form of the disease with delayed disease progression. We confirmed the existence of a rare ocular MuSK-MG phenotype, a high proportion of coexisting with other autoimmune diseases, and a good response to steroids combined with tacrolimus for our MuSK-MG series.
As an adverse immune phenomenon, graft-versus-host disease often occurs after allogeneic hematopoietic stem cell transplantation. The incidence of acute and chronic graft-versus-host disease is about 40–60% and the mortality rate can reach 15%, which is a potentially fatal disease. There are rare GvHD cases involving the central nervous system. We reported a rare case of diffuse white matter changes after haploid bone marrow transplantation, summarizing its clinical manifestations and diagnosis and treatment in conjunction with the literature.
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ObjectiveTo explore and compare the cognitive processing weakness of children with Attention deficit hyperactivity disorder (ADHD) and comorbid reading disabilities (RD) (ADHD+RD) and children with ADHD only using the Das-Naglieri Cognitive Assessment System (DN:CAS).MethodsEighty-eight children with ADHD who visited the hospital for the first time from September 2021 to November 2021 and had a Full scale intelligence quotient (IQ) of ≥85 on the Wechsler Intelligence Scale for Children revised in China (C-WISC) were selected (Age: 6–12 years; Grade: 2–6). Based on comorbidity with RD and the subtypes of ADHD (e.g., Inattention dominant type, ADHD-I, Hyperactivity/Impulse dominant type, ADHD-H and Combined type, ADHD-C), these children were divided into the ADHD+RD group (n = 30) and ADHD group (n = 58) as well as the corresponding subgroups. Clinical data on gender, age, grade, IQ scores, and DN:CAS processing scores were compared between both groups/subgroups. Spearman's correlation test was used for correlation analysis of results of interest.ResultsNo differences in age, grade, male-to-female ratio, verbal IQ, performance IQ, and full scale IQ were observed between the ADHD+RD group and ADHD group as well as the corresponding subgroups (P > 0.05). Children in the ADHD-C+RD subgroup had lower scores in Planning processing of DN:CAS than those in the ADHD-C subgroup (P = 0.040). However, there were no significant difference between the ADHD-I+RD subgroup and ADHD-I subgroup in Planning scores of DN:CAS assessment; The grade of ADHD-C+RD and ADHD-I+RD subgroups were positively correlated with the Planning scores of DN: CAS (r = 0.599, P = 0.030 and r = 0.508, P = 0.044, respectively). The grade of ADHD-C subgroup was positively correlated with the Planning and Simultaneous processing scores of DN: CAS (r = 0.409, P = 0.042 and r = 0.406, P = 0.044, respectively).ConclusionOur study confirmed that children of ADHD-C with comorbid RD have a more severe Planning processing weakness compared to children with ADHD-C only. Among the children of ADHD-C+RD, ADHD-I+RD and ADHD-C, such a Planning processing impairment may improve with increasing educational skills.
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