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  1. Article ; Online: Sporadic Late-onset Nemaline Myopathy Associated with Sjögren's Syndrome: A Case Report.

    Hamaguchi, Tsuyoshi / Nishino, Ichizo / Hirano, Yasuki / Uchida, Nobuaki / Fujita-Nakata, Michiyo / Nakanishi, Megumi / Sakai, Tomoyuki / Asahina, Masato

    Internal medicine (Tokyo, Japan)

    2024  

    Abstract: We report the case of a 46-year-old female patient who developed a subacute progression of axial and proximal muscle weakness. Laboratory findings revealed mildly elevated serum creatine kinase levels. No monoclonal gammopathy was detected. A muscle ... ...

    Abstract We report the case of a 46-year-old female patient who developed a subacute progression of axial and proximal muscle weakness. Laboratory findings revealed mildly elevated serum creatine kinase levels. No monoclonal gammopathy was detected. A muscle biopsy revealed that she had nemaline myopathy. Serological tests and a lip biopsy revealed Sjögren's syndrome (SjS). We diagnosed her as having sporadic late-onset nemaline myopathy without monoclonal gammopathy of undetermined significance associated with SjS. Her symptoms improved after methylprednisolone pulse therapy followed by intravenous immunoglobulin therapy. A good response to immunotherapy demonstrates the necessity of making a correct diagnosis, for which a muscle biopsy is required.
    Language English
    Publishing date 2024-03-04
    Publishing country Japan
    Document type Journal Article
    ZDB-ID 32371-8
    ISSN 1349-7235 ; 0021-5120 ; 0918-2918
    ISSN (online) 1349-7235
    ISSN 0021-5120 ; 0918-2918
    DOI 10.2169/internalmedicine.3092-23
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Autochthonous Cryptococcus gattii genotype VGIIb infection in a Japanese patient with anti-granulocyte-macrophage colony-stimulating factor antibodies.

    Hamaguchi, Tsuyoshi / Uchida, Nobuaki / Fujita-Nakata, Michiyo / Nakanishi, Megumi / Tsuchido, Yasuhiro / Nagao, Miki / Iinuma, Yoshitsugu / Asahina, Masato

    Journal of infection and chemotherapy : official journal of the Japan Society of Chemotherapy

    2024  

    Abstract: A 31-year-old Japanese man presented with cerebral and pulmonary cryptococcosis. Cryptococcus gattii (C. gattii) genotype VGIIb was detected in the patient's sputum and cerebrospinal fluid specimens. The serum levels of anti-granulocyte-macrophage colony- ...

    Abstract A 31-year-old Japanese man presented with cerebral and pulmonary cryptococcosis. Cryptococcus gattii (C. gattii) genotype VGIIb was detected in the patient's sputum and cerebrospinal fluid specimens. The serum levels of anti-granulocyte-macrophage colony-stimulating factor (GM-CSF) antibodies were elevated in this patient, which has been associated with pulmonary alveolar proteinosis and is considered a risk factor for C. gattii infection. After undergoing >12 months of antifungal treatments, the patient showed improvements in symptoms and findings on brain and lung imaging. Several Japanese patients who develop C. gattii infection have also been reported; however, most of these patients have been infected outside Japan, as C. gattii infection is rare in Japan. Only one patient with C. gattii genotype VGIIb infection has been reported in Japan, and it is believed that this patient contracted the infection in China. In the present case, our patient has never been outside Japan, indicating that the infection originated in Japan. Our findings suggest that C. gattii might be spreading in Japan. Therefore, patients with positive serum anti-GM-CSF antibodies should be thoroughly monitored for C. gattii infection, even those living in Japan.
    Language English
    Publishing date 2024-03-12
    Publishing country Netherlands
    Document type Case Reports
    ZDB-ID 1355399-9
    ISSN 1437-7780 ; 1341-321X
    ISSN (online) 1437-7780
    ISSN 1341-321X
    DOI 10.1016/j.jiac.2024.03.009
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article ; Online: Systemic cellular immunity and neuroinflammation during acute flare-up in multiple sclerosis and neuromyelitis optica spectrum disorder patients.

    Uchida, Nobuaki / Mori, Kentaro / Fujita-Nakata, Michiyo / Nakanishi, Megumi / Sanada, Mitsuru / Nagayama, Shigemi / Sugiyama, Hiroshi / Matsui, Makoto

    Journal of neuroimmunology

    2021  Volume 353, Page(s) 577500

    Abstract: Twenty-seven treatment-naïve patients with relapsing-remitting multiple sclerosis (MS) and 13 with neuromyelitis optica spectrum disorder (NMOSD) were enrolled during a time of acute flare-up. Common cerebrospinal fluid (CSF) features were increased CD29- ...

    Abstract Twenty-seven treatment-naïve patients with relapsing-remitting multiple sclerosis (MS) and 13 with neuromyelitis optica spectrum disorder (NMOSD) were enrolled during a time of acute flare-up. Common cerebrospinal fluid (CSF) features were increased CD29- and/or CD45RO-positive helper T cells capable of propagating inflammation in the central nervous system (CNS). B cell activation in the CSF was unique to MS, while an increase in CD4
    MeSH term(s) Adrenal Cortex Hormones/therapeutic use ; Adult ; Aged ; B-Lymphocytes/drug effects ; B-Lymphocytes/immunology ; Biomarkers/analysis ; Blood-Brain Barrier/drug effects ; Blood-Brain Barrier/pathology ; Female ; Humans ; Immunity, Cellular/drug effects ; Immunity, Cellular/immunology ; Immunologic Factors/therapeutic use ; Inflammation/blood ; Inflammation/cerebrospinal fluid ; Inflammation/immunology ; Lymphocyte Activation/drug effects ; Lymphocyte Activation/immunology ; Lymphocyte Subsets/drug effects ; Lymphocyte Subsets/immunology ; Male ; Middle Aged ; Multiple Sclerosis, Relapsing-Remitting/blood ; Multiple Sclerosis, Relapsing-Remitting/cerebrospinal fluid ; Multiple Sclerosis, Relapsing-Remitting/immunology ; Neuromyelitis Optica/blood ; Neuromyelitis Optica/cerebrospinal fluid ; Neuromyelitis Optica/immunology ; Symptom Flare Up ; T-Lymphocytes, Helper-Inducer/drug effects ; T-Lymphocytes, Helper-Inducer/immunology
    Chemical Substances Adrenal Cortex Hormones ; Biomarkers ; Immunologic Factors
    Language English
    Publishing date 2021-02-10
    Publishing country Netherlands
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 8335-5
    ISSN 1872-8421 ; 0165-5728
    ISSN (online) 1872-8421
    ISSN 0165-5728
    DOI 10.1016/j.jneuroim.2021.577500
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article ; Online: Rheumatoid meningitis developed in patient with stable rheumatoid arthritis and myasthenia gravis-detailed analysis of intracranial inflammation using flow cytometry.

    Oono, Miki / Fujita, Yoshimasa / Uchida, Nobuaki / Kawai, Ukichiro / Fujita-Nakata, Michiyo / Nakanishi, Megumi / Sanada, Mitsuru / Nagayama, Shigemi / Matsui, Makoto

    Journal of neuroinflammation

    2018  Volume 15, Issue 1, Page(s) 151

    Abstract: Background: Rheumatoid meningitis (RM) is a rare disorder that often develops during a remission phase of rheumatoid arthritis (RA). This is the first study to demonstrate differences in regard to immunological disturbance between blood and ... ...

    Abstract Background: Rheumatoid meningitis (RM) is a rare disorder that often develops during a remission phase of rheumatoid arthritis (RA). This is the first study to demonstrate differences in regard to immunological disturbance between blood and cerebrospinal fluid (CSF) samples obtained from a patient with RM using flow cytometry.
    Case presentation: A 36-year-old woman with RA and generalized myasthenia gravis (MG) developed RM during a remission phase. Although both RA and MG were stable and well controlled, she noticed fever, headache, and transient sensory disturbance. Blood and CSF examination findings suggested aseptic meningitis, while brain magnetic resonance imaging revealed restricted portions of meningitis and associated cortical lesions, compatible with a diagnosis of RM. The dose of oral prednisolone was increased, which ameliorated the symptoms within 1 week along with improvement in CSF findings. This patient exhibited features of RM that were manifested in a manner independent of the activity of RA. An investigation of cellular immunity using CSF specimens with flow cytometry showed differences in regard to the pathogenesis of inflammation in the CSF as compared to outside of the central nervous system. In contrast to results obtained with paired blood samples, CSF cells at the peak stage of RM showed a marked increase in CCR3
    Conclusions: RM is not a simple reflection of the immune status of RA, as the pathogenesis seems related to, at least in part, CSF-specific immunological dysregulation.
    MeSH term(s) Adult ; Anti-Inflammatory Agents/therapeutic use ; Antigens, CD/metabolism ; Arthritis, Rheumatoid/complications ; Arthritis, Rheumatoid/diagnostic imaging ; Female ; Flow Cytometry/methods ; Humans ; Inflammation/diagnostic imaging ; Inflammation/etiology ; Magnetic Resonance Imaging ; Meningitis/diagnostic imaging ; Meningitis/drug therapy ; Meningitis/etiology ; Meningitis/immunology ; Myasthenia Gravis/complications ; Myasthenia Gravis/diagnostic imaging ; Prednisolone/therapeutic use ; Treatment Outcome
    Chemical Substances Anti-Inflammatory Agents ; Antigens, CD ; Prednisolone (9PHQ9Y1OLM)
    Language English
    Publishing date 2018-05-18
    Publishing country England
    Document type Case Reports ; Journal Article
    ISSN 1742-2094
    ISSN (online) 1742-2094
    DOI 10.1186/s12974-018-1196-3
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article ; Online: Progressive multifocal leukoencephalopathy developed 26 years after renal transplantation.

    Nagayama, Shigemi / Gondo, Yuichiro / Araya, Shin-ichi / Minato, Naomi / Fujita-Nakata, Michiyo / Kaito, Muichi / Nakanishi, Megumi / Tanaka, Keiko / Yamaya, Hideki / Yokoyama, Hitoshi / Nakamichi, Kazuo / Saijo, Masayuki / Okamoto, Kouichirou / Toyoshima, Yasuko / Kakita, Akiyoshi / Matsui, Makoto

    Clinical neurology and neurosurgery

    2013  Volume 115, Issue 8, Page(s) 1482–1484

    MeSH term(s) Antibodies, Viral/analysis ; Autopsy ; Brain/pathology ; Cerebellar Diseases/etiology ; Cerebellar Diseases/physiopathology ; Fatal Outcome ; Female ; Humans ; Immunosuppressive Agents/adverse effects ; Immunosuppressive Agents/therapeutic use ; JC Virus ; Kidney Transplantation/adverse effects ; Leukoencephalopathy, Progressive Multifocal/etiology ; Leukoencephalopathy, Progressive Multifocal/pathology ; Leukoencephalopathy, Progressive Multifocal/virology ; Magnetic Resonance Imaging ; Middle Aged ; Neurologic Examination ; Polymerase Chain Reaction
    Chemical Substances Antibodies, Viral ; Immunosuppressive Agents
    Language English
    Publishing date 2013-08
    Publishing country Netherlands
    Document type Case Reports ; Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 193107-6
    ISSN 1872-6968 ; 0303-8467
    ISSN (online) 1872-6968
    ISSN 0303-8467
    DOI 10.1016/j.clineuro.2012.11.016
    Database MEDical Literature Analysis and Retrieval System OnLINE

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