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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
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Zs.A 240: Show issues Location:
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