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Case Reports
Sporadic Late-onset Nemaline Myopathy Associated with Sjögren's Syndrome: A Case Report.
- Tsuyoshi Hamaguchi, Ichizo Nishino, Yasuki Hirano, Nobuaki Uchida, Michiyo Fujita-Nakata, Megumi Nakanishi, Tomoyuki Sakai, and Masato Asahina.
- Department of Neurology, Kanazawa Medical University, Japan.
- Intern. Med. 2024 Oct 1; 63 (19): 268326872683-2687.
AbstractWe 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.
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