Peer-reviewedCC BY-NC-NDInternal Medicine
Sporadic Late-onset Nemaline Myopathy Presenting with Dropped Head Syndrome and Complicated with Sjögren's Syndrome
Abstract
Sporadic late-onset nemaline myopathy (SLONM) is a rare neuromuscular disorder often associated with monoclonal gammopathy of undetermined significance (MGUS). However, reports of SLONM without MGUS are limited, particularly in cases complicated by Sjögren's syndrome. We herein report the case of a 62-year-old woman with SLONM without MGUS, associated with Sjögren's syndrome. The patient presented with dropped head syndrome. A muscle biopsy confirmed SLONM, and Sjögren's syndrome was diagnosed. Treatment with intravenous immunoglobulin and corticosteroids improved her muscle strength. This case highlights the importance of considering SLONM in the differential diagnosis when dropped head is the predominant clinical presentation.
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BibTeX
@article{seki2026sporadic,
title = {{Sporadic Late-onset Nemaline Myopathy Presenting with Dropped Head Syndrome and Complicated with Sjögren's Syndrome}},
author = {Ayaka Seki and Tomonari Seki and Yuki Nagasako and Yusuke Sugiyama and Taro Bannai and Minako Yamada and Meiko Maeda and Akatsuki Kubota and Yasushi Shiio},
journal = {Internal Medicine},
year = {2026},
month = aug,
doi = {10.2169/internalmedicine.6489-25},
url = {https://www.jstage.jst.go.jp/article/internalmedicine/65/16/65_6489-25/_article},
note = {CC BY-NC-ND},
}
RIS
TY - JOUR
TI - Sporadic Late-onset Nemaline Myopathy Presenting with Dropped Head Syndrome and Complicated with Sjögren's Syndrome
AU - Ayaka Seki
AU - Tomonari Seki
AU - Yuki Nagasako
AU - Yusuke Sugiyama
AU - Taro Bannai
AU - Minako Yamada
AU - Meiko Maeda
AU - Akatsuki Kubota
AU - Yasushi Shiio
PY - 2026
DA - 2026/08/15
JO - Internal Medicine
DO - 10.2169/internalmedicine.6489-25
UR - https://www.jstage.jst.go.jp/article/internalmedicine/65/16/65_6489-25/_article
AB - Sporadic late-onset nemaline myopathy (SLONM) is a rare neuromuscular disorder often associated with monoclonal gammopathy of undetermined significance (MGUS). However, reports of SLONM without MGUS are limited, particularly in cases complicated by Sjögren's syndrome. We herein report the case of a 62-year-old woman with SLONM without MGUS, associated with Sjögren's syndrome. The patient presented with dropped head syndrome. A muscle biopsy confirmed SLONM, and Sjögren's syndrome was diagnosed. Treatment with intravenous immunoglobulin and corticosteroids improved her muscle strength. This case highlights the importance of considering SLONM in the differential diagnosis when dropped head is the predominant clinical presentation.
SN - 1349-7235
SN - 0918-2918
ER -