Sporadic Late-onset Nemaline Myopathy Presenting with Dropped Head Syndrome and Complicated with Sjögren's Syndrome

Ayaka Seki; Tomonari Seki; Yuki Nagasako; Yusuke Sugiyama; Taro Bannai; Minako Yamada; Meiko Hashimoto Maeda; Akatsuki Kubota; Yasushi Shiio

doi:10.2169/internalmedicine.6489-25

Sporadic Late-onset Nemaline Myopathy Presenting with Dropped Head Syndrome and Complicated with Sjögren's Syndrome

Internal Medicine 2026 Open access

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Authors: Ayaka Seki, Tomonari Seki, Yuki Nagasako, Yusuke Sugiyama, Taro Bannai, Minako Yamada, Meiko Hashimoto Maeda, Akatsuki Kubota, Yasushi Shiio

DOI: 10.2169/internalmedicine.6489-25

Cited by: 0

References: 18

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.

Fields of Study & Subjects

Inflammatory Myopathies and Dermatomyositis Peripheral Neuropathies and Disorders Neurogenetic and Muscular Disorders Research

Publication details

Journal / source: Internal Medicine
Volume: 65
Issue: 16
Pages: 2246–2249
Publication date: 14 January 2026
Language: EN

References 18