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Sporadic Late-onset Nemaline Myopathy Presenting with Dropped Head Syndrome and Complicated with Sjögren's Syndrome
Ayaka Seki1, Tomonari Seki1, Yuki Nagasako1
1Department of Neurology, Tokyo Teishin Hospital, Japan.
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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