Localized Immunoglobulin Light-Chain Amyloidosis of the Ulnar Nerve

Shinsuke Morisaki; Shinji Tsuchida; Eiichi Konishi; Nagaaki Katoh; Yusuke Takahashi; Kenji Takahashi

doi:10.1159/000515475

Localized Immunoglobulin Light-Chain Amyloidosis of the Ulnar Nerve

Case Rep Neurol. 2021 May 27;13(2):305-311. doi: 10.1159/000515475. eCollection 2021 May-Aug.

Authors

Shinsuke Morisaki¹, Shinji Tsuchida², Eiichi Konishi³, Nagaaki Katoh⁴, Yusuke Takahashi⁴, Kenji Takahashi²

Affiliations

¹ Department of Orthopaedics, Saiseikai Shiga Hospital, Ritto, Japan.
² Department of Orthopaedics, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kamigyo-ku, Japan.
³ Department of Surgical Pathology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kamigyo-ku, Japan.
⁴ Department of Medicine (Neurology and Rheumatology), Shinshu University, Matsumoto, Japan.

Abstract

Amyloidosis is a disorder caused by extracellular tissue deposition of insoluble fibrils. Amyloidosis can be divided into systemic or localized disease. Primary systemic amyloidosis is a multisystem disease caused by the deposition of amyloid in various tissues. Localized amyloidosis has different characteristics than those of systemic amyloidosis. In this paper, we present the case of a middle-aged woman who presented with worsening ulnar nerve palsy. Electrophysiological examination and MRI indicated a tumor surrounding the ulnar nerve in the forearm. However, the operative findings revealed that ulnar nerve fascicles were replaced with a yellow tissue, which was diagnosed as amyloid light-chain λ amyloidosis, based on histopathological examination. Systemic amyloidosis was ruled out after the screening examinations. This paper is the first report of the ulnar nerve as the sole site of localized immunoglobulin light-chain amyloidosis manifestation.

Keywords: Amyloid; Neuropathy; Ulnar nerve.

Publication types

Case Reports