AL Amyloidosis

AL amyloidosis is a systemic disease caused by misfolded immunoglobulin light chains deposited as amyloid fibrils in multiple organs. Peripheral neuropathy, which often presents with sensory dissociation and autonomic dysfunction, is a key initial manifestation that can occasionally be misdiagnosed...

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Published inBrain and nerve = Shinkei kenkyū no shinpo Vol. 77; no. 8; p. 847
Main Authors Ide, Toshihiro, Koike, Haruki
Format Journal Article
LanguageJapanese
Published Japan 01.08.2025
Subjects
Humans
Immunoglobulin Light-chain Amyloidosis - diagnosis
Immunoglobulin Light-chain Amyloidosis - therapy
Peripheral Nervous System Diseases - diagnosis
Peripheral Nervous System Diseases - etiology
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Summary:AL amyloidosis is a systemic disease caused by misfolded immunoglobulin light chains deposited as amyloid fibrils in multiple organs. Peripheral neuropathy, which often presents with sensory dissociation and autonomic dysfunction, is a key initial manifestation that can occasionally be misdiagnosed as a chronic inflammatory demyelinating polyneuropathy. This review outlines clinical features and diagnostic strategies, including electrophysiological studies, nerve biopsy findings, and recent advances in treatment. Particular focus was given to the pathological mechanisms underlying neuropathy in AL amyloidosis, including axonal degeneration and myelin-Schwann cell interface abnormalities. We emphasized the importance of early diagnosis and multidisciplinary management from a neurological perspective.
ISSN:1881-6096
DOI:10.11477/mf.188160960770080847