[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 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.

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Publication Details

Journal
PubMed
Published
2025-08-01
DOI
https://doi.org/10.11477/mf.188160960770080847
Primary Topic
Amyloidosis: Diagnosis, Treatment, Outcomes
Type
article
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article

[AL Amyloidosis].

Toshihiro Ide, Haruki Koike
PubMed
Amyloidosis: Diagnosis, Treatment, Outcomes
article

[AL Amyloidosis].

Toshihiro Ide, Haruki Koike
article en

Abstract

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.

PubMedVol. 77(8)
Saga University (JP)
Openalex Percentile: Top 81%
Amyloidosis: Diagnosis, Treatment, Outcomes
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[AL Amyloidosis]. — Toshihiro Ide, Haruki Koike · PubMed (2025) | TGRS Research Map | TGRS