Prevalence and predictors of neurological manifestations in systemic AL amyloidosis

Thimm, Andreas; Carpinteiro, Alexander; Oubari, Sara; Παπαθανασίου, Μαρία; Luedike, Peter; Kessler, Lukas; Rischpler, Christoph; Blau, Tobias; Reinhardt, Hans Christian; Rassaf, Tienush; Schmidt, Hartmut; Kleinschnitz, Christoph; Hagenacker, Tim

doi:10.1016/j.jns.2022.120341

Cited by 7 publications

(6 citation statements)

References 33 publications

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“…Nerve conduction studies are generally consistent with a length-dependent sensorimotor axonal neuropathy, but coexisting demyelinating findings (prolonged distal motor latencies, severely reduced nerve conduction velocities, abnormal temporal dispersion, prolonged F-wave latencies) are common. Rarely, definitive demyelinating features are present, fulfilling electrophysiologic criteria for CIDP 62,66 . Large fiber neuropathy becomes more overt as the disease progresses.…”

Section: Amyloid Light Chain Neuropathymentioning

confidence: 96%

“…Rarely, definitive demyelinating features are present, fulfilling electrophysiologic criteria for CIDP. 62,66 Large fiber neuropathy becomes more overt as the disease progresses.…”

Section: Key Pointsmentioning

confidence: 99%

“…Peripheral neuropathy occurs in 17% to 36% of patients with AL amyloidosis 61,62 . It usually presents with a length-dependent sensory predominant polyneuropathy with a predilection for small fibers at the initial stage 51 .…”

Section: Amyloid Light Chain Neuropathymentioning

confidence: 99%

“…Carpal tunnel syndrome can occur in up to 21% of patients with AL amyloidosis 61 . Involvement of the cranial nerves, lumbosacral roots, and lumbosacral plexus may occur 62 …”

Section: Amyloid Light Chain Neuropathymentioning

confidence: 99%

“…61 Peripheral neuropathy occurs in 17% to 36% of patients with AL amyloidosis. 61,62 It usually presents with a length-dependent sensory predominant polyneuropathy with a predilection for small fibers at the initial stage. 51 Autonomic symptoms, including orthostatic hypotension, sweating abnormalities, postprandial fullness, diarrhea, constipation, erectile dysfunction, or urinary retention, are common.…”

Section: Key Pointsmentioning

confidence: 99%

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Paraproteinemic Neuropathies

Beydoun,

Darki

2023

CONTINUUM: Lifelong Learning in Neurology

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OBJECTIVE Coexistence of polyneuropathy and gammopathy is a common but potentially challenging situation in clinical practice. This article reviews the clinical, electrophysiologic, and hematologic phenotypes of the paraproteinemic neuropathies and the diagnostic and treatment strategies for each. LATEST DEVELOPMENTS Advances in our understanding of the underlying pathophysiology of various paraproteinemic neuropathies and their corresponding phenotypes have identified potential new therapeutic targets. Therapeutic strategies to diminish anti–myelin-associated glycoprotein (MAG) IgM antibodies have shown partial and inconsistent efficacy; however, antigen-specific immune therapy is being investigated as a novel treatment to remove the presumably pathogenic anti-MAG antibody. Advances in genetic and cell signaling studies have resulted in the approval of Bruton tyrosine kinase inhibitors for Waldenström macroglobulinemia. Monoclonal antibodies are being investigated for the treatment of light chain amyloidosis. ESSENTIAL POINTS Early recognition and treatment of underlying plasma cell disorders improves clinical outcomes in patients with paraproteinemic neuropathy. Despite significant progress, our knowledge regarding underlying mechanisms for paraproteinemic neuropathy is still limited. Clinicians’ awareness of clinical phenotypes, electrophysiologic hallmarks, and hematologic findings of the different paraproteinemic neuropathies is crucial to promptly identify and treat patients and to avert misdiagnosis. Multidisciplinary collaboration among specialists, including neurologists and hematologists, is paramount for the optimal treatment of these patients with overlapping conditions.

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Section: Amyloid Light Chain Neuropathymentioning

confidence: 96%

“…Rarely, definitive demyelinating features are present, fulfilling electrophysiologic criteria for CIDP. 62,66 Large fiber neuropathy becomes more overt as the disease progresses.…”

Section: Key Pointsmentioning

confidence: 99%

Section: Amyloid Light Chain Neuropathymentioning

confidence: 99%

“…Carpal tunnel syndrome can occur in up to 21% of patients with AL amyloidosis 61 . Involvement of the cranial nerves, lumbosacral roots, and lumbosacral plexus may occur 62 …”

Section: Amyloid Light Chain Neuropathymentioning

confidence: 99%

Section: Key Pointsmentioning

confidence: 99%

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Paraproteinemic Neuropathies

Beydoun,

Darki

2023

CONTINUUM: Lifelong Learning in Neurology

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Immunoglobulin light chain amyloidosis: 2024 update on diagnosis, prognosis, and treatment

Gertz

2023

American J Hematol

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Disease OverviewImmunoglobulin light chain amyloidosis is a clonal, nonproliferative plasma cell disorder in which fragments of immunoglobulin light or heavy chain are deposited in tissues. Clinical features depend on organs involved but can include heart failure with preserved ejection fraction, nephrotic syndrome, hepatic dysfunction, peripheral/autonomic neuropathy, and “atypical smoldering multiple myeloma or MGUS.”DiagnosisTissue biopsy stained with Congo red demonstrating amyloid deposits with apple‐green birefringence is required for the diagnosis of AL amyloidosis. Organ biopsy is not required in 85% of patients. Verification that amyloid is composed of immunoglobulin light chains is mandatory. The gold standard is laser capture mass spectroscopy.PrognosisN‐terminal pro–brain natriuretic peptide (NT‐proBNP or BNP), serum troponin T(or I), and difference between involved and uninvolved immunoglobulin free light chain values are used to classify patients into four stages; 5‐year survivals are 82%, 62%, 34%, and 20%, respectively.TherapyAll patients with a systemic amyloid syndrome require therapy to prevent deposition of amyloid in other organs and prevent progressive organ failure. Current first‐line therapy with the best outcome is daratumumab, bortezomib, cyclophosphamide, and dexamethasone. The goal of therapy is a ≥VGPR. In patients failing to achieve this depth of response options for consolidation include pomalidomide, stem cell transplantation, venetoclax, and bendamustine.Future ChallengesDelayed diagnosis remains a major obstacle to initiating effective therapy prior to the development of end‐stage organ failure. Trials of antibodies to deplete deposited fibrils are underway.

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Primary systemic amyloidosis: A brief overview

Hughes,

Lentzsch

2025

La Presse Médicale

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Prevalence and predictors of neurological manifestations in systemic AL amyloidosis

Cited by 7 publications

References 33 publications

Paraproteinemic Neuropathies

Paraproteinemic Neuropathies

Immunoglobulin light chain amyloidosis: 2024 update on diagnosis, prognosis, and treatment

Primary systemic amyloidosis: A brief overview

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