Amyloid Neuropathies (Amyloid Neuropathy)

Disorders of the peripheral nervous system associated with the deposition of AMYLOID in nerve tissue. Familial, primary (nonfamilial), and secondary forms have been described. Some familial subtypes demonstrate an autosomal dominant pattern of inheritance. Clinical manifestations include sensory loss, mild weakness, autonomic dysfunction, and CARPAL TUNNEL SYNDROME. (Adams et al., Principles of Neurology, 6th ed, p1349)

Also Known As:

Networked: 170 relevant articles (4 outcomes, 14 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Immunoglobulin Light-chain Amyloidosis
2.	Hyperuricemia
3.	Gout
4.	Amyloidosis
5.	Cardiomyopathies (Cardiomyopathy)

Experts

1.	Ando, Yukio: 9 articles (06/2021 - 04/2002)
2.	Amass, Leslie: 8 articles (01/2021 - 09/2016)
3.	Stewart, Michelle: 6 articles (10/2021 - 03/2017)
4.	Conceição, Isabel: 6 articles (06/2020 - 02/2005)
5.	Coelho, Teresa: 5 articles (06/2021 - 02/2005)
6.	Adams, David: 5 articles (10/2018 - 10/2012)
7.	Lozeron, Pierre: 5 articles (10/2018 - 10/2012)
8.	Benson, Merrill D: 4 articles (06/2021 - 02/2002)
9.	Dyck, Peter J: 4 articles (06/2021 - 02/2002)
10.	Botteman, Marc F: 4 articles (06/2020 - 03/2017)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Amyloid Neuropathies:

1.	Prealbumin (Transthyretin)IBA 10/01/2014 - "Recent therapeutic advances showed efficacy of a molecule stabilizing transthyretin in early forms of amyloid neuropathy due to transthyretin mutations." 01/01/2022 - "The present study was intended to analyze the characteristics of myelinated nerve fibers density (MFD) of transthyretin amyloid polyneuropathy (ATTR-PN) and other similar neuropathies. " 09/01/2009 - "This study assesses the value of standard quantitative autonomic (QAT) and sensation (QST) tests in detecting, characterizing, and quantitating the severity of transthyretin amyloid polyneuropathy (TTR-A-PN). " 11/01/2003 - "Genetic study of transthyretin amyloid neuropathies: carrier risks among French and Portuguese families." 12/01/2002 - "Finally, structural studies and mutation analysis of transthyretin in patients with amyloid neuropathy have opened a new area of research."
2.	tafamidisIBA 09/13/2017 - "In a subset of TTR amyloid polyneuropathy patients, the probes also detected a circulating TTR fragment that disappeared after tafamidis treatment. " 09/13/2017 - "Misfolded oligomers decrease in TTR amyloid polyneuropathy patients treated with disease-modifying therapies (tafamidis or liver transplant-mediated gene therapy). " 09/01/2017 - "The objective of the present study was to evaluate the long-term safety and efficacy of tafamidis in treating hereditary transthyretin amyloid polyneuropathy. " 09/01/2017 - "Long-term safety and efficacy of tafamidis for the treatment of hereditary transthyretin amyloid polyneuropathy: results up to 6 years." 01/01/2023 - "Updated Evaluation of the Safety, Efficacy and Tolerability of Tafamidis in the Treatment of Hereditary Transthyretin Amyloid Polyneuropathy."
3.	Xanthine OxidaseIBA 08/29/2011 - "This newly developed methodology has been successfully applied to the syntheses of febuxostat (a xanthine oxidase inhibitor that is effective for the treatment of gout and hyperuricemia), tafamidis (effective for the treatment of TTR amyloid polyneuropathy), and texaline (a natural product having antitubercular activity)."
4.	FebuxostatFDA Link 08/29/2011 - "This newly developed methodology has been successfully applied to the syntheses of febuxostat (a xanthine oxidase inhibitor that is effective for the treatment of gout and hyperuricemia), tafamidis (effective for the treatment of TTR amyloid polyneuropathy), and texaline (a natural product having antitubercular activity)."
5.	texalineIBA 08/29/2011 - "This newly developed methodology has been successfully applied to the syntheses of febuxostat (a xanthine oxidase inhibitor that is effective for the treatment of gout and hyperuricemia), tafamidis (effective for the treatment of TTR amyloid polyneuropathy), and texaline (a natural product having antitubercular activity)."
6.	Proteins (Proteins, Gene)FDA Link 03/01/2009 - "[Amyloid neuropathy resulting from an unknown protein]." 03/01/1998 - "Twenty-six patients with sural nerve biopsy-proven amyloid neuropathy and a documented monoclonal protein in the serum or urine were studied. " 10/01/2001 - "The responsibility of the monoclonal protein in the occurrence of amyloid neuropathy related to multiple myeloma is also recognized. " 08/01/2001 - "Acquired amyloid neuropathy concerns only immunoglobulin light chain amyloidosis (AL) and are frequently associated with renal manifestations and monoclonal protein in serum or urine. " 09/01/2020 - "The differential diagnosis for CIDP-like neuropathies includes amyloid neuropathy and POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) and amyloidosis. "
7.	DNA (Deoxyribonucleic Acid)IBA 01/01/1992 - "This study suggests that investigations in amyloid polyneuropathy with no overt family history should include systematic DNA analysis." 08/10/1992 - "In one patient with amyloid polyneuropathy, the exon 3 DNA showed a unique, aberrant migration pattern. " 01/01/1988 - "Results of DNA analysis in 8 patients with type I amyloid neuropathy (ANI) are reported. " 07/01/2001 - "In the present study, we investigated molecular abnormalities of transthyretin in elderly patients with cardiac amyloidosis or amyloid polyneuropathy, using DNA sequencing and protein sequencing. "
8.	Amyloid (Amyloid Fibrils)IBA 01/01/2017 - "C1q ablation exacerbates amyloid deposition: A study in a transgenic mouse model of ATTRV30M amyloid neuropathy." 06/01/2012 - "Six of 21 DLT recipients who survived for more than 3 years developed amyloid depositions within organs, and de novo amyloid neuropathy was reported in three patients. " 01/01/1999 - "Familial amyloid and light chain amyloid polyneuropathies were subsequently confirmed by relevant tests. " 01/01/1996 - "Ultrastructural immunolabelling of amyloid fibrils in acquired and hereditary amyloid neuropathies." 08/01/1984 - "We investigated two patients with Portuguese amyloid polyneuropathy to learn more about the role played by amyloid in this condition. "
9.	Diflunisal (Dolobid)FDA Link 01/01/2022 - "Conclusion: Our study suggests that most domino liver transplant recipients with acquired amyloid neuropathy will develop neurological deterioration by 12 months of treatment with diflunisal. " 01/01/2022 - "Methods: We performed a retrospective longitudinal study of prospectively collected data for all domino liver transplant recipients with acquired amyloid neuropathy who received diflunisal at our hospital. " 01/01/2022 - "Objectives: To analyze the efficacy and tolerability of diflunisal for the treatment of acquired amyloid neuropathy in domino liver transplant recipients. " 01/01/2022 - "Treatment With Diflunisal in Domino Liver Transplant Recipients With Acquired Amyloid Neuropathy." 04/01/2016 - "Amyloid Neuropathy Following Domino Liver Transplantation: Response to Diflunisal."
10.	Antisense OligonucleotidesIBA 06/01/2021 - "Design and Rationale of the Global Phase 3 NEURO-TTRansform Study of Antisense Oligonucleotide AKCEA-TTR-LRx (ION-682884-CS3) in Hereditary Transthyretin-Mediated Amyloid Polyneuropathy."

Therapies and Procedures

1.	Liver Transplantation 01/01/2018 - "Amyloid polyneuropathy in 2 patients after liver transplantation." 01/28/2017 - "Iatrogenic amyloid polyneuropathy after domino liver transplantation." 06/01/2012 - "The detail mechanisms of the onset are not well understood in FAP and domino liver transplantation-induced amyloid neuropathy. " 06/01/2012 - "Amyloid polyneuropathy following domino liver transplantation." 11/01/2010 - "Acquired amyloid neuropathy in a Portuguese patient after domino liver transplantation."
2.	Stem Cell Transplantation 03/01/2010 - "Treatment remains a challenge, although chemotherapy and autologous stem cell transplantation offer hope for those with primary systemic amyloidosis, whereas liver transplantation is effective for some forms of hereditary amyloid neuropathy. " 01/01/2010 - "Primary AL amyloid polyneuropathy successfully treated with high-dose melphalan followed by autologous stem cell transplantation."
3.	Drug Therapy (Chemotherapy) 03/01/2010 - "Treatment remains a challenge, although chemotherapy and autologous stem cell transplantation offer hope for those with primary systemic amyloidosis, whereas liver transplantation is effective for some forms of hereditary amyloid neuropathy. " 08/01/2001 - "Specific treatment of amyloid polyneuropathy varies with the variety of amyloidosis including liver transplantation in TTR-FAP, at the onset of the disease or chemotherapy for immunoglobulin light chain amyloidosis."
4.	Therapeutics 10/01/2012 - "The main recent articles on amyloid neuropathy cover its description, methods for diagnosis and therapies. " 01/01/2010 - "In addition to amyloid deposits in tissues, an amyloidogenic light chain itself produced by abnormal plasma cells might be harmful to peripheral nerve function, and thus HDM/SCT seems to be a promising therapy for primary AL amyloid polyneuropathy." 01/01/2022 - "As neurological manifestations of amyloidosis may precede involvement of other organs by several years, recognizing amyloid neuropathy and myopathy are crucial, especially in this new and exciting era of effective therapies for AL and ATTR neuropathy. " 03/01/2017 - "Antibody therapy for transthyretin-related hereditary amyloid polyneuropathy: another therapeutic option." 09/13/2017 - "Misfolded oligomers decrease in TTR amyloid polyneuropathy patients treated with disease-modifying therapies (tafamidis or liver transplant-mediated gene therapy). "
5.	Denervation 04/05/2003 - "Cardiac denervation in amyloid polyneuropathy." 06/06/2017 - "Early skin denervation in hereditary and iatrogenic transthyretin amyloid neuropathy." 08/10/2010 - "Clinical presentations and skin denervation in amyloid neuropathy due to transthyretin Ala97Ser."