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Article ; Online: Vutrisiran: a new drug in the treatment landscape of hereditary transthyretin amyloid polyneuropathy.

Planté-Bordeneuve, Violaine / Perrain, Valentine

2024 Volume 19, Issue 4, Page(s) 393–402

Abstract: Introduction: Hereditary transthyretin (ATTRv) amyloidosis is a progressive, fatal disorder caused by mutations in the transthyretin (TTR) gene leading to deposition of the misfolded protein in amyloid fibrils. The main phenotypes are peripheral ... ...

Abstract	Introduction: Hereditary transthyretin (ATTRv) amyloidosis is a progressive, fatal disorder caused by mutations in the transthyretin (TTR) gene leading to deposition of the misfolded protein in amyloid fibrils. The main phenotypes are peripheral neuropathy (PN) and cardiomyopathy (CM). Areas covered: Gene silencing therapy, by dramatically reducing liver production of TTR, has transformed ATTRv-PN patient care in the last decade. In this drug discovery case history, the authors discuss the treatment history of ATTRv-PN and focus on the latest siRNA therapy: vutrisiran. Vutrisiran is chemically enhanced and N-acetylgalactosamin-conjugated, allowing increased stability and specific liver delivery. HELIOS-A, a phase III, multicenter randomized study, tested vutrisiran in ATTRv-PN and showed significant improvement in neuropathy impairment, disability, quality of life (QoL), gait speed, and nutritional status. Tolerance was acceptable, no safety signals were raised. Expert opinion: Vutrisiran offers a new treatment option for patients with ATTRv-PN. Vutrisian's easier delivery and administration route, at a quarterly frequency, as well as the absence of premedication, are major improvements to reduce patients' disease burden and improve their QoL. Its place in the therapeutic strategy is to be determined, considering affordability.
MeSH term(s)	Humans ; Quality of Life ; Prealbumin/genetics ; Amyloid Neuropathies ; Drug Discovery ; Gene Silencing
Chemical Substances	Prealbumin
Language	English
Publishing date	2024-01-27
Publishing country	England
Document type	Randomized Controlled Trial ; Multicenter Study ; Journal Article
ZDB-ID	2259618-5
ISSN	1746-045X ; 1746-0441
ISSN (online)	1746-045X
ISSN	1746-0441
DOI	10.1080/17460441.2024.2306843
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Article ; Online: Study of the parent-of-origin effect in monogenic diseases with variable age of onset. Application on ATTRv.

Alarcon, Flora / Planté-Bordeneuve, Violaine / Nuel, Grégory

PloS one

2023 Volume 18, Issue 8, Page(s) e0288958

Abstract: In genetic diseases with variable age of onset, an accurate estimation of the survival function for the mutation carriers and also modifying factors effects estimations are important for the management of asymptomatic gene carriers across life. Among the ...

Abstract	In genetic diseases with variable age of onset, an accurate estimation of the survival function for the mutation carriers and also modifying factors effects estimations are important for the management of asymptomatic gene carriers across life. Among the modifying factors, the gender of the parent transmitting the mutation (i.e. the parent-of-origin effect) has been shown to have a significant effect on survival curve estimation on transthyretin familial amyloid polyneuropathy (ATTRv) families. However, as most genotypes are unknown, the parent-of-origin must be calculated through a probability estimated from the pedigree. We propose in this article to extend the method providing mutation carrier survival estimates in order to estimate the parent-of-origin effect. The method is both validated on simulated data and applied to familly samples with ATTRv.
MeSH term(s)	Humans ; Age of Onset ; Amyloid Neuropathies, Familial/genetics ; Genotype ; Heterozygote ; Prealbumin/genetics ; Mutation
Chemical Substances	Prealbumin
Language	English
Publishing date	2023-08-10
Publishing country	United States
Document type	Journal Article
ZDB-ID	2267670-3
ISSN	1932-6203 ; 1932-6203
ISSN (online)	1932-6203
ISSN	1932-6203
DOI	10.1371/journal.pone.0288958
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Article ; Online: Transthyretin familial amyloid polyneuropathy: an update.

Plante-Bordeneuve, Violaine

Journal of neurology

2017 Volume 265, Issue 4, Page(s) 976–983

Abstract: Transthyretin familial amyloid polyneuropathy (TTR-FAP) is a progressive, fatal, inherited disorder first identified in Portugal and now recognized in all continents. Over the past decade, thanks to the availability of the genetic test, our knowledge on ... ...

Abstract	Transthyretin familial amyloid polyneuropathy (TTR-FAP) is a progressive, fatal, inherited disorder first identified in Portugal and now recognized in all continents. Over the past decade, thanks to the availability of the genetic test, our knowledge on the range of clinical expressions of this disorder has expanded, including different patterns and progression rates of the neuropathy, as well as aspects of the cardiomyopathy, which can be prominent. In the mean time, new tools are being developed to detect earlier TTR amyloid deposition such as cardiac scintigraphy with technetium-labelled pyrophosphate tracers or small nerve fiber alterations from skin biopsies, or using neurophysiological approaches as well as magnetic resonance neurography (MRN). Such refinements, along with an increased awareness of the disease, should reduce the diagnostic delay and facilitate early treatment. In this regard, thanks to a better understanding of the TTR amyloid formation, major advances have been made, allowing for therapeutic developments which are less invasive than liver transplantation (LT). TTR stabilizer drugs are safe and seem to delay the disease progression in some groups of patients. Indeed, positive results have just been released from 2 phase III trials on TTR gene modifiers, namely silencing RNA and antisense oligonucleotide therapies. These recent advances open a new area in the field with the hope that we can safely bring about long-term stabilization of the disease. Furthermore, immunotherapies targeting the amyloid deposits are being explored.
MeSH term(s)	Amyloid Neuropathies, Familial/diagnostic imaging ; Amyloid Neuropathies, Familial/epidemiology ; Amyloid Neuropathies, Familial/genetics ; Amyloid Neuropathies, Familial/therapy ; Humans ; Liver Transplantation ; Magnetic Resonance Imaging ; Mutation/genetics ; Prealbumin/genetics
Chemical Substances	Prealbumin
Language	English
Publishing date	2017-12-16
Publishing country	Germany
Document type	Journal Article ; Review
ZDB-ID	187050-6
ISSN	1432-1459 ; 0340-5354 ; 0012-1037 ; 0939-1517 ; 1619-800X
ISSN (online)	1432-1459
ISSN	0340-5354 ; 0012-1037 ; 0939-1517 ; 1619-800X
DOI	10.1007/s00415-017-8708-4
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Article ; Online: Strategies to improve the quality of life in patients with hereditary transthyretin amyloidosis (hATTR) and autonomic neuropathy.

Gendre, Thierry / Planté-Bordeneuve, Violaine

Clinical autonomic research : official journal of the Clinical Autonomic Research Society

2019 Volume 29, Issue Suppl 1, Page(s) 25–31

Abstract: Purpose: Hereditary transthyretin amyloidosis (hATTR) is a severe adult-onset progressive disease mainly involving the peripheral nervous system and the heart, with a prominent impact on the autonomic nervous system. This review summarizes the clinical ... ...

Abstract	Purpose: Hereditary transthyretin amyloidosis (hATTR) is a severe adult-onset progressive disease mainly involving the peripheral nervous system and the heart, with a prominent impact on the autonomic nervous system. This review summarizes the clinical aspects of autonomic dysfunction in hATTR, and their impact on quality of life as well as potential therapeutic options. Methods: Literature review. Results: Autonomic dysfunction, causing neurogenic orthostatic hypotension, gastroparesis, constipation, diarrhea, bladder dysfunction, and erectile dysfunction in males, has a major impact on the quality of life of patients with hATTR. Improvement of qualify of life in patients with hATTR implies periodic symptomatic screening and early management, taking into consideration comorbidities and medication side effects. The specific effect of the disease-modifying treatment on this aspect remains to be unraveled. Conclusions: Management of autonomic dysfunction in patients with hAATR is feasible and can result in improved qualify of life. Novel disease-modifying treatments for hAATR may contribute to improve autonomic dysfunction, although specific studies are required.
MeSH term(s)	Amyloid Neuropathies, Familial/complications ; Autonomic Nervous System Diseases/etiology ; Humans ; Polyneuropathies ; Quality of Life
Language	English
Publishing date	2019-09-10
Publishing country	Germany
Document type	Journal Article ; Review
ZDB-ID	1080007-4
ISSN	1619-1560 ; 0959-9851
ISSN (online)	1619-1560
ISSN	0959-9851
DOI	10.1007/s10286-019-00624-w
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Article ; Online: 3-T MR neurography of lumbo-sacral plexus in hereditary transthyretin-related amyloidosis with polyneuropathy.

Hodel, Jérôme / Benadjaoud, Samir / Benadjaoud, Mohamed Amine / Lefaucheur, Jean-Pascal / Planté-Bordeneuve, Violaine

European radiology

2022 Volume 32, Issue 11, Page(s) 7865–7871

Abstract: Objectives: Our aim was to evaluate the ability of magnetic resonance neurography (MRN) of the lumbo-sacral plexus (LSP) to distinguish patients with hereditary transthyretin-related amyloidosis with polyneuropathy (ATTRv-PN) from asymptomatic variant ... ...

Abstract	Objectives: Our aim was to evaluate the ability of magnetic resonance neurography (MRN) of the lumbo-sacral plexus (LSP) to distinguish patients with hereditary transthyretin-related amyloidosis with polyneuropathy (ATTRv-PN) from asymptomatic variant carriers (AVC) and healthy controls and to assess its prognostic value. Methods: Three-Tesla MRN was performed in 25 consecutive ATTRv-PN patients, 18 AVC, and 10 controls including T2-w DIXON and DWI MR sequences. Two blinded readers independently assessed LSP root diameter and intraneural signal on the MRN images of each subject. MRN findings were compared between groups and correlated with clinical impairment scored on the Neuropathy Impairment Score (NIS) and the modified Polyneuropathy Disability score (mPND). Results: The agreement between readers on MRN images was excellent (Cohen's kappa = 0.82). LSP root enlargement was significantly more frequent in ATTRv-PN patients compared to AVC (ratio = 4.38, p = 0.038). Increased LSP root intraneural signal on T2-w images was significantly more frequent in ATTRv-PN patients compared to AVC (ratio = 3.4, p = 0.016). In contrast, there were no MRN abnormalities in controls. In ATTRv-PN patients, LSP root enlargement was associated with higher mPND scores (p = 0.03) and increased intraneural signal on T2-w images was associated with significantly higher NIS and mPND scores (p = 0.004 and 0.02, respectively). Conclusions: MRN of the LSP can help differentiate ATTRv-PN patients from AVC. LSP root enlargement and increased intraneural signal are significantly associated with clinical impairment, suggesting potential implications for patient care. Key points: • ATTRv-PN patients showed abnormal LSP changes on MRN. • MRN of the LSP can help to differentiate ATTRv-PN patients from AVC and healthy controls. • LSP root enlargement and increased intraneural signal were significantly associated with clinical impairment in ATTRv-PN patients.
MeSH term(s)	Humans ; Prealbumin ; Amyloid Neuropathies, Familial/diagnostic imaging ; Magnetic Resonance Imaging/methods ; Lumbosacral Plexus/diagnostic imaging ; Polyneuropathies/diagnostic imaging ; Hypertrophy
Chemical Substances	Prealbumin
Language	English
Publishing date	2022-04-19
Publishing country	Germany
Document type	Journal Article
ZDB-ID	1085366-2
ISSN	1432-1084 ; 0938-7994 ; 1613-3749
ISSN (online)	1432-1084
ISSN	0938-7994 ; 1613-3749
DOI	10.1007/s00330-022-08748-w
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Article ; Online: Update in the diagnosis and management of transthyretin familial amyloid polyneuropathy.

Plante-Bordeneuve, Violaine

Journal of neurology

2014 Volume 261, Issue 6, Page(s) 1227–1233

Abstract: Transthyretin familial amyloid polyneuropathy (TTR-FAP) is a progressive neurodegenerative and systemic genetic disease first identified in Portugal, now reported worldwide. During the past few years our knowledge on the phenotypic presentation of this ... ...

Abstract	Transthyretin familial amyloid polyneuropathy (TTR-FAP) is a progressive neurodegenerative and systemic genetic disease first identified in Portugal, now reported worldwide. During the past few years our knowledge on the phenotypic presentation of this devastating condition has remarkably expanded including a wide variation in age of onset, different neuropathic patterns and patients presenting with isolated or predominant cardiac involvement. Liver transplantation, the first therapeutic approach, although invasive, has been shown to halt the progression of the neuropathy in young onset patients. Fortunately, several disease-modifying treatments are now available or in clinical development including TTR stabilizers and gene therapy. Their efficacy is higher if administered at the earliest disease stage. Thus, management of TTR-FAP patients is a moving field with need for early diagnosis using new diagnostic tools and new therapeutic options.
MeSH term(s)	Amyloid Neuropathies, Familial/diagnosis ; Amyloid Neuropathies, Familial/therapy ; Disease Management ; Female ; Humans ; Male ; Portugal
Language	English
Publishing date	2014-06-03
Publishing country	Germany
Document type	Journal Article ; Research Support, Non-U.S. Gov't ; Review
ZDB-ID	187050-6
ISSN	1432-1459 ; 0340-5354 ; 0012-1037 ; 0939-1517 ; 1619-800X
ISSN (online)	1432-1459
ISSN	0340-5354 ; 0012-1037 ; 0939-1517 ; 1619-800X
DOI	10.1007/s00415-014-7373-0
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Article ; Online: Rate and characteristics of inflammatory neuropathies associated with brentuximab vedotin therapy.

Matthys, Arthur / Bardel, Benjamin / Le Bras, Fabien / Créange, Alain / Nordine, Tarik / Gounot, Romain / Ingen-Housz-Oro, Saskia / Carvalho, Muriel / Lefaucheur, Jean-Pascal / Haioun, Corinne / Planté-Bordeneuve, Violaine / Gendre, Thierry

European journal of neurology

2024 , Page(s) e16285

Abstract: Background and purpose: Peripheral neuropathy is a frequent complication of brentuximab vedotin (BV), used in CD30+ lymphoma treatment. Classic BV-induced neuropathy (BV-CN) is a mild distal sensory axonal polyneuropathy. Severe BV-induced inflammatory ... ...

Abstract	Background and purpose: Peripheral neuropathy is a frequent complication of brentuximab vedotin (BV), used in CD30+ lymphoma treatment. Classic BV-induced neuropathy (BV-CN) is a mild distal sensory axonal polyneuropathy. Severe BV-induced inflammatory neuropathies (BV-IN) have been described. BV-IN contribute to lymphoma-associated morbidity but might be immunotherapy-responsive. Our primary objective was to evaluate the rate of BV-IN. Our secondary objectives were to determine risk factors and warning signs. Methods: We conducted a retrospective cohort study on all patients treated with BV at our center between April 2014 and September 2021. Clinical, biological, and electrophysiological data were collected. BV-induced neuropathy was defined as the occurrence of neuropathy up to 3 months after BV discontinuation. BV-IN was defined with criteria adapted from European Academy of Neurology/Peripheral Nerve Society 2021 electrodiagnostic criteria for chronic inflammatory demyelinating polyradiculoneuropathy. Other neuropathies were classified as BV-CN. Results: Among 83 patients, 41 (49%) developed neuropathy: 35 BV-CN and 6 BV-IN. Thus, the rate of BV-IN was 7.2%. Compared to patients with BV-CN, no predisposing factor was identified. However, patients with BV-IN more frequently presented muscle weakness (67% vs. 5.7%, p < 0.05), gait disorders (83% vs. 20%, p < 0.05), or acute or subacute onset (67% vs. 14%, p < 0.05). BV-IN was frequently more severe (Common Terminology Criteria for Adverse Events grade ≥3; 50% vs. 0%, p < 0.05). Four patients were treated with immunotherapy. Conclusions: Brentuximab vedotin-induced neuropathy is an overlooked complication. Based on four easily identifiable "red flags", we provide an algorithm to help non-neurologist physicians that care for BV-treated patients to detect BV-IN. The aim of the algorithm is to decrease the diagnostic and management delay of this disabling neuropathy.
Language	English
Publishing date	2024-03-21
Publishing country	England
Document type	Journal Article
ZDB-ID	1280785-0
ISSN	1468-1331 ; 1351-5101 ; 1471-0552
ISSN (online)	1468-1331
ISSN	1351-5101 ; 1471-0552
DOI	10.1111/ene.16285
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Article: Current and Future Treatment Approaches in Transthyretin Familial Amyloid Polyneuropathy.

Kerschen, Philippe / Planté-Bordeneuve, Violaine

Current treatment options in neurology

2016 Volume 18, Issue 12, Page(s) 53

Abstract: Opinion statement: Treatment of transthyretin familial amyloid polyneuropathy (TTR FAP) must be tailored to disease stage. Patients with early stage disease (i.e., without major impairment in walking ability), especially younger patients, should be ... ...

Abstract	Opinion statement: Treatment of transthyretin familial amyloid polyneuropathy (TTR FAP) must be tailored to disease stage. Patients with early stage disease (i.e., without major impairment in walking ability), especially younger patients, should be referred as soon as possible for liver transplantation (LT) in the absence of major comorbid conditions. LT remains the most effective treatment option to date and should be offered to these patients as early as possible. Bridging therapy with an oral TTR stabilizer (tafamidis or diflunisal, according to local access to these treatments) should be started as soon as the diagnosis of TTR FAP is established. Early stage patients who do not wish to or have contraindications to LT should be treated with an oral TTR stabilizer or get access to the newly developed therapeutic options (IONIS TTR-Rx, patisiran, doxycycline/TUDCA). Late stage patients (presenting with significant walking impairment) are usually older and notoriously difficult to treat. They should be offered an oral TTR stabilizer but are not candidates for LT due to a significant rate of perioperative complications and increased risk of progressive neurological and especially cardiac disease despite LT. Access to the different therapies in development should also be considered depending on respective inclusion and exclusion criteria. The abovementioned treatment options were mostly validated in Val30Met mutation patients, but should also be offered to non-Val30Met patients, although mortality rates after LT are higher in these patients. Treatment decisions should be made on an individual basis. Screening for heart, eye, and renal involvement is mandatory for every patient at disease diagnosis and regularly thereafter, even in transplanted patients. Symptomatic treatment should be offered as needed, as well as genetic counseling to at-risk family members. Asymptomatic mutation carriers should benefit from regular screening for early symptoms of disease. Current therapeutic management of TTR FAP will hopefully be changed in the near future with data from the ongoing phase 2/3 studies testing the TTR gene silencing agents. In the longer term, it is likely that combined therapeutic approaches will be necessary to reverse the disease process.
Language	English
Publishing date	2016-11-21
Publishing country	United States
Document type	Review ; Journal Article
ZDB-ID	2057342-X
ISSN	1534-3138 ; 1092-8480
ISSN (online)	1534-3138
ISSN	1092-8480
DOI	10.1007/s11940-016-0436-z
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Article ; Online: Broadening the spectrum of the neurological complications in systemic lupus erythematosus: A patient with meningoradiculitis.

Matthys, Arthur / Megdiche, Imen / Bardel, Benjamin / Remy, Philipe / Limal, Nicolas / Planté-Bordeneuve, Violaine / Gendre, Thierry

Lupus

2022 Volume 31, Issue 3, Page(s) 378–381

Abstract: Background: Neurological complications of systemic lupus erythematosus (SLE) are wide and may rarely involve the peripheral nervous system. However, no case of meningoradiculitis has been well-detailed.: Methods: We report a patient with lupus- ... ...

Abstract	Background: Neurological complications of systemic lupus erythematosus (SLE) are wide and may rarely involve the peripheral nervous system. However, no case of meningoradiculitis has been well-detailed. Methods: We report a patient with lupus-associated meningoradiculitis. Results: A 57-year-old woman had SLE without neurological involvement, treated with hydroxychloroquine, methotrexate, and prednisone for 10 years. Six months after the drug discontinuation for SLE, she acutely developed gait instability, paresthesia, and neuropathic pain of the four limbs. The neurological examination and nerve conduction studies were consistent with radiculopathies. Cerebrospinal fluid (CSF) analysis showed lymphocytic meningitis. The spinal magnetic resonance imaging (MRI) revealed thickening and an enhancement of the lumbosacral roots consistent with meningoradiculitis. The extensive investigations did not argue for a differential diagnosis of SLE. The patient dramatically improved upon corticosteroids. At the last follow-up, the patient still reported mild paresthesia but the clinical examination, the CSF, and the spinal MRI were normal. Conclusion: This well-detailed case of meningoradiculitis broadens the spectrum of neurological complications in SLE. Early recognition of such complications might lead to efficient immunotherapy.
MeSH term(s)	Female ; Humans ; Hydroxychloroquine/therapeutic use ; Lupus Erythematosus, Systemic/complications ; Lupus Erythematosus, Systemic/diagnosis ; Lupus Erythematosus, Systemic/drug therapy ; Magnetic Resonance Imaging ; Middle Aged ; Nervous System Diseases ; Paresthesia/complications
Chemical Substances	Hydroxychloroquine (4QWG6N8QKH)
Language	English
Publishing date	2022-02-08
Publishing country	England
Document type	Case Reports ; Journal Article
ZDB-ID	1154407-7
ISSN	1477-0962 ; 0961-2033
ISSN (online)	1477-0962
ISSN	0961-2033
DOI	10.1177/09612033221078227
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Article ; Online: Revisiting the spectrum of IgM-related neuropathies in a large cohort of IgM monoclonal gammopathy.

Bardel, Benjamin / Molinier-Frenkel, Valérie / Le Bras, Fabien / Ayache, Samar S / Nordine, Tarik / Lefaucheur, Jean-Pascal / Planté-Bordeneuve, Violaine

Journal of neurology

2022 Volume 269, Issue 9, Page(s) 4955–4960

Abstract: Introduction: A significant number of patients with a peripheral neuropathy have IgM monoclonal gammopathy (IgM-MG). In this work, we encompassed the spectrum and outcome of IgM-related neuropathies (IgM-NP) in a large monocentric cohort of patients ... ...

Abstract	Introduction: A significant number of patients with a peripheral neuropathy have IgM monoclonal gammopathy (IgM-MG). In this work, we encompassed the spectrum and outcome of IgM-related neuropathies (IgM-NP) in a large monocentric cohort of patients with IgM-MG. Methods: We retrospectively reviewed the neurological and hematological findings and the course of neuropathy in all patients with IgM-MG over a five-year period in our center (Henri Mondor hospital, Assistance Publique Hôpitaux de Paris (APHP), France). Results: Among 550 patients with IgM-MG, 83 patients (15%) had IgM-NP (55 males, mean age 67 y.o.). The median serum level of IgM-MG was 3.4 g/L, mostly kappa light chain component. The hematological diagnosis was Monoclonal Gammopathy of Undetermined Significance (MGUS) in 62 patients. Anti-MAG antibodies were detected in 38 patients with heterogeneous clinical and neurophysiological features. Four patients had neurolymphomatosis presenting as a non-length dependent predominantly motor neuropathy, which occurred long after the finding of IgM-MG and was responsive to hematological treatment. Five patients had an AL amyloid neuropathy revealed by a small fiber neuropathy. Finally, 30 patients were classified as "Neuropathy of Uncertain Relationship with the IgM" (NURIM) with characteristics close to those of an anti-MAG-NP at the time of diagnosis, except for the neurophysiological features with a predominant axonal pattern. Conclusion: This study emphasizes the wide spectrum of IgM-NP associated with a variety of hematological diagnoses. In particular, the course and prognosis vary considerably. In this setting, further studies are needed to unravel the group of patients classified as NURIM.
MeSH term(s)	Aged ; Autoantibodies ; Female ; Humans ; Immunoglobulin M ; Male ; Monoclonal Gammopathy of Undetermined Significance/complications ; Monoclonal Gammopathy of Undetermined Significance/diagnosis ; Myelin-Associated Glycoprotein ; Paraproteinemias/complications ; Peripheral Nervous System Diseases/complications ; Retrospective Studies
Chemical Substances	Autoantibodies ; Immunoglobulin M ; Myelin-Associated Glycoprotein
Language	English
Publishing date	2022-05-03
Publishing country	Germany
Document type	Journal Article
ZDB-ID	187050-6
ISSN	1432-1459 ; 0340-5354 ; 0012-1037 ; 0939-1517 ; 1619-800X
ISSN (online)	1432-1459
ISSN	0340-5354 ; 0012-1037 ; 0939-1517 ; 1619-800X
DOI	10.1007/s00415-022-11139-2
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