Publications by authors named "Guillemette Beaudonnet"

9 Publications

  • Page 1 of 1

Impact of Coronavirus Disease 2019 in a French Cohort of Myasthenia Gravis.

Neurology 2021 Feb 10. Epub 2021 Feb 10.

Referral Center for Neuromuscular Diseases and ALS, Timone University Hospital, Aix-Marseille University, Marseille, France.

Objective: To describe the clinical characteristics and outcomes of COVID-19 among patients with MG and identify factors associated with COVID-19 severity in MG patients.

Methods: The CO-MY-COVID registry was a multicenter, retrospective, observational cohort study conducted in neuromuscular referral centers and general hospitals of the FILNEMUS network (between March 1, 2020, and June 8, 2020), including MG patients with a confirmed or highly-suspected diagnosis of COVID-19. COVID-19 was diagnosed based on a polymerase chain reaction (PCR) test from a nasopharyngeal swab and/or SARS-CoV-2 serology, thoracic computed tomography (CT-scan), or typical symptoms. The main outcome was COVID-19 severity based on location of treatment/management (home, hospitalized in a medical unit, or in an intensive care unit). We collected information on demographic variables, general history, and risk factors for severe COVID-19. Multivariate ordinal regression models were used to identify factors associated with severe COVID-19 outcomes.

Results: Among 3,558 MG patients registered in the French database for rare disorders, 34 (0.96%) had COVID-19. The mean age at COVID-19 onset was 55.0 ±19.9 years (mean MG duration: 8.5 ± 8.5 years). By the end of the study period, 28 patients recovered from COVID-19, 1 remained affected, and 5 died. Only high Myasthenia Gravis Foundation of America (MGFA) class (≥IV) before COVID-19 was associated with severe COVID-19 (p=0.004); factors that were not associated included gender, MG duration, and medium MGFA classes (≤IIIb). The type of MG treatment had no independent effect on COVID-19 severity.

Conclusions: This registry-based cohort study shows that COVID-19 had a limited effect on most patients, and immunosuppressive medications and corticosteroids used for MG management are not risk factors for poorer outcomes. However, the risk of severe COVID-19 is elevated in patients with high MGFA classes [odds ratio: 102.6 (4.4; 2,371.9)]. These results are important for establishing evidence-based guidelines for the management of MG patients during the COVID-19 pandemic.
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http://dx.doi.org/10.1212/WNL.0000000000011669DOI Listing
February 2021

Motor unit number index as an individual biomarker: Reference limits of intra-individual variability over time in healthy subjects.

Clin Neurophysiol 2020 Sep 9;131(9):2209-2215. Epub 2020 Jul 9.

Referral Centre for Neuromuscular Diseases and ALS, La Timone Hospital, Marseille, France.

Objective: Motor unit number index (MUNIX) is proposed to monitor neuromuscular disorders. Our objective is to determine the intra-individual variability over time of the MUNIX.

Methods: In 11 different hospital centres, MUNIX was assessed twice, at least 3 months apart (range 90-360 days), in tibialis anterior (TA), abductor pollicis brevis (APB), abductor digiti minimi (ADM) and deltoid muscles in 118 healthy subjects. MUNIX sum score 2, 3 and 4 were respectively the sum of the MUNIX of the TA and ADM, of the TA, APB and ADM and of the TA, APB, ADM and deltoid muscles.

Results: The repeatability of the MUNIX was better for sum scores than for single muscle recordings. The variability of the MUNIX was independent of sex, age, interval between measurements and was lower for experienced than non-experienced operators. The 95th percentile of the coefficient of variability of the MUNIX sum score 2, 3 and 4 were respectively 22%, 18% and 15% for experienced operators.

Conclusions: The MUNIX technique must be performed by experienced operators on several muscles to reduce its variability and improve its reliability.

Significance: A variation of the MUNIX sum score ≥20% can be interpreted as a significant change of muscle innervation.
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http://dx.doi.org/10.1016/j.clinph.2020.06.019DOI Listing
September 2020

Ischemic strokes

Rev Prat 2017 Dec;67(10):e489-e490

Service de neurologie, hôpital Bicêtre, 94270 Le Kremlin-Bicêtre, France.

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December 2017

Motor and/or sensory deficit of the limbs

Rev Prat 2017 Dec;67(10):e479-e488

Service de neurologie, hôpital Bicêtre, 94270 Le Kremlin-Bicêtre, France.

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December 2017

Transthyretin amyloid polyneuropathies mimicking a demyelinating polyneuropathy.

Neurology 2018 07 15;91(2):e143-e152. Epub 2018 Jun 15.

From Service de Physiologie Clinique-Explorations Fonctionnelles (P.L.), AP-HP, Hôpital Lariboisière, Paris; INSERM UMR965 (P.L.), Paris; Université Paris Diderot Sorbonne Paris Cité (P.L., B.A.), Paris; French National Reference Center for FAP (NNERF) (L.-L.M., P.D., G.B., M.T., C.A., D.A.), Le Kremlin-Bicêtre; Service de Neurologie (L.-L.M., P.D., M.T., D.A.) and Service d'anatomopathologie (C.A.), APHP, Hôpital Bicêtre, Le Kremlin-Bicêtre; Unité de Neurophysiologie Clinique et d'épileptologie (G.B.), Hôpital Bicêtre, Le Kremlin-Bicêtre; Immuno-Hematology Department (B.A.), Saint-Louis Hospital, Paris; Université Paris 11 (D.A.); and INSERM UMR1195 (D.A.), Le Kremlin-Bicêtre, France.

Objective: To clearly define transthyretin familial amyloid polyneuropathies (TTR-FAPs) fulfilling definite clinical and electrophysiologic European Federation of Neurological Societies/Peripheral Nerve Society criteria for chronic inflammatory demyelinating polyneuropathy (CIDP).

Methods: From a cohort of 194 patients with FAP, 13 of 84 patients (15%) of French ancestry had late-onset demyelinating TTR-FAP. We compared clinical presentation and electrophysiology to a cohort with CIDP and POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes) syndrome. We assessed nerve histology and the correlation between motor/sensory amplitudes/velocities. Predictors of demyelinating TTR-FAP were identified from clinical and electrophysiologic data.

Results: Pain, dysautonomia, small fiber sensory loss above the wrists, upper limb weakness, and absence of ataxia were predictors of demyelinating TTR-FAP ( < 0.01). The most frequent demyelinating features were prolonged distal motor latency of the median nerve and reduced sensory conduction velocity of the median and ulnar nerves. Motor axonal loss was severe and frequent in the median, ulnar, and tibial nerves ( < 0.05) in demyelinating FAP. Ulnar nerve motor amplitude <5.4 mV and sural nerve amplitude <3.95 μV were distinguishing characteristics of demyelinating TTR-FAP. Nerve biopsy showed severe axonal loss and occasional segmental demyelination-remyelination.

Conclusion: Misleading features of TTR-FAP fulfilling criteria for CIDP are not uncommon in sporadic late-onset TTR-FAP, which highlights the limits of European Federation of Neurological Societies/Peripheral Nerve Society criteria. Specific clinical aspects and marked electrophysiologic axonal loss are red flag symptoms that should alert to this diagnosis and prompt gene sequencing.
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http://dx.doi.org/10.1212/WNL.0000000000005777DOI Listing
July 2018

Motor neuron disease of paraneoplastic origin: a rare but treatable condition.

J Neurol 2018 Jul 3;265(7):1590-1599. Epub 2018 May 3.

Département de Neurophysiologie clinique, Groupe Hospitalier Pitié-Salpêtrière, Paris, France.

Paraneoplastic motor neuron disorders (MND) are rare conditions; their exact clinical and electrophysiological phenotype have not been exhaustively described yet. The purpose of this study is to depict the main characteristics of paraneoplastic MND to highlight the features that may allow its diagnosis. Based on the description of eight original cases, and on the revision of 21 patients identified from a systematic review of the literature, the main features of paraneoplastic MND can be summarized as follows: (1) subacute; (2) lower motor neuron syndrome, associated or not with upper motor neuron involvement; (3) predominant asymmetric upper limb involvement; (4) presence of other non-motor neurological manifestations, including sensory neuronopathy; (5) signs of inflammation in the cerebrospinal fluid (CSF); (6) neurological improvement or stabilization after immunotherapy and tumor treatment. The diagnosis of paraneoplastic MND may be difficult because of its rarity, the absence of pathognomonic clinical features, and the frequent absence of prior tumor history. However, it is of capital importance to correctly identify patients with paraneoplastic MND, as this represents a potentially treatable condition. In the presence of subacute lower motor neuron impairment, especially when atypical clinical features for degenerative MND or other non-motor neurological manifestations are present, we recommend testing for onconeural antibodies. In the case, the search for onconeural antibodies is negative, but it exists a strong clinical suspicion for a paraneoplastic etiology; CSF analysis and total-body 18FDG-PET/CT imaging should be performed to circumstantiate diagnosis.
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http://dx.doi.org/10.1007/s00415-018-8881-0DOI Listing
July 2018

Expression of Yo Antigen in a Prostatic Adenocarcinoma.

Can J Neurol Sci 2017 Mar;44(2):221-223

6CRMR NAF et autres Neuropathies RaresService de Neurologie,CHU Bicêtre78 rue du Général Leclerc94270 Le Kremlin-Bicêtre,France.

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http://dx.doi.org/10.1017/cjn.2016.400DOI Listing
March 2017

TTR kinetic stabilizers and TTR gene silencing: a new era in therapy for familial amyloidotic polyneuropathies.

Expert Opin Pharmacother 2016 10;17(6):791-802. Epub 2016 Mar 10.

a Neurology , CHU Bicêtre, APHP , Le Kremlin Bicêtre , France.

Introduction: Transthyretin Familial Amyloid Polyneuropathy (TTR-FAP) is a rare disease with autosomal dominant transmission due to a point mutation of the TTR gene. By removing the main source of systemic mutant TTR, liver transplantation (LT) has become the reference therapy of this severe and fatal polyneuropathy of adult-onset, stopping disease progression in subgroup of patients. Recently, new therapeutic strategies have emerged, which intend to stabilize TTR or to silence the TTR gene. Amongst them, the TTR kinetic stabilizer tafamidis is the first drug approved in the EU.

Areas Covered: We shall review the natural history of TTR-FAP and the best indications for LT. Data on the efficacy, safety and tolerability of the TTR kinetic stabilizers, tafamidis and diflunisal, have been reviewed, from the pivotal Phase III clinical trials published in PubMed medical journals or presented at international meetings. We will review the ongoing phase III clinical trials of TTR gene silencing with RNAi therapeutics and ASO published in clinicaltrialgov.

Expert Opinion: Due to the data on efficacy, tolerability, safety, tafamidis and diflunisal became the first line anti-amyloid treatment in stage 1 TTR-FAP. Both drugs slow progression of the disease. Only tafamidis got marketing authorization. We are waiting for results of the 2 phase III clinical trials of TTR gene silencing in varied stages of the disease.
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http://dx.doi.org/10.1517/14656566.2016.1145664DOI Listing
August 2016