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    المساهمون: UCL - SSS/IONS - Institute of NeuroScience, UCL - (SLuc) Service de neurologie, UCL - (SLuc) Centre de référence neuromusculaire, Neurology, AII - Inflammatory diseases, ANS - Neuroinfection & -inflammation, EURO-NMD

    المصدر: European journal of neurology, Vol. 28, no.11, p. 3556-3583 (2021)
    Journal of the peripheral nervous system, Vol. 26, no.3, p. 242-268 (2021)
    Van den Bergh, P Y K, van Doorn, P A, Hadden, R D M, Avau, B, Vankrunkelsven, P, Allen, J A, Attarian, S, Blomkwist-Markens, P H, Cornblath, D R, Eftimov, F, Goedee, H S, Harbo, T, Kuwabara, S, Lewis, R A, Lunn, M P, Nobile-Orazio, E, Querol, L, Rajabally, Y A, Sommer, C & Topaloglu, H A 2021, ' European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy : Report of a joint Task Force—Second revision ', Journal of the Peripheral Nervous System, vol. 26, no. 3, pp. 242-268 . https://doi.org/10.1111/jns.12455Test
    Van den Bergh, P Y K, van Doorn, P A, Hadden, R D M, Avau, B, Vankrunkelsven, P, Allen, J A, Attarian, S, Blomkwist-Markens, P H, Cornblath, D R, Eftimov, F, Goedee, H S, Harbo, T, Kuwabara, S, Lewis, R A, Lunn, M P, Nobile-Orazio, E, Querol, L, Rajabally, Y A, Sommer, C & Topaloglu, H A 2021, ' European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy : Report of a joint Task Force—Second revision ', European Journal of Neurology, vol. 28, no. 11, pp. 3556-3583 . https://doi.org/10.1111/ene.14959Test
    European journal of neurology, 28(11), 3556-3583. Wiley-Blackwell
    JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM
    r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau
    instname
    Journal of the Peripheral Nervous System, 26(3), 242-268. Wiley-Blackwell Publishing Ltd
    EUROPEAN JOURNAL OF NEUROLOGY
    Journal of the peripheral nervous system, 26(3), 242-268. Wiley-Blackwell
    European Journal of Neurology, 28(11), 3556-3583. Wiley-Blackwell Publishing Ltd

    الوصف: Objective: To revise the 2010 consensus guideline on chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Methods: Seventeen disease experts, a patient representative, and two Cochrane methodologists constructed 12 Population/Intervention/Comparison/Outcome (PICO) questions regarding diagnosis and treatment to guide the literature search. Data were extracted and summarized in GRADE summary of findings (for treatment PICOs) or evidence tables (for diagnostic PICOs). Results: Statements were prepared according to the GRADE Evidence-to-Decision frameworks. Typical CIDP and CIDP variants were distinguished. The previous term “atypical CIDP” was replaced by “CIDP variants” because these are well characterized entities (multifocal, focal, distal, motor, or sensory CIDP). The levels of diagnostic certainty were reduced from three (definite, probable, possible CIDP) to only two (CIDP and possible CIDP), because the diagnostic accuracy of criteria for probable and definite CIDP did not significantly differ. Good Practice Points were formulated for supportive criteria and investigations to be considered to diagnose CIDP. The principal treatment recommendations were: (a) intravenous immunoglobulin (IVIg) or corticosteroids are strongly recommended as initial treatment in typical CIDP and CIDP variants; (b) plasma exchange is strongly recommended if IVIg and corticosteroids are ineffective; (c) IVIg should be considered as first-line treatment in motor CIDP (Good Practice Point); (d) for maintenance treatment, IVIg, subcutaneous immunoglobulin or corticosteroids are recommended; (e) if the maintenance dose of any of these is high, consider either combination treatments or adding an immunosuppressant or immunomodulatory drug (Good Practice Point); and (f) if pain is present, consider drugs against neuropathic pain and multidisciplinary management (Good Practice Point).

    وصف الملف: application/pdf

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    المصدر: Muscle & Nerve. 58:23-28

    الوصف: Introduction There is uncertainty as to whether the Guillain-Barre syndrome (GBS) subtypes, acute inflammatory demyelinating polyradiculoneuropathy (AIDP) and acute motor axonal neuropathy (AMAN), can be diagnosed electrophysiologically. Methods We prospectively included 58 GBS patients. Electrodiagnostic testing (EDX) was performed at means of 5 and 33 days after disease onset. Two traditional and one recent criteria sets were used to classify studies as demyelinating or axonal. Results were correlated with anti-ganglioside antibodies and reversible conduction failure (RCF). Results No classification shifts were observed, but more patients were classified as axonal with recent criteria. RCF and anti-ganglioside antibodies were present in both subtypes, more frequently in the axonal subtype. Discussion Serial EDX has no effect on GBS subtype proportions. The absence of exclusive correlation with RCF and anti-ganglioside antibodies may challenge the concept of demyelinating and axonal GBS subtypes based upon electrophysiological criteria. Frequent RCF indicates that nodal/paranodal alterations may represent the main pathophysiology. Muscle Nerve, 2018.

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    المصدر: Muscle & Nerve. 56:828-832

    الوصف: Introduction Cramp-fasciculation syndrome is a peripheral nerve hyperexcitability disorder, which could be caused by inflammatory neuropathy. Case report We describe a 51-year-old woman who presented with a 4- to 5-year history of fasciculations and painful cramping of the right thenar eminence. Results Electrophysiological studies showed motor conduction block in the right median nerve between the axilla and the elbow with fasciculation potentials and cramp discharges on electromyography in the right abductor pollicis brevis muscle. High titers of serum anti-GM1 immunoglobulin M antibodies were detected. Conclusions Monofocal motor neuropathy of the right median nerve was diagnosed. Intravenous immunoglobulin treatment led to significant improvement of symptoms and signs. Although fasciculations and cramps have been reported in multifocal motor neuropathy and are considered supporting criteria for the diagnosis, the occurrence of cramp-fasciculation syndrome as the presenting feature and predominant manifestation in monofocal motor neuropathy, a variant of multifocal motor neuropathy, is unique. Muscle Nerve 56: 828-832, 2017.

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    المساهمون: AII - Amsterdam institute for Infection and Immunity, ANS - Amsterdam Neuroscience, Neurology

    المصدر: Journal of the peripheral nervous system, 15(4), 295-301. Wiley-Blackwell
    JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM
    r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau
    instname

    الوصف: A European Federation of Neurological Societies/Peripheral Nerve Society consensus guideline on the definition, investigation, and treatment of multifocal motor neuropathy (MMN) was published in 2006. The aim is to revise this guideline. Disease experts considered references retrieved from MEDLINE and Cochrane Systematic Reviews published between August 2004 and July 2009 and prepared statements that were agreed to in an iterative fashion. The Task Force agreed on Good Practice Points to define clinical and electrophysiological diagnostic criteria for MMN, investigations to be considered, and principal recommendations for treatment.

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    المصدر: Muscle & Nerve. 41:423-426

    الوصف: We examined a 27-year-old woman who developed rapidly progressive quadriplegia and acute respiratory failure that required mechanical ventilation in the intensive care unit. It was unclear whether this was a presentation of Guillain-Barre syndrome (GBS) or acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (A-CIDP). Remarkable features included multiple cranial nerve involvement, respiratory failure, dysautonomia, and skin manifestations. Several autoantibodies were elevated, including antinuclear (ANA), anticardiolipin (aCL), thyroid, and calcium-sensing receptor (CaSR) autoantibodies. The patient was initially diagnosed with GBS and treated with intravenous immunoglobulin (IVIg). After almost complete recovery, relapse with quadriplegia and respiratory failure was observed 12 weeks after motor symptom onset. She then received IVIg and steroid pulse therapy followed by maintenance oral methylprednisolone and plasma exchange. She recovered completely 4 months after the relapse. The further clinical and serological course was consistent with systemic lupus erythematosus (SLE)-associated CIDP. Herein we evaluate the association between A-CIDP and some biological markers of autoimmunity.

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    المؤلفون: Peter Van den Bergh, F Piéret

    المصدر: Muscle & Nerve. 29:565-574

    الوصف: Electrodiagnosis plays an important role in the early detection and characterization of inflammatory demyelinating polyradiculoneuropathies, because timely treatment reduces morbidity and disability. The challenge consists of defining electrodiagnostic criteria that are highly specific for primary demyelination but sufficiently sensitive to be useful in clinical practice. We compared 10 published sets of criteria in 53 patients with demyelinating Guillain-Barre syndrome (GBS) and 28 with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Specificity of criteria sets was tested in 40 patients with amyotrophic lateral sclerosis (ALS) and 32 with diabetic polyneuropathy (DPN). Sensitivity ranged from 24 to 83% (mean, 54.3%) in GBS and 39 to 89% (mean, 64.9%) in CIDP. With regard to ALS, specificity was 100% for nine sets but was 97% in one. In contrast, 3-66% of DPN patients fulfilled criteria in eight of ten sets. We propose a set of criteria with 72% and 75% sensitivity in our GBS and CIDP patient series, respectively, and 100% specificity with regard to ALS and DPN. Our data illustrate that most, but not all, patients can be electrodiagnostically ascertained.

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    المصدر: European Journal of Neurology. 10:687-694

    الوصف: The aim of this study was to provide an incremental cost-effectiveness analysis comparing intravenous immunoglobulin (IVIg) and prednisolone treatment for chronic inflammatory demyelinating polyradiculoneuropathy. Patients were recruited to a double-blind randomized crossover trial from nine European centres and received either prednisolone or IVIg during the first 6-week treatment period on which the economic evaluation was based. A societal perspective was adopted in measuring service use and costs, although the costs of lost employment were not included. The main outcome measure in the economic evaluation was the number of quality adjusted life years (QALYs) gained, with change in a 11-point disability scale used to measure clinical outcomes. Service use and quality of life data were available for 25 patients. Baseline costs were controlled for using a bootstrapped multiple regression model. The cost difference between the two treatments was estimated to be e3754 over the 6-week period. Health-related quality of life, as measured by the EuroQol EQ-5D instrument, increased more in the IVIg group but the difference was not statistically significant. Using a net-benefit approach it was shown that the probability of IVIg being cost-effective in comparison with prednisolone was 0.5 or above (i.e. was more likely to be cost-effective than cost-ineffective) only if one QALY was valued at over e250 000. The cost-effectiveness of IVIg is greatly affected by the price of IVIg and the amount administered. The impact of later side-effects of prednisolone on long-term costs and quality of life are likely to reduce the cost per QALY of IVIg treatment.

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    المصدر: Annals of Neurology. 50:195-201

    الوصف: This multicenter, randomized, double-blind, crossover trial compared a six week course of oral prednisolone tapering from 60 mg to 10 mg daily with intravenous immunoglobulin (IVIg) 2.0 g/kg given over one to two days for treating chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Twenty-four of the thirty-two randomized patients completed both treatment periods. Both treatments produced significant improvements in the primary outcome measure, change in an 11-point disability scale two weeks after randomization. There was slightly, but not significantly, more improvement after IVIg than with prednisolone, the mean difference between the groups in change in disability grade being 0.16 (95% CI = -0.35 to 0.66). There were also slightly, but not significantly, greater improvements favoring IVIg in the secondary outcome measures: time to walk 10 meters after two weeks and improvement in disability grade after six weeks. Results may have been biased against IVIg by the eight patients who did not complete the second arm of the trial. A serious adverse event (psychosis) attributable to treatment occurred in one patient while on prednisolone and in none with IVIg.

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    المصدر: Muscle & Nerve. 23:283-288

    الوصف: A patient with unilateral, painless, chronic progressive upper limb sensorimotor deficit showed electrophysiological evidence of a focal demyelinating neuropathy with almost complete conduction block across the brachial plexus. Magnetic resonance imaging disclosed marked brachial plexus hypertrophy. Intravenous immunoglobulin led to fast and complete recovery, maintained by intermittent perfusions. Hypertrophic brachial plexus neuropathy can be a presentation of focal chronic inflammatory demyelinating polyradiculoneuropathy. Objective and quantitative assessment of hand function is useful to evaluate treatment results and to optimize treatment regimens.

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