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P Y K Van den Bergh, R D M Hadden,
P Bouche,
D R Cornblath,
A Hahn,
I Illa,
C L Koski,
J-M Léger,
E Nobile-Orazio,
J Pollard,
C Sommer,
P A van Doorn,
I N van Schaik
[show abstract]
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ABSTRACT: Consensus guidelines on the definition, investigation, and treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) have been previously published in European Journal of Neurology and Journal of the Peripheral Nervous System.
To revise these guidelines.
Disease experts, including a representative of patients, considered references retrieved from MEDLINE and Cochrane Systematic Reviews published between August 2004 and July 2009 and prepared statements that were agreed in an iterative fashion.
The Task Force agreed on Good Practice Points to define clinical and electrophysiological diagnostic criteria for CIDP with or without concomitant diseases and investigations to be considered. The principal treatment recommendations were: (i) intravenous immunoglobulin (IVIg) (Recommendation Level A) or corticosteroids (Recommendation Level C) should be considered in sensory and motor CIDP; (ii) IVIg should be considered as the initial treatment in pure motor CIDP (Good Practice Point); (iii) if IVIg and corticosteroids are ineffective, plasma exchange (PE) should be considered (Recommendation Level A); (iv) if the response is inadequate or the maintenance doses of the initial treatment are high, combination treatments or adding an immunosuppressant or immunomodulatory drug should be considered (Good Practice Point); (v) symptomatic treatment and multidisciplinary management should be considered (Good Practice Point).
European Journal of Neurology 03/2010; 17(3):356-63. · 3.69 Impact Factor
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R. D. M. Hadden,
E. Nobile-Orazio,
C. Sommer,
A. Hahn,
I. Illa,
E. Morra,
J. Pollard,
R. Hughes,
P. Bouche,
D. Cornblath,
E. Evers,
C. L. Koski,
J. M. Léger,
P. Van den Bergh,
P. van Doorn,
I. N. van Schaik
01/2008: pages 362 - 375; , ISBN: 9780470753279
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R D M Hadden,
E Nobile-Orazio,
C Sommer,
A Hahn,
I Illa,
E Morra,
J Pollard,
R A C Hughes,
P Bouche,
D Cornblath,
E Evers,
C L Koski,
J M Léger,
P Van den Bergh,
P van Doorn,
I N van Schaik
[show abstract]
[hide abstract]
ABSTRACT: Paraprotein-associated neuropathies have heterogeneous clinical, neurophysiological, neuropathological and haematological features. Objectives. To prepare evidence-based and consensus guidelines on the clinical management of patients with both a demyelinating neuropathy and a paraprotein (paraproteinaemic demyelinating neuropathy, PDN).
Search of MEDLINE and the Cochrane library, review of evidence and consensus agreement of an expert panel.
In the absence of adequate data, evidence based recommendations were not possible but the panel agreed the following good practice points: (1) Patients with PDN should be investigated for a malignant plasma cell dyscrasia. (2) The paraprotein is more likely to be causing the neuropathy if the paraprotein is immunoglobulin (Ig)M, antibodies are present in serum or on biopsy, or the clinical phenotype is chronic distal sensory neuropathy. (3) Patients with IgM PDN usually have predominantly distal and sensory impairment, with prolonged distal motor latencies, and often anti-myelin associated glycoprotein antibodies. (4) IgM PDN sometimes responds to immune therapies. Their potential benefit should be balanced against their possible side-effects and the usually slow disease progression. (5) IgG and IgA PDN may be indistinguishable from chronic inflammatory demyelinating polyradiculoneuropathy, clinically, electrophysiologically, and in response to treatment. (6) For POEMS syndrome, local irradiation or resection of an isolated plasmacytoma, or melphalan with or without corticosteroids, should be considered, with haemato-oncology advice.
European Journal of Neurology 09/2006; 13(8):809-18. · 3.69 Impact Factor
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I N van Schaik,
P Bouche,
I Illa,
J-M Léger,
P Van den Bergh,
D R Cornblath,
E M A Evers, R D M Hadden,
R A C Hughes,
C L Koski,
E Nobile-Orazio,
J Pollard,
C Sommer,
P A van Doorn
[show abstract]
[hide abstract]
ABSTRACT: Several diagnostic criteria for multifocal motor neuropathy have been proposed in recent years and a beneficial effect of intravenous immunoglobulin (IVIg) and various other immunomodulatory drugs has been suggested in several trials and uncontrolled studies. The objectives were to prepare consensus guidelines on the definition, investigation and treatment of multifocal motor neuropathy. Disease experts and a patient representative considered references retrieved from MEDLINE and the Cochrane Library in July 2004 and prepared statements which were agreed in an iterative fashion. The Task Force agreed good practice points to define clinical and electrophysiological diagnostic criteria for multifocal motor neuropathy and investigations to be considered. The principal recommendations and good practice points were: (i) IVIg (2 g/kg given over 2-5 days) should be considered as the first line treatment (level A recommendation) when disability is sufficiently severe to warrant treatment. (ii) Corticosteroids are not recommended (good practice point). (iii) If initial treatment with IVIg is effective, repeated IVIg treatment should be considered (level C recommendation). The frequency of IVIg maintenance therapy should be guided by the individual response (good practice point). Typical treatment regimens are 1 g/kg every 2-4 weeks or 2 g/kg every 4-8 weeks (good practice point). (iv) If IVIg is not or not sufficiently effective then immunosuppressive treatment may be considered. Cyclophosphamide, ciclosporin, azathioprine, interferon beta1a, or rituximab are possible agents (good practice point). (v) Toxicity makes cyclophosphamide a less desirable option (good practice point).
European Journal of Neurology 09/2006; 13(8):802-8. · 3.69 Impact Factor
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R A C Hughes,
P Bouche,
D R Cornblath,
E Evers, R D M Hadden,
A Hahn,
I Illa,
C L Koski,
J M Léger,
E Nobile-Orazio,
J Pollard,
C Sommer,
P Van den Bergh,
P A van Doorn,
I N van Schaik
[show abstract]
[hide abstract]
ABSTRACT: Numerous sets of diagnostic criteria have sought to define chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and randomized trials and systematic reviews of treatment have been published. The objective is to prepare consensus guidelines on the definition, investigation and treatment of CIDP. Disease experts and a patient representative considered references retrieved from MEDLINE and Cochrane Systematic Reviews in May 2004 and prepared statements which were agreed in an iterative fashion. The Task Force agreed on good practice points to define clinical and electrophysiological diagnostic criteria for CIDP with or without concomitant diseases and investigations to be considered. The principal treatment recommendations were: (1) intravenous immunoglobulin (IVIg) or corticosteroids should be considered in sensory and motor CIDP (level B recommendation); (2) IVIg should be considered as the initial treatment in pure motor CIDP (Good Practice Point); (3) if IVIg and corticosteroids are ineffective plasma exchange (PE) should be considered (level A recommendation); (4) If the response is inadequate or the maintenance doses of the initial treatment are high, combination treatments or adding an immunosuppressant or immunomodulatory drug should be considered (Good Practice Point); (5) Symptomatic treatment and multidisciplinary management should be considered (Good Practice Point).
European Journal of Neurology 05/2006; 13(4):326-32. · 3.69 Impact Factor
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[show abstract]
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ABSTRACT: The efficacy of plasma exchange as a therapy for Guillain-Barré syndrome (GBS) suggests that humoral factors might contribute to the axonal conduction block responsible for the major symptoms of the disease. To explore this possibility, we have applied sera to rat spinal roots in vitro while monitoring axonal conduction. Neither fresh sera from 12 patients with GBS or Miller-Fisher syndrome (MFS), nor serum from rabbits immunised with Campylobacter jejuni from patients with GBS, MFS or gastroenteritis were effective in causing acute conduction block, despite the presence of antibodies to gangliosides GD3, GM1, GQ1b and GT1a. Potential explanations are advanced.
Journal of Neuroimmunology 07/2003; 139(1-2):133-40. · 2.96 Impact Factor
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ABSTRACT: At the onset of Guillain-Barré syndrome, disruption of diffusion barriers, such as the blood-nerve barrier, probably increases the exposure of spinal roots and peripheral nerves to macromolecules, some of which might be pathogenic. As a measure of such disruption, we measured the accumulation in the endoneurium of spinal roots and sciatic nerve of systemically administered 125I-labelled immunoglobulin in adoptive transfer experimental autoimmune neuritis (AT-EAN) in the rat. AT-EAN is a model of Guillain-Barré syndrome, induced by injection of activated T lymphocytes sensitized to myelin P2 protein. Immunoglobulin accumulation was expressed as counts/min/mg in fixative-perfused roots as a percentage of that in serum, measured 24 h after intraperitoneal injection of 0.1 micro Ci 125I-labelled immunoglobulin. Immunoglobulin accumulation in the roots of normal rats was 3 +/- 1% (mean +/- SE), but this first increased 3(1/2) days after cell injection, peaked at 22 +/- 2% on day 4(1/2), and declined to normal by day 8. T lymphocytes and polymorphonuclear leucocytes first appeared within the endoneurium at day 3(1/2), and macrophages and a few erythrocytes at day 4. Neurological deficit appeared on day 4 and was maximal on day 6. Demyelination and axonal degeneration began at day 5. The first abnormality detected in AT-EAN was a rapid increase in the passage of immunoglobulin into spinal roots, together with endoneurial infiltration of T lymphocytes and polymorphonuclear leucocytes. Accumulation of immunoglobulin was maximal during the worsening of neurological deficit, and declined rapidly before the onset of neurological recovery.
Neuropathology and Applied Neurobiology 01/2003; 28(6):489-97. · 3.80 Impact Factor
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[show abstract]
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ABSTRACT: Staphylococcal protein A immunoadsorption and plasma exchange were compared for treating chronic inflammatory demyelinating polyradiculoneuropathy. In a single patient, plasma exchange had a more beneficial effect than immunoadsorption on clinical outcome measures. Serum IgM antibody activity to peripheral nerve fell significantly following plasma exchange. Serum IgM and IgA fell more and IgG less after plasma exchange than after immunoadsorption. The superior efficacy of plasma exchange to immunoadsorption in this case may have been the result of removal of an IgM antibody.
Journal of Neurology Neurosurgery & Psychiatry 06/2002; 72(5):644-6. · 4.76 Impact Factor
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Journal of Applied Microbiology 12/2001; 90(S6):145S - 154S. · 2.34 Impact Factor
