International Inflammatory Neuropathy Consortium

Neurologists, other experts and patient representatives from Europe and the USA met under the auspices of this ENMC workshop to consider the management of inflammatory neuropathy. Existing treatments for Guillain-Barré syndrome (GBS), chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and related disorders leave unacceptable numbers of patients with persistent severe disability. Despite the evident need for better treatments, no trials are in progress for GBS and few for CIDP and related disorders. This workshop has launched an international consortium to discover new treatments for these conditions. Participants reviewed the evidence from Cochrane systematic reviews, randomised trials and observational studies for treating GBS, CIDP, multifocal motor neuropathy (MMN) and paraprotein associated demyelinating neuropathy (PDN). They then debated ways of making advances and reached the following conclusions.

Conclusions

· There is a need for better education of health care professionals to enable the earlier diagnosis of these potentially treatable diseases

· There is a need for more research into the pathogenesis of inflammatory neuropathy

· There is a need for more evidence in children

· In GBS there is a need for trials of

o Intravenous immunoglobulin (IVIg) in mild GBS and the related disorder Fisher syndrome

o Plasma exchange versus IVIg in axonal forms

o complement inhibitors in acute severe GBS

o a second course of IVIg in patients who are still bed bound 2 weeks after the first course

o sodium channel blockers

· In CIDP there is a need for trials of high doses of methotrexate or else of rituximab additional to existing treatments depending on the result of the trial of methotrexate which will conclude in February 2008.

· In MMN there is a need for trials of methotrexate additional to existing treatments.

· In PDN the choice of trials depends on the outcome of the ongoing French-Swiss trial of rituximab with an intended 60 participants. However there would be likely to be a need for trials of higher dose or more frequent rituximab. Chlorambucil was also considered worth trying.

· For all these diseases, there is a need for improved outcome measures and the Consortium should support the PERINOMS study designed by the Rotterdam-Maastricht group.

· Treatments for symptoms of particular concern to patients, particularly fatigue and pain, should also be tested. A trial of exercise for residual symptoms after GBS and in CIDP should be further considered.

· For all these rare disorders there is a need to implement statistical trial methods for identifying candidate treatments more efficiently

Future plans

• The consortium will seek official status as a special interest group of the Peripheral Nerve Society
• The Consortium will liaise closely with the GBS-CIDP Foundation International to ensure the relevance of their work to the needs of patients
• The Consortium will maintain an up to date account of the current trial evidence and support the Cochrane Collaboration in maintaining up-to-date systematic reviews of the evidence
• The consortium will maintain a register of relevant ongoing and planned trials, drawing on existing sources of evidence
• The consortium will prioritise the agents which need to be tried
• The consortium will establish and maintain initially retrospective but eventually prospective registers of patients with rare inflammatory neuropathies and their treatments. An initial register of multifocal motor neuropathy will be set up.
• The consortium will facilitate development of prioritised trials
• The consortium will publish its recommendations in an appropriate journal, the newsletters of the GBS-CIDP Foundation International and on the World Wide Web.

Participants: J. Berciano (Spain), P. van den Bergh (The Netherlands), P. Blomkwist (The Netherlands) D. Cornblath (USA), S. Delsignore (Italy), P. van Doorn (The Netherlands), H. Hartung (Germany), R. Hughes (United Kingdom, chair) I. Illa (Spain), R. Korinthenberg (Germany), J.M. Leger (France), R. Lewis (USA), M. Lunn (United Kingdom), I. Merkies (The Netherlands), J. Pouget (France), G. Sanders (United Kingdom), I. Van Schaik (The Netherlands) E. Steyerberg (The Netherlands)

A full report of this meeting has been published in Neuro Muscular Diseases. (pdf)

Next meeting

Peripheral Nerve Society meeting Monday July 16 2007 Snowbird, Utah.

This meeting will be open to other interested members of the PNS and relevant pharmaceutical company representatives will be invited.