Inflammatory Neuropathy Consortium (TNC) – Special Interest Group of the Peripheral Nerve Society
The Inflammatory Neuropathy Consortium is a Special Interest Group (SIG) of the Peripheral Nerve Society. Its inaugural meeting took place in April 2007 as the 151st Workshop of the European Neuromuscular Centre. The SIG is composed of neurologists who are committed to improving and advancing the investigation and treatment of inflammatory neuropathies.
Mission: To improve the care and treatment of patients with inflammatory and immune-mediated neuropathies. This includes Guillain-Barré syndrome (GBS), chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), multifocal motor neuropathy (MMN) and paraproteinemic neuropathies (PDN). Further details of the aims, objectives and structure of the INC are set out in its constitution.
The INC recognizes the need for:
• Better education of health care professionals to enable earlier diagnosis and treatment,
• More research into the pathogenesis of the inflammatory neuropathies,
• More trials of treatment for all forms of inflammatory neuropathy,
• More trials in children with inflammatory neuropathies,
• Improved outcome measures,
• Treatments for symptoms of particular concern to patients, such as fatigue and pain, and
• A strategy for prioritizing and undertaking trials of candidate treatments efficiently.
On Going Work (Presentations at INC 2016):
1. PeriNomS (Peripheral Neuropathy Outcome Measures Standardization) – Ingemar Merkies – 9 papers have been published within the last year on validating outcome measures in GBS/CIDP/MMN/MGUS using Rasch analysis. The results of this body of work are expected to strongly influence adoption of more proper outcome measures in treatment trials of these conditions.
2. IMAGiNe (IgM Anti-MAG peripheral Neuropathy: from proper assessment to trial needs) – Ingemar Merkies – this study is planned as an observational outcomes study to identify more responsive outcome measures in IgM-MGUS patients, including a RODS under development, ataxia scores, QOL, pain measures, and biobanking. An organizing ENMC workshop is planned for the Fall of 2016.
3. SID-GBS – Christa Walgaard and Pieter van Doorn – RCT, 60 hospitals in The Netherlands. GBS patients randomized to a 2nd dose of IVIg or placebo in patients with a poor prognostic score based on mEGOS one week after presentation. 244 patients enrolled, 72 with poor prognosis, enrollment will be completed with an additional 16 patients with poor prognosis, anticipated by end of 2017.
4. FORCIDP (Fingolimod vs. placebo in CIDP patients on stable doses of IVIg or corticosteroids) – Richard Hughes – 106 patients randomized. Interim analysis was performed after 50 patients enrolled assessing rate of relapse after discontinuing IVIg/corticosteroids, measuring time to 1st relapse with INCAT > 1 point decline. Trial halted for futility (no difference in rate of relapse between active and placebo treatment).
5. PATH trial (SCIg vs. placebo in IVIg dependent patients with CIDP) – Ivo van Schaik – 172 patients randomized, last randomized subject was in April of 2016. The trial should be completed by the end of the 2016, results planned in 2017.
6. Advance CIDP – planned trial of IVIg vs. HyQvia (SCIg monthly) in patients with CIDP. Primary outcome is time to relapse.
7. Mycophenolate in CIDP – Viala – planned trial in patients stable on IVIg randomized to mycophenolate or placebo for 3 months; outcome is time to relapse after stopping IVIg.
8. IVIg cessation trial – Eftimov/van Schaik – study to determine the best method of weaning IVIg in stable CIDP patients, reducing dose and increasing frequency of infusions vs. increasing dose and reducing infusion frequency.
9. ICA-GBS (eculizumab complement inhibition in severe GBS patients treated with IVIg) – Amy Davidson/Hugh Willison – 900 mg vs. placebo, 4 weekly infusions. Outcome measures are GBS score/RODS/ONLS/MRC score at 4 weeks and 6 months. Primary endpoint is safety and tolerability assessment and change in GBS score of 1 grade at 4 weeks. Target enrollment is 30 patients.
10. IGOS – Bart Jacobs et al. – over 1300 patients have been enrolled, and the data has been gleaned for over 800 thus far. An initial manuscript describing the study design and cohort with data analysis is under preparation and is planned to be submitted by the end of 2016. There are several centers in Brazil organizing a substudy of ZIKA-IGOS using the same data collection platform. This has been an enormous effort involving 144 centers and 18 countries.
11. Annexon is in the planning stages of initiating a clinical trial assessing the safety/tolerability/efficacy of a novel C1q inhibitor in patients with GBS, using the IGOS network and data collection platform. The study is planned to start in 2017.
12. ICOS (International CIDP Outcomes Study) – Eftimov/Jacobs – Pilot trial in The Netherlands to collect prospective clinical data and treatment responses to various therapies in patients with CIDP