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  • Chronic inflammatory demyelinating polyradiculoneuropathy: search for factors associated with treatment dependence or successful withdrawal

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Neuromuscular
Review
Chronic inflammatory demyelinating polyradiculoneuropathy: search for factors associated with treatment dependence or successful withdrawal
  1. Magalie Rabin1,
  2. Gurkam Mutlu2,
  3. Tanya Stojkovic1,3,
  4. Thierry Maisonobe1,4,5,
  5. Timothée Lenglet1,4,
  6. Emmanuel Fournier1,3,4,
  7. Pierre Bouche1,
  8. Jean-Marc Léger3,
  9. Karine Viala1,4
  1. 1Département de Neurophysiologie Clinique, Groupe Hospitalier Pitié-Salpêtrière Paris, Université Pierre et Marie Curie–Paris VI, AP-HP, Paris, France
  2. 2Groupe Hospitalier Pitié-Salpêtrière Paris, Service d'urgence cérébrovasculaire Université Pierre et Marie Curie–Paris VI, AP-HP, Paris, France
  3. 3Groupe Hospitalier Pitié-Salpêtrière Paris, Centre de Référence des Pathologies Neuromusculaires Paris Est, Université Pierre et Marie Curie–Paris VI, AP-HP, Paris, France
  4. 4Centre diagnostique et thérapeutique des neuropathies périphériques, Département de Neurophysiologie et Service de Neurologie 1, Groupe Hospitalier Pitié-Salpêtriére Paris, Université Pierre et Marie Curie–Paris VI, AP-HP, Paris, France
  5. 5Département de Neuropathologie R Escourolle, Groupe Hospitalier Pitié-Salpêtrière Paris, Université Pierre et Marie Curie–Paris VI, AP-HP, Paris, France
  1. Correspondence to Dr Karine Viala, Département de Neurophysiologie Clinique, Hôpital de la Salpêtrière, 47 bd de l'Hôpital, 75651 Paris, cedex 13, France; karine.viala{at}psl.aphp.fr

Abstract

Background About 40% of responders to treatment for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) remain treatment dependent and have a relapse if treatment is interrupted.

Objective To look for factors associated with treatment dependence or successful withdrawal in CIDP patients.

Methods We retrospectively studied 70 responder CIDP patients comprising 34 patients who remained treatment dependent (treatment-dependent group) and 36 patients whose treatment could be discontinued (treatment withdrawal group). Clinical, biological, electrophysiological and therapeutic features were compared between these groups.

Results A multifocal deficit was more frequent in the treatment-dependent group (35%) than in the treatment withdrawal group (8%) (p<0.01). The most frequent effective treatment was intravenous immunoglobulin (IVIG) for the treatment-dependent group (79%). In this group, more patients were resistant to corticosteroids in first-line therapy (93%) than in the treatment withdrawal group (40%) (p=0.002). The delay to effective treatment was significantly shorter for the treatment withdrawal group than for the treatment-dependent group (mean 11.1 vs 31.2 months; p<0.01). The rate of successful withdrawal was lower with IVIG (29%) than with corticosteroids (83%) (p<0.001).

Conclusions When compared with the treatment withdrawal group, the treatment-dependent group was more frequently responsive to IVIG, more frequently resistant to corticosteroids in first-line treatment, had a longer delay to effective treatment and was more likely to present a multifocal deficit. The rate of successful withdrawal seems to be higher with corticosteroids, but a prospective study with a long-term follow-up is needed to confirm these features.

  • NEUROPATHY

https://doi.org/10.1136/jnnp-2013-306105

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