Journal of Neurology

, Volume 254, Issue 11, pp 1518–1523

Severe steroid-resistant post-infectious encephalomyelitis

General features and effects of IVIg
  • S. Ravaglia
  • G. Piccolo
  • M. Ceroni
  • D. Franciotta
  • A. Pichiecchio
  • S. Bastianello
  • E. Tavazzi
  • L. Minoli
  • E. Marchioni
ORIGINAL COMMUNICATION

DOI: 10.1007/s00415-007-0561-4

Cite this article as:
Ravaglia, S., Piccolo, G., Ceroni, M. et al. J Neurol (2007) 254: 1518. doi:10.1007/s00415-007-0561-4

Abstract

Based on their presumed immuno-mediated etiology, post-infectious CNS disorders are commonly treated with high-dose steroids. Factors influencing treatment effectiveness, possible alternative options for steroid-resistant cases, and their outcome profiles, remain unclear. We here describe the clinical features, the prognosis and the efficacy of i. v. immunoglobulins (IVIg) in a series of severe ADEM refractory to steroids. We performed an inception cohort study on inpatients of the Neurologic and Infectious Disease Clinics, consecutively admitted over eight years, with a minimum two-year follow-up. Nineteen patients affected by classic and site-restricted ADEM were treated with IVIg after steroid failure. Five other patients received IVIg as first-line treatment due to steroids contraindications: although not included in the analysis, they were monitored for anecdotal comparison. Steroids were administered as IV 6-methylprednisolone (6-MP) 500/1000 mg daily until a maximum dose of 6–8 g; IVIg were administered at 0.4 g/kg/day for 5 days. The outcome was assessed by the Scripps Neurological Rating Scale (SNRS) score with determined periodicity. We observed that steroid-resistant patients showed high prevalence of PNS damage (89%) and myelitis (95 %). Other features were old age, severe disability at onset, and moderate to severe blood-brain-barrier (BBB) damage on CSF. In 10/19 patients (53 %) IVIg were effective, the clinical improvement beginning within the end of the five-day cycle,without relapses. Prominent effects of IVIg were detectable on motor dysfunction. Milder onset disability (p = 0.013) and lower CSF albumin (p = 0.006) were the predictors of IVIg response.Among steroid-free patients, 3/5 were responsive to IVIg. We conclude that IVIg can be useful in a portion of patients with severe steroid-resistant ADEM and prominent motor dysfunction. Unsolved issues regard the usefulness of IVIg in less selected groups, and the spectrum of their clinical effects.

Key words

encephalomyelitisADEMmyelo-radiculo-nevritisintravenous immunoglobulins

Copyright information

© Steinkopff-Verlag 2007

Authors and Affiliations

  • S. Ravaglia
    • 1
    • 3
  • G. Piccolo
    • 1
  • M. Ceroni
    • 3
  • D. Franciotta
    • 1
  • A. Pichiecchio
    • 1
  • S. Bastianello
    • 1
  • E. Tavazzi
    • 1
  • L. Minoli
    • 2
  • E. Marchioni
    • 1
  1. 1.Institute of Neurology "C. Mondino"PaviaItaly
  2. 2.Infectious Diseases ClinicPoliclinico San MatteoPaviaItaly
  3. 3.Dept. of Neurological Sciencesc/o Institute of Neurology "C. Mondino"PaviaItaly