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Severe steroid-resistant post-infectious encephalomyelitis

General features and effects of IVIg

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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.

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Authors and Affiliations

  1. Institute of Neurology "C. Mondino", Pavia, Italy

    S. Ravaglia MD, PhD, G. Piccolo MD, D. Franciotta MD, A. Pichiecchio MD, S. Bastianello MD, E. Tavazzi MD & E. Marchioni MD

  2. Infectious Diseases Clinic, Policlinico San Matteo, Pavia, Italy

    L. Minoli MD

  3. Dept. of Neurological Sciences, c/o Institute of Neurology "C. Mondino", Via Mondino 2, 27100, Pavia, Italy

    S. Ravaglia MD, PhD & M. Ceroni MD

Authors
  1. S. Ravaglia MD, PhD
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  2. G. Piccolo MD
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  3. M. Ceroni MD
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  4. D. Franciotta MD
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  5. A. Pichiecchio MD
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  6. S. Bastianello MD
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  7. E. Tavazzi MD
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  8. L. Minoli MD
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  9. E. Marchioni MD
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Correspondence to S. Ravaglia MD, PhD.

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Ravaglia, S., Piccolo, G., Ceroni, M. et al. Severe steroid-resistant post-infectious encephalomyelitis. J Neurol 254, 1518–1523 (2007). https://doi.org/10.1007/s00415-007-0561-4

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  • DOI: https://doi.org/10.1007/s00415-007-0561-4

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