title: Severe steroid-resistant post-infectious encephalomyelitis: general features and effects of IVIg.
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.
authors.affiliation: Institute of Neurology C. Mondino, Pavia, Italy. sabrina.ravaglia@mondino.it
authors.forename: Sabrina
authors.initials: S
authors.lastname: Ravaglia
authors.forename: Giovanni
authors.initials: G
authors.lastname: Piccolo
authors.forename: Mauro
authors.initials: M
authors.lastname: Ceroni
authors.forename: Diego
authors.initials: D
authors.lastname: Franciotta
authors.forename: Anna
authors.initials: A
authors.lastname: Pichiecchio
authors.forename: Stefano
authors.initials: S
authors.lastname: Bastianello
authors.forename: Eleonora
authors.initials: E
authors.lastname: Tavazzi
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Minoli
authors.forename: Enrico
authors.initials: E
authors.lastname: Marchioni
mesh_terms: D000368:Aged; D015331:Cohort Studies; D004305:Dose-Response Relationship, Drug; D004673:Encephalomyelitis, Acute Disseminated / Q000145:classification / Q000188:drug therapy* / Q000473:pathology; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007155:Immunologic Factors / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D012720:Severity of Illness Index; D013256:Steroids / Q000627:therapeutic use; D017211:Treatment Failure; D016896:Treatment Outcome
chemical_list: D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D013256:Steroids