|
Severe steroid-resistant post-infectious encephalomyelitis: General features and effects of IVIg.
J Neurol. 2007 Nov 2
Ravaglia S, Piccolo G, Ceroni M, Franciotta D, Pichiecchio A, Bastianello S, Tavazzi E, Minoli L, Marchioni E.
Institute of Neurology "C.Mondino", Pavia, Italy.
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.
|
