Severe steroid-resistant post-infectious encephalomyelitis: general features and effects of IVIg.

J Neurol 2007 Nov 14;254(11):1518-23. Epub 2007 Nov 14.

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.

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00415-007-0561-4DOI Listing
November 2007
18 Reads

Publication Analysis

Top Keywords

ivig
11
severe steroid-resistant
8
effects ivig
8
motor dysfunction
8
patients
6
severe
5
failure patients
4
disability 0013
4
patients received
4
damage 89%
4
treated ivig
4
0013 lower
4
ivig steroid
4
received ivig
4
steroid failure
4
first-line treatment
4
milder onset
4
contraindications included
4
dysfunction milder
4
included analysis
4

References

(Supplied by CrossRef)

Menge et al.
Arch Neurol 2005

Wingerchuk et al.
Curr Neurol Neurosci Rep 2003

al Deeb et al.
Brain 1997

Aimoto et al.
No To Shinkei 1996

Amit et al.
Childs Nerv Syst 1992

Dowling et al.
Neurology 1980

Ellis et al.
J Neuro-Ophthalmol 1994

Sahlas et al.
Neurology 2000

Kanter et al.
Neurology 1995

Murthy et al.
Pediatrics 2002

Hahn et al.
Neurology 1996

Similar Publications