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NEUROLOGY 2005;65:1057-1065
© 2005 American Academy of Neurology

Postinfectious inflammatory disorders

Subgroups based on prospective follow-up

E. Marchioni, MD, S. Ravaglia, MD, PhD, G. Piccolo, MD, M. Furione, MD, E. Zardini, BS, D. Franciotta, MD, E. Alfonsi, MD, L. Minoli, MD, A. Romani, MD, A. Todeschini, MD, C. Uggetti, MD, E. Tavazzi, MD and M. Ceroni, MD

From the Institute of Neurology IRCCS "C. Mondino," (Drs. Marchioni, Ravaglia, Piccolo, Franciotta Alfonsi, Romani, Todeschini, Uggetti, and Tavazzi, E. Zardini), University of Pavia, Infectious Diseases Clinic (Dr. Minoli), Policlinico San Matteo, and Laboratory of Virology (Dr. Furione), Policlinico San Matteo, Pavia, and Department of Neurology (Dr. Ceroni), Policlinico di Monza, Monza, Italy.

Address correspondence and reprint requests to Dr. E. Marchioni, Institute of Neurology IRCCS "C. Mondino," University of Pavia, Via Mondino 2, 27100 Pavia, Italy; e-mail: enrico.marchioni{at}mondino.it

Background: Acute disseminated encephalomyelitis (ADEM) refers to a monophasic acute multifocal inflammatory CNS disease. However, both relapsing and site-restricted variants, possibly associated with peripheral nervous system (PNS) involvement, are also observed, and a systematic classification is lacking.

Objective: To describe a cohort of postinfectious ADEM patients, to propose a classification based on clinical and instrumental features, and to identify subgroups of patients with different prognostic factors.

Methods: Inpatients of a Neurologic and Infectious Disease Clinic affected by postinfectious CNS syndrome consecutively admitted over 5 years were studied.

Results: Of 75 patients enrolled, 60 fulfilled criteria for ADEM after follow-up lasting from 24 months to 7 years. Based on lesion distribution, patients were classified as encephalitis (20%), myelitis (23.3%), encephalomyelitis (13.3%), encephalomyeloradiculoneuritis (26.7%), and myeloradiculoneuritis (16.7%). Thirty patients (50%) had a favorable outcome. Fifteen patients (25%) showed a relapsing course. Poor outcome was related with older age at onset, female gender, elevated CSF proteins, and spinal cord and PNS involvement. All but two patients received high-dose steroids as first-line treatment, with a positive response in 39 (67%). Ten of 19 nonresponders (53%) benefited from high-dose IV immunoglobulin; 9 of 10 had PNS involvement. The data were not controlled.

Conclusions: A high prevalence of "atypical variants" was found in this series, with site-restricted damage or additional peripheral nervous system (PNS) involvement. Prognosis and response to steroids were generally good, except for some patient subgroups. In patients with PNS involvement and steroid failure, a favorable effect of IV immunoglobulin was observed.

Disclosure: The authors report no conflicts of interest.

Received February 9, 2005. Accepted in final form June 21, 2005.




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