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Postinfectious inflammatory disorders: subgroups based on prospective follow-up
E Marchioni1, S Ravaglia, G Piccolo
1Institute of Neurology IRCCS C. Mondino, University of Pavia, Via Mondino 2, 27100 Pavia, Italy. enrico.marchioni@mondino.it
Neurology
|October 12, 2005
Summary
This study describes atypical variants of acute disseminated encephalomyelitis (ADEM), including those affecting the peripheral nervous system (PNS). While many patients responded well to steroids, IV immunoglobulin showed promise for those with PNS involvement and steroid failure.
Area of Science:
- Neurology
- Immunology
- Infectious Diseases
Background:
- Acute disseminated encephalomyelitis (ADEM) is typically a monophasic inflammatory disease of the central nervous system (CNS).
- However, relapsing and site-restricted variants, sometimes involving the peripheral nervous system (PNS), are observed without a clear classification.
- This study addresses the lack of systematic classification for ADEM variants.
Purpose of the Study:
- To characterize a cohort of postinfectious ADEM patients.
- To propose a classification system for ADEM based on clinical and instrumental findings.
- To identify prognostic factors and patient subgroups with distinct outcomes.
Main Methods:
- A 5-year retrospective study of inpatients diagnosed with postinfectious CNS syndrome.
- Patients were classified based on lesion distribution and clinical presentation.
- Outcomes were assessed over a follow-up period of 24 months to 7 years.
Main Results:
- Of 75 enrolled patients, 60 met ADEM criteria. Variants included encephalitis, myelitis, encephalomyelitis, encephalomyeloradiculoneuritis, and myeloradiculoneuritis.
- Fifty percent of patients had a favorable outcome; 25% experienced a relapsing course.
- Poor outcomes were associated with older age, female gender, elevated CSF proteins, and spinal cord/PNS involvement.
Conclusions:
- A significant prevalence of atypical ADEM variants with CNS and PNS involvement was identified.
- Prognosis and response to steroid treatment were generally favorable, with exceptions in specific subgroups.
- Intravenous immunoglobulin (IVIG) demonstrated a beneficial effect in patients with PNS involvement who did not respond to steroids.