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Updated: Aug 15, 2026

Primed Mycobacterial Uveitis (PMU) as a Model for Post-Infectious Uveitis
Published on: December 17, 2021
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
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.
Insights
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.
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