Postinfectious inflammatory disorders: Subgroups based on prospective follow-up

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

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish
Pages (from-to)1057-1065
Number of pages9
JournalNeurology
Volume65
Issue number7
DOIs
Publication statusPublished - Oct 11 2005

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Peripheral Nervous System
Acute Disseminated Encephalomyelitis
Intravenous Immunoglobulins
Steroids
Myelitis
Encephalomyelitis
Central Nervous System Diseases
Encephalitis
Nervous System Diseases
Age of Onset
Communicable Diseases
Inpatients
Spinal Cord
Proteins

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Postinfectious inflammatory disorders : Subgroups based on prospective follow-up. / Marchioni, E.; Ravaglia, S.; Piccolo, G.; Furione, M.; Zardini, E.; Franciotta, D.; Alfonsi, E.; Minoli, L.; Romani, A.; Todeschini, A.; Uggetti, C.; Tavazzi, E.; Ceroni, M.

In: Neurology, Vol. 65, No. 7, 11.10.2005, p. 1057-1065.

Research output: Contribution to journalArticle

Marchioni, E. ; Ravaglia, S. ; Piccolo, G. ; Furione, M. ; Zardini, E. ; Franciotta, D. ; Alfonsi, E. ; Minoli, L. ; Romani, A. ; Todeschini, A. ; Uggetti, C. ; Tavazzi, E. ; Ceroni, M. / Postinfectious inflammatory disorders : Subgroups based on prospective follow-up. In: Neurology. 2005 ; Vol. 65, No. 7. pp. 1057-1065.
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AU - Marchioni, E.

AU - Ravaglia, S.

AU - Piccolo, G.

AU - Furione, M.

AU - Zardini, E.

AU - Franciotta, D.

AU - Alfonsi, E.

AU - Minoli, L.

AU - Romani, A.

AU - Todeschini, A.

AU - Uggetti, C.

AU - Tavazzi, E.

AU - Ceroni, M.

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Y1 - 2005/10/11

N2 - 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.

AB - 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.

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