Postinfectious inflammatory disorders: subgroups based on prospective follow-up.
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.
Marchioni
E
E
Institute of Neurology IRCCS C. Mondino, University of Pavia, Via Mondino 2, 27100 Pavia, Italy. enrico.marchioni@mondino.it
Ravaglia
S
S
Piccolo
G
G
Furione
M
M
Zardini
E
E
Franciotta
D
D
Alfonsi
E
E
Minoli
L
L
Romani
A
A
Todeschini
A
A
Uggetti
C
C
Tavazzi
E
E
Ceroni
M
M
D000328:Adult; D000367:Age Factors; D000368:Aged; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001921:Brain / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D002490:Central Nervous System / Q000276:immunology / Q000473:pathology / Q000503:physiopathology*; D015331:Cohort Studies; D004673:Encephalomyelitis, Acute Disseminated / Q000145:classification* / Q000175:diagnosis* / Q000503:physiopathology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D010525:Peripheral Nerves / Q000276:immunology / Q000473:pathology / Q000503:physiopathology*; D011379:Prognosis; D011446:Prospective Studies; D012008:Recurrence; D012737:Sex Factors; D013116:Spinal Cord / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D013126:Spinal Nerve Roots / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D013256:Steroids / Q000627:therapeutic use; D016896:Treatment Outcome
D000893:Anti-Inflammatory Agents; D016756:Immunoglobulins, Intravenous; D013256:Steroids