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Recurrence of Acute Disseminated Encephalomyelitis at the Previously Affected Brain Site
Oren Cohen, MD;
Bettina Steiner-Birmanns, MD;
Iftah Biran, MD;
Oded Abramsky, MD, PhD;
Sylvia Honigman, MD;
Israel Steiner, MD
Arch Neurol. 2001;58:797-801.
Background Acute disseminated encephalomyelitis (ADEM) is a usually monophasic
demyelinating disorder of the central nervous system. Recurrences pose a diagnostic
challenge because they can be overlooked or suggest an alternative diagnosis.
Objective To examine the frequency, nature, and outcome of recurrent ADEM.
Design Review of the medical records of patients diagnosed in our institution
as having ADEM between January 1, 1983, and May 31, 1998. Recurrences were
defined as appearance of new symptoms and signs at least 1 month after the
previous episode.
Results Five (24%) of 21 patients with ADEM developed recurrent disease episodes.
In all, diagnosis was confirmed by brain biopsy. One patient had 4 disease
episodes, 2 had 3, and the other 2 each had 2. Recurrence appeared 1.5 to
32 months after initial presentation and involved the same brain territory
in 6 of 9 recurrences in 3 of 5 patients. In 2 patients, recurrences included
neuropsychiatric signs. A good response to corticosteroid therapy was observed
in 10 of 13 of treated ADEM attacks: in 3 of the 4 treated initial events
and in 7 of 9 recurrences.
Conclusions Recurrent ADEM may be more prevalent than previously recognized. Patients
who relapse tend to have more than 1 recurrence that usually involves, clinically
and radiologically, a brain territory that was affected before and can simulate
a space-occupying lesion that requires histologic diagnosis. Neuropsychiatric
features may be the main presentation of a relapse. Since recurrent ADEM is
a corticosteroid-responsive condition, awareness and early diagnosis are mandatory.
From the Department of Neurology and The Agnes Ginges Center for Human
Neurogenetics, Hadassah University Hospital and the Hebrew University Medical
School, Jerusalem, Israel (Drs Cohen, Steiner-Birmanns, Biran, Abramsky, and
Steiner); and the Department of Neurology, Carmel Medical Center, Haifa, Israel
(Dr Honigman).
Corresponding author: Israel Steiner, MD, Department of Neurology,
Hadassah University Hospital, PO Box 12000, Ein Karem, Jerusalem 91120, Israel
(e-mail: isteiner{at}md2.huji.ac.il).
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