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Recurrence of acute disseminated encephalomyelitis at the previously affected brain site
O Cohen1, B Steiner-Birmanns, I Biran
1Department of Neurology, Hadassah University Hospital, PO Box 12000, Ein Karem, Jerusalem 91120, Israel.
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.
Insights
Recurrent acute disseminated encephalomyelitis (ADEM) occurs in 24% of patients and often involves previously affected brain areas. Early diagnosis and corticosteroid treatment are crucial for managing recurrent ADEM.
Area of Science:
- Neurology
- Neuroimmunology
- Demyelinating Diseases
Background:
- Acute disseminated encephalomyelitis (ADEM) is typically a single-episode demyelinating disorder of the central nervous system.
- Recurrent ADEM presents diagnostic challenges, often being misdiagnosed or overlooked.
Observation:
- This study reviewed 21 patients diagnosed with ADEM between 1983 and 1998.
- Recurrence was defined as new symptoms appearing at least one month after a prior episode.
- Brain biopsy confirmed ADEM diagnosis in all reviewed cases.
Findings:
- 24% of patients (5 out of 21) experienced recurrent ADEM episodes.
- Recurrences occurred 1.5 to 32 months post-initial presentation.
- Recurrent episodes often affected previously impacted brain regions and could mimic space-occupying lesions, necessitating biopsy.
- Neuropsychiatric symptoms were present in some relapses.
- Corticosteroid therapy showed good response in 7 out of 9 recurrent ADEM attacks.
Implications:
- Recurrent ADEM may be more common than previously thought.
- Awareness of recurrent ADEM is vital for timely diagnosis and management.
- Prompt recognition and corticosteroid treatment are essential for favorable outcomes in recurrent ADEM.