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Updated: May 20, 2026

Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis
Published on: July 4, 2007
Isolated brain stem lesion in children: is it acute disseminated encephalomyelitis or not?
G Alper1, G Sreedher, G Zuccoli
1Division of Child Neurology, Department of Pediatrics, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA. gulay.alper@chp.edu
Insights
Acute demyelinating lesions in the brain stem can be challenging to diagnose in children. This study suggests that some isolated brain stem lesions may represent a rare, localized form of acute disseminated encephalomyelitis (ADEM).
Area of Science:
- Pediatric Neurology
- Neuroimmunology
- Demyelinating Diseases
Background:
- Isolated brain stem lesions with acute neurological symptoms pose a diagnostic challenge in pediatric patients.
- While acute disseminated encephalomyelitis (ADEM) often involves the brain stem, solitary lesions are uncommon.
Purpose of the Study:
- To investigate the characteristics and outcomes of children presenting with inflammatory lesions confined to the brain stem.
- To determine if these cases represent a distinct clinical entity or a variant of ADEM.
Main Methods:
- Retrospective review of 6 pediatric patients with brain stem-confined inflammatory lesions.
- Clinical assessment, neuroimaging (MRI), and diagnostic workup for underlying etiologies.
- Evaluation of treatment response, particularly to high-dose corticosteroids.
Main Results:
- Two patients were diagnosed with connective tissue disorders (CNS lupus, localized scleroderma).
- One case had an undetermined etiology, and three cases showed features of monophasic demyelination.
- The three monophasic cases exhibited vasogenic edema, responded dramatically to corticosteroids, and achieved full recovery with no relapses.
- Follow-up MRI in these three cases showed complete lesion resolution.
Conclusions:
- Monofocal demyelination confined to the brain stem can occur in children.
- These cases, particularly those with a monophasic course and good response to steroids, are best classified as a localized form of ADEM.
- ADEM, typically multifocal, can rarely present as a solitary brain stem lesion.
Abstract:
Isolated brain stem lesions presenting with acute neurologic findings create a major diagnostic dilemma in children. Although the brain stem is frequently involved in ADEM, solitary brain stem lesions are unusual. We performed a retrospective review in 6 children who presented with an inflammatory lesion confined to the brain stem. Two children were diagnosed with connective tissue disorder, CNS lupus, and localized scleroderma. The etiology could not be determined in 1, and clinical features suggested monophasic demyelination in 3. In these 3 children, initial lesions demonstrated vasogenic edema; all showed dramatic response to high-dose corticosteroids and made a full clinical recovery. Follow-up MRI showed complete resolution of lesions, and none had relapses at >2 years of follow-up. In retrospect, these cases are best regarded as a localized form of ADEM. We conclude that though ADEM is typically a disseminated disease with multifocal lesions, it rarely presents with monofocal demyelination confined to the brain stem.
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