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Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis
Published on: July 4, 2007
Acute disseminated encephalomyelitis in children: a single institution experience of 28 patients
Po-Cheng Hung1, Huei-Shyong Wang, Ming-Liang Chou
1Division of Pediatric Neurology, Chang Gung Children's Hospital, Chang Gung University, Taoyuan, Taiwan. h2918@cgmh.org.tw
Insights
This study details acute disseminated encephalomyelitis (ADEM) in Taiwanese children, noting common symptoms like impaired consciousness and white matter lesions. Steroid treatment led to rapid recovery, with most children experiencing complete recovery.
Area of Science:
- Pediatric Neurology
- Neuroimmunology
- Demyelinating Diseases
Background:
- Acute disseminated encephalomyelitis (ADEM) is an immune-mediated demyelinating disease affecting the central nervous system.
- Understanding ADEM's presentation and outcomes in diverse pediatric populations is crucial for effective management.
Purpose of the Study:
- To characterize the clinical, laboratory, and neuroimaging features of ADEM in Taiwanese children.
- To compare these findings with international cohorts from the United States and Japan.
- To evaluate treatment responses and outcomes in this pediatric cohort.
Main Methods:
- Retrospective review of medical records and MRI scans for 28 Taiwanese children diagnosed with ADEM (2001-2009).
- Analysis of clinical presentation, prodromal illness, cerebrospinal fluid findings, and neuroimaging results.
- Assessment of treatment protocols, primarily steroid therapy, and patient outcomes.
Main Results:
- The mean age of onset was 6 years 9 months, with 24 children having a preceding illness.
- Common symptoms included impaired consciousness and headache; MRI revealed white matter lesions, frequently in frontal/parietal lobes and deep gray matter, with no cortical involvement.
- Intrathecal oligoclonal bands were absent in cerebrospinal fluid samples.
Conclusions:
- Steroid treatment, including high-dose intravenous methylprednisolone and dexamethasone, was associated with rapid recovery.
- All patients survived, with 23 achieving complete recovery and five experiencing mild sequelae.
- Taiwanese pediatric ADEM shares similarities with international cohorts but highlights specific neuroimaging patterns and favorable outcomes with prompt steroid intervention.
Abstract:
The aim of this study is to describe the clinical, laboratory, and neuroimaging features, treatment and outcome of acute disseminated encephalomyelitis (ADEM) in Taiwanese children to compare with two series from United States of America and Japan. We retrospectively reviewed the medical records and magnetic resonance images of 28 children, 23 boys and 5 girls, with ADEM between January 2001 and December 2009. Their mean age at disease onset was 6 years 9 months. Twenty four children experienced a prodromal illness. There was no special seasonal distribution in our patients. They presented mostly with impaired consciousness and headache. Cerebrospinal fluid samples of 21 patients were analyzed and none showed intrathecal oligoclonal bands. Magnetic resonance imaging showed variable findings: lesions with abnormal signal changes frequently found in the subcortical white matter of frontal and parietal lobes. No patient showed cortical gray matter involvement. We also found a high rate of deep gray matter involvement including thalami and basal ganglia. Treating with steroids was usually associated with a rapid recovery and both intravenous high dose methylprednisolone and dexamethasone had the same effect. All patients survived. Twenty three patients recovered completely with only mild sequelae in the remaining five children.
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