J Korean Child Neurol Soc.  2017 Jun;25(2):106-112. 10.0000/jkcns.2017.25.2.106.

Acute Combined Central and Peripheral Demyelination in Children: in Comparison with Isolated Demyelinating Disease

• Byun JH
• Song JY
• Kong J
• Nam SO
• Kim YM
• Yeon GM
• Lee YJ

• KMID: 2385931
• DOI: http://doi.org/10.0000/jkcns.2017.25.2.106

Abstract

PURPOSE
This study aimed to describe the clinical characteristics and outcomes of children with acute combined central and peripheral nervous system demyelination (CCPD); and compare with the children of isolated acute central or peripheral nervous system demyelination.
METHODS
A retrospective chart review of 145 children with acute demyelinating disease between 2010 and 2015 was undertaken in children with younger than 18 years old. Among these, 96 fulfilled criteria (clinical features and positive neuroimaging or electromyography/nerve conduction studies) for either acute central (group A, n=60, 62.5%) or peripheral (group B, n=30, 31.3%) nervous system demyelination, or a CCPD (group C, n=6, 6.3%).
RESULTS
Significant differences among the groups (A vs B vs C) were evident for occurrence of disease between 2013-2015 (45.0% vs 43.3% vs 83.3%; P=0.024), admission to intensive care unit (8.3% vs 26.7% vs 50.0%; P=0.027), length of hospitalization (median, 9.7 vs 12.3 vs 48.3 days; P<0.001), treatment with steroids (88.3% vs 10.0 vs 100.0%; P=0.003), immunoglobulins (13.3% vs 100.0% vs 100.0%; P=0.002) and plasmapheresis (0.0% vs 3.3% vs 50.0%; P=0.037) and severe disability at discharge (3.3% vs 16.7% vs 33.3%; P=0.012). Children of group C showed good response to simultaneous use of immunoglobulin and high-dose corticosteroids and earlier try of plasmapheresis, however, two patients had moderate degree of neurological disability.
CONCLUSION
Systemic studies using neuroimaing and electromyography/nerve conduction studies in all patients with demyelinating disease will be necessary to verify the combined or isolated disease, because CCPD might have the poorer outcome than isolated disease.

Keyword

Demyelinating disease; Child; Acute disseminated encephalomyelitis; Transverse myelitis; Guillain-Barre syndrome; Optic neuritis; Miller-Fisher syndrome

MeSH Terms

Adrenal Cortex Hormones
Child*
Demyelinating Diseases*
Encephalomyelitis, Acute Disseminated
Guillain-Barre Syndrome
Hospitalization
Humans
Immunoglobulins
Intensive Care Units
Miller Fisher Syndrome
Myelitis, Transverse
Nervous System
Neuroimaging
Optic Neuritis
Peripheral Nervous System
Plasmapheresis
Retrospective Studies
Steroids
Adrenal Cortex Hormones
Immunoglobulins
Steroids