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Acute severe combined demyelination.

R Amit1, B Glick, Y Itzchak

  • 1Division of Neurology, Children's Hospital of Pittsburgh, PA 15213.

Child'S Nervous System : Chns : Official Journal of the International Society for Pediatric Neurosurgery
|September 1, 1992
PubMed
Summary
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This case study details a rare simultaneous occurrence of Guillain-Barré syndrome (GBS) and acute disseminated encephalomyelitis (ADEM) in a child. Prompt corticosteroid treatment led to a full recovery, suggesting a potential distinct demyelinating entity.

Area of Science:

  • Neuroimmunology
  • Neurology
  • Pediatric Neurology

Background:

  • Guillain-Barré syndrome (GBS) and acute disseminated encephalomyelitis (ADEM) are distinct autoimmune neurological disorders.
  • Simultaneous presentation of GBS and ADEM is exceptionally rare.

Observation:

  • A 10-year-old girl presented with acute coma and severe quadriparesis.
  • Cerebrospinal fluid (CSF) analysis showed elevated protein, and electrophysiological studies revealed delayed F waves, consistent with GBS.
  • Magnetic Resonance Imaging (MRI) demonstrated extensive multifocal demyelination, indicative of ADEM.

Findings:

  • The patient exhibited a fulminant, acute onset of both GBS and ADEM.
  • Treatment with corticosteroids resulted in a rapid clinical improvement and complete recovery.

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Implications:

  • This case supports the hypothesis that a severe, acute combined central and peripheral demyelination could represent a distinct entity.
  • The simultaneous occurrence suggests a potential cross-reactivity of immune responses against myelin components in both the central and peripheral nervous systems.