Acute severe combined demyelination

✍ Scribed by R. Amit; B. Glick; Y. Itzchak; Y. Dgani; S. Meyeir

• Book ID: 104710426
• Publisher: Springer
• Year: 1992
• Tongue: English
• Weight: 536 KB
• Volume: 8
• Category: Article
• ISSN: 0256-7040
• DOI: 10.1007/bf00296569

✦ Synopsis

We present a second case in which Guillain-Barré syndrome (GBS) and acute disseminated encephalomyelitis (ADEM) appeared simultaneously, both in acute and fulminant form. The patient, a 10-year-old girl, presented with acute onset of coma and flaccid, are-flexic quadriparesis. The elevated CSF protein levels and delayed F waves fulfilled the criteria of GBS and an MRI study revealed extensive multifocal demyelination compatible with a diagnosis of ADEM. Prompt clinical response followed by complete recovery was achieved by treatment with corticosteroids. It is suggested that acute severe combined demyelination might constitute a separate entity in which the demyelinating process, involving simultaneously the central and the peripheral nervous systems, indicates immune response against a component of the myelin of one system carrying cross-antigenicity with the other.