Acute severe combined demyelination.
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.
Amit
R
R
Division of Neurology, Children's Hospital of Pittsburgh, PA 15213.
Glick
B
B
Itzchak
Y
Y
Dgani
Y
Y
Meyeir
S
S
D000208:Acute Disease; D002648:Child; D003711:Demyelinating Diseases / Q000209:etiology*; D004679:Encephalomyelitis / Q000150:complications*; D005260:Female; D006801:Humans; D011129:Polyradiculoneuropathy / Q000150:complications*