Abstract
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, areflexic 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.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Amit R, Shapira Y, Blank A, Aker M (1986) Acute, severe, central and peripheral nervous system combined demyelination. Pediatr Neurol 2:47–50
Atlas SW, Grossman RI, Goldberg HI, Hackey DB, Bilaniuk LT, Zimmerman RA (1986) MRI diagnosis of acute disseminated encephalo-myelitis. J Comput Assist Tomogr 10:798–801
Baker AB (1943) Guillain-Barré's disease (encephalo-myeloradiculitis): a review of 33 cases. J Lancet 63:384–398
Blennow G, Gamstorp I, Rosenberg R (1968) Encephalomyelo-radiculo-neuropathy. Dev Med Child Neurol 10:485–490
Gamstorp I (1974) Encephalo-myelo-radiculo-neuropathy: involvement of the CNS in children with Guillain-Baré-Strohl syndrome. Dev Med Child Neurol 16:654–658
Lukes SA, Norman D (1983) Computed tomography in acute disseminated encephalomyelitis. Ann Neurol 13:567–572
Mendell JR, Kolkin S, Kissel JT, Weiss KL, Chakeres DW, Rammohan KW (1987) Evidence for central nervous system demyelination in chronic inflammatory demyelinating polyradiculoneuropathy. Neurology 37:1291–1294
Morley JB, Reynolds EH (1966) Papilledema and Landry-Guillain-Barré syndrome. Case report and review. Brain 89:205
Rossi LN (1977) L'encéphalomyclo-radiculoneuropathy: la participation du système nerveux centrale dans la polyradiculite aigüe de l'enfant. Arch Fr Pediatr 134:399–405
Rubin M, Karpati G, Carpenter S (1987) Combined central and peripheral myelinopathy. Neurology 37:1287–1290
Whitaker JN (1981) The protein antigen of peripheral nerve myelin. Ann Neurol 9 [Suppl]:56–64
Willis J, Van Den Berg P (1988) Cerebral involvement in children with acute relapsing polyneuropathy. J Child Neurol 3:200–204
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Amit, R., Glick, B., Itzchak, Y. et al. Acute severe combined demyelination. Child's Nerv Syst 8, 354–356 (1992). https://doi.org/10.1007/BF00296569
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF00296569