European Journal of Pediatrics

, Volume 158, Issue 2, pp 133–137

Encephalopathy associated with haemophagocytic lymphohistiocytosis following rotavirus infection

  • S. Takahashi
  • J. Oki
  • A. Miyamoto
  • S. Koyano
  • K. Ito
  • H. Azuma
  • A. Okuno
INFECTIOUS DISEASES

DOI: 10.1007/s004310051033

Cite this article as:
Takahashi, S., Oki, J., Miyamoto, A. et al. Eur J Pediatr (1999) 158: 133. doi:10.1007/s004310051033

Abstract

A 2-year-old Japanese boy with a haemophagocytic lymphohistiocytosis (HLH) associated encephalopathy which developed after rotavirus infection is described. The neurological symptoms consisted of coma, seizures and spastic quadriplegia. On therapy with steroids, etoposide and cyclosporin A, the patient recovered without any neurological deficits. The interferon-gamma levels in serum and CSF were elevated at onset of the disease but had returned to normal at the time of clinical remission. Brain MRI revealed diffuse white matter abnormalities and parenchymal volume loss. Proton magnetic resonance spectroscopy revealed elevated lactate in the abnormal lesions observed on MRI, indicating that macrophages not exhibiting aerobic metabolism had infiltrated the CNS. At the time of clinical remission, the white matter abnormalities and brain lactate had disappeared. These findings suggested that the neurological symptoms resulted from the overproduction of cytokines by activated T-cells and macrophages. The pathophysiology of a HLH associated encephalopathy was considered to be a local immune response within the CNS, because interferon-gamma can induce the expression of major histocompatibility complex class I and II antigens on glial cells in the CNS.

Conclusion Haemophagocytic lymphohistiocytosis associated encephalopathy should be considered early in the differential diagnosis of cases with acute onset neuropathy.

Key words EncephalopathyHaemophagocytic lymphohistiocytosisRotavirusCyclosporin A

Copyright information

© Springer-Verlag Berlin Heidelberg 1999

Authors and Affiliations

  • S. Takahashi
    • 1
  • J. Oki
    • 1
  • A. Miyamoto
    • 1
  • S. Koyano
    • 1
  • K. Ito
    • 1
  • H. Azuma
    • 1
  • A. Okuno
    • 1
  1. 1.Department of Paediatrics, Asahikawa Medical College, 4-5-3-11 Nishikagura, Asahikawa 078-8510, Japan, e-mail: joki5p@asahikawa-med.ac.jp, Tel.: +81-166-68-2483, Fax: +81-166-68-2489JP