Guillain-Barré syndrome and hemophagocytic lymphohistiocytosis in a patient with severe chronic active Epstein-Barr virus infection syndrome.

Abstract

Epstein-Barr virus (EBV) infection causes a wide range of neurologic and hematologic manifestations. We report a 72-year-old Japanese male patient with severe chronic active EBV infection syndrome (SCAEBV) who presented with Guillain-Barré syndrome (GBS) and developed hemophagocytic lymphohistiocytosis (HLH) several months after the onset of GBS. He showed acute onset of distal muscle weakness, ophthalmoplegia and bulbar palsy. Results of nerve conduction study revealed acute motor-sensory axonal neuropathy (AMSAN). His serum was positive for anti-LM1 IgG and anti-GM1b IgM. Titers of antibodies to EBV-related antigens indicated chronic reactivated EBV infection. Treatment with IVIg resolved the acute ophthalmoplegia, but there was no notable improvement in the AMSAN and bulbar palsy despite repeated. Finally, he developed refractory HLH resulting in a fatal outcome. In the present patient, it seems that SCAEBV was associated with the development of GBS and fatal HLH via parainfectious autoimmunity and direct infectious immune mechanisms, respectively.

• Authors: Koji Takahashi, Makoto Kunishige, Masayuki Shinohara, Katsuyuki Kubo, Hideo Inoue, Hiide Yoshino, Atsuko Asano, Souichi Honda, Toshio Matsumoto, Takao Mitsui
• Journal: Clinical neurology and neurosurgery (Clin Neurol Neurosurg) Vol. 108 Issue 1 Pg. 80-3 (Dec 2005) ISSN: 0303-8467 [Print] Netherlands
• PMID: 16311154 (Publication Type: Case Reports, Journal Article)

Topics

• Aged
• Chronic Disease
• Epstein-Barr Virus Infections (complications, diagnosis, therapy)
• Fatal Outcome
• Guillain-Barre Syndrome (diagnosis, therapy, virology)
• Humans
• Lymphohistiocytosis, Hemophagocytic (diagnosis, therapy, virology)
• Male
• Syndrome