Case reportGuillain-Barré syndrome and hemophagocytic lymphohistiocytosis in a patient with severe chronic active Epstein–Barr virus infection syndrome
Introduction
Epstein–Barr virus (EBV) causes a wide range of immunological disorders, such as infectious mononucleosis, nasopharlyngeal carcinoma and lymphoma, and occasionally produces chronic active infection [1]. Chronic EBV infection is characterized by chronic or recurrent infectious mononucleosis-like symptoms. Severe chronic active EBV infection syndrome (SCAEBV) is a serious form of this infection that shows more severe clinical and hematological features associated with extremely high antibody titers to EBV-related antigens [2]. SCAEBV has high rates of mortality and morbidity because of life-threatening complications, such as hemophagocytic lymphohistiocytosis (HLH) [2], [3]. On the other hand, EBV infection is occasionally associated with the development of various neurologic disorders. EBV is one of the infectious agents causing onset of Guillain-Barré syndrome (GBS) [4], [5], [6]. We report a patient with SCAEBV who presented with GBS and developed fatal HLH several months after the onset of GBS.
Section snippets
Case report
A 72-year-old Japanese man suffered from upper respiratory tract infection, which improved on October 15, 2001. On October 22, blepharoptosis, diplopia and distal muscle weakness occurred. He was admitted to Tokushima University Hospital because he developed dysphagia and muscle weakness resulting in him becoming chair-bound on November 16, 2001.
As shown in Fig. 1, he had pancytopenia and pneumonia in November 1999 at the age of 70 years. Serum titers of antibodies to EBV-related antigens
Discussion
The present patient harbored persistently high titers of antibodies to EBV-VCA (IgG), EA-DR (IgG) and EBNA, indicating chronic reactivated EBV infection. He also had intermittent fever, pancytopenia and polyclonal gammopathy. These findings fulfilled the diagnostic criteria for SCAEBV [2]. Increased CD16 and CD56 positive cells suggested active natural killer cell subpopulations, which was compatible for active EBV infection. EBV ubiquitously infects humans and persists for the life time of the
Acknowledgement
We would like to thank Dr. Keiko Tanaka, Department of Neurology, Brain Research Institute, Niigata University, for an extensive analysis of anti-neuronal antibodies.
References (15)
Overview and problematic standpoints of severe chronic active Epstein–Barr virus infection syndrome
Crit Rev Oncol Hematol
(2002)Clinical features and treatment strategies of Epstein–Barr virus-associated hemophagocytic lymphohistiocytosis
Crit Rev Oncol Hematol
(2002)- et al.
Fine specificities of anti-LM1 IgG antibodies in Guillain-Barré syndrome
J Neurol Sci
(2002) - et al.
Encephalitis in association with chronic active Epstein–Barr virus infection
J Neuroimmunol
(1988) Epstein–Barr virus infection
N Engl J Med
(2000)- et al.
The spectrum of antecedent infections in Guillain-Barré syndrome
Neurology
(1998) - et al.
Epstein–Barr virus and the nervous system
Curr Opin Neurol
(2000)
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