Korean J Pediatr.  2014 Oct;57(10):457-460. 10.3345/kjp.2014.57.10.457.

Overlapping Guillain-Barre syndrome and Bickerstaff's brainstem encephalitis associated with Epstein Barr virus

• Rho YI

• KMID: 2284386
• DOI: http://doi.org/10.3345/kjp.2014.57.10.457

Abstract

A flaccid tetraparesis in Bickerstaff's brainstem encephalitis (BBE) is presumed to be a sign of overlapping Guillain-Barre syndrome (GBS). In addition, BBE and Fisher syndrome, which are clinically similar and are both associated with the presence of the immunoglobulin G anti-GQ1b antibody, represent a specific autoimmune disease with a wide spectrum of symptoms that include ophthalmoplegia and ataxia. A 2-year-old boy presented with rapidly progressive ophthalmoplegia, ataxia, hyporeflexia, weakness of the lower extremities, and, subsequently, disturbance of consciousness. He experienced bronchitis with watery diarrhea and had laboratory evidence of recent infection with Epstein-Barr virus (EBV). He was diagnosed as having overlapping GBS and BBE associated with EBV and received treatment with a combination of immunoglobulin and methylprednisolone, as well as acyclovir, and had recovered completely after 3 months. In addition, he has not experienced any relapse over the past year. We suggest that combinations of symptoms and signs of central lesions (disturbance of consciousness) and peripheral lesions (ophthalmoplegia, facial weakness, limb weakness, and areflexia) are supportive of a diagnosis of overlapping GBS and BBE and can be helpful in achieving an early diagnosis, as well as for the administration of appropriate treatments.

Keyword

Bickerstaff brainstem encephalitis; Guillain-Barre syndrome; Epstein-Barr virus

MeSH Terms

Acyclovir
Ataxia
Autoimmune Diseases
Brain Stem*
Bronchitis
Child, Preschool
Consciousness
Diagnosis
Diarrhea
Early Diagnosis
Encephalitis*
Extremities
Guillain-Barre Syndrome*
Herpesvirus 4, Human*
Humans
Immunoglobulin G
Immunoglobulins
Lower Extremity
Male
Methylprednisolone
Miller Fisher Syndrome
Ophthalmoplegia
Recurrence
Reflex, Abnormal
Acyclovir
Immunoglobulin G
Immunoglobulins
Methylprednisolone