Bickerstaff’s brainstem encephalitis, Miller Fisher syndrome and Guillain-Barré syndrome overlap in an asthma patient with negative anti-ganglioside antibodies

Springer Science and Business Media LLC - 2012
Chongyu Han1, Yuan Wang2, Jianping Jia2, Xunming Ji2, Vance L Fredrickson3, Yuchuan Ding3, Wei Sun2, Jia Xu1, Yong Sun2
1Department of Neurology, You Anmen Hospital, 100069, Beijing, People's Republic ofz China
2Department of Neurology, Xuan Wu Hospital of Capital Medical University, 100053, Beijing, People's Republic of China
3Department of Neurological Surgery, Wayne State University School of Medicine, Detroit, MI 48201, USA

Tóm tắt

Abstract Background Bickerstaff’s brainstem encephalitis (BBE), together with Miller Fisher syndrome (MFS) and Guillain-Barré syndrome (GBS) were considered to form a continuous clinical spectrum. An anti-GQ1b antibody syndrome has been proposed to underlie the common pathophysiology for the three disorders; however, other studies have found a positive anti-GM1 instead of anti-GQ1b antibody. Case presentation Here we report a 20-year-old male patient with overlapping BBE, MFS and GBS. The patient had a positive family history of bronchial asthma and had suffered from the condition for over 15 years. He developed BBE symptoms nine days after an asthma exacerbation. During the course of illness, he had significantly elevated IgE levels in both serum and cerebrospinal fluid. Serologic analysis of antibodies against ganglioside complexes (anti-GDIa, anti-GDIb, anti-GM1, anti-GM2, anti-GM3, anti-GQIb and anti-GTIb antibodies) showed negative results. Conclusions Since asthma has recently been related to autoimmune disease, our case supports an autoimmune mechanism underlying the clinical spectrum composed of BBE, MFS and GBS. However, contrary to a proposed anti-GQ1b antibody syndrome, we would suggest that pathogenesis of this clinical spectrum is not limited to anti-ganglioside antibodies.

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