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


METHODS
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.
Department of Neurology, You Anmen Hospital, 100069 Beijing, People's Republic of China.
Chongyu
C
Han
Yuan
Y
Wang
Jianping
J
Jia
Xunming
X
Ji
Vance
V
Fredrickson
Yuchuan
Y
Ding
Wei
W
Sun
Jia
J
Xu
Yong-Xin
YX
Sun
D000906:Antibodies / Q000276:immunology*; D001249:Asthma / Q000150:complications* / Q000276:immunology / Q000503:physiopathology; D001933:Brain Stem / Q000473:pathology* / Q000503:physiopathology; D004569:Electroencephalography; D004660:Encephalitis / Q000150:complications* / Q000175:diagnosis / Q000276:immunology / Q000503:physiopathology; D005732:Gangliosides / Q000276:immunology*; D020275:Guillain-Barre Syndrome / Q000150:complications* / Q000175:diagnosis / Q000276:immunology / Q000503:physiopathology; D006801:Humans; D008297:Male; D019846:Miller Fisher Syndrome / Q000150:complications* / Q000175:diagnosis / Q000276:immunology / Q000503:physiopathology; D055815:Young Adult
D000906:Antibodies; D005732:Gangliosides