N. Yuki et al., AN IMMUNOLOGICAL ABNORMALITY COMMON TO BICKERSTAFFS BRAIN-STEM ENCEPHALITIS AND FISHERS SYNDROME, Journal of the neurological sciences, 118(1), 1993, pp. 83-87
The nosological position of Bickerstaff's brain stem encephalitis (BBE
) has yet to be established, and its etiology is not clear. Because an
ti-G(Q1b) antibody frequently occurs in patients with Fisher's syndrom
e (FS) and there are clinical similarities between FS and BBE, we inve
stigated anti-ganglioside antibodies in sera from 3 BBE patients who h
ad transient long tract signs in addition to acute ophthalmoplegia and
cerebellar-like ataxia in order to clarify the etiology and nosologic
al position of BBE. High IgG anti-G(Q1b) antibody titers were present
in all 3 sera samples but decreased with the clinical course of the il
lness. In contrast, no anti-G(Q1b) antibody was found in sera from pat
ients with other neurologic diseases which were able to produce transi
ent brain stem disturbance: multiple sclerosis, neuro-Behcet's disease
, brain stem infarction, herpes simplex virus encephalitis, and Wernic
ke's encephalopathy. The finding that BBE and FS shared common autoant
ibody suggests that autoimmune mechanism common to FS is likely in BBE
, and that both conditions represent a distinct disease with a wide sp
ectrum of symptoms that include ophthalmoplegia and ataxia.