Bickerstaff’ s brainstem encephalitis (BBE), Miller Fisher syndrome (MFS) an d Guillain- Barré syndrome (GBS) are thought to be closely related and to for m a continuous spectrum. However, chronic polyneuropathy...Bickerstaff’ s brainstem encephalitis (BBE), Miller Fisher syndrome (MFS) an d Guillain- Barré syndrome (GBS) are thought to be closely related and to for m a continuous spectrum. However, chronic polyneuropathy in BBE has not been rep orted. We report the temporal profile of anti- ganglioside antibody titer in a case of BBE- like brainstem encephalitis complicated with chronic polyneuropath y. A 71- year- old Japanese woman presented with drowsiness and cerebellar ata xia in addition to mild weakness in distal limb muscles. Anti- GalNAc- GD1a Ig G and anti- GalNAc- GM1b IgG antibodies were positive in her serum. Brain magn etic resonance imaging revealed high- intensity signals in the midbrain, pons, and middle cerebellar peduncles on T2- weighted images. Central nervous system manifestations improved after immunomodulating therapy that included prednisolon e, plasmapheresis and intravenous immunoglobulin. Nevertheless, the distal muscl e weakness was exacerbated when the anti- GalNAc- GD1a IgG titer was elevated. Nerve conduction study indicated motor and sensory neuropathy which was develop ed motor dominant axonal damage. These findings suggest that anti- ganglioside antibodies, including anti- GalNAc- GD1a IgG, may be involved in a common auto immune mechanism in BBE- like brainstem encephalitis and chronic motor dominant axonal neuropathy. However, the fact that the latter manifestation exacerbated after the improvement of former one possibly indicates different thresholds of neurolog ic symptoms mediated by anti- ganglio- side antibodies in the present patient.展开更多
文摘Bickerstaff’ s brainstem encephalitis (BBE), Miller Fisher syndrome (MFS) an d Guillain- Barré syndrome (GBS) are thought to be closely related and to for m a continuous spectrum. However, chronic polyneuropathy in BBE has not been rep orted. We report the temporal profile of anti- ganglioside antibody titer in a case of BBE- like brainstem encephalitis complicated with chronic polyneuropath y. A 71- year- old Japanese woman presented with drowsiness and cerebellar ata xia in addition to mild weakness in distal limb muscles. Anti- GalNAc- GD1a Ig G and anti- GalNAc- GM1b IgG antibodies were positive in her serum. Brain magn etic resonance imaging revealed high- intensity signals in the midbrain, pons, and middle cerebellar peduncles on T2- weighted images. Central nervous system manifestations improved after immunomodulating therapy that included prednisolon e, plasmapheresis and intravenous immunoglobulin. Nevertheless, the distal muscl e weakness was exacerbated when the anti- GalNAc- GD1a IgG titer was elevated. Nerve conduction study indicated motor and sensory neuropathy which was develop ed motor dominant axonal damage. These findings suggest that anti- ganglioside antibodies, including anti- GalNAc- GD1a IgG, may be involved in a common auto immune mechanism in BBE- like brainstem encephalitis and chronic motor dominant axonal neuropathy. However, the fact that the latter manifestation exacerbated after the improvement of former one possibly indicates different thresholds of neurolog ic symptoms mediated by anti- ganglio- side antibodies in the present patient.
