title: Delayed motor and sensory neuropathy in a patient with brainstem encephalitis.
abstract: Bickerstaff's brainstem encephalitis (BBE), Miller Fisher syndrome (MFS) and Guillain-Barré syndrome (GBS) are thought to be closely related and to form a continuous spectrum. However, chronic polyneuropathy in BBE has not been reported. We report the temporal profile of anti-ganglioside antibody titer in a case of BBE-like brainstem encephalitis complicated with chronic polyneuropathy. A 71-year-old Japanese woman presented with drowsiness and cerebellar ataxia in addition to mild weakness in distal limb muscles. Anti-GalNAc-GD1a IgG and anti-GalNAc-GM1b IgG antibodies were positive in her serum. Brain magnetic 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 prednisolone, plasmapheresis and intravenous immunoglobulin. Nevertheless, the distal muscle weakness was exacerbated when the anti-GalNAc-GD1a IgG titer was elevated. Nerve conduction study indicated motor and sensory neuropathy which was developed motor dominant axonal damage. These findings suggest that anti-ganglioside antibodies, including anti-GalNAc-GD1a IgG, may be involved in a common autoimmune 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 neurologic symptoms mediated by anti-ganglioside antibodies in the present patient.
authors.affiliation: Department of Medicine and Bioregulatory Sciences, University of Tokushima Graduate School of Medicine, 3-18-15 Kuramoto-cho, Tokushima 770-8503, Japan.
authors.forename: Hiro
authors.initials: H
authors.lastname: Chikakiyo
authors.forename: Makoto
authors.initials: M
authors.lastname: Kunishige
authors.forename: Hiide
authors.initials: H
authors.lastname: Yoshino
authors.forename: Atsuko
authors.initials: A
authors.lastname: Asano
authors.forename: Yuka
authors.initials: Y
authors.lastname: Sumitomo
authors.forename: Itsuro
authors.initials: I
authors.lastname: Endo
authors.forename: Toshio
authors.initials: T
authors.lastname: Matsumoto
authors.forename: Takao
authors.initials: T
authors.lastname: Mitsui
mesh_terms: D000368:Aged; D001323:Autoantibodies / Q000097:blood / Q000276:immunology; D015153:Blotting, Western / Q000379:methods; D001933:Brain Stem / Q000276:immunology / Q000473:pathology / Q000503:physiopathology*; D002855:Chromatography, Thin Layer / Q000379:methods; D004660:Encephalitis / Q000276:immunology / Q000503:physiopathology*; D019054:Evoked Potentials, Motor / Q000502:physiology; D005073:Evoked Potentials, Somatosensory / Q000502:physiology; D005260:Female; D005500:Follow-Up Studies; D005732:Gangliosides / Q000276:immunology; D006801:Humans; D007074:Immunoglobulin G / Q000378:metabolism; D008279:Magnetic Resonance Imaging / Q000379:methods; D009431:Neural Conduction / Q000502:physiology; D010396:Penicillamine / Q000031:analogs & derivatives / Q000378:metabolism; D010523:Peripheral Nervous System Diseases / Q000276:immunology / Q000503:physiopathology*
chemical_list: D001323:Autoantibodies; D005732:Gangliosides; D007074:Immunoglobulin G; C110881:S-nitro-N-acetylpenicillamine; C055253:IV(4)-galactosyl-N-acetylganglioside GD1a; D010396:Penicillamine