Anti-galactocerebroside Antibody-Associated Bickerstaff's Brainstem Encephalitis With Dysautonomia: A Case Report and a Review of Associated Central Nervous System Diseases

A 68-year-old man developed diplopia, unsteady walking, and bladder and bowel dysfunction followed by consciousness disturbance within four weeks. On physical examination, consciousness disturbance, bilateral ptosis, ophthalmoplegia, disappearing of doll's eye phenomenon, dysarthria, and diminished deep tendon reflexes were observed. Cerebrospinal fluid (CSF) examination showed oligoclonal bands. Autoantibody to galactocerebroside (Gal-C) was only positive in the serum. Blink reflex and auditory brainstem response showed abnormal findings although brain MRI and peripheral nerve conduction study were negative. The diagnosis of Bickerstaff's brainstem encephalitis (BBE) associated with anti-Gal-C antibody was made. Methylprednisolone (mPSL) pulse therapy was administered but it was not effective. The patient developed a paralytic ileus with complications and required artificial ventilation. Intravenous immunoglobulin (IVIG) was administered, and he was weaned from the ventilator two weeks later. His symptoms slowly improved, and he was discharged after four months. Anti-Gal-C antibody causes a variety of central nervous system (CNS) diseases, including brainstem encephalitis, in all ages but not many cases have been accumulated. Although reports on anti-Gal-C antibody-associated BBE are scarce, the clinical presentation of this case clearly differed from that of classic BBE.