An atypical case of ataxia, headache, and vertigo associated with ganglionic acetylcholine receptor autoantibodies

: Ganglionic acetylcholine receptor autoantibodies (AChR Ab) typically cause autoimmune dysautonomia and are commonly associated with malignancy. : We report a case of a 56-year-old man with longstanding refractory headaches, vertigo, and cerebellar ataxia who was found to have α3-AChR Ab seropositivity in the absence of other neurological symptoms including dysautonomia. His-neurological symptoms improved significantly after treatment with intravenous immunoglobulin. : This is the first reported case of AChR Ab causing headache, vertigo, and ataxia and expands the spectrum of neurological symptoms associated with AChR Ab. The case highlights the importance of recognizing possible central nervous system manifestations of AChR Ab.