Anti-glutamic acid decarboxylase (anti-GAD) autoantibody syndrome produces symptoms and signs related to loss of the inhibitory neurotransmitter gamma aminobutyric acid. Patients with GAD antibody may present with hyperexcitability disorders, including stiff-person syndrome, limbic encephalitis, and nystagmus. There has been only one previous report of convergence spasm in a patient with GAD antibody. Here we present a case of convergence spasm and, to our knowledge, the first case in the English-language ophthalmic literature to have concomitant horizontal nystagmus, in a patient with anti-GAD65 antibody syndrome.A 40-year-old Hispanic woman presented with end-gaze nystagmus and convergence spasm. She was previously healthy with no medical or psychiatric history until age 30 years. Since then, she had a 10-year history of progressive neurocognitive decline, seizures and pseudoseizures, depression and anxiety, limbic encephalitis, and cerebellar ataxia. Her medical history was significant for hypothyroidism. Family history was negative for epilepsy and psychiatric conditions. She did not have stiffperson syndrome or latent autoimmune diabetes of adults (LADA). Her surgical history was noncontributory. Her medications were lamotrigine, dicyclomine, pantoprazole, melatonin, and vitamin supplementation.Neuro-ophthalmic examination demonstrated visual acuity of 20/30 in the right eye (OD) and 20/40 in the left eye (OS). Pupils were equal and reactive without a relative afferent pupillary defect. Ishihara color plates were 14/14 OU. Motility testing showed end gaze horizontal nystagmus with episodes of convergence-spasm-related intermittent esotropia and pupillary miosis (see Video 1 included in supplementary materials, available online). Slit-lamp, intraocular pressure, and external examinations and dilated fundus examination were all normal. Humphrey visual field testing was normal in both eyes (OU). Serial neuroimaging studies with magnetic resonance imaging (MRI) of the head were normal. Cerebrospinal fluid composition was normal. Laboratory testing for syphilis, human immunodeficiency virus, sarcoidosis, tuberculosis, hypercoagulable state, toxin screen, and paraneoplastic antibody panel were all negative. Anti-GAD antibodies were positive at 457 nmol/L and was repeated and persistently elevated. The patient was treated with corticosteroids, intravenous immunoglobulin, and plasma exchange with some variable improvement over the next several years. Search for underlying neoplasm, including whole-body imaging, was negative.Antibody against the GAD enzyme inhibits the formation of inhibitory neurotransmitter gamma aminobutyric acid, resulting in an excitatory or hyperexcitable state. The most common GAD antibody is GAD65, the type present in this patient. The neurological findings in patients with anti-GAD are variable and include stiff-person syndrome,