We described the clinical and neuroimaging characteristics of seven Chinese patients with anti-GAD65 antibody-associated neurological disorders of whom epileptic seizures were the initial and main symptoms. All patients were given immunotherapy and followed up monthly. The outcome demonstrates that immunotherapy is helpful for non-seizure manifestations of anti-GAD65-associated neurological autoimmunity and is less effective in the treatment of seizures, yet partial responses can still occur in the early stage. Taken together we suggest a trial with immunotherapy in all patients in the early stage of the disease, and in patients with non-epilepsy symptoms in the later stage. Unlabelled Image • The long-term observation on the clinical manifestations, neuroimaging, and outcome of immunotherapy in a series of patients with neurological syndromes mediated by anti-GAD65 Ab. • GAD65 associated SPS and CA manifestations tend to respond well to immunotherapy regardless of the treatment delays, warranting an immunotherapy trial for non-epilepsy manifestations even in late stage of disease. • GAD65 associated epilepsy had poorer response to immunotherapy compared to other antibody-mediated epilepsy, but some patients partially responded to immunotherapy in the early stage, hence it is still justifiable to commence a trial of immunotherapy in GAD65 associated epilepsy patients as soon as possible. • A comprehensive antibody panel should be utilized as patients can have more than one antibody, and a significant response may occur if one Ab is potentially immune-responsive. [ABSTRACT FROM AUTHOR]