In conclusion, the great variability of syndromes associated with anti-CASPR2 antibodies, despite its rarity, should be noted in clinical practice as a differential diagnosis of conditions, such as limbic encephalitis and epilepsy, especially in older patients, newly onset psychiatric symptoms; neuropathic pain; and idiopathic ataxia, as immunotherapy has been identified to be associated with improved outcomes in patients with CASPR2-associated encephalitis. This evidence concerns the gene CNTNAP2 and encephalitis.