Telitacicept as a potential therapeutic alternative in autoantibody-negative probable autoimmune encephalitis: a case report

BACKGROUND: The diagnosis and treatment of autoantibody-negative autoimmune encephalitis (AbNAE) remain challenging due to the absence of definitive serological markers and established treatment strategies. CASE PRESENTATION: We present a case of a 54-year-old male with acute memory impairment. Cerebrospinal fluid (CSF) analysis and cranial magnetic resonance imaging (MRI) revealed features consistent with neuroinflammation. However, the autoimmune encephalitis (AE) antibody panel in both CSF and serum returned negative results. Initially misdiagnosed as viral encephalitis (VE), the patient received a three-week antiviral regimen. Subsequently, the patient developed seizures and neuropsychiatric symptoms, with concurrent deterioration on serial electroencephalography (EEG) and MRI. Repeat AE antibody testing remained negative. The diagnosis of autoantibody-negative but probable autoimmune encephalitis (ANPRA), a subtype of AbNAE, was established. Despite prompt initiation of first-line immunotherapies, including high-dose corticosteroids and intravenous immunoglobulin (IVIg), the patient's neurological status continued to decline. Therefore, subcutaneous telitacicept (160 mg weekly) was administered for four consecutive weeks. This intervention led to progressive neurological improvement without clinically significant adverse events. CONCLUSION: This case highlights the aggressive nature and suboptimal response to conventional immunotherapy in ANPRA, and suggests that telitacicept may represent a viable therapeutic alternative for this challenging condition.