Anti-IgLON5 disease in a pediatric patient with Langerhans cell histiocytosis

Anti-IgLON5 disease is a rare autoimmune disease of the central nervous system. Different from the previous autoimmune encephalitis, the disease is a chronic progressive disease characterized by abnormal sleep, sleep apnea and motor disorders, which is prone to misdiagnosis and missed diagnosis. We report a unique case of anti-IgLON5 disease in a pediatric patient with Langerhans cell histiocytosis (LCH). He gradually developed increased muscle tone and nystagmus during chemotherapy and showed signs of meningeal enhancement on cranial imaging. Due to insufficient evidence of LCH invasion of the central nervous system, the presence of autoimmune encephalitis-related antibodies was investigated by using cell-based assay (CBA) experiment in indirect immuno-fluorescence assay (IFA). Clinical manifestations of sleep disorders and motor disorders, plus the presence of IgLON5 IgG antibodies (1:30) in the serum leading to a confirmed diagnosis of anti-IgLON5 disease. Anti-IgLON5 disease is rare and almost no cases of children have been reported. In view of the difficult to recognize symptoms in pediatric patients, especially those with other comorbidities. Clinicians should raise their awareness of this disease and pay attention to the detection of autoimmune antibodies.