Clinical Features of CASPR2 Autoimmune Encephalitis in Children

Liu X#, Chen J*, Yue X#, Huan B and Liu N

Department of Neurology, Xuzhou Children's Hospital, China
#These authors contributed equally to this work

^*Correspondance to: Jiao Chen 

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Abstract:

Objective: To investigate the clinical features, treatment and prognosis of CASPR2-antibody encephalitis in children. Methods: One preschool male child diagnosed with CASPR2 antibody encephalitis was admitted to the Department of Neurology of Xuzhou Children's Hospital. The clinical features, laboratory tests, EEG manifestations, treatment and prognosis were retrospectively analyzed. The relevant literature was searched. Results: The clinical features of this case were mental and behavioral disturbance. His cranial imaging was normal. The abnormal video EEG showed slow waves, multifocal sharp slow wave and spike wave. His serum anti-CASPR2 antibody titer was 1:10. After 3 weeks of treatment with gamma globulin and steroids, visual hallucination and fear disappeared, hypersomnia and hyperhidrosis were alleviated. The patient could communicate with others, but calculation was limited in simple addition and subtraction. After 6 months, there remained only involuntary movements such as shaking hands, the EEG was normal, the antibodies were negative. These symptoms disappeared one month after oral administration of tiapride. In the literature review, there was no similar domestic case reported in child; while foreign literature reported three cases of children with CASPR2 antibody positive, and two cases were diagnosed with CASPR2 encephalitis. They firstly received treatment of methylprednisolone combined with gamma globulin and achieved significant effect. Another case was diagnosed with Kleine-Levin syndrome, and the symptoms were relieved after oral administration of oxcarbazepine. Conclusion: Children with CASPR2 encephalitis have a variety of clinical symptoms. They respond well to gamma globulin and methylprednisolone, and the short-term prognosis is optimistic after treatment.

Keywords:

Children; Autoimmune Encephalitis; CASPR2; Visual Hallucination; Mental Disturbance; Behavioral Disturbance

Cite the Article:

Liu X, Chen J, Yue X, Huan B, Liu N. Clinical Features of CASPR2 Autoimmune Encephalitis in Children. Ann Clin Case Rep. 2023; 8: 2471..