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Clinical characteristics and long-term outcome of CASPR2 antibody-associated autoimmune encephalitis in children.
Liao, Donglei; Zhu, Saying; Yang, Lifen; Zhang, Ciliu; He, Fang; Yin, Fei; Peng, Jing.
Afiliación
  • Liao D; Department of Pediatrics, Xiangya Hospital of Central South University, Changsha, 410008, Hunan Province, China.
  • Zhu S; Department of Pediatrics, Xiangya Hospital of Central South University, Changsha, 410008, Hunan Province, China.
  • Yang L; Department of Pediatrics, Xiangya Hospital of Central South University, Changsha, 410008, Hunan Province, China.
  • Zhang C; Department of Pediatrics, Xiangya Hospital of Central South University, Changsha, 410008, Hunan Province, China.
  • He F; Department of Pediatrics, Xiangya Hospital of Central South University, Changsha, 410008, Hunan Province, China.
  • Yin F; Department of Pediatrics, Xiangya Hospital of Central South University, Changsha, 410008, Hunan Province, China.
  • Peng J; Clinical Research Center for Children Neurodevelopmental disabilities of Hunan Province, Central South University, XiangyaHospital, Changsha, 410008, China.
Ital J Pediatr ; 50(1): 158, 2024 Aug 26.
Article en En | MEDLINE | ID: mdl-39183357
ABSTRACT

BACKGROUND:

Contactin-associated protein-2(CASPR2) antibody-associated autoimmune encephalitis(AE) is rare in children. This study aimed to report the clinical characteristics and long-term outcome of CASPR2 autoimmunity in children to expand the disease spectrum.

METHODS:

Children who were hospitalized in our hospital with clinically suspected AE from May 2015 to April 2022 and underwent neuronal surface antibodies detections were retrospectively analyzed. Clinical data of patients with CASPR2 autoimmunity were collected.

RESULTS:

Patients who were positive for NMDAR-IgG, CASPR2-IgG, LGI1-IgG and IgLON5-IgG occupied 95.2%(119/125),3.2%(4/125),0.8%(1/125) and 0.8%(1/125), respectively.The median onset age of the 4 patients with CASPR2-IgG was 5.6 years. The most common symptoms were psychiatric symptoms/abnormal behavior(3/4) and sleep dysfunction(3/4). One patient developed a phenotype of Rasmussen encephalitis(RE). Tumor was absent in our patients. Two patients showed abnormal findings on initial brain magnetic resonance imaging(MRI) scans. All the patients showed favorable response to immunotherapy except the patient with RE experienced recurrent symptoms who finally achieved remission after surgery. All the patients had a favorable long-term outcome at the last follow-up(33-58months).

CONCLUSIONS:

CASPR2 autoimmunity may be the second most common anti-neuronal surface antibodies associated neurological disease in children. Psychiatric symptoms/abnormal behavior and sleep disorder were common in children with CASPR2-associated AE. Tumor was rare in those patients. Most pediatric patients had a favorable long-term outcome.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Autoanticuerpos / Encefalitis / Proteínas de la Membrana / Proteínas del Tejido Nervioso Límite: Adolescent / Child / Child, preschool / Female / Humans / Male Idioma: En Revista: Ital J Pediatr Asunto de la revista: PEDIATRIA Año: 2024 Tipo del documento: Article País de afiliación: China Pais de publicación: Reino Unido
Texto completo
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XML
PubMed Links
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Autoanticuerpos / Encefalitis / Proteínas de la Membrana / Proteínas del Tejido Nervioso Límite: Adolescent / Child / Child, preschool / Female / Humans / Male Idioma: En Revista: Ital J Pediatr Asunto de la revista: PEDIATRIA Año: 2024 Tipo del documento: Article País de afiliación: China Pais de publicación: Reino Unido