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Clinical characteristics of anti-N-methyl-D-aspartate receptor encephalitis in children.
Zhong Nan Da Xue Xue Bao Yi Xue Ban. 2020 Jan 28; 45(1):47-54.ZN

Abstract

OBJECTIVES

To analyze the clinical characteristics and prognosis of children with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis and to provide a basis for early clinical identification of this disease.

METHODS

The clinical data of 42 cases of anti-NMDAR encephalitis at Department of Pediatrics, Second Xiangya Hospital, Central South University from January 2015 to March 2018 were collected. The clinical features and followed-up outcomes were analyzed retrospectively.

RESULTS

There were 15 cases (35.7%) of males and 27 cases (64.3%) of females in 42 children, with a ratio of 1꞉1.8. They were aged from 4 months to 17 years, with an average of (9.20±4.66) years. The most common initial symptoms were seizures (47.6%, 20/42) and mental behavior disorder (35.7%, 15/42). During the course of the disease, 85.7% patients(36/42) had mental and behavior disorder, 85.7% patients (36/42) had epilepsy, 76.2% (32/42) had speech disorder, 66.7% patients (28/42) had dyskinesia, 66.7% patients (28/42) had the decreased level of consciousness, 61.9% patients (26/42) had autonomic instability, and 57.1% (24/42) patients had sleep disorder. All the children had positive antibody against NMDA receptor resistance encephalitis in cerebrospinal fluid. Head MRI showed the abnormal incidence was 50.0% (21/42), and the lesions involved in parietal lobe, frontal lobe, temporal lobe, occipital lobe, midbrain, thalamus, basal ganglia and optic nerve. There was a patient with optic nerve damage combined with myelin oligodendrocyte glycoprotein (MOG) antibody positive. Forty cases were examined by electroencephalogram (EEG), 92.5% cases (37/40) were abnormal, mainly showing diffuse slow waves, and δ brushes could be seen in severe cases. And there was 1 patient (2.4%) complicated with mesenteric teratoma. The mRS score (2.14±1.46) at discharge was significantly lower than the highest mRS score (3.88±1.38) during hospitalization (P<0.05). After 3-39 months of follow-up, mRS score at 3 months after discharge was only 0.81±1.29, which was still improved compared with that at discharge, 76.2% cases (32/42) experienced complete or near-complete recovery (mRS score≤2), and 4.8% (2/42) cases relapsed. There was no mortality; the initial time of immunotherapy and the highest mRS score in the course of the disease were the factors affecting the prognosis. The earlier the starting time for immunotherapy and the lower mRS score in the course of the disease were, the better the prognosis was.

CONCLUSIONS

Seizures, mental and behavior disorder, dyskinesias, speech disorder and autonomic instability are common clinical manifestations of anti-NMDAR encephalitis in children. The effect of immunotherapy is significant, and the time to start immunotherapy and the severity of the disease are important factors affecting the prognosis. Anti-NMDAR encephalitis can be combined with other autoantibodies, but its clinical significance and mechanism need further study.

Authors+Show Affiliations

Department of Pediatrics, Second Xiangya Hospital, Central South University, Changsha 410011, China. Childern's Brain Development and Brain Injury Research Office, Second Xiangya Hospital, Central South University, Changsha 410011, China.Department of Pediatrics, Second Xiangya Hospital, Central South University, Changsha 410011, China. Childern's Brain Development and Brain Injury Research Office, Second Xiangya Hospital, Central South University, Changsha 410011, China.Department of Pediatrics, Second Xiangya Hospital, Central South University, Changsha 410011, China. Childern's Brain Development and Brain Injury Research Office, Second Xiangya Hospital, Central South University, Changsha 410011, China.Department of Pediatrics, Second Xiangya Hospital, Central South University, Changsha 410011, China. Childern's Brain Development and Brain Injury Research Office, Second Xiangya Hospital, Central South University, Changsha 410011, China.Department of Pediatrics, Second Xiangya Hospital, Central South University, Changsha 410011, China. Childern's Brain Development and Brain Injury Research Office, Second Xiangya Hospital, Central South University, Changsha 410011, China.Department of Pediatrics, Second Xiangya Hospital, Central South University, Changsha 410011, China. Childern's Brain Development and Brain Injury Research Office, Second Xiangya Hospital, Central South University, Changsha 410011, China.Department of Pediatrics, Second Xiangya Hospital, Central South University, Changsha 410011, China. Childern's Brain Development and Brain Injury Research Office, Second Xiangya Hospital, Central South University, Changsha 410011, China.Department of Pediatrics, Second Xiangya Hospital, Central South University, Changsha 410011, China. Childern's Brain Development and Brain Injury Research Office, Second Xiangya Hospital, Central South University, Changsha 410011, China.Department of Pediatrics, Second Xiangya Hospital, Central South University, Changsha 410011, China. Childern's Brain Development and Brain Injury Research Office, Second Xiangya Hospital, Central South University, Changsha 410011, China.Department of Pediatrics, Second Xiangya Hospital, Central South University, Changsha 410011, China. Childern's Brain Development and Brain Injury Research Office, Second Xiangya Hospital, Central South University, Changsha 410011, China.

Pub Type(s)

Journal Article

Language

eng chi

PubMed ID

32132297

Citation

Zheng, Jing, et al. "Clinical Characteristics of anti-N-methyl-D-aspartate Receptor Encephalitis in Children." Zhong Nan Da Xue Xue Bao. Yi Xue Ban = Journal of Central South University. Medical Sciences, vol. 45, no. 1, 2020, pp. 47-54.
Zheng J, Shen J, Wang A, et al. Clinical characteristics of anti-N-methyl-D-aspartate receptor encephalitis in children. Zhong Nan Da Xue Xue Bao Yi Xue Ban. 2020;45(1):47-54.
Zheng, J., Shen, J., Wang, A., Liu, L., Xiong, J., Li, X., Xiao, Y., Li, J., Mao, D., & Liu, L. (2020). Clinical characteristics of anti-N-methyl-D-aspartate receptor encephalitis in children. Zhong Nan Da Xue Xue Bao. Yi Xue Ban = Journal of Central South University. Medical Sciences, 45(1), 47-54. https://doi.org/10.11817/j.issn.1672-7347.2020.180761
Zheng J, et al. Clinical Characteristics of anti-N-methyl-D-aspartate Receptor Encephalitis in Children. Zhong Nan Da Xue Xue Bao Yi Xue Ban. 2020 Jan 28;45(1):47-54. PubMed PMID: 32132297.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Clinical characteristics of anti-N-methyl-D-aspartate receptor encephalitis in children. AU - Zheng,Jing, AU - Shen,Jinfeng, AU - Wang,Aiping, AU - Liu,Lingjuan, AU - Xiong,Jie, AU - Li,Xingfang, AU - Xiao,Yangyang, AU - Li,Jian, AU - Mao,Ding'an, AU - Liu,Liqun, PY - 2020/3/6/entrez PY - 2020/3/7/pubmed PY - 2020/3/10/medline KW - anti-N-methyl- D-aspartate receptor encephalitis KW - children KW - clinical characteristics KW - immuno- therapy KW - prognosis SP - 47 EP - 54 JF - Zhong nan da xue xue bao. Yi xue ban = Journal of Central South University. Medical sciences JO - Zhong Nan Da Xue Xue Bao Yi Xue Ban VL - 45 IS - 1 N2 - OBJECTIVES: To analyze the clinical characteristics and prognosis of children with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis and to provide a basis for early clinical identification of this disease. METHODS: The clinical data of 42 cases of anti-NMDAR encephalitis at Department of Pediatrics, Second Xiangya Hospital, Central South University from January 2015 to March 2018 were collected. The clinical features and followed-up outcomes were analyzed retrospectively. RESULTS: There were 15 cases (35.7%) of males and 27 cases (64.3%) of females in 42 children, with a ratio of 1꞉1.8. They were aged from 4 months to 17 years, with an average of (9.20±4.66) years. The most common initial symptoms were seizures (47.6%, 20/42) and mental behavior disorder (35.7%, 15/42). During the course of the disease, 85.7% patients(36/42) had mental and behavior disorder, 85.7% patients (36/42) had epilepsy, 76.2% (32/42) had speech disorder, 66.7% patients (28/42) had dyskinesia, 66.7% patients (28/42) had the decreased level of consciousness, 61.9% patients (26/42) had autonomic instability, and 57.1% (24/42) patients had sleep disorder. All the children had positive antibody against NMDA receptor resistance encephalitis in cerebrospinal fluid. Head MRI showed the abnormal incidence was 50.0% (21/42), and the lesions involved in parietal lobe, frontal lobe, temporal lobe, occipital lobe, midbrain, thalamus, basal ganglia and optic nerve. There was a patient with optic nerve damage combined with myelin oligodendrocyte glycoprotein (MOG) antibody positive. Forty cases were examined by electroencephalogram (EEG), 92.5% cases (37/40) were abnormal, mainly showing diffuse slow waves, and δ brushes could be seen in severe cases. And there was 1 patient (2.4%) complicated with mesenteric teratoma. The mRS score (2.14±1.46) at discharge was significantly lower than the highest mRS score (3.88±1.38) during hospitalization (P<0.05). After 3-39 months of follow-up, mRS score at 3 months after discharge was only 0.81±1.29, which was still improved compared with that at discharge, 76.2% cases (32/42) experienced complete or near-complete recovery (mRS score≤2), and 4.8% (2/42) cases relapsed. There was no mortality; the initial time of immunotherapy and the highest mRS score in the course of the disease were the factors affecting the prognosis. The earlier the starting time for immunotherapy and the lower mRS score in the course of the disease were, the better the prognosis was. CONCLUSIONS: Seizures, mental and behavior disorder, dyskinesias, speech disorder and autonomic instability are common clinical manifestations of anti-NMDAR encephalitis in children. The effect of immunotherapy is significant, and the time to start immunotherapy and the severity of the disease are important factors affecting the prognosis. Anti-NMDAR encephalitis can be combined with other autoantibodies, but its clinical significance and mechanism need further study. SN - 1672-7347 UR - https://www.unboundmedicine.com/medline/citation/32132297/Clinical_characteristics_of_anti_N_methyl_D_aspartate_receptor_encephalitis_in_children_ L2 - http://xbyxb.csu.edu.cn/xbwk/fileup/PDF/20200147.pdf DB - PRIME DP - Unbound Medicine ER -