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Encephalitis Lethargica With Isolated Substantia Nigra Lesions Followed by a Second Encephalitis in a Child With Humoral Immunodeficiency.
Pediatr Neurol. 2015 Dec; 53(6):519-22.PN

Abstract

BACKGROUND

Encephalitis lethargica is an encephalitic illness with multiple nervous system symptoms. Lesions only involving substantia nigra on magnetic resonance imaging are uncommon, especially in children. A second encephalitis illness after encephalitis lethargica has never been reported before.

PATIENT DESCRIPTION

We describe a 7-year-old boy with humoral immunity deficiency who developed encephalitis lethargica associated with bilateral substantia nigra lesions on magnetic resonance imaging. After a nearly complete recovery, he developed encephalitis once again. He was diagnosed with encephalitis lethargica with somnolence, akinetic mutism, and ophthalmoplegia after intermittent fever. Cerebrospinal fluid pleocytosis and positive oligoclonal bands were documented. Symmetrical substantia nigra lesions on high-intensity magnetic resonance imaging gradually evolved into a liquid signal. He had almost recovered when he developed fatigue and hypersomnia and was diagnosed with encephalitis again, supported by mild pleocytosis in cerebrospinal fluid and subcortical white matter lesions in the frontal lobes. His symptoms resolved following administration of corticosteroids and immunoglobulins.

CONCLUSION

This is the first report of an immune-deficient child to develop encephalitis lethargica with isolated substantia nigra lesions on magnetic resonance imaging and a second encephalitis illness after recovery from encephalitis lethargica.

Authors+Show Affiliations

Pediatric Department of Shandong University Qilu Hospital, Jinan City, Shandong Province, China.Pediatric Department of Shandong University Qilu Hospital, Jinan City, Shandong Province, China.Pediatric Department of Shandong University Qilu Hospital, Jinan City, Shandong Province, China.Pediatric Department of Shandong University Qilu Hospital, Jinan City, Shandong Province, China.Pediatric Department of Shandong University Qilu Hospital, Jinan City, Shandong Province, China. Electronic address: srpyl2008@sina.com.cn.

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

26431898

Citation

Yang, Lu, et al. "Encephalitis Lethargica With Isolated Substantia Nigra Lesions Followed By a Second Encephalitis in a Child With Humoral Immunodeficiency." Pediatric Neurology, vol. 53, no. 6, 2015, pp. 519-22.
Yang L, Jia G, Li B, et al. Encephalitis Lethargica With Isolated Substantia Nigra Lesions Followed by a Second Encephalitis in a Child With Humoral Immunodeficiency. Pediatr Neurol. 2015;53(6):519-22.
Yang, L., Jia, G., Li, B., Lei, G., & Sun, R. (2015). Encephalitis Lethargica With Isolated Substantia Nigra Lesions Followed by a Second Encephalitis in a Child With Humoral Immunodeficiency. Pediatric Neurology, 53(6), 519-22. https://doi.org/10.1016/j.pediatrneurol.2015.03.030
Yang L, et al. Encephalitis Lethargica With Isolated Substantia Nigra Lesions Followed By a Second Encephalitis in a Child With Humoral Immunodeficiency. Pediatr Neurol. 2015;53(6):519-22. PubMed PMID: 26431898.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Encephalitis Lethargica With Isolated Substantia Nigra Lesions Followed by a Second Encephalitis in a Child With Humoral Immunodeficiency. AU - Yang,Lu, AU - Jia,Guijuan, AU - Li,Baomin, AU - Lei,Gefei, AU - Sun,Ruopeng, Y1 - 2015/06/18/ PY - 2015/03/21/received PY - 2015/03/21/accepted PY - 2015/10/4/entrez PY - 2015/10/4/pubmed PY - 2016/9/28/medline KW - encephalitis lethargica KW - immunological deficiency KW - parkinsonism KW - substantia nigra SP - 519 EP - 22 JF - Pediatric neurology JO - Pediatr Neurol VL - 53 IS - 6 N2 - BACKGROUND: Encephalitis lethargica is an encephalitic illness with multiple nervous system symptoms. Lesions only involving substantia nigra on magnetic resonance imaging are uncommon, especially in children. A second encephalitis illness after encephalitis lethargica has never been reported before. PATIENT DESCRIPTION: We describe a 7-year-old boy with humoral immunity deficiency who developed encephalitis lethargica associated with bilateral substantia nigra lesions on magnetic resonance imaging. After a nearly complete recovery, he developed encephalitis once again. He was diagnosed with encephalitis lethargica with somnolence, akinetic mutism, and ophthalmoplegia after intermittent fever. Cerebrospinal fluid pleocytosis and positive oligoclonal bands were documented. Symmetrical substantia nigra lesions on high-intensity magnetic resonance imaging gradually evolved into a liquid signal. He had almost recovered when he developed fatigue and hypersomnia and was diagnosed with encephalitis again, supported by mild pleocytosis in cerebrospinal fluid and subcortical white matter lesions in the frontal lobes. His symptoms resolved following administration of corticosteroids and immunoglobulins. CONCLUSION: This is the first report of an immune-deficient child to develop encephalitis lethargica with isolated substantia nigra lesions on magnetic resonance imaging and a second encephalitis illness after recovery from encephalitis lethargica. SN - 1873-5150 UR - https://www.unboundmedicine.com/medline/citation/26431898/Encephalitis_Lethargica_With_Isolated_Substantia_Nigra_Lesions_Followed_by_a_Second_Encephalitis_in_a_Child_With_Humoral_Immunodeficiency_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0887-8994(15)00178-2 DB - PRIME DP - Unbound Medicine ER -