title: Encephalitis Lethargica With Isolated Substantia Nigra Lesions Followed by a Second Encephalitis in a Child With Humoral Immunodeficiency.
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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.affiliation: Pediatric Department of Shandong University Qilu Hospital, Jinan City, Shandong Province, China.
authors.forename: Lu
authors.initials: L
authors.lastname: Yang
authors.affiliation: Pediatric Department of Shandong University Qilu Hospital, Jinan City, Shandong Province, China.
authors.forename: Guijuan
authors.initials: G
authors.lastname: Jia
authors.affiliation: Pediatric Department of Shandong University Qilu Hospital, Jinan City, Shandong Province, China.
authors.forename: Baomin
authors.initials: B
authors.lastname: Li
authors.affiliation: Pediatric Department of Shandong University Qilu Hospital, Jinan City, Shandong Province, China.
authors.forename: Gefei
authors.initials: G
authors.lastname: Lei
authors.affiliation: Pediatric Department of Shandong University Qilu Hospital, Jinan City, Shandong Province, China. Electronic address: srpyl2008@sina.com.cn.
authors.forename: Ruopeng
authors.initials: R
authors.lastname: Sun
mesh_terms: D002648:Child; D004660:Encephalitis / Q000473:pathology* / Q000503:physiopathology*; D006801:Humans; D056724:Immunity, Humoral / Q000276:immunology*; D007153:Immunologic Deficiency Syndromes / Q000276:immunology*; D053609:Lethargy / Q000503:physiopathology*; D008297:Male; D013378:Substantia Nigra / Q000473:pathology*
keywords: encephalitis lethargica; immunological deficiency; parkinsonism; substantia nigra