Outcome of acute necrotizing encephalopathy in relation to treatment with corticosteroids and gammaglobulin

• Published in: Brain & development (Tokyo. 1979) Vol. 31; no. 3; pp. 221 - 227
• Main Authors: Okumura, Akihisa, Mizuguchi, Masashi, Kidokoro, Hiroyuki, Tanaka, Manabu, Abe, Sinpei, Hosoya, Mitsuaki, Aiba, Hideo, Maegaki, Yoshihiro, Yamamoto, Hitoshi, Tanabe, Takuya, Noda, Eiko, Imataka, George, Kurahashi, Hirokazu
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier B.V 01.03.2009
• Subjects: Acute necrotizing encephalopathy; Adrenal Cortex Hormones - administration & dosage; Adrenal Cortex Hormones - adverse effects; Adrenal Cortex Hormones - therapeutic use; Brain Diseases - drug therapy; Brain Diseases - mortality; Brain Diseases - pathology; Brain Stem - drug effects; Brain Stem - immunology; Brain Stem - pathology; Brainstem lesion; Child; Child, Preschool; Cognition Disorders; Female; gamma-Globulins - therapeutic use; Humans; Infant; Male; Necrosis; Neurology; Outcome; Retrospective Studies; Sex Characteristics; Steroid; Treatment Outcome; Steroid; Acute necrotizing encephalopathy; Outcome; Brainstem lesion
• ISSN: 0387-7604; 1872-7131
• DOI: 10.1016/j.braindev.2008.03.005

Abstract Objective. To examine the relation between outcome and treatment with steroids and gammaglobulin in children with acute necrotizing encephalopathy. Methods. We retrospectively evaluated the clinical course and outcome of 34 children with acute necrotizing encephalopathy. They were divided into two groups; 17 patients with brainstem lesion and 17 patients without brainstem lesion. Early steroid use was defined as when steroids were administered within 24 h after the onset. The outcome was judged as good when a patient had no or mild cognitive impairment and poor when a patient had more severe sequelae, or died. Results. Among patients without brainstem lesions, the outcome was good in 7 of 12 with early steroid, whereas it was poor in all 5 patients without early steroid. There was no significant difference in sex, age, and laboratory data between patients with and without early steroid. The outcome was not correlated with gammaglobulin treatment. As to patients without brainstem lesions, the outcome was not correlated with early steroid or gammaglobulin treatment. Conclusions. Steroid within 24 h after the onset was related to better outcome of children with acute necrotizing encephalopathy without brainstem lesions. Early steroid treatment will be an important option of the treatment for acute necrotizing encephalopathy.