Underlying Disorders in Children With Infection-Related Acute Encephalopathy

Tomohiko Nakata; Jun Natsume; Hiroyuki Yamamoto; Yuji Ito; Takeshi Suzuki; Masahiro Kawaguchi; Anna Shiraki; Sumire Kumai; Fumi Sawamura; Ryosuke Suzui; Takamasa Mitsumatsu; Hajime Narita; Takeshi Tsuji; Tetsuo Kubota; Shinji Saitoh; Akihisa Okumura; Hiroyuki Kidokoro

doi:10.1016/j.pediatrneurol.2024.03.017

Underlying Disorders in Children With Infection-Related Acute Encephalopathy

Pediatr Neurol. 2024 Mar 22:155:126-132. doi: 10.1016/j.pediatrneurol.2024.03.017. Online ahead of print.

Authors

Tomohiko Nakata¹, Jun Natsume², Hiroyuki Yamamoto³, Yuji Ito³, Takeshi Suzuki⁴, Masahiro Kawaguchi⁵, Anna Shiraki⁶, Sumire Kumai³, Fumi Sawamura⁷, Ryosuke Suzui³, Takamasa Mitsumatsu³, Hajime Narita³, Takeshi Tsuji⁸, Tetsuo Kubota⁹, Shinji Saitoh¹⁰, Akihisa Okumura¹¹, Hiroyuki Kidokoro³

Affiliations

¹ Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan. Electronic address: tomonakata@med.nagoya-u.ac.jp.
² Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan; Department of Developmental Disability Medicine, Nagoya University Graduate School of Medicine, Nagoya, Japan.
³ Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan.
⁴ Department of Pediatrics, Okazaki City Hospital, Okazaki, Japan.
⁵ Division of Neurology, Aichi Children's Health and Medical Center, Obu, Japan.
⁶ Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan; Department of Pediatrics, Nagoya Memorial Hospital, Nagoya, Japan.
⁷ Department of Pediatrics, Aichi Aoitori Medical Welfare Center, Nagoya, Japan.
⁸ Department of Pediatrics, Aichi Prefecture Mikawa Aoitori Medical and Rehabilitation Center for Developmental Disabilities, Okazaki, Japan.
⁹ Department of Pediatrics, Anjo Kosei Hospital, Anjo, Japan.
¹⁰ Department of Pediatrics and Neonatology, Nagoya City University Graduate School of Medical Sciences, Aichi, Japan.
¹¹ Department of Pediatrics, Aichi Medical University, Aichi, Japan.

PMID: 38636169
DOI: 10.1016/j.pediatrneurol.2024.03.017

Abstract

Background: Various factors contribute to the development of infection-related acute encephalopathy (AE) in children, such as infectious agents and chronic underlying disorders. We studied underlying disorders in children with AE to identify predisposing factors of AE.

Methods: We investigated underlying disorders or past histories in patients with two types of AE from the database in the Tokai area of Japan between 2009 and 2022: 204 patients with AE with reduced subcortical diffusion (AED) and 137 with clinically mild encephalopathy with a reversible splenial lesion (MERS). We compared them with 89 patients with acute disseminated encephalomyelitis (ADEM) to clarify the specific disorders in the two AE types.

Results: The prevalence of underlying disorders in AED (34%, 70 patients) was significantly higher than that in ADEM (12%, 11 patients) (P < 0.01). The prevalence of underlying disorders in MERS was 23% (32 patients). The underlying disorders included seizure disorders, premature birth, genetic/congenital disorders, and endocrine/renal diseases. In patients with seizure disorders in AED, five patients (18%) had Dravet syndrome and four (15%) had West syndrome, whereas none with MERS had these syndromes. Twenty-five (12%) of 204 patients with AED, three (2%) with MERS, and one (1%) with ADEM were preterm or low birth weight.

Conclusions: The high prevalence of seizure disorders suggests that seizure susceptibility is an important predisposing factor in AED. Premature birth also has an impact on the development of AED. Caution is required regarding the development of AE in patients with chronic seizure disorders or premature birth.

Keywords: Acute disseminated encephalomyelitis; Acute encephalopathy; Acute encephalopathy with biphasic seizures and late reduced diffusion; Clinically mild encephalitis/encephalopathy with a reversible splenial lesion; Magnetic resonance imaging; Underlying disorder.