Cerebral blood flow abnormalities with central sparing on arterial spin labeling in mild encephalopathy associated with excitotoxicity: a case report

BMC Neurology

卷:22

Case Report

Tsutomu Kageyama¹  Yuki Nakajima²  Hideki Tanoue²  Mari Asakura²  Daichi Suzuki²  Kenta Hayashi²  Ayako Kamiya²  Natsuko Obana²  Kunihiro Oba²  Nanako Kawata²  Sayaka Ishihara²  Takahiro Kawaguchi²  Masahiro Noda²  Tatsuo Katori²  Shinya Kobayashi²  Masashi Ogasawara³ 

[1] Center for Emergency Preparedness and Response, National Institute of Infectious Diseases, Tokyo, Japan;

[2] Department of Pediatrics, Showa General Hospital, Tokyo, Japan;

[3] Department of Pediatrics, Showa General Hospital, Tokyo, Japan;Department of Neuromuscular Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry (NCNP), 4-1-1 Ogawahigashi, Kodaira, Tokyo, Japan;

关键词: Acute encephalopathy with biphasic seizures and late reduced diffusion;    Mild encephalopathy associated with excitotoxicity;    Arterial spin labeling;    Magnetic resonance imaging;    Cerebral blood flow;    Glutamate;    Glutamine;    Early diagnosis;   

DOI  :  10.1186/s12883-022-02942-5

 received in 2021-10-29, accepted in 2022-10-27,  发布年份 2022

来源: Springer

【 摘 要 】

BackgroundAcute encephalopathy with biphasic seizures and late reduced diffusion (AESD) and mild encephalopathy associated with excitotoxicity (MEEX) are the most frequent acute encephalopathies in pediatric patients in Japan. AESD typically presents with biphasic seizures and delayed reduced diffusion in the subcortical area, called bright tree appearance (BTA), on radiological examination. In patients with AESD, arterial spin labeling (ASL) shows decreased cerebral blood flow (CBF) in the hyperacute stage and increased CBF in the acute stage, suggesting the usefulness of ASL for the early diagnosis of AESD. Additionally, proton magnetic resonance spectroscopy (MRS) shows elevated glutamate (Glu) and glutamine (Gln) in AESD. MEEX is a group of mild encephalopathies with transient elevation of Gln on MRS similar to that in AESD; however, MEEX does not include any clinical biphasic course or abnormalities, including BTA on diffusion-weighted imaging. Although the usefulness of ASL for AESD has been reported, there are no reports for patients with MEEX. In this study, we report our experience with a 4-year-old girl diagnosed with MEEX who showed unique findings on ASL.Case presentationThe patient was a 4-year-old girl admitted to the emergency room with febrile status epilepticus. Considering the possibility of AESD, vitamin therapy was initiated. ASL-MR imaging (MRI) of the brain performed on the second day showed increased blood flow in the frontal, temporal, and occipital regions with spared central sulcus, which indicated AESD with central sparing. The patient was diagnosed with AESD, and the treatment included pulse steroid therapy and immunoglobulin therapy from day 3. The patient remained mildly unconscious but gradually became conscious by day 7 with no seizures. Brain MRI performed on day 8 did not show any characteristic AESD findings, such as BTA. Furthermore, MRS showed elevated Gln, which, along with the clinical course, led to the diagnosis of MEEX. The patient was discharged on day 16 without obvious sequelae.ConclusionsASL may be useful in the early diagnosis of MEEX as well as AESD, facilitating early intervention.

【 授权许可】

CC BY   
© The Author(s) 2022

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