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Published in: BMC Neurology 1/2022

Open Access 01-12-2022 | Magnetic Resonance Imaging | Case report

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

Authors: Yuki Nakajima, Shinya Kobayashi, Hideki Tanoue, Sayaka Ishihara, Ayako Kamiya, Nanako Kawata, Mari Asakura, Daichi Suzuki, Natsuko Obana, Kenta Hayashi, Takahiro Kawaguchi, Masahiro Noda, Kunihiro Oba, Tatsuo Katori, Tsutomu Kageyama, Masashi Ogasawara

Published in: BMC Neurology | Issue 1/2022

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Abstract

Background

Acute 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 presentation

The 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.

Conclusions

ASL may be useful in the early diagnosis of MEEX as well as AESD, facilitating early intervention.
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Metadata
Title
Cerebral blood flow abnormalities with central sparing on arterial spin labeling in mild encephalopathy associated with excitotoxicity: a case report
Authors
Yuki Nakajima
Shinya Kobayashi
Hideki Tanoue
Sayaka Ishihara
Ayako Kamiya
Nanako Kawata
Mari Asakura
Daichi Suzuki
Natsuko Obana
Kenta Hayashi
Takahiro Kawaguchi
Masahiro Noda
Kunihiro Oba
Tatsuo Katori
Tsutomu Kageyama
Masashi Ogasawara
Publication date
01-12-2022
Publisher
BioMed Central
Published in
BMC Neurology / Issue 1/2022
Electronic ISSN: 1471-2377
DOI
https://doi.org/10.1186/s12883-022-02942-5

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