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Unusual presentation of acute encephalopathy with biphasic seizures and late reduced diffusion in Miller–Dieker syndrome
  1. Satoru Kobayashi1,
  2. Mai Kamishima2,
  3. Kyoko Yokoi1 and
  4. Satoshi Suzuki1
  1. 1Department of Pediatrics, Nagoya City University West Medical Center, Nagoya, Aichi, Japan
  2. 2Department of Radiology, Nagoya City University West Medical Center, Nagoya, Aichi, Japan
  1. Correspondence to Dr Satoru Kobayashi; s.kobayashi.22{at}


Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is a unique subtype of acute encephalopathy that occurs in children. A girl aged 2 years and 8 months with Miller-Dieker syndrome (MDS) was admitted for status epilepticus and high fever. Brain MRI performed on the third day postadmission showed abnormally high intensities in the subcortical white matter on diffusion-weighted images. Acute encephalitis/encephalopathy was diagnosed based on the electroencephalography (EEG) findings of diffuse high-voltage delta waves. Six days postadmission, frequent apnoeic episodes were observed, with oxygen desaturation due to cluster seizures. Subclinical seizures were found on amplitude-integrated EEG (aEEG). The disturbance of consciousness was difficult to recognise because of severe developmental disabilities due to MDS. EEG aids in the evaluation of consciousness, and aEEG can be helpful in monitoring and controlling subclinical seizures in the biphasic phase of AESD, especially in patients with underlying neurological disorders.

  • congenital disorders
  • developmental paediatrics
  • cerebral palsy
  • clinical neurophysiology
  • epilepsy and seizures

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  • Contributors SK and MK collected and analysed data. SK created the initial draft. KY and SS provided critical revision of the manuscript for important intellectual content. All authors read and approved the final manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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