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Modeling Mitochondrial Disease Using Brain Organoids: A Focus on Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-like Episodes
Published on: October 10, 2025
Elevated thyroid peroxidase antibodies with encephalopathy in MELAS syndrome
Derrick W S Chan1, C C Tchoyoson Lim, Stacey K H Tay
1Pediatric Neurology Service, Department of Pediatric Medicine, KK Women's and Children's Hospital, Singapore. derrickchanws@gmail.com
Pediatric Neurology
|June 15, 2007
Summary
Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome can mimic Hashimoto's encephalopathy, presenting with steroid-responsive encephalopathy and elevated thyroid antibodies. Suspect MELAS syndrome if lactic acidosis and characteristic MRI findings are present.
Area of Science:
- Neurology
- Endocrinology
- Genetics
Background:
- Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome and Hashimoto's encephalopathy (HE) can present with nonspecific encephalopathy.
- HE is characterized by steroid-responsive encephalopathy and elevated thyroid peroxidase antibodies (TPOAbs).
- MELAS syndrome typically presents with stroke-like episodes and lactic acidosis, and steroid-responsive encephalopathy is not a common feature.
Observation:
- A patient with goiter, recurrent encephalopathy, and elevated TPOAbs showed apparent response to steroids.
- Magnetic resonance imaging (MRI) findings were atypical for HE.
- Diagnosis of MELAS syndrome was established.
Findings:
- MELAS syndrome can present with apparent steroid-responsive encephalopathy and elevated TPOAbs, mimicking HE.
- Lactic acidosis and characteristic MRI findings are crucial for diagnosing MELAS syndrome in such cases.
Implications:
- This case highlights the importance of considering MELAS syndrome in patients with apparent HE.
- Diagnostic workup for MELAS syndrome should include assessment for lactic acidosis and characteristic neuroimaging.
- Accurate diagnosis is critical for appropriate management and prognosis of these distinct neurological conditions.
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