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Updated: Jul 3, 2026
08:09
Generation of Human Brain Organoids for Mitochondrial Disease Modeling
Published on: June 21, 2021
Diaschisis in cerebral mitochondrial disorder
Vinod V Narla1, Ashok Muthukrishnan, James M Mountz
1Department of Radiology, Division of Nuclear Medicine, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania 15213, USA.
Clinical Nuclear Medicine
|July 23, 2008
Summary
Cerebral mitochondrial disorder can cause extensive brain dysfunction. Regional cerebral blood flow (rCBF) SPECT imaging effectively detects and monitors brain damage, especially when MRI findings are subtle.
Area of Science:
- Neuroscience
- Medical Imaging
- Biochemistry
Background:
- Cerebral mitochondrial disorders are rare genetic conditions affecting brain function.
- Early diagnosis and monitoring are crucial for managing neurological deficits.
Observation:
- A 10-year-old girl with a history of neurological issues presented with symptoms suggestive of cerebral mitochondrial disorder.
- Advanced imaging techniques including 1H magnetic resonance spectroscopic imaging, MRI, and Tc-99m ethylcysteinate dimer brain SPECT were employed.
Findings:
- Spectroscopy revealed decreased N-acetylaspartate and increased choline in cerebellar hemispheres, suggesting lipid accumulation.
- MRI showed delayed myelination, while rCBF SPECT indicated reduced blood flow in the occipital lobes.
- Parametric statistical mapping of rCBF confirmed significant reductions, consistent with gray matter abnormality or white matter diaschisis.
Implications:
- Regional cerebral blood flow (rCBF) SPECT with parametric statistical mapping is valuable for detecting subtle brain damage in mitochondrial disorders.
- This imaging approach aids in assessing the extent of cerebral dysfunction and monitoring disease progression over time.
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