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Published on: March 20, 2013
Lowe syndrome: proton mr spectroscopy, and diffusion mr imaging
1Department of Radiology, Ege University Hospital, Bornova, 35100 Izmir, Turkey. rnsener@med.ege.edu.tr
Journal of Neuroradiology = Journal De Neuroradiologie
|September 10, 2004
Summary
Lowe syndrome in a child showed white matter lesions on MRI. Diffusion imaging revealed high apparent diffusion coefficient values, indicating gliosis, not restricted diffusion.
Area of Science:
- Neurology
- Pediatric Radiology
- Neuroimaging
Background:
- Lowe syndrome is a rare X-linked disorder affecting multiple organs, including the brain.
- Cerebral white matter abnormalities are common in Lowe syndrome, but their specific imaging characteristics require further elucidation.
Observation:
- A 3-year-old boy diagnosed with Lowe syndrome presented with bilateral periatrial white matter lesions and cyst-like changes on MRI.
- Proton MR spectroscopy indicated prominent myoinositol peaks, suggestive of gliosis.
Findings:
- Diffusion-weighted imaging (b=1000 s/mm2) did not show restricted diffusion in the periatrial lesions.
- Apparent Diffusion Coefficient (ADC) maps demonstrated significantly elevated ADC values in the lesions (1.76 and 1.66 x 10(-3) mm2/s) compared to normal brain parenchyma (0.81 x 10(-3) mm2/s).
Implications:
- Elevated ADC values in the periatrial lesions are indicative of gliosis, a reactive proliferation of glial cells.
- These findings differentiate gliosis from other potential pathologies like cytotoxic edema or acute infarcts in the context of Lowe syndrome.
- This neuroimaging pattern aids in the accurate diagnosis and understanding of white matter changes in pediatric Lowe syndrome.
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