Schizencephaly and Septo optic dysplasia
Heather Borders, MD
over 7 years ago
Please choose a workflow. A standard workflow allows you to browse the repository with full case detail; the academic workflow allows you to browse the repository with limited case detail revealed. Double click on the images to launch image viewer.
Joseph Junewick, MD FACR
|Diagnostic Category: Metabolic
|Created: over 7 years ago
|Updated: over 7 years ago
2 week old with jaundice.
MR – Sagittal and axial T1 and axial GRE images demonstrate hyperintensity in the thalami and globus pallidi.
Kernicterus is the result of toxic accumulation of free bilirubin in the brain. It may present with somnolence, hypotonia, opisthotonus, rigidity, and/or high pitched cry. Many of these symptoms overlap with sepsis, asphyxia, and hypoglycemia.
Bilirubin is usually bound by albumin and is nontoxic but free bilirubin is toxic. Bilirubin is symmetrically deposited in the pallidum, subthalamic nuclei and horn of Ammon and less often in the thalami, striatum and cranial nerve nuclei (III, IV and VI). Bilirubin inhibits astrocyte uptake of glutamine; increased glutamine in the synaptic spaces leads to overstimulation of neurons and neuronal death.
Kernicterus is manifest as high signal on MR, most pronounced on T1 and GRE. Glutamine and glutamate levels are increased and choline levels are decreased on MR spectroscopy. Differential diagnosis for the conventional MR abnormalities in this case would include hypoxia, hypoglycemia, carbon monoxide intoxication, and inborn errors of metabolism.
Matrich-Kriss V, Kollias SS, Ball WS. MR findings in kernicterus. AJNR (1995);16:819-821.
Oakden WK, et al. 1-H MR spectroscopic changes of kernicterus: A possible metabolic signature. AJNR (2005); 26:1571-1574.