Year : 2009 | Volume
: 4 | Issue : 2 | Page : 120--121
Magnetic resonance imaging of bilateral mesial temporal sclerosis in chronic kernicterus
Virginia C Mendes1, Fernando Costa1, Antonia Silva2, Maria Jose Rosas2,
1 Department of Neuroradiology, Hospital de S. Joćo, Porto, Portugal
2 Department of Neurology, Hospital de S. Joćo, Porto, Portugal
Virginia C Mendes
Serviēo de Neurorradiologia, Hospital de S. Joćo, Alameda Prof. Hernāni Monteiro, 4000 - Porto
Kernicterus is an encephalopathy resulting from the cerebral deposition of unconjugated bilirubin in the neonatal period. We report a case of kernicterus where MRI demonstrated bilateral symmetric high signal intensity and volume loss in the hippocampus in addition to globus pallidus and subthalamic nucleus hyperintensity onT2-weighted images. The authors believe that hippocampal sclerosis, plays a role in adding specificity to the imaging diagnosis of kernicterus.
|How to cite this article:|
Mendes VC, Costa F, Silva A, Rosas MJ. Magnetic resonance imaging of bilateral mesial temporal sclerosis in chronic kernicterus.J Pediatr Neurosci 2009;4:120-121
|How to cite this URL:|
Mendes VC, Costa F, Silva A, Rosas MJ. Magnetic resonance imaging of bilateral mesial temporal sclerosis in chronic kernicterus. J Pediatr Neurosci [serial online] 2009 [cited 2020 Jun 5 ];4:120-121
Available from: http://www.pediatricneurosciences.com/text.asp?2009/4/2/120/57339
Kernicterus is an encephalopathy resulting from the cerebral deposition of unconjugated bilirubin in the neonatal period. The most common finding on magnetic resonance imaging (MRI) is increased signal intensity of the globus pallidus on T2-weighted images.  We present a case of kernicterus where MRI demonstrated bilateral symmetric high signal intensity and volume loss in the hippocampus in addition to globus pallidus and subthalamic nucleus hyperintensity on T2-weighted images.
A 9-year-old black girl was admitted for investigation of dystonia and severe developmental delay. She was born after a term pregnancy and normal delivery. Her mother had malaria during pregnancy and had been treated with chloroquine. The patient had no history of perinatal asphyxia, metabolic abnormalities, or ABO/Rh isoimmunization. At the third postnatal day, she presented with jaundice (the level of total bilirubin is unknown), fever, and irritability. She underwent antibiotics and phototherapy. After treatment, axial hypotonia and limitation of vertical gaze movements were noted. At 6 months old age, serious psicomotor delay and generalized dystonia developed. Currently, neurological examination revealed mental retardation, dystonia, and spastic tetraparesis. Brain MRI demonstrated bilaterally symmetric hyperintensities in the globus pallidus and subthalamic nucleus. Bilateral hippocampal volume loss and high signal, resembling bilateral mesial temporal sclerosis, were also observed [Figure 1]. Blood analyses were normal, including copper and ceruloplasmin; bacterial cultures and polymerase chain reactions studies for CMV, toxoplasma, and HSV were negative. The screening for mitochondrial disorders, including muscle biopsy, respiratory chain enzymes activity, and analysis for mutations of mitochondrial DNA, was negative. The patient was treated with trihexyphenidyl (Artane® ) until 30 mg/day, with improvement of the generalized dystonia.
The widespread use of phototherapy and exchange transfusion has greatly reduced the incidence of kernicterus. MRI findings in chronic kernicterus are characteristic, with bilateral high signal of the pallida and subthalamic nucleus, but non-specific, and can be found in various disorders, such as hypoxic ischemic encephalopathy, mitochondrial disorders, inborn errors of metabolism, hemolytic uremic syndrome, and carbon monoxide poisoning.  Although mesial temporal sclerosis is a common pathological finding in patients with kernicterus, it is only exceptionally described in MRI reports.  Interestingly, as in our patient, none of those had epilepsy. This is in contradistinction to the pallidal involvement that shows a good clinical correlation, as movement disorders are typical. Nevertheless, we believe that hippocampal sclerosis, demonstrated by MRI, plays a role in adding specificity to the imaging diagnosis of kernicterus.
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