Evoked potential abnormalities in children with chronic cholestasis

Area of Science:

• Pediatric Neurology
• Hepatology
• Neurophysiology

Background:

• Chronic cholestasis in children can impact nervous system function.
• Vitamin E and A deficiencies are potential contributors to neurological issues in liver disease.

Purpose of the Study:

• To evaluate the effects of chronic cholestasis and vitamin E deficiency on nervous system function in children.
• To investigate the relationship between cholestasis, vitamin status, and evoked potential abnormalities.

Main Methods:

• Multimodality evoked potential testing (visual, brainstem auditory, somatosensory) in 17 children with chronic liver disease.
• Periodic retesting in 11 patients over 1-33 months.
• Correlation of evoked potential findings with serum bile acid levels and vitamin E/A status.

Main Results:

• Eight children showed abnormal visual evoked potentials (P100 peak delay), correlated with higher serum bile acid levels.
• Six patients initially had auditory evoked potential abnormalities; four with arteriohepatic dysplasia had persistent hearing loss.
• Somatosensory evoked potentials were mildly abnormal in four children due to peripheral neuropathy.
• Vitamin E and A levels did not consistently correlate with evoked potential abnormalities.
• Visual evoked potentials normalized in four patients following cholestasis improvement, particularly after liver transplantation.

Conclusions:

• Frequent, potentially reversible visual system abnormalities are associated with cholestasis in children.
• These visual abnormalities are not solely attributable to vitamin E and/or A deficiency.
• Evoked potential testing is a valuable tool for assessing neurological function in pediatric liver disease.