A progressive neurologic syndrome in children with chronic liver disease.
A progressive neurologic syndrome developed in six children with longstanding cholestatic liver disease. The neurologic abnormalities included areflexia, gait disturbance, decreased proprioceptive and vibratory sensation, and paresis of gaze. Serum vitamin E concentrations were uniformly low. Neuropathological studies carried out in two of the three fatal cases revealed degeneration of the posterior column, selective loss of large-caliber, myelinated axons in peripheral nerve, and spheroids in the gracile and cuneate nuclei. These lesions are similar to those found in animals with experimentally induced vitamin E deficiency. We therefore speculate that the neurologic syndrome in these children may be the result of chronic vitamin E malabsorption.
D000293:Adolescent; D002648:Child; D002779:Cholestasis / Q000150:complications*; D002780:Cholestasis, Intrahepatic / Q000150:complications*; D005133:Eye Movements; D005684:Gait; D006801:Humans; D009422:Nervous System Diseases / Q000209:etiology* / Q000473:pathology; D010525:Peripheral Nerves / Q000473:pathology; D011434:Proprioception; D012026:Reflex, Stretch; D012677:Sensation; D013116:Spinal Cord / Q000473:pathology; D013577:Syndrome; D014732:Vibration; D014811:Vitamin E Deficiency / Q000150:complications* / Q000473:pathology