Symptomatic manganese neurotoxicity in a patient with chronic liver disease: correlation of clinical symptoms with MRI findings.
Hyperintense symmetric pallidal lesions have been described in chronic hepatic failure. Similar lesions are reported in experimental models of manganese neurotoxicity. We describe an 8-year-old girl with chronic hepatic failure and dystonia in association with an elevated whole blood manganese level and symmetric hyperintense pallidal lesions on magnetic resonance imaging. After hepatic transplantation, her symptoms and signs resolved with normalization of magnetic resonance imaging and the whole blood manganese suggesting that in chronic hepatic failure, the pallidal lesions may be secondary to manganese deposition.
Department of Pediatrics, Milton S. Hershey Medical Center, Hershey, PA 17033.
T F
TF
Barron
A G
AG
Devenyi
A C
AC
Mamourian
D016738:Alagille Syndrome / Q000097:blood / Q000175:diagnosis* / Q000601:surgery; D001480:Basal Ganglia Diseases / Q000097:blood / Q000139:chemically induced* / Q000601:surgery; D002648:Child; D005260:Female; D005917:Globus Pallidus / Q000187:drug effects* / Q000473:pathology; D006501:Hepatic Encephalopathy / Q000097:blood / Q000175:diagnosis* / Q000601:surgery; D006801:Humans; D016031:Liver Transplantation / Q000502:physiology; D008279:Magnetic Resonance Imaging; D008345:Manganese / Q000009:adverse effects* / Q000097:blood; D011183:Postoperative Complications / Q000097:blood / Q000175:diagnosis; D014057:Tomography, X-Ray Computed
D008345:Manganese