1043-3155 Pediatr Neurol Briefs pedneurbriefs Pediatr Neurol Briefs Pediatric Neurology Briefs Pediatr Neurol Briefs 2166-6482 1043-3155 2166-3155 Pediatric Neurology Briefs Publishers Chicago, IL, USA PNB-6-83-a 10.15844/pedneurbriefs-6-11-4 Toxic Disorders Neurology Pediatrics Nervous System Diseases Child Development Brain Diseases Neurosurgery Child Infant Manganese Poisoning in Alagille’s Syndrome http://orcid.org/0000-0002-0173-7931 Millichap J. Gordon MD 1 2 * Division of Neurology, Children's Memorial Hospital, Chicago, IL Departments of Pediatrics and Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL Correspondence: Dr. J. Gordon Millichap, E-mail: jgmillichap@northwestern.edu 11 1992 01 07 2016 6 11 83 83 Copyright: © 1992 The Author(s) 1992 This work is licensed under the Creative Commons Attribution 4.0 International License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Hepatic ductular hypoplasia associated with characteristic facies, vertebral malformations, retarded physical, mental, and sexual development, and cardiac murmur

An 8 year old girl with Alagille’s syndrome who developed dystonia secondary to manganese toxicity is reported from the Pediatric Department, Hershey Medical Center, Hershey, PA.

 Manganese Toxicity Alagille’s Syndrome Neurological Syndromes

An 8 year old girl with Alagille’s syndrome who developed dystonia secondary to manganese toxicity is reported from the Pediatric Department, Hershey Medical Center, Hershey, PA. She had developed a mild sensory neuropathy secondary to vitamin E deficiency which was corrected. MRI revealed hyperintense lesions in the globus pallidus bilaterally on T1 weighted images. A CT scan was normal. The serum manganese was 27 ug/1 (normal less than 3). Treatment with ursodeoxycholic acid resulted in a normal serum manganese level. The dystonia cleared within 2 months of therapy. [1]

COMMENT. Long exposure to inhalation of manganese has been known to be associated with neurological syndromes. The present case is unusual because the manganese toxicity was dietary in origin and caused by an impairment of the normal excretion of manganese through the biliary tree. Also, the symptoms were reversible. Wilson’s disease was considered and excluded.

Alagille D. and colleagues described a syndrome of hepatic duct hypoplasia associated with characteristics facies, vertebral malformations, retarded physical, mental and sexual development and cardiac murmur [2]. He also published on the clinical aspects of neonatal hepatitis [3]. The above case of Alagille’s syndrome was characterized by paucity of intrahepatic bile ducts with chronic cholestasis and pulmonic stenosis. Toxicity from dietary sources of manganese appears to require a prolonged period of exposure before neurological symptoms develop.

 Barron TF Manganese neurotoxicity in Alagille’s syndrome: a case report Ann Neurol 1992 Sept 32 453 (abstr) Alagille D Odievre M Gautier M Dommergues JP Hepatic ductular hypoplasia associated with characteristic facies, vertebral malformations, retarded physical, mental, and sexual development, and cardiac murmur J Pediatr 1975 Jan 86 1 63 71 803282 Alagille D Clinical aspects of neonatal hepatitis Am J Dis Child 1972 Apr 123 4 287 291 5027537