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. 
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 . He also published on the clinical aspects of neonatal hepatitis . 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.