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Dyskinesia from manganism in a hepatic dysfunction patient
1Department of Pediatrics, Taipei Municipal Women and Children Hospital, Taiwan, ROC.
Summary
Manganese intoxication can cause involuntary movements (dyskinesia) and specific brain MRI changes, even without direct environmental exposure. Chronic liver issues or medical treatments can lead to toxic manganese levels.
Area of Science:
- Neurology
- Toxicology
- Hepatology
Background:
- Common variable hypogammaglobulinemia is an immunodeficiency disorder.
- Hepatosplenomegaly and jaundice can indicate liver dysfunction.
Observation:
- A 14-year-old boy presented with involuntary movements (choreaoathetosis) and signs of liver disease.
- Brain MRI revealed characteristic signal changes in the basal ganglia and brainstem.
- Elevated body manganese levels were confirmed.
Findings:
- The patient exhibited hyperbilirubinemia, hypoalbuminemia, and impaired liver function tests.
- Magnetic resonance imaging (MRI) showed T1-weighted signal abnormalities in manganese-sensitive brain regions.
- High manganese loading was identified as the likely cause of neurological symptoms.
Implications:
- Manganese intoxication should be considered in patients with unexplained dyskinesia and specific neuroimaging findings.
- Chronic liver dysfunction, total parenteral nutrition, or porto-systemic shunts can contribute to manganese accumulation.
- Prompt recognition and management of manganese overload are crucial, although outcomes can be poor in severe cases.