Selenium Deficiency Triggering Intractable Seizures

• DOI: 10.1055/s-2008-1073025
• Publication Date: 2008-11-07T07:28:59Z
• Authors: V. Ramaekers; M. Calomme; D. Vanden Berghe; W. Makropoulos
• Keywords: Selenium deficiency; Liver function

Two children with severe neurodevelopmental retardation and elevated liver function tests developed intractable seizures during the first year of life. Detectable neurometabolic conditions have been ruled out. At time evidence for systemic selenium deficiency could be documented. The youngest patient, who manifested fits from fourth day life, died at age ten months. Neuropathologic examination was consistent Progressive Neuronal Degeneration Childhood (PNDC) disease or formerly known as Alpers disease. In oldest child, whose diet normally balanced, started 11 months features long-standing became apparent 1 1/2 years consisted disturbances, depigmented hair osteoarthropathy Oral substitution supplements in both (3-5 µg/kg body weight) resulted reduction improvement EEG recordings after two weeks while normal.