SELENIUM DEFICIENCY TRIGGERING INTRACTABLE SEIZURES

Two children with severe neurodevelopmental retardation and elevated l iver function tests developed intractable seizures during the first ye ar of Life. Detectable neurometabolic conditions have been ruled out. At the time of seizures evidence for systemic selenium deficiency coul d be documented. The youngest patient, who manifested intractable fits from the fourth day of life, died at the age of ten months. Neuropath ologic examination was consistent with Progressive Neuronal Degenerati on of Childhood (PNDC) with liver disease or formerly known as Alpers disease. In the oldest child, whose diet was normally balanced, fits s tarted from the age of 11 months and features of long-standing seleniu m deficiency became apparent from the age of 11/2 years and consisted of liver function disturbances, depigmented hair and osteoarthropathy. Oral substitution with selenium supplements in both children (3-5 mu g/kg body weight) resulted in reduction of seizures and improvement of the EEG recordings after two weeks while Liver function became normal . Two of the seleno-dependent enzymes Glutathione Peroxidase (GPX) and Phospholipid Hydroperoxide Glutathione Peroxidase (PHGPX) are specula ted to play a key-role in the defence of neuronal cells against oxygen radical formation and peroxidative processes. Our findings support th e hypothesis that the presence of selenium depletion in the brain amon gst patients with epilepsy constitutes an important triggering factor for the origin of intractable seizures and subsequent neuronal damage.