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Selenium deficiency triggering intractable seizures
V T Ramaekers1, M Calomme, D Vanden Berghe
1Department of Paediatric Neurology, University of Aachen, Germany.
Insights
Severe neurodevelopmental issues and intractable seizures in children were linked to selenium deficiency. Selenium supplementation improved seizures and liver function, suggesting its crucial role in neuronal health.
Area of Science:
- Biochemistry
- Neurology
- Pediatrics
Background:
- Investigating severe neurodevelopmental retardation and intractable seizures in infants.
- Ruling out common neurometabolic disorders as the cause.
Observation:
- Two children presented with intractable seizures and elevated liver function tests within the first year of life.
- Systemic selenium deficiency was documented concurrently with seizure onset.
- One infant died at ten months with neuropathology consistent with Progressive Neuronal Degeneration of Childhood (PNDC) / Alpers disease.
Findings:
- Selenium deficiency was implicated in intractable seizures and neuronal damage.
- Oral selenium supplementation (3-5 µg/kg) led to seizure reduction and EEG improvement.
- Liver function normalized following selenium supplementation in both patients.
Implications:
- Selenium deficiency may trigger intractable seizures and subsequent neuronal damage in susceptible individuals.
- Seleno-dependent enzymes like Glutathione Peroxidase (GPX) and Phospholipid Hydroperoxide Glutathione Peroxidase (PHGPX) are vital for neuronal defense against oxidative stress.
- This study highlights the critical role of selenium in pediatric neurological health and warrants further investigation into selenium's impact on epilepsy and neurodegenerative disorders.
Abstract:
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 the time of seizures evidence for systemic selenium deficiency could be documented. The youngest patient, who manifested intractable fits from the fourth day of life, died at the age of ten months. Neuropathologic examination was consistent with Progressive Neuronal Degeneration of Childhood (PNDC) with liver disease or formerly known as Alpers disease. In the oldest child, whose diet was normally balanced, fits started from the age of 11 months and features of long-standing selenium deficiency became apparent from the age of 1 1/2 years and consisted of liver function disturbances, depigmented hair and osteoarthropathy. Oral substitution with selenium supplements in both children (3-5 micrograms/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 speculated to play a key-role in the defence of neuronal cells against oxygen radical formation and peroxidative processes. Our findings support the hypothesis that the presence of selenium depletion in the brain amongst patients with epilepsy constitutes an important triggering factor for the origin of intractable seizures and subsequent neuronal damage.