May 7, 2015: Childhood stroke: think metabolic
May 7, 2015
Childhood stroke: think metabolic
Childhood stroke is rare and occurs in approximately two to thirteen out of one hundred thousand children per year. Several known conditions place children at a higher risk for stroke, both ischemic and hemorrhagic. More common conditions include vascular malformations, coagulopathic diseases, hemophilias, amongst others. Metabolic disease should also be included in the differential when evaluating a child with stroke. Children with mitochondrial diseases are predisposed to stroke during periods of stress and have even been associated with Moyamoya disease. A classic example is MELAS, mitochondrial encephalopathy with lactic acidosis and stroke-like symptoms. In homocystinuria, children develop elevated levels of homocysteine, leading to vascular endothelial damage and promotion of thrombosis. Glycolipids accumulate in the endothelial wall in Fabry’s disease, a deficiency in alpha-galactodsidase. In addition, a condition such as alpha-1 antitrypsin syndrome can also predispose a child to stroke due to an imbalance of proteases and formation of aneurysms. As childhood stroke is quite rare, a broad differential is crucial and metabolic disease should be considered.
- Giroud M, Lemesle M, Gouyon JB, Nivelon JL, Milan C, Dumas R. Cerebrovascular disease in children under 16 years of age in the city of Dijon, France: a study of incidence and clinical features from 1985 to 1993. Journal of Clinical Epidemiology 1995; 48: 1343-1348.
- Longo N, Schrijver I, Vogel H, et al. Progressive cerebral vascular degeneration with mitochondrial encephalopathy. American Journal of Medical Genetics Part A 2008; 146: 361-367.
Submitted by James Addington, M.D. Resident Physician, Department of Neurology, University of Virginia
Disclosures: Dr. Addington is a member of the Residents & Fellows Section of Neurology.