PEDIATRICS Vol. 69 No. 3 March 1982, pp. 308-316
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Pediatric Victims of Unexplained Stroke and Their Families: Familial Lipid and Lipoprotein Abnormalities

Charles J. Glueck MD1, Stephen R. Daniels MD1, Stephen Bates MD1, Corning Benton MD1, Trent Tracy BS1, and Jane L.H.C. Third MD1

1 Departments of Pediatrics and Medicine, and Children's Hospital Medical Center, Divisions of Pediatric Neurology and Radiology, and Lipid Research Clinic, General Clinical Research Center and CLINFO Center, Lipid Research Division, University of Cincinnati, College of Medicine, Cincinnati

The finding of low levels of high-density lipoprotein cholesterol (C-HDL) and/or high levels of triglyceride in several children with "unexplained" ischemic cerebrovascular accidents led to a systematic review of lipids and lipoproteins in 11 pediatric victims of unexplained stroke and their families. Each of the children, 1 to 17 years of age, had an acute, nonhemorrhagic cerebrovascular accident documented by symptoms, signs, history, physical examination, and laboratory radiographic studies; no known causes or strong preclisposing factors were elicited. Of the 11 pediatric stroke probands, only one had normal lipid and lipoprotein levels; five had low C-HDL levels alone, two had elevated levels of plasma triglycerides with low C-HDL levels, two had high levels of triglycerides with normal C-HDL levels, and one had high levels of low-density lipoprotein cholesterol (C-LDL) alone. A consistent trend toward familial clustering of low C-HDL, elevated triglyceride, and/or C-LDL levels was observed. Nine of the 11 kindreds had two generation parent:child stroke proband, or parent:child clustering of elevated triglyceride and/or depressed C-HDL levels, whereas one parent:child stroke proband pair had elevated levels of C-LDL. In five of nine kindreds for whom siblings of the pediatric stroke probands were available for testing, there were low C-HDL and/or high triglyceride levels in otherwise healthy and entirely asymptomatic siblings. In nine of the 11 kindreds premature coronary heart disease and/or ischemic cerebrovascular disease was observed in the probands' adult relatives. We speculate that familial lipoprotein abnormalities, particularly those involving low levels of C-HDL and/or high levels of triglycerides, may mediate occlusive cerebrovascular arteriosclerosis, and thus may predispose children to ischemic cerebrovascular strokes. The apparent association of lipoproteins and ischemic strokes in children and their families merits further exploration with a particular focus on familial aggregation of low C-HDL levels, already known to be a significant risk factor for ischemic cerebrovascular and cardiovascular disease in adults.

Submitted on April 29, 1981
Accepted on June 19, 1981




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