A Japanese family with familial elevation of plasma histidine-rich glycoprotein (HRG) is described. The propositus was a 41-year-old man, who complained of left hemiparesis and dysarthria. He was made a diagnosis of pontine and cerebellar infarctions by brain CT, brain MRI and cerebral angiography. His parents were not consanguineous, and none of his relatives suffered from thrombosis. His plasma HRG antigen measured by Laurell's method was 190% (normal range: 58-161%). No other hemostatic abnormalities were found. Family studies revealed the elevation of plasma HRG antigen in 10 family members. None of them had rheumatic heart disease or Behcet's disease, in which elevation of plasma HRG antigen was reported. These data suggest that familial elevation of plasma HRG was inherited in this family.
