Cerebral cholesterol granuloma in homozygous familial hypercholesterolemia

CMAJ. 2005 Feb 15;172(4):495-7. doi: 10.1503/cmaj.1041152.

Abstract

Familial hypercholesterolemia (FH) is characterized by the accumulation of excess cholesterol in tissues including the artery wall and tendons. We describe a patient with homozygous FH who presented with asymptomatic cholesterol granuloma of the brain. The patient's plasma low-density lipoprotein cholesterol level was remarkably responsive to combination hypolipidemic therapy with statin plus ezetimibe. This case illustrates another potential complication of whole-body cholesterol excess and underscores the differences in phenotype and in response to therapy among patients with FH.

Publication types

  • Case Reports

MeSH terms

  • Anticholesteremic Agents / therapeutic use
  • Azetidines / therapeutic use
  • Brain Diseases / etiology*
  • Brain Diseases / surgery
  • Cholesterol*
  • Cholesterol, LDL / blood
  • Conjunctival Diseases / etiology
  • Drug Therapy, Combination
  • Edema / etiology
  • Ezetimibe
  • Female
  • Granuloma, Foreign-Body / etiology*
  • Granuloma, Foreign-Body / surgery
  • Homozygote
  • Humans
  • Hydroxymethylglutaryl-CoA Reductase Inhibitors / therapeutic use
  • Hyperlipoproteinemia Type II / complications*
  • Hyperlipoproteinemia Type II / drug therapy
  • Middle Aged
  • Ophthalmic Solutions / adverse effects
  • Point Mutation
  • Vasodilation

Substances

  • Anticholesteremic Agents
  • Azetidines
  • Cholesterol, LDL
  • Hydroxymethylglutaryl-CoA Reductase Inhibitors
  • Ophthalmic Solutions
  • Cholesterol
  • Ezetimibe