[Lacunar brain infarction in patients with pseudoxanthoma elasticum]

Brain infarctions in patients with pseudoxanthoma elasticum (PXE) are thought to be associated with the damage of cerebral arteries injured by connective tissue change caused by PXE. We experienced 3 cases of PXE with lacunar brain infarction and evaluated the relationship between the damage of small penetrating arteries and PXE. All patients' diagnosis of PXE were confirmed by histological examination. They were a 47-year-old man and two women with 54 and 62 years of age old. All patients had pseudoxanthoma and angioid streaks and showed pyramidal tract signs. In addition, one patient presented Parkinsonism and another presented involuntary movement of the left lower extremities. None of these cases showed cortical symptoms. Brain CT and MRI revealed multiple lacuna in the areas of penetrating arteries, but their cerebral angiogram were almost normal. Severe small artery diseases, which cannot be attributable only to hypertension, were present. The relationship between such severe small artery disease and damage of the origin of small penetrating arteries caused by PXE was strongly suggested.