Hirano T, Hashimoto Y, Kimura K, Uchino M
Department of Neurology, Kumamoto University School of Medicine.
Rinsho Shinkeigaku. 1996 May;36(5):633-9.
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.
弹性假黄瘤(PXE)患者的脑梗死被认为与PXE引起的结缔组织变化所损伤的脑动脉有关。我们诊治了3例伴有腔隙性脑梗死的PXE患者,并评估了小穿通动脉损伤与PXE之间的关系。所有患者PXE的诊断均经组织学检查证实。他们分别是一名47岁男性和两名女性,年龄分别为54岁和62岁。所有患者均有假黄瘤和血管样条纹,并表现出锥体束征。此外,一名患者出现帕金森综合征,另一名患者出现左下肢不自主运动。这些病例均未出现皮质症状。脑部CT和MRI显示穿通动脉区域有多个腔隙,但他们的脑血管造影基本正常。存在严重的小动脉疾病,且不能仅归因于高血压。强烈提示这种严重的小动脉疾病与PXE引起的小穿通动脉起源损伤之间存在关联。