Abstract
Although pseudoxanthcma elasticum (PXE) is known to involve multiple systems including the skin, the eye and other several organs, a few cases with the manifestation of central nervous system have been reported. In this report, a case of PXE associated with two episodes of brainstem infarction is described. A 38 year old man suffered from the second attack of brainstem infarction one year after the first attack, which was followed by complete recovery. The patient had loosening of the skin in the neck and the trunk and angioid streaks of the optic fundi. Based on neurologic examination, he was diagnosed as Wallenberg's syndrome. Vertebral angiography revealed occlusion of the right posterior inferior cerebellar artery. The ischemic attacks were presumed to be due to cerebrovascular involvement of PXE which was confirmed by skin biopsy.
Only 23 cases of PXE with the involvement of central nervous system have been found in Japanese literature, since Kawamura reported the first case in 1957. Following results were obtained by reviewing the 23 cases :
1. The mean age of the onset of neurological symptoms was 37.9 years (range 14 to 67).
2. Mortality was low, but functional recovery of the nervous system was poor.
3. The involvement of central nervous system was for the most part due to ischemic cerebrovascular disease.
4. In the majority of cases, cerebral angiography revealed the lesion in the internal carotid artery.
