Involvement of central nervous system in pseudoxanthoma elasticum

A 24- year-old female with typical pseudoxanthoma elasticum of the skin, angioid streaks of the optic fundi and cardiovascular involvement developed neurasthenic symptoms, epilepsy, progressive mental deterioration and focal neurological deficits. Cerebral angiography revealed marked generalized arteriosclerotic change and occlusion of the left internal carotid artery. The literature of pseudoxanthoma elasticum is reviewed with special reference to central nervous system (CNS) manifestations. The possible role of the involvement of cerebral arteries in the genesis of CNS manifectations is discussed. And the importance of neurasthenic syndrome as an initial sign, which suggests that CNS involvement has already started in this disease, is stressed.