Neuropsychological findings with pseudoxanthoma elasticum

It has been reported that the disease pseudoxanthoma elasticum (PXE) is associated with a high incidence of neurologic and psychiatric symptoms, which are possibly due to cerebrovascular ischemia. These reports are based mainly upon clinical observations made without reference to control group data. We have compared the results of 27 PXE patients with results of a control group on a battery of objective neuropsychological and personality tests. The PXE group showed only very mild deficits on extensive neuropsychological testing, a finding which argues against any marked involvement of cerebral vessels in these patients. Similarly, the personality test results did not reveal significant psychiatric disturbances which could be attributed to PXE. Possible reasons for the descrepancy with previous reports include the subjective data and much older PXE patients on which those reports are based. The need for systematic neuropsychological research with older PXE patients and control groups is suggested.