Kidney transplantation in Fabry's disease.

Abstract

The right abducens nerve palsy gradually improved. Six weeks after discharge lateral movement of the right eye was apparently full but the diplopia persisted and he complained of blurring in the left eye. Prednisolone was then given, but a week later vision in the right eye became blurred. The left carotid and the left radial pulses were absent and the right carotid pulse was weak. A short, soft systolic bruit was audible over the right carotid artery. Visual impairment was believed to be due to retinal ischaemia. Three months after discharge the patient still had headache and intermittent blurring of vision. The left auriculotemporal arterial pulsation was absent. Nicotinic acid added to the treatment gave no benefit. Four months after dischage he developed another crop of papulonecrotic eruptions over the back, buttocks, arms, legs, and face. The headache was intense and there was polyarthralgia involving the wrists, ankles, and knees. These symptoms persisted for over a month despite prednisolone in doses of up to 60 mg/day. Prednisolone was stopped and cyclophosphamide by mouth 150 mg/day gradually reducing to 100 mg/day was given instead. The headache became less frequent and less severe.