[Paget's disease as a cause for symptomatic basilar impression--a case report and review of the literature]

Abstract

Introduction: We report a case of a 52-years old women with Paget's disease. A secondary symptomatic basilar impression was observed, causing quadriparesis, bulbar palsy and ataxia

Clinical presentation: We report a case of a 52-year old woman with history of/periodic headache. In the last 6 months the complaints became more intensive and continuous. Additionally quadriparesis, disphagia, hoarsness and gait instability occurred. A cranial form of Paget's disease was found and a secondary basilar impression with compression of the cerebellum and brain stem was proven. A median suboccipital decompression and C1 laminectomy were performed. The occipital bone was thick, porous, with lacunas full of blood, causing unusually intensive hemorrhage. The postoperative period was uneventful with resolution of the preoperative symptomatology.

Conclusion: In cases with Paget's disease a secondary basilary impression with ensuing cerebellar and brain stem compression may be observed. Decompressive suboccipital craniectomy may be a therapeutic option. The surgical team should be prepared for an excessive hemorrhage from the porous occipital bone.