Daniel J Rubin1, Robert M Levin. 1. Section of Endocrinology, Diabetes, and Nutrition, Boston University Medical Center, Boston, Massachusetts 02118, USA.
Abstract
OBJECTIVE: To review the epidemiology, evaluation, and management of the neurologic complications associated with Paget disease of bone (PDB). METHODS: We reviewed the English-language medical literature using MEDLINE data sources from 1950 to August 2008 and manually searched cross-references from original articles and reviews. Search terms included "Paget* disease of bone" and "neurologic* complications," "cranial nerve," "spinal cord," or "peripheral nerve." RESULTS: Several neurologic problems in the central and peripheral nervous systems may complicate PDB. Up to 76% of patients may have some form of neurologic involvement. Neurologic complications can occur in patients with a long history of PDB as well as in patients with previously unrecognized disease. The primary mechanisms of nerve damage in PDB involving the spine are ischemic myelitis and compression due to bone hypertrophy. Evaluation includes determining the serum alkaline phosphatase level and imaging by radiography, bone scintigraphy, computed tomographic scanning, and, for lesions of the central nervous system, magnetic resonance imaging. If a soft-tissue mass is found, biopsy should be considered to exclude the presence of sarcoma. Treatment strategies include calcium, vitamin D, bisphosphonates, and possibly surgical intervention for refractory cases. CONCLUSION: Neurologic sequelae of PDB may be underappreciated. Despite the paucity of data guiding treatment, zoledronic acid is a reasonable first-line therapy. Lack of response to treatment or relapse should prompt diagnostic reevaluation with a heightened suspicion for tumor.
OBJECTIVE: To review the epidemiology, evaluation, and management of the neurologic complications associated with Paget disease of bone (PDB). METHODS: We reviewed the English-language medical literature using MEDLINE data sources from 1950 to August 2008 and manually searched cross-references from original articles and reviews. Search terms included "Paget* disease of bone" and "neurologic* complications," "cranial nerve," "spinal cord," or "peripheral nerve." RESULTS: Several neurologic problems in the central and peripheral nervous systems may complicate PDB. Up to 76% of patients may have some form of neurologic involvement. Neurologic complications can occur in patients with a long history of PDB as well as in patients with previously unrecognized disease. The primary mechanisms of nerve damage in PDB involving the spine are ischemic myelitis and compression due to bone hypertrophy. Evaluation includes determining the serum alkaline phosphatase level and imaging by radiography, bone scintigraphy, computed tomographic scanning, and, for lesions of the central nervous system, magnetic resonance imaging. If a soft-tissue mass is found, biopsy should be considered to exclude the presence of sarcoma. Treatment strategies include calcium, vitamin D, bisphosphonates, and possibly surgical intervention for refractory cases. CONCLUSION:Neurologic sequelae of PDB may be underappreciated. Despite the paucity of data guiding treatment, zoledronic acid is a reasonable first-line therapy. Lack of response to treatment or relapse should prompt diagnostic reevaluation with a heightened suspicion for tumor.