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Paget's disease and the nervous system.

R G Feldman, A Culebras, H H Schmidek

    Journal of the American Geriatrics Society
    |January 1, 1979
    PubMed
    Summary
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    Paget's disease, typically affecting those over 40, causes bone overgrowth leading to neurological issues. Diagnosis involves imaging and lab tests, with treatments including medication and surgery.

    Area of Science:

    • Neurology
    • Orthopedics
    • Radiology

    Background:

    • Paget's disease of bone is a chronic disorder characterized by abnormal bone remodeling.
    • It commonly affects individuals over 40, with cranial vault involvement posing significant risks.
    • Symptoms can include headaches, hearing loss, tinnitus, and neurological deficits due to bony overgrowth.

    Purpose of the Study:

    • To review the clinical presentation, diagnostic methods, and management strategies for Paget's disease.
    • To highlight the neurological complications arising from cranial and spinal involvement.
    • To discuss current therapeutic options for Paget's disease.

    Main Methods:

    • Review of clinical literature and diagnostic criteria for Paget's disease.
    • Analysis of radiographic and biochemical markers for diagnosis.

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  • Evaluation of treatment outcomes for pharmacological and surgical interventions.

    • Main Results:

    • Diagnosis is confirmed by characteristic radiographic findings and elevated serum alkaline phosphatase and urinary hydroxyproline levels.
    • Bony overgrowth can compress neural structures, leading to symptoms like brain stem stretching and obstructive hydrocephalus.
    • Potential complications include sarcoma of the cranial vault and cervicomedullary or spinal cord compression.

    Conclusions:

    • Early diagnosis and management are crucial to prevent severe neurological complications in Paget's disease.
    • Surgical decompression may be required for significant neural compression.
    • Pharmacological treatments such as calcitonin, disodium etidronate, and mithramycin offer therapeutic benefits.