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  • Title: Intracellular extraocular muscle light- and heavy-chain deposition disease contributing to compressive optic neuropathy in a patient with preexisting Graves' orbitopathy.
    Author: Zakrzewski PA, Weis E, White VA, Rootman J.
    Journal: Ophthalmologica; 2010; 224(1):59-62. PubMed ID: 19707026.
    Abstract:
    PURPOSE: Light- and heavy-chain deposition disease (LHCDD) is a rare form of nonamyloidal monoclonal immunoglobulin deposition disease (MIDD) in which light- and heavy-chain immunoglobulin fragments accumulate systemically, typically leading to end organ dysfunction. Herein we describe the case of a 64-year-old female with a history of Graves' orbitopathy and multiple myeloma who presented with bilateral asymmetric compressive optic neuropathies. PROCEDURE: A biopsy of the right medial rectus muscle was taken during orbital decompression surgery. RESULTS: Light and electron microscopy of the biopsy specimen led to a diagnosis of intracellular skeletal muscle LHCDD. CONCLUSION: This is the first published report to describe the findings of: (1) intracellular deposition of nonamyloidal MIDD; (2) orbital involvement of nonamyloidal MIDD, and (3) compressive optic neuropathy resulting from any form of MIDD.
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