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.

Keywords:
Monoclonal immunoglobulin deposition disease, Light- and heavy-chain deposition disease, Compressive optic neuropathy, Graves’ orbitopathy, Multiple myeloma
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© 2009 S. Karger AG, Basel
2009
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