Monoclonal paraproteinemia with subacute encephalopathy, seizures, and scleromyxedema

For 3 months, a 37-year-old man developed memory loss and personality changes, followed by fever, seizures, bilateral upper motor neuron signs, and increased CSF protein with skin induration that was compatible with scleromyxedema. An IgG-type paraproteinemia was identified. He improved with plasmap...

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Bibliographic Details
Published in:	Neurology Vol. 37; no. 6; pp. 1054 - 1057
Main Authors:	REID, T. L, SPOTO, D. V, LARRABEE, G. J, SHLAMOWITZ, M. A, HOROWITZ, S. A
Format:	Journal Article
Language:	English
Published:	Hagerstown, MD Lippincott Williams & Wilkins 01-06-1987
Subjects:	Adult Biological and medical sciences Brain Diseases - complications Brain Diseases - pathology Humans Immunodeficiencies. Immunoglobulinopathies Immunoglobulin G Immunoglobulinopathies Immunopathology Male Medical sciences Paraproteinemias - complications Paraproteinemias - pathology Seizures - complications Seizures - pathology Skin Diseases - complications Skin Diseases - pathology Human Immunopathology Papular mucinosis Skin disease Nervous system diseases Immunoglobulinopathy Encephalopathy Epilepsy Monoclonal immunoglobulinemia IgG Hemopathy Subacute
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Summary:	For 3 months, a 37-year-old man developed memory loss and personality changes, followed by fever, seizures, bilateral upper motor neuron signs, and increased CSF protein with skin induration that was compatible with scleromyxedema. An IgG-type paraproteinemia was identified. He improved with plasmapheresis, but died in acute respiratory and renal failure. Autopsy revealed right temporal demyelination and meningeal inflammation.
ISSN:	0028-3878 1526-632X
DOI:	10.1212/WNL.37.6.1054