Secondary amyloidosis as a life-ending event in multifocal motor neuropathy.
Multifocal motor neuropathy (MMN) is a disorder of peripheral nerve often associated with a high monosialoganglioside (GM1) antibody and multifocal conduction block. It has a chronic, indolent course with involvement of predominantly peripheral motor nerves, usually in an asymmetric fashion. There have been few reported cases of progression to frank quadriplegia. Secondary amyloidosis refers to the deposition of amyloid in various tissues due to an underlying chronic inflammatory state. We report the first case, to our knowledge, of a patient with MMN associated with high titer of GM1 antibody who developed acute paraplegia with both cranial nerve and worsening sensory involvement associated with multiorgan compromise due to a secondary amyloidosis involving the myocardium.
Beydoun
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SR
Keck University of Southern California School of Medicine, Department of Neurology, 1450 San Pablo Street, Suite 1900, Los Angeles, California 90033, USA. sbeydoun@hsc.usc.edu
Rison
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RA
Commins
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D000686:Amyloidosis / Q000209:etiology / Q000473:pathology*; D001323:Autoantibodies / Q000097:blood; D017809:Fatal Outcome; D005267:Femoral Nerve / Q000473:pathology; D005677:G(M1) Ganglioside / Q000276:immunology; D006801:Humans; D008297:Male; D008475:Median Nerve / Q000503:physiopathology; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000150:complications / Q000473:pathology* / Q000503:physiopathology; D009431:Neural Conduction; D013979:Tibial Nerve / Q000503:physiopathology; D014459:Ulnar Nerve / Q000503:physiopathology
D001323:Autoantibodies; D005677:G(M1) Ganglioside