Severe cranial nerve involvement in a patient with monoclonal anti-MAG/SGPG IgM antibody and localized hard palate amyloidosis.
We report a patient with severe cranial polyneuropathy as well as sensory limb neuropathy. Biclonal serum IgM-kappa/IgM-lambda gammopathy was found and serum anti-myelin-associated glycoprotein (MAG)/sulfoglucuronyl paragloboside (SGPG) IgM antibody was also detected. Immunofluorescence analysis of a sural nerve biopsy specimen revealed binding of IgM and lambda-light chain on myelin sheaths. No amyloid deposition was detected in biopsied tissues except for the hard palate, suggesting that the amyloidosis was of the localized type and had no relation to the pathogenesis of cranial neuropathy. Our observations indicate that the anti-MAG/SGPG IgM antibody may be responsible for this patient's cranial polyneuropathy, which is a rare manifestation in anti-MAG/SGPG-associated neuropathy.
Yoshida
Takuhiro
T
Department of Medicine (Neurology), Shinshu University School of Medicine, Matsumoto 390-8621, Japan.
Yazaki
Masahide
M
Gono
Takahisa
T
Tazawa
Ko-ichi
K
Morita
Hiroshi
H
Matsuda
Masayuki
M
Funakoshi
Kei
K
Yuki
Nobuhiro
N
Ikeda
Shu-ichi
S
D000682:Amyloid / Q000276:immunology / Q000378:metabolism; D000686:Amyloidosis / Q000150:complications* / Q000503:physiopathology; D001323:Autoantibodies / Q000097:blood*; D001706:Biopsy; D003389:Cranial Nerve Diseases / Q000175:diagnosis / Q000276:immunology* / Q000503:physiopathology; D003391:Cranial Nerves / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D003937:Diagnosis, Differential; D005155:Facial Nerve Diseases / Q000175:diagnosis / Q000276:immunology / Q000503:physiopathology; D005915:Globosides / Q000276:immunology*; D006801:Humans; D007075:Immunoglobulin M / Q000097:blood; D008297:Male; D046529:Microscopy, Electron, Transmission; D008875:Middle Aged; D009186:Myelin Sheath / Q000276:immunology / Q000473:pathology; D018992:Myelin-Associated Glycoprotein / Q000276:immunology*; D021362:Palate, Hard / Q000473:pathology / Q000503:physiopathology; D011115:Polyneuropathies / Q000175:diagnosis / Q000276:immunology* / Q000503:physiopathology; D013497:Sural Nerve / Q000276:immunology / Q000473:pathology / Q000648:ultrastructure
D000682:Amyloid; D001323:Autoantibodies; D005915:Globosides; D007075:Immunoglobulin M; D018992:Myelin-Associated Glycoprotein; C051917:sulfate-3-glucuronyl paragloboside