[A case of IgM paraproteinemic neuropathy associated with anti-sulfated glucuronic paragloboside (SGPG) IgG antibody without anti-myelin-associated glycoprotein (MAG) activity].
We report a case of IgM paraproteinemic neuropathy associated with anti-sulfated glucuronic paragloboside (SGPG) IgG antibody. An 84-year old man complained of numbness on the left side of the face and in the distal portions of the limbs. Neurological examination showed mild sensory ataxia. The laboratory tests revealed the presence of IgM lambda paraproteinemia and anti-SGPG IgG antibody without anti-myelin-associated glycoprotein (MAG) activity and anti-MAG/SGPG IgM antibody. Results of nerve conduction study showed decreased sensory nerve action potential (SNAP) amplitude, indicating the presence of sensory-dominant axonal polyneuropathy, and the prolongation of distal latency was not observed. Treatment with corticosteroids resulted in a rapid improvement in neurological abnormalities. In IgM paraproteinemic neuropathy associated with anti-MAG/SGPG antibody, distal acquired demyelinating sensory neuropathy and resistance to immunological treatments are the characteristic pathologic and clinical features, respectively. On the other hand our rare case of IgM paraproteinemic neuropathy positive for anti-SGPG IgG antibody presented with axonal sensory polyneuropathy and a good responsiveness to corticosteroids.
Department of Neurology, National Hospital Organization Yokohama Medical Center.
Haruko
H
Nakamura
Masanao
M
Endo
Eriko
E
Sugawara
Motoi
M
Kuwahara
Susumu
S
Kusunoki
Fumiaki
F
Tanaka
Tatsuya
T
Takahashi
D000369:Aged, 80 and over; D000888:Antibodies, Anti-Idiotypic / Q000032:analysis*; D001323:Autoantibodies / Q000276:immunology*; D005915:Globosides / Q000276:immunology*; D006801:Humans; D007075:Immunoglobulin M / Q000276:immunology*; D008297:Male; D018992:Myelin-Associated Glycoprotein / Q000276:immunology; D010265:Paraproteinemias / Q000276:immunology*; D011115:Polyneuropathies / Q000276:immunology*
D000888:Antibodies, Anti-Idiotypic; D001323:Autoantibodies; D005915:Globosides; D007075:Immunoglobulin M; C115082:MAG protein, human; D018992:Myelin-Associated Glycoprotein; C048844:anti-IgG; C051917:sulfate-3-glucuronyl paragloboside