Central nervous system involvement in patients with monoclonal gammopathy and polyneuropathy.
OBJECTIVE
To evaluate clinical presentation of patients with the clinical triad of monoclonal gammopathy, polyneuropathy and signs of CNS involvement.


METHODS
Nineteen patients with monoclonal protein (M-protein, 9 IgM, 10 IgG) were studied. Clinical examination, MRI, cerebrospinal fluid analysis and immune reactivity against myelin-associated glycoprotein and gangliosides in serum were obtained. By immunohistochemistry, different binding patterns of M-proteins to human CNS tissue were investigated.


RESULTS
Nine out of 19 patients (four IgM, five IgG) showed one or more clinical signs of CNS involvement. Clinical features associated with signs of CNS pathology were disease duration and greater concentration of IgM paraprotein. The IgM M-protein of two patients strongly stained the cortex/cerebellar neurons in human brain sections.


CONCLUSIONS
Our results complement previous reports that some patients with monoclonal gammopathy and polyneuropathy can develop solitary or disseminated signs of CNS involvement. It indicates that pathological effects of M-proteins are not necessarily restricted to the peripheral nervous system. The specificity and affinity of circulating M-protein to antigens in the CNS might be critical for the development of different clinical phenotypes.
Department of Neurology, Heinrich-Heine University, Düsseldorf, Germany. helmar.lehmann@uni-duesseldorf.de
H C
HC
Lehmann
F R
FR
Hoffmann
G
G
Meyer Zu Hörste
H-P
HP
Hartung
B C
BC
Kieseier
D000368:Aged; D000369:Aged, 80 and over; D001259:Ataxia / Q000150:complications / Q000276:immunology / Q000503:physiopathology*; D002531:Cerebellum / Q000276:immunology; D005260:Female; D005625:Frontal Lobe / Q000276:immunology; D006801:Humans; D007075:Immunoglobulin M / Q000276:immunology; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D010265:Paraproteinemias / Q000150:complications / Q000276:immunology / Q000503:physiopathology*; D011115:Polyneuropathies / Q000150:complications / Q000276:immunology / Q000503:physiopathology*; D012720:Severity of Illness Index; D018709:Statistics, Nonparametric; D014202:Tremor / Q000150:complications / Q000276:immunology / Q000503:physiopathology*
D007075:Immunoglobulin M