BACKGROUND AND PURPOSE: 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. CONCLUSION: 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.
BACKGROUND AND PURPOSE: 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. CONCLUSION: 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.
Authors: Abraham C J Stork; W-Ludo van der Pol; Hessel Franssen; Bart C Jacobs; Nicolette C Notermans Journal: J Neurol Date: 2014-04-30 Impact factor: 4.849
Authors: Jonas Graf; Lea Jansen; Jens Ingwersen; Marius Ringelstein; Jens Harmel; Jana Rybak; Robert Kolbe; Laura Rhöse; Lena Gemerzki; John-Ih Lee; Alexander Klistorner; Rainer Guthoff; Hans-Peter Hartung; Orhan Aktas; Philipp Albrecht Journal: Ann Clin Transl Neurol Date: 2018-07-07 Impact factor: 4.511