BACKGROUND: The disease course of polyneuropathy associated with immunoglobulin M monoclonal gammopathy (IgM MGUSP) can be highly variable. In order to identify factors that influence long-term disease outcome, a prospective cohort study was performed of 140 patients with IgM MGUSP over a period of 23 years. METHODS: All patients with IgM MGUSP who were diagnosed in our tertiary referral center for polyneuropathy were eligible. All patients underwent nerve conduction studies and were tested for anti-MAG antibodies. The modified Rankin Scale, graded muscle strength, quantified sensory function, and laboratory testing were performed at 0, 1, 2, and 5 years and at last visit. The primary outcome measure was the risk of developing a modified Rankin Scale score of > or = 3 points. RESULTS: A total of 140 patients with IgM MGUSP fulfilled inclusion criteria (101 [72%] demyelinating, 39 [28%] axonal, 63 [44%] MAG positive). The median age at onset was 59 years (interquartile range 52-67), median disease duration at baseline was 3.2 years (interquartile range 1.9-6). Anti-MAG antibodies were associated with a lower risk of Rankin Scale score > or = 3. Demyelination and a higher age at onset were associated with a higher risk for Rankin Scale score > or = 3. Based on these 3 factors, a Web-based prognostic model was developed that directly allows clinicians to estimate the probability of developing disability (http://www.umcutrecht.nl/subsite/Prognosis-MGUS-Neuropathy). CONCLUSION: Higher age at onset and demyelination increase the risk, whereas anti-MAG antibodies decrease the risk, of developing Rankin Scale score > or = 3 in polyneuropathy associated with immunoglobulin M monoclonal gammopathy (IgM MGUSP). Our Web-based prognostic model allows determination of prognosis in IgM MGUSP.
BACKGROUND: The disease course of polyneuropathy associated with immunoglobulin M monoclonal gammopathy (IgM MGUSP) can be highly variable. In order to identify factors that influence long-term disease outcome, a prospective cohort study was performed of 140 patients with IgM MGUSP over a period of 23 years. METHODS: All patients with IgM MGUSP who were diagnosed in our tertiary referral center for polyneuropathy were eligible. All patients underwent nerve conduction studies and were tested for anti-MAG antibodies. The modified Rankin Scale, graded muscle strength, quantified sensory function, and laboratory testing were performed at 0, 1, 2, and 5 years and at last visit. The primary outcome measure was the risk of developing a modified Rankin Scale score of > or = 3 points. RESULTS: A total of 140 patients with IgM MGUSP fulfilled inclusion criteria (101 [72%] demyelinating, 39 [28%] axonal, 63 [44%] MAG positive). The median age at onset was 59 years (interquartile range 52-67), median disease duration at baseline was 3.2 years (interquartile range 1.9-6). Anti-MAG antibodies were associated with a lower risk of Rankin Scale score > or = 3. Demyelination and a higher age at onset were associated with a higher risk for Rankin Scale score > or = 3. Based on these 3 factors, a Web-based prognostic model was developed that directly allows clinicians to estimate the probability of developing disability (http://www.umcutrecht.nl/subsite/Prognosis-MGUS-Neuropathy). CONCLUSION: Higher age at onset and demyelination increase the risk, whereas anti-MAG antibodies decrease the risk, of developing Rankin Scale score > or = 3 in polyneuropathy associated with immunoglobulin M monoclonal gammopathy (IgM MGUSP). Our Web-based prognostic model allows determination of prognosis in IgM MGUSP.
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: Lotte Vlam; Sanne Piepers; Nadia A Sutedja; Bart C Jacobs; Anne P Tio-Gillen; Marloes Stam; Hessel Franssen; Jan H Veldink; Elisabeth A Cats; Nicolette C Notermans; Andries C Bloem; Renske I Wadman; W-Ludo van der Pol; Leonard H van den Berg Journal: J Neurol Date: 2015-01-01 Impact factor: 4.849