Limitations in daily activities and general perception of quality of life: Long term follow‐up in patients with anti‐myelin‐glycoprotein antibody polyneuropathy

Abstract

In this study, we assessed the modifications over time of daily activities and quality of life (QoL) in 32 subjects with anti‐myelin‐glycoprotein (MAG) antibody neuropathy. A widespread panel including clinical scores and patient‐reported questionnaires, in compliance of the terms by the International Classification of Functioning, Disability, and Health (ICF) of the World Health Organization (WHO), was employed at enrollment (T0) and at follow‐up evaluation (T1) after a mean interval of 15.4 ± 5.7 months. The Sensory Modality Sum score (SMS) at four limbs showed a significant worsening over time (mean score 27.2 ± 3.9 at T0 vs 25.7 ± 3 at T1 at upper limbs, P = .03; 20.5 ± 4.8 at T0 vs 18.6 ± 5.9 at T1 at lower limbs, P = .04). The Visual Analogue Scale (VAS) for pain significantly worsened at upper limbs at T1 (mean values 0.84 ± 1.95 at T0 vs 1.78 ± 2.6 at T1, P = .03). All the other tests did not show significant differences between T0 and T1. In the subgroup who underwent rituximab (15/32 treated before T0, 3/32 patients treated between T0 and T1 with median interval of 1 year), no significant differences were observed between T0 and T1. Despite the quite long follow‐up, statistical significance was not achieved either for the limited number of patients or for the lack of sensitive outcome measures. In our cohort, the significant worsening of the SMS and VAS after a median of 14 months can be considered as a reliable expression of the natural history of the disease, and suggest that these scales might represent possible outcome measures in anti‐MAG antibody neuropathy.

Therapeutic Monoclonal Antibody Therapies in Chronic Autoimmune Demyelinating Neuropathies

“…Neuropathy with anti-MAG antibodies is the most common IgM paraproteinemic neuropathy, characterized by predominant sensory symptoms, ataxic gait, tremor at upper limbs, with motor involvement and disability occurring late in the course of the disease [ 62 ] . Despite slowly progressive, the neuropathy may severely affect functional activities and quality of life [ 63 – 65 ].…”

IgM anti‐MAG± peripheral neuropathy (IMAGiNe) study protocol: An international, observational, prospective registry of patients with IgM M‐protein peripheral neuropathies

“…IgM monoclonal gammopathy-associated peripheral neuropathy (IgM PNP) is a rare and clinically heterogeneous disease that may cause functional disabilities and severe limitations in daily activities and quality of life. [1][2][3] To date, a direct causal relationship between gammopathies of any class and neuropathies has only been commonly recognized for the IgM subtype. Approximately 30%-60% of neuropathies associated with gammopathies are IgM related.…”

Mutational Profile in 75 Patients With Anti–Myelin-Associated Glycoprotein Neuropathy

“… 1 , 2 Despite being slowly progressive, the neuropathy may severely influence patients' functionality and quality of life. 3 Among possible therapies, rituximab, an anti-CD20 chimeric monoclonal antibody, remains the most used treatment efficacious in almost half of the patients and capable of improving disability scales and the response to questionnaires in the global impression of the disease. 4 - 8 Recently, the discovery of the mutational profile of the MYD88 and CXCR4 genes has radically changed the diagnostic and prognostic evaluation of IgM monoclonal gammopathies.…”