title: Long-term disability and prognostic factors in polyneuropathy associated with anti-myelin-associated glycoprotein (MAG) antibodies.
abstract: OBJECTIVE
  Neuropathy associated with IgM monoclonal gammopathy (MGUS) represents distinctive clinical syndrome, characterized by male predominance, late age of onset, slow progression, predominantly sensory symptoms, deep sensory loss, ataxia, minor motor impairment. More than 50% of patients with neuropathy-associated MGUS possess antibodies against myelin-associated glycoprotein (MAG). Purpose of our study was to assess effects on disease progression of demographic, clinical and neurophysiological variables in our large cohort of patients.
  
  
  METHODS
  Forty-three Caucasians patients were followed every eight months for median duration time of 93 months. Extremity strength was assessed with Medical Research Council (MRC) Scale, disability with overall disability status scale (ODSS), modified Rankin Scale and sensory function with Inflammatory Neuropathy Cause and Treatment (INCAT) sensory scale (ISS). Statistical analyses were conducted with parametric or non-parametric measures as appropriate. Survival analysis was used to test predictive value of clinical, demographical and neurophysiological variables. Variance analysis was conducted to explain difference on MRC between patients and groups at different time from onset.
  
  
  RESULTS
  Results showed that demyelinating pattern, older age and absence of treatment were significant risk factors for disability worsening. No other factors emerged as predictors including gender, ataxia and tremor at baseline, level of anti-MAG and IgM protein concentration in serum. Despite worsening of all outcome measures between first and last visit, quality of life (HRQol) judged by patients did not vary significantly.
  
  
  CONCLUSIONS
  Our study provides evidence that electrophysiologic pattern, age of onset and absence of treatment are strong predictor of prognosis in anti-MAG polyneuropathy.
authors.affiliation: a Department of Biomedical, Metabolic and Neural Science , University of Modena & Reggio Emilia , Modena , Italy.
authors.forename: Giuliana
authors.initials: G
authors.lastname: Galassi
authors.affiliation: a Department of Biomedical, Metabolic and Neural Science , University of Modena & Reggio Emilia , Modena , Italy.
authors.forename: Manuela
authors.initials: M
authors.lastname: Tondelli
authors.affiliation: a Department of Biomedical, Metabolic and Neural Science , University of Modena & Reggio Emilia , Modena , Italy.
authors.forename: Alessandra
authors.initials: A
authors.lastname: Ariatti
authors.affiliation: a Department of Biomedical, Metabolic and Neural Science , University of Modena & Reggio Emilia , Modena , Italy.
authors.forename: Francesca
authors.initials: F
authors.lastname: Benuzzi
authors.affiliation: a Department of Biomedical, Metabolic and Neural Science , University of Modena & Reggio Emilia , Modena , Italy.
authors.forename: Paolo
authors.initials: P
authors.lastname: Nichelli
authors.affiliation: a Department of Biomedical, Metabolic and Neural Science , University of Modena & Reggio Emilia , Modena , Italy.
authors.forename: Franco
authors.initials: F
authors.lastname: Valzania
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D000906:Antibodies / Q000097:blood*; D015331:Cohort Studies; D004185:Disability Evaluation; D006233:Persons with Disabilities; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D018992:Myelin-Associated Glycoprotein / Q000276:immunology*; D011115:Polyneuropathies / Q000097:blood* / Q000150:complications / Q000175:diagnosis; D012044:Regression Analysis; D012720:Severity of Illness Index
chemical_list: D000906:Antibodies; C115082:MAG protein, human; D018992:Myelin-Associated Glycoprotein
keywords: IgM titer; anti-MAG neuropathy; chronic inflammatory demyelinating polyradiculoneuropathy; health-related quality of life; intravenous immnonoglobulin; monoclonal gammopathy of undetermined significance