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III Meeting Neuroscienze Toscane 5-7 Aprile 2019 Viareggio

This study aims to investigate the clinical phenotype and long-term outcome of patients with MGUS-associated polyneuropathy. The study includes a retrospective review of patients evaluated at a neurology unit over a 15-year period, focusing on demographic data, clinical findings, hematologic and immunological data, EMG vs. immunological findings, therapy vs. immunological findings, and immune-based differences. The results will contribute to a better understanding of the treatment response and factors influencing disability recovery in patients with MGUS-associated polyneuropathy.

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III Meeting Neuroscienze Toscane 5-7 Aprile 2019 Viareggio

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  1. III Meeting Neuroscienze Toscane 5-7 Aprile 2019 Viareggio POLINEUROPATIA ASSOCIATA A GAMMOPATIA MONOCLONALE: UNO STUDIO OSSERVAZIONALE Stefano Filippini, Sabrina Matà, Alessandro Barilaro, Luca Massacesi, Sandro Sorbi (Firenze)

  2. Monoclonalgammopathy MGUS (monoclonal gammopathy of undetermined significance) is a common, age-related medical condition characterized by an accumulation of bone marrow plasma cells derived from a single abnormal clone without proliferation of malignant cells. There are threetypesof MGUS -IgM -non-IgM -light chains Paraproteins are detected in the serum of approximately 1 % of the general population. The prevalence of paraproteinemia rises with age, up to 5.3 % among individuals over 70 years and up to 10 % in people older than 80 years 

  3. MGUS-associatedneuropathies Distaldemyelinatingacquiredsymmetricneuropathy (DADS) Chronicinflammatorydemyelinatingpolyneuropathy (CIDP) –like Axonalsensorimotorperipheralneuropathy Multifocal motor neuropathy Multifocalmotor-sensoryneuropathy POEMS CANOMAD Few treatment options are nowavailableforthesepatients, whichincludingIVIg, plasma exchange and, recently, Rituximab.

  4. MGUS-associatedneuropathy: a localexperience Aimofthisstudywastoinvestigate the clinical phenotype and long-term outcome of patients with MGUS-associated polyneuropathy Retrospectivereviewof a consecutive seriesofpatientswithneuropathy and MGUS evaluatedduring a 15 yearperiodwhowereseen at NeurologyUnitofCareggi’s Hospital The patientswereclassifiedashavingaxonalneuropathy, demyelinatingneuropathy, or a mixedneuropathy, accordingto EMG Antibodies againstgangliosides, sulfatides and MAG weretestedby ELISA. The modifiedRankindisability scale wasusedtodeterminefunctionalimpairment at the timeof the maximal neurological deficit and after treatment A treatment responsewasdefinedasimprovementof at leastonegradeof the Rankin score for at leasttwomonths. Patientswithmalignancies, amiloidosis or otherdysimmune or metabolicdisorderswereexcluded.

  5. Results: Demographic Data

  6. Results: ClincalFindings

  7. Results: Haematologic Data

  8. Results: ImmunologicalFindings

  9. Results: EMG vsImmunologicalfindings

  10. Results: TherapyvsImmunologicalfindings

  11. Results: Immune-baseddifferences No reactivity MAG Gangliosides/Sulf No reactivity MAG Gangliosides/Sulf No reactivity MAG Gangliosides/Sulf

  12. Conclusions - 1 Distribution of electrophysiological, haematologic and immunologicalfindings in ourpatientsmirror the data from literature MGUS neuropathies are oftenmildlydisablingatpresentation (mRS=1/2 in 70% of patients), but 20% of patientshad a high mRSatonset (mRS=4 in 20% of cohort) Patients with reactivity to gangliosides/sulfatideshad a higher burden of disabilityatonset, butshowedmarked benefit from therapy (the majoritywastreated with IVIg)

  13. Conclusions - 2 Patients with reactivity to MAG and no reactivityhad a lower burden of disabilityatonset, butshowedless benefit from therapy (mostlytreated with rituximab) An higherIgMtiter and most severe deficit atpresentation can be predictive of a betterresponse to IGIv therapy in anti-ganglioside/sulfatidePNPs, asalreadydescribed in literature for Rituximab in anti-MAG PNPs (Dalakas, 2009). Nevertheless, IgMtiter and severity of disease are notcorrelated.

  14. What’snext Isresponse to therapy in anti-GM/S patientinfluenced by EMG pattern? Do Anti-GM/S patientshave the same benefit whentreated with IVIG and then Rituximab vs. rituximab only? Isthere an associationbetweendisability recovery and time from diagnosis to treatment?

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