Treatment for IgG and IgA paraproteinaemic neuropathy

Derivation and validation of diagnostic criteria for chronic inflammatory demyelinating polyneuropathy

“…First, the evaluation of patients with suspected CIDP demonstrated the relevance of obtaining a careful family history, as several patients initially diagnosed as CIDP had upon further investigation a genetic neuropathy in the absence of a family history as has been previously reported, [21,22]. Second, the laboratory evaluation in those with neuropathy should include serum immunofixation electrophoresis or an equivalent test, since the presence of a serum paraprotein may be indicative of a specific diagnosis, such as osteosclerotic myeloma or Waldenström's macroglobulinemia, with implications for therapy and prognosis [18][19][20]. Lastly, in our cohort lumbar puncture and nerve biopsy were infrequently performed.…”

“…The primary rationale for this decision was evidence suggesting that the course and response to therapy may be different in patients with CIDP with and without a serum paraprotein [18][19][20] and the fact that hereditary neuropathies have a clear genetic basis. These patients were considered, by definition, to be true negatives, which may have resulted in inflated specificity estimates.…”

Intravenous Immunoglobulins in Neurological Diseases: Established and Novel Clinical Applications

“…A third randomized study was an open parallel group trial with 20 patients which compared IVIg and recombinant interferona (class II). The results of these three trials have been summarized in a Cochrane review, which concluded that IVIg is relatively safe and may produce some short-term benefit (Lunn & Nobile-Orazio, 2006 (Allen et al, 2007). Recommendations: -IVIg should be considered as initial treatment of demyelinating IgM MGUS-related neuropathy (level B recommendation).…”

Immunomodulatory Therapies in Neurologic Critical Care

“…More recently, rituximab has shown efficacy as well [44]. There is currently limited data for the use of immunomodulatory therapies for IgG and IgA paraproteinemic neuropathies [45].…”