Amyloid Neuropathy and Tremor in Waldenstrom's Macroglobulinemia

Clinical and pathological heterogeneity in a series of 31 patients with IgM-related neuropathy

“…Multiple mononeuropathy was observed in 5/13 patients, with predominant involvement of the upper limbs in three (#1, 6, 7) and of cranial nerves in the other two (#2, 8). Symmetric polyneuropathy with predominant motor impairment was observed in 7/13 patients (#3, 4,5,9,10,11,13). The remaining one patient (#12) had painful neuropathy, characterized by shooting pain and burning in her lower limbs and hands, due to predominant small-fibre involvement, as previously described [16].…”

“…We treated with glucocorticoids 10/13 patients (#2, 4,5,6,7,9,10,11,12,13), all of whom reported symptomatic improvement except for one (#12) who responded poorly, as we have previously reported [16]. Of the remaining 3/13 patients, two refused therapy (#1, 3) and one (#8) was treated with chemotherapy for an underlining WM with a good response [9].…”

“…However, the pathogenic mechanisms of IgM-related neuropathy are heterogeneous, including an autoimmune demyelinating process associated with anti-MAG (myelin associated glycoprotein) antibodies [8], endoneurial deposition of immunoglobulins or light chains [9][10][11], vasculitis [12], amyloid deposition [13] and infiltration by malignant cells [14].…”

Light chain deposition in peripheral nerve as a cause of mononeuritis multiplex in Waldenström's macroglobulinaemia

“…Neurological complications develop in 50% of patients with WM and generally consist of symmetrical sensorimotor peripheral neuropathy, whereas mononeuritis or mononeuritis multiplex are less common [3,4]. The pathogenetic mechanisms of IgM-related neuropathy are heterogeneous, including an autoimmune demyelinating process associated with anti-MAG (myelin associated glycoprotein) antibodies [5], endoneurial deposition of immunoglobulins or light chains [6,7], vasculitis [8], amyloid deposition [9] and infiltration by malignant cells [10].…”

Hereditary and acquired amyloid neuropathies

“…Monoclonal immunoglobulins or light chains are detected in serum and/or urine in 75 % of these patients at referral [2,20]. In 40 % of patients, amyloid neuropathy occurs in a context of overt malignant lymphoproliferative disorders [2] including multiple myeloma [2,14,31,55] or Waldenström macroglobulinemia [2,9,10,39] which are usually diagnosed at the time of the neuropathy. Neuropathy does not occur in secondary "AA" amyloidosis which may complicate chronic inflammatory or infectious diseases [20].…”