Light chain deposition disease neuropathy resembling amyloid neuropathy in a multiple myeloma patient

Abstract

A 65-year-old man with IgG lambda multiple myeloma developed severe polyneuropathy with prominent thermal-pain sensory impairment and autonomic failure. Although the clinical presentation suggested amyloid neuropathy, nerve biopsy showed the immunohistochemical and ultrastructural features typical of light chain deposition disease (LCDD). A precise morphologic and clinical description of LCDD neuropathy is given for the first time in the present report.

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].…”

“…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 disease restricted to the brain: the first case report

“…In cases of systemic LCDD, the peripheral nerves were the only part of the nervous system to be occasionally affected, clinically manifesting as polyneuropathy [15]. The blood-brain barrier protects the central nervous system from the circulating, polymerized, misfolded proteins [10], preventing any type of systemic amyloidosis or systemic nonamyloid MIDD causing any harm to the central nervous system.…”

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].…”