The natural history of peripheral neuropathy in primary systemic amyloidosis

Abstract

The records of 31 patients with primary systemic amyloidosis and peripheral neuropathy seen during a 17-year period were analyzed to define the natural history of the neuropathy. Patients tended to be older men with a painful, distal, symmetrical sensorimotor neuropathy and prominent autonomic features. Loss of pain and temperature sensation was frequently more striking than loss of mechanoreception. Renal, cardiac, hematological, and gastrointestinal dysfunction often overshadowed the neuropathy. Clinical, neurophysiological, and histopathological studies pointed to axonal degeneration with predominant but not exclusive involvement of small myelinated and unmyelinated fibers. The neuropathy was progressive in all patients with or without treatment, but death was typically due to supervening medical complications.

Lymphoma and peripheral neuropathy: A clinical review

“…37 Also, in the setting of a monoclonal paraprotein, amyloidosis may cause neuropathy. 68,99 Lymphoma cells can affect peripheral nerves remotely, causing generalized neuropathies. 57,58,96,132 Examples include NHL, chronic lymphocytic leukemia, Waldenström's macroglobulinemia, and osteosclerotic myeloma.…”

“…81 Chronic lymphocytic leukemia and Waldenström's macroglobulinemia may cause polyneuropathy by nerve infiltration or autoimmunity. Waldenström's macroglobulinemia can cause amyloid neuropathy 68 and neuropathy with antibodies directed at myelin-associated glycoproteins. 65,66 In addition to these disorders, osteosclerotic myeloma and Castleman's disease (angiofollicular lymph node hyperplasia), a nonmalignant lymphoproliferative syndrome, can also cause neuropathies.…”

Upper limb neuropathy such as carpal tunnel syndrome as an initial manifestation of ATTR Val30Met familial amyloid polyneuropathy

“…Bilateral CTS usually accompanies various systemic diseases including amyloidosis, producing heavy deposition of amyloid on the tenosynovium of the wrist. CTS is well known to be an initial symptom in long term hemodialysis-related amylodosis [1], primary systemic AL amyloidosis [2], senile systemic amyloidosis [3,4], and some forms of familial amyloid polyneuropathy (FAP) with amyloidogenic transthyretin (ATTR) non-Val30Met (other mutations except for Val30Met) [5,6]. However, in the most common form of FAP with ATTR Val30Met CTS has not been noted [7].…”

Axoval neuropathy as initial manifestation of primary amyloidosis: report of a case submitted to bone marrow transplantation

“…It is more prevalent in males, in a proportion of 2:1. Deposition of amyloid fibrils in peripheral nerves occurs in up to 20% of AL cases, and most patients have more prominent sensory symptoms; however, some individuals might have greater involvement of the autonomous nervous system and carpal tunnel [5][6] . Autonomic neuropathy may cause orthostatic hypotension, diarrhea or impotence.…”

“…Symptoms of distal sensorial involvement are common, and pain and temperature sensations are more significant than deep sensibility. The course of neuropathy is gradually progressive, involving the sensory, motor and autonomic portions, making most patients unable to walk during the advanced stages of the disease 5 . In most cases CSF examination in AL patients shows increased protein levels, and protein electrophoresis may reveal elevated IgG fractions 6,9 .…”