Amyloid neuropathy

Systemic amyloidosis complicates the course of myeloma in 7-10% of patients. Amyloid neuropathy occurs in about 15% of patients with systemic AL amyloidosis.5677 In the nervous system, amyloid may be deposited, often focally, in epineurial and endoneurial connective tissues and vessel walls of peripheral nerves, dorsal ganglia, and autonomic ganglia.78 Sural nerve biopsy reveals axonal degeneration with a lesser degree of demyelination, affecting mostly unmyelinated or lightly myelinated fibers.79 The absence of blood-nerve barrier in dorsal root and sympathetic ganglia may predispose these sites to amyloid deposi-tion.80 Electrodiagnostic studies show decreased action potential amplitude, reduced sensory responses, and normal or mildly reduced conduction velocity.81 The neuropathy is progressive, sensory dominant, with prominent pain and temperature sense loss. The most common initial presentation is burning or painful paresthesia with mild weakness of lower extremities. Symptoms of autonomic dysfunction include ortho-static hypotension, impotence, and urinary retention.

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