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Home > West Medicine > Internal Medicine > Rheumatology
E85.101†

Amyloid Peripheral Neuropathy

风湿免疫科

Common symptoms

肝脾肿大、腹泻、肌肉萎缩、淀粉样变性、蛋白尿、低血压、感觉障碍

The pathology of disease

Amyloid peripheral neuropathy is amyloid-fibril deposition in peripheral nerves/autonomic ganglia, seen in AL (light-chain) and hereditary ATTR (transthyretin) amyloidosis. Clinical: progressive distal symmetric sensory loss (pain/temperature first), motor weakness, and autonomic neuropathy (orthostatic hypotension, diarrhea/constipation, erectile dysfunction). Treatment: treat underlying amyloid (chemotherapy for AL, liver transplant/tafamidis for hereditary ATTR), supportive neuropathic and autonomic care.

Common Symptoms

Distal sensory loss; painful neuropathy; motor weakness; orthostatic hypotension; GI dysautonomia; carpal tunnel syndrome

Causes and Pathogenesis

Misfolded light-chain (AL) or mutant transthyretin (ATTR) fibrils deposit in peripheral nerve/autonomic fibers, causing axonal degeneration.

Treatment

Treat underlying amyloid (chemotherapy/liver transplant/tafamidis); neuropathic pain control; autonomic support; physical therapy

Preventive Measures

Early diagnosis; family screening in ATTR; manage symptoms.

Department to Visit

It is recommended to visit the Neurology department; an appointment can be booked on this platform before the visit.

Warm reminder: The above information is for health science reference only and cannot replace professional medical diagnosis. If you have related symptoms, please seek medical attention at a regular hospital promptly.

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