Amyloid Neuropathies

Overview

Disorders of peripheral nerves are the most common neurological complications of systemic amyloidosis; an illness where a protein called amyloid is deposited in tissues and organs. Amyloidosis can affect peripheral sensory, motor or autonomic nerves and deposition of amyloid lead to degeneration and dysfunction in these nerves.

Symptoms

  • Sensory impairment
  • Autonomic dysfunction
  • Painful paresthesia
  • Unusual sensations
  • Numbness
  • Balance problems
  • Chronic nausea
  • Vomiting
  • Chronic diarrhea
  • Chronic constipation
  • Incontinence
  • Sweating abnormalities
  • Sexual dysfunction

Diagnosis

Diagnosis of amyloid neuropathies is based on history, clinical examination and supporting laboratory investigations. These include electromyography with nerve conduction studies, skin biopsies to evaluate cutaneous nerve innervation, and nerve and muscle biopsies for histopathological evaluation. In cases of familial amyloidosis, genetic testing in the blood may be useful.

Treatment

Treatment of amyloid neuropathies is directed at both preventing further deposition of amyloid in peripheral nerves and treating painful symptoms. Depending on the type of amyloid protein, patients may benefit from liver or bone marrow transplant. Neuropathic pain due to amyloid neuropathy can be treated with anti-seizure medications such as gabapentin (Neurontin), pregabalin (Lyrica) and topiramate (Topamax), antidepressants such as amitritptylline (Elavil) and duloxetine (Cymbalta), or analgesics including opiate drugs. In severe painful conditions patients may be referred to the Blaustein Chronic Pain Clinic for a multidisciplinary approach to pain management.