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Area of Science:

  • Neurology
  • Genetics
  • Internal Medicine

Background:

  • Peripheral neuropathy is a common manifestation of both hereditary transthyretin amyloidosis (hATTR) and acquired immunoglobulin light chain (AL) amyloidosis.
  • hATTR stems from TTR gene mutations, while AL amyloidosis is the most frequent acquired form.
  • Neurologic symptoms include sensorimotor polyneuropathy, focal neuropathy (e.g., carpal tunnel syndrome), and autonomic neuropathy, often delaying diagnosis when presenting solely.

Purpose of the Study:

  • To review the epidemiology, clinical presentations, pathophysiology, diagnostic workup, and treatment of neuropathy in systemic amyloidosis.
  • To highlight the neurologist's role in early diagnosis and treatment initiation.
  • To discuss advancements in therapeutic options for AL and hATTR amyloidosis.

Main Methods:

  • Review of existing literature on amyloidosis and associated neuropathies.
  • Analysis of clinical presentations and diagnostic approaches.
  • Summary of current and emerging treatment strategies.

Main Results:

  • Neuropathy is a key indicator for both hATTR and AL amyloidosis.
  • Delayed diagnosis is common when neuropathy is the primary symptom.
  • New therapies (proteasome inhibitors, TTR silencers, TTR stabilizers) offer improved treatment outcomes.

Conclusions:

  • Early diagnosis of neurologic manifestations of systemic amyloidosis is crucial.
  • Neurologists are vital in identifying patients who can benefit from emerging therapies.
  • Advances in treatment offer new hope for patients with hATTR and AL amyloidosis.