Test Your Knowledge of hATTR Polyneuropathy
Reviewed by: HU Medical Review Board | Last reviewed: July 2026 | Last updated: August 2026
Hereditary transthyretin amyloidosis with polyneuropathy (hATTR-PN) is a progressive, treatable neuropathy that is frequently mislabeled and remains frequently underrecognized. Delayed diagnosis can result in irreversible nerve injury and missed opportunities for disease-modifying treatment.
This 5-question clinical challenge spans the knowledge that most directly shapes early recognition and management: red-flag clustering, the chronic inflammatory demyelinating polyneuropathy (CIDP) differential, the mechanism of disease-modifying therapy, autonomic presentation, and disease monitoring. See how sharp your diagnostic and therapeutic reasoning is.
Clinical Challenge
In a patient with progressive idiopathic axonal polyneuropathy, which combination of features should most prompt transthyretin (TTR) genetic testing?
Clinical Challenge
Non-response to immunotherapy in a patient labeled with CIDP should prompt reconsideration of the diagnosis. Which combination of electrophysiologic and clinical findings should instead raise suspicion for hATTR-PN?
Clinical Challenge
TTR silencer therapies and TTR stabilizers act by fundamentally different mechanisms. How do the silencer classes, RNA interference (RNAi) and antisense oligonucleotide (ASO), act compared with stabilizers?
Clinical Challenge
A patient with a length-dependent sensory neuropathy also reports orthostatic lightheadedness, early satiety, and unintentional weight loss. What best explains these non-sensorimotor features?
Clinical Challenge
Which measure tracks neuropathy progression and treatment response in hATTR-PN and is being studied as an early marker of axonal damage?