Corneal nerves as a biomarker of peripheral neuropathy : the example of transthyretin amyloidosis

• Published in: Acta ophthalmologica (Oxford, England) England), 2017-09, Vol.95 (S259), p.n/a
• Main Authors: Rousseau, A., Cauquil, C., Dupas, B., Labbé, A., Baudouin, C., Lacroix, C., Guiochon‐Mantel, A., Benmalek, A., Labetoulle, M., Adams, D.
• Format: Article
• Language: English
• Subjects: Amyloidosis; Autonomic nervous system; Biopsy; Cornea; Evaluation; Nerves; Peripheral neuropathy; Rare diseases; Sensorimotor system; Skin; Transthyretin
• ISSN: 1755-375X; 1755-3768
• DOI: 10.1111/j.1755-3768.2017.03123

Transthyretin amyloidosis (TTR‐A) is a rare autosomic disease associated with severe sensorimotor and autonomic neuropathy. A practical and objective method for the clinical evaluation of TTR‐A neuropathy is needed to improve the management of this disease. We conducted a study on 15 patients and 15 controls to determine the correlations of sensorimotor and autonomic neuropathy with corneal nerve parameters in TTR‐A patients. We found that corneal nerve fiber length (CNFL) measured using IVCM was inversely correlated with the severity of autonomic and sensorimotor neuropathy, assessed both with clinical and paraclinical tests, including intra‐epidermal nerve length in skin biopsy. In these 15 subjects, IVCM measurement permitted rapid, non‐invasive evaluation of small‐fiber alterations in TTR‐A patients and could be used to assess neuropathy. CNFL could be measured in all patients, thus avoiding the floor effect seen with other neuropathy measures. Through the example of this rare disease, our study illustrates the emerging idea that corneal nerve parameters assessed with IVCM could become a potent surrogate biomarker for peripheral neuropathy.