August 2015
Volume 56, Issue 9
Letters to the Editor  |   August 2015
Small Fiber Neuropathy and Wilson Disease
Author Affiliations & Notes
  • Francisco de Assis Aquino Gondim
    Neurology Division Hospital Universitário Walter Cantídio, Fortaleza, Brazil; and
  • Joana Gurgel Holanda Filha
    Ophthalmology Division, Hospital Universitário Walter, Fortaleza, Brazil.
Investigative Ophthalmology & Visual Science August 2015, Vol.56, 5330. doi:
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      Francisco de Assis Aquino Gondim, Joana Gurgel Holanda Filha; Small Fiber Neuropathy and Wilson Disease. Invest. Ophthalmol. Vis. Sci. 2015;56(9):5330. doi:

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      © ARVO (1962-2015); The Authors (2016-present)

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We read with interest the article written by Sturniolo and colleagues1 and recently published in Investigative Ophthalmology and Visual Science. The authors evaluated 24 patients with Wilson disease (WD) and 24 controls by corneal confocal microscopy and reported that patients with WD exhibited significantly altered corneal subbasal nerve plexus (reduced number of fibers, number of branchings and beadings, as well as reduced nerve fiber length density, with associated increased fiber tortuosity). 
The cornea is densely innervated by sensory and autonomic fibers (C and Aδ fibers) from the ophthalmic branch of the trigeminal nerve.1 Confocal microscopy of the Meissner corpuscles also has been used to evaluate the presence of sensory neuropathy.2 A recent report by Chen et al.3 demonstrated that several parameters of corneal confocal microscopy and intraepidermal nerve fiber density strongly correlate with each other for the diagnosis of distal symmetric peripheral neuropathy in patients with diabetes mellitus. 
As pointed out by Sturniolo et al.,1 there are few studies evaluating the impact of WD on peripheral nervous system function. Initial reports described predominant demyelinating involvement, whereas subsequent reports focused on axonal impairment.1 We also recently reported small fiber neuropathy mostly correlating with somatic rather than autonomic small fibers in WD patients.4 Our patients had normal nerve conduction studies, but half of them had abnormal skin wrinkling test that, similar to corneal confocal microscopy, also strongly correlated with abnormalities on intraepidermal nerve fiber density.4 Interestingly, our patients with small fiber involvement and WD also had parkinsonism. In his series, Sturniolo et al.1 included 10 patients with significant central nervous system disease and 14 patients with predominant hepatic involvement. Although the authors did not perform autonomic or nerve conduction studies, we were curious to find out whether there was a difference between the small fiber changes in patients with central nervous system involvement in comparison with WD patients with predominant hepatic presentation. We are currently attempting to evaluate the extent of peripheral nervous system involvement in WD patients in a larger series of patients and follow those changes prospectively, correlating with the disease progression and the extent of central nervous system involvement. 
Sturniolo GC, Lazzarini D, Bartolo O, et al. Small fiber peripheral neuropathy in Wilson disease: an in vivo documentation by corneal confocal microscopy. Invest Ophthalmol Vis Sci. 2015; 56: 1390–1395.
Nolano M, Provitera V, Santoro L, et al. In vivo confocal microscopy of Meissner corpuscles as a measure of sensory neuropathy. Neurology. 2008; 71: 536–537.
Chen X, Graham J, Dabbah MA, et al. Small nerve fiber quantification in the diagnosis of diabetic sensorimotor polyneuropathy: comparing corneal confocal microscopy with intraepidermal nerve fiber density. Diabetes Care. 2015; 38: 1138–1144.
Gondim Fde A, Araújo DF, Oliveira IS, Vale OC. Small fiber dysfunction in patients with Wilson's disease. Arq Neuropsiquiatr. 2014; 72: 592–595.

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