ArticleOrphanet journal of rare diseases2022
Small fiber neuropathy for assessment of disease severity in amyotrophic lateral sclerosis: corneal confocal microscopy findings.
Article in Orphanet journal of rare diseases, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers, 1 of them a synthesis that pooled it.
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Who cites it
11 citing papers in PubMed, 1 synthesis or guideline pooled it, 18 citations in OpenAlex.
- Exploring amyotrophic lateral sclerosis through the visual system: A systematic review.European journal of neurology · 2024Pooled it
- Influence of Immune Cells on Corneal Nerve Morphological Analysis and Clinical Relevance in Diabetes-Related Dry Eye.Translational vision science & technology · 2025Article
- Corneal confocal microscopy differentiates patients with secondary parkinsonism from idiopathic Parkinson's disease.NPJ Parkinson's disease · 2025Article
- Cornea Oculomics: A Clinical Blueprint for Extending Corneal Diagnostics and Artificial Intelligence in Systemic Health Insights.Diagnostics (Basel, Switzerland) · 2025Review
- Influence of dendritic cells on corneal nerve morphological analysis and clinical relevance in chronic dry eye disease after femtosecond laser-assisted laserFrontiers in medicine · 2025Article
- Small Fiber Neuropathy Associated with Post-COVID-19 and Post-COVID-19 Vaccination Arthritis: A Rare Post-Infective Syndrome or a New-Onset Disease?Journal of personalized medicine · 2024Article
- Corneal nerves and amyotrophic lateral sclerosis: an in vivo corneal confocal imaging study.Journal of neurology · 2024Article
- Corneal Sub-Basal Nerve Plexus in Non-Diabetic Small Fiber Polyneuropathies and the Diagnostic Role of In Vivo Corneal Confocal Microscopy.Journal of clinical medicine · 2023Review
- The severity of corneal nerve loss differentiates motor subtypes in patients with Parkinson's disease.Therapeutic advances in neurological disorders · 2023Article
- Corneal Confocal Microscopy and the Nervous System: Introduction to the Special Issue.Journal of clinical medicine · 2022Article
- Article
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Authors and funding
8 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundAmyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder with progressive motor system impairment, and recent evidence has identified the extra-motor involvement. Small fiber neuropathy reflecting by sensory and autonomic disturbances in ALS has been reported to accompany the motor damage. However, non-invasive assessment of this impairment and its application in disease evaluation of ALS is scarce. We aim to evaluate the use of corneal confocal microscopy (CCM) to non-invasively quantify the corneal small fiber neuropathy in ALS and explore its clinical value in assessing disease severity of ALS.
methodsSixty-six patients with ALS and 64 healthy controls were included in this cross-sectional study. Participants underwent detailed clinical assessments and corneal imaging with in vivo CCM. Using ImageJ, the following parameters were quantified: corneal nerve length (IWL) and dendritic cell density (IWDC) in the inferior whorl region and corneal nerve fiber length (CNFL), nerve fiber density (CNFD), nerve branch density (CNBD), and dendritic cell density (CDC) in the peripheral region. Disease severity was evaluated using recognized scales.
resultsCorneal nerve lengths (IWL and CNFL) were lower while dendritic cell densities (IWDC and CDC) were higher in patients with ALS than controls in peripheral and inferior whorl regions (p < 0.05). Additionally, corneal nerve complexity in the peripheral region was greater in patients than controls with higher CNBD (p = 0.040) and lower CNFD (p = 0.011). IWL was significantly associated with disease severity (p < 0.001) and progression (p = 0.002) in patients with ALS. Patients with bulbar involvement showed significantly lower IWL (p = 0.014) and higher IWDC (p = 0.043) than patients without bulbar involvement.
conclusionsCCM quantified significant corneal neuropathy in ALS, and alterations in the inferior whorl region were closely associated with disease severity. CCM could serve as a noninvasive, objective imaging tool to detect corneal small fiber neuropathy for clinical evaluation in ALS.
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