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Huntington’s disease and neurovascular structure of retina

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Abstract

Background

Retinal biomarkers in neurodegenerative disorders have attracted much attention in recent years. Recent studies have reported visual dysfunction in Huntington’s disease (HD). However, little is known about retinal structural changes in HD.

Methods

A total of 50 subjects, including 25 motor-manifest HD patients and 25 gender- and age-matched controls, were enrolled. Unified Huntington’s Disease Rating Score-Motor part was assessed in HD patients. Spectral-domain Optical Coherence Tomography (SD-OCT) was used to evaluate the macular thickness and peripapillary retinal nerve fiber layer (pRNFL). Superficial and deep capillary plexus densities were measured using OCT angiography (OCTA). To account for inter-eye correlation, generalized estimating equation (GEE) model was used.

Results

HD patients had a significant reduction in macular thickness in both inner and outer superior sectors and the inferior outer sector. Inferior pRNFLs were significantly decreased in thickness. There was no significant difference in retinal capillary plexus density between the two groups. Age and disease duration were negatively correlated with macular thickness in HD patients. However, the severity of motor involvement was not correlated with SD-OCT or OCTA parameters.

Conclusions

We observed attenuated pRNFL and macular retinal thickness in patients with HD, independent of macular capillary plexus parameters. It can support the hypothesis that the retina may be a potential biomarker for monitoring the neurodegenerative process in HD.

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Authors and Affiliations

  1. Skull Base Research Center, The Five Senses Health Institute, Rasoul Akram Hospital, Iran University of Medical Sciences, Tehran, Iran

    Elahe Amini, Mehdi Moghaddasi, Zahra Azad & Mohammad Rohani

  2. Department of Neurology, Rasoul Akram Hospital, Iran University of Medical Sciences, Tehran, Iran

    Seyed Amir Hassan Habibi, Nahid Mohammadzadeh & Mohammad Rohani

  3. Neuro-Ophthalmology Division, Wilmer Eye Institute, Johns Hopkins Medicine, Baltimore, MD, USA

    Shahnaz Miri

  4. Eye Research Center, The Five Senses Health Institute, Rasoul Akram Hospital, Iran University of Medical Sciences, Tehran, Iran

    Naveed Nilforushan & Reza Mirshahi

  5. Neurology Department Hospital Universitario Burgos, University of Burgos, Burgos, Spain

    Esther Cubo

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  1. Elahe Amini
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Correspondence to Mohammad Rohani.

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Amini, E., Moghaddasi, M., Habibi, S.A.H. et al. Huntington’s disease and neurovascular structure of retina. Neurol Sci 43, 5933–5941 (2022). https://doi.org/10.1007/s10072-022-06232-3

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