Huntington's disease and neurovascular structure of retina.


Journal

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
ISSN: 1590-3478
Titre abrégé: Neurol Sci
Pays: Italy
ID NLM: 100959175

Informations de publication

Date de publication:
Oct 2022
Historique:
received: 08 05 2022
accepted: 17 06 2022
pubmed: 1 7 2022
medline: 17 9 2022
entrez: 30 6 2022
Statut: ppublish

Résumé

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. 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. 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. 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.

Sections du résumé

BACKGROUND 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 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 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 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.

Identifiants

pubmed: 35771295
doi: 10.1007/s10072-022-06232-3
pii: 10.1007/s10072-022-06232-3
doi:

Substances chimiques

Biomarkers 0

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

5933-5941

Informations de copyright

© 2022. Fondazione Società Italiana di Neurologia.

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Auteurs

Elahe Amini (E)

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

Mehdi Moghaddasi (M)

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

Seyed Amir Hassan Habibi (SAH)

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

Zahra Azad (Z)

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

Shahnaz Miri (S)

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

Naveed Nilforushan (N)

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

Reza Mirshahi (R)

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

Esther Cubo (E)

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

Nahid Mohammadzadeh (N)

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

Mohammad Rohani (M)

Skull Base Research Center, The Five Senses Health Institute, Rasoul Akram Hospital, Iran University of Medical Sciences, Tehran, Iran. mohammadroohani@gmail.com.
Department of Neurology, Rasoul Akram Hospital, Iran University of Medical Sciences, Tehran, Iran. mohammadroohani@gmail.com.

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