AUTHOR=Wong Bryan M. , Hudson Christopher , Snook Emily , Tayyari Faryan , Jung Hyejung , Binns Malcolm A. , Samet Saba , Cheng Richard W. , Balian Carmen , Mandelcorn Efrem D. , Margolin Edward , Finger Elizabeth , Black Sandra E. , Tang-Wai David F. , Zinman Lorne , Tan Brian , Lou Wendy , Masellis Mario , Abrahao Agessandro , Frank Andrew , Beaton Derek , Sunderland Kelly M. , Arnott Stephen R. , ONDRI Investigators , Tartaglia Maria Carmela , Hatch Wendy V. , Adamo Sabrina , Arnott Stephen , Bartha Rob , Beaton Derek , Berezuk Courtney , Black Alanna , Bonnick Alisia , Breen David , Brien Don , Bronskill Susan , Bulman Dennis , Casaubon Leanne , Chen Ying , Chum Marvin , Coe Brian , Cornish Ben , Dewar Jane Lawrence , Dixon Roger A. , El-Defrawy Sherif , Farhan Sali M.K. , Faria Frederico , Fraser Julia , Ghani Mahdi , Greenberg Barry , Haddad Hassan , Hatch Wendy , Holmes Melissa , Hudson Chris , Kleinstiver Peter , Kwan Donna , Leontieva Elena , Levine Brian , Lou Wendy , Mandelcorn Efrem D. , Margolin Ed , Marras Connie , McIlroy Bill , McLaughlin Paula , Odasso Manuel Montero , Munoz Doug , Munoz David , Nanayakkara Nuwan , Orange JB , Ozzoude Miracle , Peltsch Alicia , Raamana Pradeep , Ramirez Joel , Rashkovan Natalie , Roberts Angela , Adamson Yanina Sarquis , Scott Christopher , Strong Michael , Strothers Stephen , Sujanthan Sujeevini , Sunderland Kelly M. , Symons Sean , Tayyari Faryan , Theyers Athena , Troyer Angela , Uthirakumaran Abiramy , Ooteghem Karen Van , Woulfe John , Zamyadi Mojdeh , Zou Guangyong (GY) TITLE=Retinal nerve fiber layer in frontotemporal lobar degeneration and amyotrophic lateral sclerosis JOURNAL=Frontiers in Neuroscience VOLUME=16 YEAR=2022 URL=https://www.frontiersin.org/journals/neuroscience/articles/10.3389/fnins.2022.964715 DOI=10.3389/fnins.2022.964715 ISSN=1662-453X ABSTRACT=Purpose

Tauopathy and transactive response DNA binding protein 43 (TDP-43) proteinopathy are associated with neurodegenerative diseases. These proteinopathies are difficult to detect in vivo. This study examined if spectral-domain optical coherence tomography (SD-OCT) can differentiate in vivo the difference in peripapillary retinal nerve fibre layer (pRNFL) thickness and macular retinal thickness between participants with presumed tauopathy (progressive supranuclear palsy) and those with presumed TDP-43 proteinopathy (amyotrophic lateral sclerosis and semantic variant primary progressive aphasia).

Study design

Prospective, multi-centre, observational study.

Materials and methods

pRNFL and macular SD-OCT images were acquired in both eyes of each participant using Heidelberg Spectralis SD-OCT. Global and pRNFL thickness in 6 sectors were analyzed, as well as macular thickness in a central 1 mm diameter zone and 4 surrounding sectors. Linear mixed model methods adjusting for baseline differences between groups were used to compare the two groups with respect to pRNFL and macular thickness.

Results

A significant difference was found in mean pRNFL thickness between groups, with the TDP-43 group (n = 28 eyes) having a significantly thinner pRNFL in the temporal sector than the tauopathy group (n = 9 eyes; mean difference = 15.46 μm, SE = 6.98, p = 0.046), which was not significant after adjusting for multiple comparisons. No other significant differences were found between groups for pRNFL or macular thickness.

Conclusion

The finding that the temporal pRNFL in the TDP-43 group was on average 15.46 μm thinner could potentially have clinical significance. Future work with larger sample sizes, longitudinal studies, and at the level of retinal sublayers will help to determine the utility of SD-OCT to differentiate between these two proteinopathies.