What is it about?
Hereditary spastic paraplegia (HSP) is a rare inherited neurodegenerative disorder characterized by progressive degeneration of distal axons in the long corticospinal tracts. Loss of retinal cells and microvascular networks has neither been suspected nor investigated. In this study we explored whether patients with HSP display concurrent changes in retinal microvascular networks and retinal morphology detectable by optical coherence tomography angiography (OCT-A) and optical coherence tomography (OCT).
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Why is it important?
This study shows for the first time that microvascular and structural retinal changes can be detected with OCT-A and OCT imaging in HSP, indicating that both cells and vascular networks in the retina are compromised. Future focus should be placed particularly on the status of the retinal ganglion cell layer, inner plexiform layer and superficial vascular plexus for disease monitoring, and even as novel structural outcome measures for future clinical trials.
Perspectives
Since the first clinical and pathological description of HSP in the late 19th century, HSP has been widely understood and accepted as a distal axonopathy of the longest large myelinated fibers of the spinal tract. Impairment of the retinal ganglion cell layer along with its synapses in the inner plexiform layer, however, has not been reported or suggested as a possible disease characterizing feature. We believe these novel findings may further enhance our understanding of this rare group of disorders and be an initiative for future research to delve deeper into the underlying disease mechanisms.
Dr. Gabrielle Turski
University of Virginia
Read the Original
This page is a summary of: Retinal ganglion cell and microvascular density loss in hereditary spastic paraplegia, Restorative Neurology and Neuroscience, January 2024, IOS Press,
DOI: 10.3233/rnn-231380.
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