Clinical implications of photophobia in progressive supranuclear palsy

Mohanty, Deepankar; Hay, Kaitlyn R; Berkowitz, Sean T.; Patel, Shriji; Lin, Ya-Chen; Kang, Hakmook; Claassen, Daniel O.

doi:10.1016/j.prdoa.2021.100097

Cited by 4 publications

(7 citation statements)

References 13 publications

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“…Recently, photophobia has attracted increasing attention and is considered a frequent symptom of PSP that significantly negatively affects daily activities [ 6 ]. We found that 42.1% of our PSP patients had photophobia, which is slightly lower than the prevalence of 43%–100% reported in previous studies [ 1 , 6 , 31 ]. To date, the pathophysiology of photophobia in PSP patients has not been elucidated.…”

Section: Discussionmentioning

confidence: 99%

“…The various visual symptoms suggest not only oculomotor dysfunction but also abnormalities in the retina and central processing mechanisms in PSP patients. Notably, the prevalence of photophobia in patients with PSP varies from 43% to 100% (depending on the evaluation tool that is used), which is significantly higher than that in patients with Parkinson’s disease (PD) and corticobasal degeneration [ 5 , 6 ]. It has been shown that intrinsically photosensitive retinal ganglion cells (ipRGCs) in the retina play an important role in photophobia [ 5 ].…”

Section: Introductionmentioning

confidence: 99%

“…Notably, the prevalence of photophobia in patients with PSP varies from 43% to 100% (depending on the evaluation tool that is used), which is significantly higher than that in patients with Parkinson's disease (PD) and corticobasal degeneration. 5,6 It has been shown that intrinsically photosensitive retinal ganglion cells (ipRGCs) in the retina play an important role in photophobia. 5 However, it remains unknown as to whether photophobia in patients with PSP is related to retinal thickness.…”

Section: Introductionmentioning

confidence: 99%

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Retinal Thinning as a Marker of Disease Severity in Progressive Supranuclear Palsy

Chen,

Wang,

Wang

et al. 2024

JMD

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Objective Progressive supranuclear palsy (PSP) involves a variety of visual symptoms that are thought to be partially caused by structural abnormalities of the retina. However, the relationship between retinal structural changes, disease severity, and intracranial alterations remains unknown. We investigated distinct retinal thinning patterns and their relationship with clinical severity and intracranial alterations in a PSP cohort.Methods We enrolled 19 patients with PSP (38 eyes) and 20 age-matched healthy controls (40 eyes). All of the participants underwent peripapillary and macular optical coherence tomography. Brain 11C-2β-carbomethoxy-3β-(4-fluorophenyl) tropane (11C-CFT) and 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography imaging were also performed in patients with PSP. We investigated the association between retinal thickness changes and clinical features, striatal dopamine transporter availability, and cerebral glucose metabolism.Results The peripapillary retinal nerve fiber layer (pRNFL) and macula were significantly thinner in patients with PSP than in controls. The thickness of the superior sector of the pRNFL demonstrated a significant negative relationship with the Movement Disorder Society-Unified Parkinson’s Disease Rating Scale part III and Hoehn and Yahr staging scale scores. A significant negative correlation was found between outer inferior macular thickness and disease duration. Outer temporal macular thickness was positively correlated with Montreal Cognitive Assessment scores. In PSP, lower outer temporal macular thickness was also positively correlated with decreased dopamine transporter binding in the caudate.Conclusion The pRNFL and macular thinning may be candidate markers for monitoring disease severity. Additionally, macular thinning may be an in vivo indicator of nigrostriatal dopaminergic cell degeneration in PSP patients.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Retinal Thinning as a Marker of Disease Severity in Progressive Supranuclear Palsy

Chen,

Wang,

Wang

et al. 2024

JMD

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“…Since dysfunction may be associated with disease involvement of midbrain parasympathetic pathways [22], photophobia may reflect a greater alpha-synuclein burden in midbrain regions generally including the substantia nigra and therefore faster motor decline. Alternatively, light sensitivity may impair vision, thereby impacting function, which has been observed in progressive supranuclear palsy [23].…”

Section: Discussionmentioning

confidence: 99%

Early Autonomic Symptoms Predict Functional Decline in Parkinson’s Disease Independent of Dopaminergic Therapy

2022

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Introduction: Parkinson’s disease (PD) has variable progression; identifying determinants of functional decline in PD is needed for accurate prognostication. Autonomic symptoms can result from dopaminergic therapy, but may also independently predict functional decline. Methods: The sample included individuals with newly-diagnosed PD in Parkinson’s Progression Markers Initiative. Autonomic symptoms were measured with the Scales-for-Outcomes-in-Parkinson’s-Disease-Autonomic (SCOPA-AUT). Presence/absence of autonomic symptoms for SCOPA-AUT total scale and seven subscales was defined and baseline demographic/clinical data were compared between groups with and without autonomic symptoms. Time-to-functional-dependence, or Schwab-and-England Activities-of-Daily-Living (SE-ADL) ≤70, was compared between groups using hazard models, adjusting for covariates including time-varying levodopa-equivalent daily dosage (LEDD). If a subscale was associated with a significant subhazard ratio (SHR), hazard models for items in that subscale were generated and gender was evaluated as an effect modifier. Results: 399 participants were included. Over a median of 72 months (range 6-84), 91 (22.81%) reached SE-ADL≤70. SCOPA-AUT total/gastrointestinal/urinary/pupillomotor scales were associated with SE-ADL≤70; respective multivariable SHRs (95% CI, p-value) were 1.98 (1.06-3.70, .03), 1.71 (1.04-2.81, .03), 1.94 (1.25-3.01, <.01), 2.56 (1.24-5.31, .01). Individual items in the gastrointestinal and urinary scales drove associations. Urinary scale associations were seen in males only. Conclusions: Symptoms of gastrointestinal, urinary, pupillomotor dysfunction are associated with functional decline risk in PD, independent of dopaminergic therapy. Detailed assessments of autonomic symptomatology should be utilized in studies attempting to refine predictive models of PD progression.

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“…Photophobia was first associated with ophthalmological diseases, such as lesions of the cornea, and subsequently with neurologic and neurogeriatric conditions, such as migraine [5], blepharospasm [6], and progressive supranuclear palsy (PSP) [7]. Photophobia, present in 43%-100% of PSP patients [8], appears to cause a reduction in quality of life, with an impact on outdoor activities [9]. Photophobia has been very rarely studied in other neurocognitive diseases.…”

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confidence: 99%

Neural correlates of photophobia in prodromal and mild dementia with Lewy bodies

Tisserand

Crétin

Mondino

et al. 2023

Euro J of Neurology

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Background and objectives Photophobia is a sensory disturbance provoked by light. Little is known about the association between photophobia and dementia with Lewy bodies (DLB). In this study, we aimed to identify the frequency and the neural basis of photophobia in prodromal and mild DLB. Methods One hundred and thirteen DLB patients, 53 Alzheimer's disease (AD) patients, 20 AD and DLB patients, 31 patients with other neurocognitive diseases (including prodromal and mild demented patients), and 31 healthy elderly controls were included in this case–control study. Photophobia was systematically looked for and compared between groups. Among a selection of 77 DLB patients, we used voxel‐based morphometry (VBM) to compare those with and those without photophobia (gray matter volume; SPM12, XjView, and Matlab R2021b software). Results The frequency of photophobia was higher in the DLB group (47.3%) than in the other groups (p = 0.002). The photophobia questionnaire score was higher in the DLB group than in the AD group (p = 0.001). Comparison between DLB patients with and those without photophobia showed decreased gray matter in the photophobia subgroup, in the right precentral cortex, in the eyelid motor region of Penfield's homunculus (p = 0.007, family‐wise error [FWE] corrected). Conclusions Photophobia is a quite frequent symptom of prodromal and mild DLB. The neural basis of photophobia in DLB involves the right precentral cortex, which could have a role in the decrease of cerebral excitability, but also the motricity of the eyelids.

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Clinical implications of photophobia in progressive supranuclear palsy

Cited by 4 publications

References 13 publications

Retinal Thinning as a Marker of Disease Severity in Progressive Supranuclear Palsy

Retinal Thinning as a Marker of Disease Severity in Progressive Supranuclear Palsy

Early Autonomic Symptoms Predict Functional Decline in Parkinson’s Disease Independent of Dopaminergic Therapy

Neural correlates of photophobia in prodromal and mild dementia with Lewy bodies

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