Objective: We present here two cases of atypical parkinsonism with visual field loss to help increase recognition and drive research efforts forward to elucidate the underlying causes and management of visual field loss in atypical parkinsonian syndromes.

Background: Atypical parkinsonian syndromes, including MSA, DLB, and PSP, are characterized by parkinsonism (e.g., rest tremors, rigidity, bradykinesia, and postural instability) along with atypical features such as early onset dementia, dysautonomia, frequent falls, or ataxia. There are currently limited reports and research describing why patients with atypical parkinsonism develop reproducible visual field loss despite lack of discernable damage in the visual pathway system.

Method: Two patients with atypical parkinsonism were evaluated in the neuro-ophthalmology clinic after being found to have persistent homonymous hemianopsia on both confrontation visual field exam and formal automated perimetry visual field testing. Further diagnostic tests were performed to investigate the potential etiology of their atypical parkinsonism and visual field deficits.

Results: Both patients also exhibited features of simultanagnosia and color agnosia on formal neuro-ophthalmological testing. They also had evidence of cognitive impairments as evident by low Montreal Cognitive Assessment (MoCA) scores. Neuro-imaging did not reveal corresponding cerebral lesion, specifically in the occipital lobes. One patient was later diagnosed with corticobasal syndrome, while the other patient had Dementia with Lewy Bodies.

Conclusion: We propose that the visual field defects in our patients were likely due to the co-existing posterior cortical atrophy (a variant of Alzheimer’s disease) in addition to their atypical parkinsonism. These two cases highlight the potential implications of the comorbid neurodegenerative diseases. Clinicians should conduct extensive counselling with patients and their care partners to adapt to the progressive visual deficits and other neurological deficits the patients may experience as their diseases progress.

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References: McFarland NR. Diagnostic Approach to Atypical Parkinsonian Syndromes. Continuum (Minneap Minn). 2016;22(4 Movement Disorders):1117-1142. doi:10.1212/CON.0000000000000348
Bashir K, Elble RJ, Ghobwith M, et al. Hemianopsia in Dementia with Lewy Bodies. Arch Neurol. 1998;55(8):1132–1135. doi:10.1001/archneur.55.8.1132
Schott JM, Crutch SJ. Posterior Cortical Atrophy. Continuum (Minneap Minn). 2019;25(1):52-75. doi:10.1212/CON.0000000000000696
Margolin E, Donaldson L. Homonymous hemianopia as the presenting sign of posterior cortical atrophy. Pract Neurol. 2022;22(2):160-161. doi:10.1136/practneurol-2021-003249
Crutch SJ, Schott JM, Rabinovici GD, et al. Consensus classification of posterior cortical atrophy. Alzheimers Dement. 2017;13(8):870-884. doi:10.1016/j.jalz.2017.01.014

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MDS Abstracts - https://www.mdsabstracts.org/abstract/atypical-parkinsonian-syndromes-presenting-with-homonymous-hemianopsia/