(274) A Case Study of Progressive Supranuclear Palsy in a 68-year-old with Pronounced Frontal-Executive Deficits Complicated by Cerebrovascular Disease

Objective: Individuals with Progressive Supranuclear Palsy (PSP) often experience cognitive decline, repeated falls, restricted gaze, bradykinesia, and depression/behavioral changes. Despite having certain distinguishing features neuroanatomically and neurologically, PSP continues to be difficult to classify and distinguish from some other more well-known neurodegenerative and movement-related disorders.

Method: A 68-year-old white male presented for a neuropsychological evaluation (2022) initially after his neurologist became suspicious of him having cerebellar degeneration or multisystem atrophy. He reported a progressive one-year decline in memory, motor abilities, and executive functions, along with having personality changes and a loss of independence in his activities of daily living (ADLs). To complicate the differential diagnosis, an MRI of his brain from six months prior simply concluded “age-related changes”. Upon revisiting this MRI and closer visual inspection, focal atrophy of the midbrain became apparent on a sagittal section, in addition to bilateral ischemic changes in the white matter. He was later diagnosed with PSP.

Results: Despite demonstrating adequate performance on simple cognitive screening measures, he displayed marked behavioral disinhibition, executive/attention problems, visuospatial concerns, decreased motor abilities, and variable memory functioning that were found to impact his ability to carry out instrumental and even some basic ADLs.

Conclusions: Cases such as this one requiring diagnostic clarification lend support to the “hummingbird sign” (midbrain atrophy) being a useful imaging marker in distinguishing PSP from other clinical disorders. Additionally, in contrast to other neurodegenerative disorders, frontal-executive deficits (e.g., disinhibition) were a particularly pronounced feature in his PSP neurocognitive profile, followed by milder difficulties in memory, language, and visuospatial functions.