A Case of Progressive Supranuclear Palsy/Steele—Richardson—Olszewski Syndrome

Abstract

Progressive supranuclear palsy (PSP) is an uncommon neurological disorder, the hallmark of which is supranuclear ophthalmoplegia involving vertical gaze. Other clinical features include pseudobulbar palsy (dysphagia and dysarthria), neck dystonia (retrocollis), bradykinesia, postural instability, and repeated falls occurring early in the course of the disease, personality changes, a staring unblinking facies, mild dementia, and cerebellar and corticospinal tract signs. Magnetic resonance imaging (MRI) of the brain on midsagittal images may reveal a characteristic atrophy of the midbrain in a shape that suggests a bird, particularly a humming bird. The PSP may resemble Parkinson's disease (PD), but the pathophysiology is distinct from PD. Here, we report a rare case of a 72-year-old man who came with difficulty in naming objects and persons, dysphagia, dysarthria, difficulty in vertical gaze, and history of recurrent fall even while in sitting down position. He was diagnosed as PSP based on clinical examination and neuroimaging. The PSP has poor prognosis.