A case of Steele-Richardson-Olszewski syndrome

Abstract

Background: Progressive supranuclear palsy (PSP) also known as Steele-Richardson-Olszewski syndrome is a rare neurological disorder that affects your balance, cause supranuclear gaze palsy, dysarthria and dysphagia. It is a neurogenerative disease with an unknown cause that involves the accumulation of tau protein within the brain. Approximately 6 per 100000 people are diagnosed with PSP annually and the mean age of onset for this syndrome is 65. Case report: In 2020 57-year-old female came to the Emergency department because of a fall. A CT scan of the brain did not show any damage, the wound was sutured and the patient was discharged. After 6 months the female started presenting changes in personality. She gradually started having issues with her vision, walking and speech. She was sent to the neurologist for a consultation. During evaluation the neurologist found that she cannot lookup, has dysphonia, increased muscle tone in the limbs, impaired amplitude of alternating movements in the hands and feet. She also could not turn around 360 degrees and do a tandem gait. MRI showed hyperintensity in midbrain and pontine tegmentum, reduction of the volume of the midbrain, the upper contour concaved inwards. In frontal, parietal lobes and deeper white matter small hyperintensive parts were seen, most likely of angiopathic origin. The patient was prescribed with bensarazide in combination with levodopa. Regardless, her condition keeps getting worse. Now the woman is in a wheelchair, her speech is barely understandable to others and she cannot do anything on her own. Conclusion: PSP is a complex condition that affects the brain. Patients, who are diagnosed with it usually die in 6 to 9 years. There is no cure for this syndrome or drugs that provide significant symptomatic benefits but multidisciplinary interventions are essential in promoting longer independence in daily living.