Uncommon Neurological Disorder Affecting Movement and Balance

Previously referred to as Steele-Richardson-Olszewski syndrome, progressive supranuclear palsy (PSP) is an uncommon neurological disorder that mainly affects the brain regions responsible for controlling movement, including those of the eyes, and balance. During the disease process, abnormal deposits of a protein called tau accumulate in nerve cells, causing these cells to not function properly and die. PSP often is misdiagnosed as Parkinson’s disease since both conditions have symptoms that affect movement, such as stiffness, slowness, gait impairment and loss of coordination and balance.

Currently, the exact causes of PSP are unknown. Like many other diseases, it may not have a single cause, but could be caused by a combination of genetic, environmental and behavioral factors.

Symptoms, Diagnosis & Outlook

Symptoms

As the onset of PSP is a gradual process, it is difficult to pinpoint when and how initial symptoms begin. At times, it may be difficult to distinguish from Parkinson’s disease, especially at the beginning of the disease process. Although these two conditions share similar symptoms, those with PSP rarely experience tremor or hunched posture. Symptom onset generally occurs after 60 years of age and may include:

  • Difficulty controlling eye movements, such as blinking, shifting gazes and opening the eyes
  • Loss of balance when walking
  • Unexplained falls
  • Abnormal gait
  • Stiffness
  • Tilting of the head backward
  • Speech and swallowing difficulties
  • Slowed thinking
  • Impaired memory
  • Inappropriate emotional reactions, such as laughing or crying for no apparent reason

Diagnosis

A diagnosis of PSP is made after careful consideration of multiple factors, including review of extensive clinical histories and exams, symptom progression and responsiveness to medications. For instance, unlike those with Parkinson’s disease, individuals with PSP do not experience markedly improved symptoms from the drug levodopa. As multiple organ systems may be affected by PSP, a team of multidisciplinary physicians often provide care for these patients.

Outlook

Currently, there is no cure for PSP and no treatments available to delay its progression. Instead, current treatment therapies are designed to alleviate symptoms.

Generally, PSP patients become severely disabled within three to five years of symptom onset and are at risk for serious complications, such as pneumonia, choking, head injuries and fractures.

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Why Choose Penn State Health for Care

Along with researchers around the world, the clinicians and scientists at Penn State College of Medicine’s Translational Brain Research Center are devoted to identifying the causes of PSP and developing better diagnostic tools through partnership with the National Institute of Neurological Disorders and Stroke (NINDS) Parkinson’s Disease Biomarkers Program and clinical trials. By partnering with patients and their families, this multidisciplinary team is committed to developing effective therapies to ensure others do not have to endure this difficult journey.

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