Steele-Richardson-Olszewski syndrome, commonly known as Progressive Supranuclear Palsy, is an uncommon brain disorder that causes serious problems for patients in terms of walking, balance and eye movements. The disorder arises as a result of deterioration of cells in areas of the brain that control body movement and thinking. PSP worsens over time and can lead to life-threatening complications, such as pneumonia and difficulty in swallowing. The disease also damages certain parts of the brain above nerve cell clusters called nuclei (supranuclear), which control eye movements. Thus, individuals diagnosed with PSP experience a classic symptom i.e. blurring of the vision. The US epidemiological review says that only about three to six in every 100,000 people worldwide, or approximately 20,000 Americans, have PSP—making it less common than Parkinson’s disease. Symptoms of PSP occur on an average after the age of 60, but may occur earlier in some cases. The male population is more affected as compared to the female population.

Recent research work conducted demonstrated that deteriorating brain cells of people with Progressive Supranuclear Palsy have abnormal amounts of a protein called Tau. A positron emission tomography (PET) scan is recommended that can help in diagnosing early signs of brain abnormalities that may not appear on Magnetic Resonance Imaging (MRI). Although there is no cure for Progressive Supranuclear Palsy, but treatments are available to help ease symptoms of the disorder including Parkinson’s disease medications, eyeglasses with bifocal or prism lenses, speech and swallowing evaluations, and physical therapy and occupational therapy.

Progressive Supranuclear Palsy does not have a robust pipeline as only a few active products are under development but have the potential to boost the future market since majority of the products are in mid-stage (Phase II) and early stage (Phase I) of development. AB Science is developing a drug in the Phase III stage which is an emerging therapy for this indication. Another pipeline product being developed by Biogen utilizes induced Pluripotent Stem Cell (iPSC) technology acquired from iPierian. Some companies are also working in collaboration with other institutional and non-institutional organizations. Most of the drugs have been granted Orphan drug and Fast-track drug designations by different regulatory bodies, providing companies added incentive to introduce a product into the market.

Insight by:
Ayushi Sinha
Associate Analyst

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