Progressive Supranuclear Palsy

OVERVIEW & AGE OF ONSET

Progressive Supranuclear Palsy (PSP) is a progressive condition that affects mobility, vision, speech, and cognition. Symptoms can begin as young as 40, but the average age of diagnosis is often in the 60s.

SYMPTOMS

COMMON SYMPTOMS OF PSP: 

•  Loss of eye movement. This is due to difficulty in moving the eyes up and down, a core characteristic of the disorder. 
•  Frequent falls. Balance and stability can be affected in PSP, resulting in numerous falls. 
•  Bradykinesia, or slow movements. Individuals may notice that they are walking slower or taking more time to do things around the house. 
•  Dysphagia, or difficulty swallowing. 
•  Difficulty speaking, such as speaking slowly, speaking very softly, or using, slurred speech. 
•  Tremor is much more uncommon than in Parkinson’s disease, but can be observed in PSP.

Individuals with PSP may feel as if they are losing their balance and fall frequently. This is particularly worrisome because there can be a slowed protective response such as putting one’s hand in front to protect oneself during a fall. There may be a slowness and stiffness to the individual’s movements. Walking and other visually-guided activities can be compromised additionally by difficulty with moving the eyes up and down. Eventually the eyes will become fixed in the midline, with loss of voluntary eye movements. Individuals may also report difficulty swallowing and speaking. 

COGNITIVE AND PERSONALITY CHANGES

Additionally, some individuals with PSP may experience cognitive and personality changes. These changes may affect personality, behavior, and the ability to complete complex activities like planning a meal or managing finances, and can result in visuospatial difficulties. Common changes include: 

•  Apathy, or showing little interest in previous hobbies and decreased participation ingroup activities, such as family gatherings.  
•  Disinhibition, which may present as hypersexuality, making inappropriate or embarrassing comments in social situations, or approaching strangers in public. 
•  Executive difficulty, or problems in planning and completing complex activities, organizing, and problem solving. Individuals with executive difficulty may have trouble with things like balancing a check book, shopping for groceries, or preparing a meal. 
•  Visuospatial impairments, or difficulty determining the spatial relationships between the self and objects, or between two objects in the environment.

PATHOLOGY AND TREATMENT

Microscopic inspection of the brain reveals that PSP is associated with the accumulation of a specific form of misfolded tau. Researchers at the Penn FTD Center and elsewhere are developing medications that are directed towards slowing or blocking the accumulation of tau. Unfortunately, there are few medications available that can help PSP symptomatically. Some individuals can have unprovoked emotional outbursts of laughing or crying, and some medications can help manage these forms of disinhibition.