DISEASE SUMMARY:
Progressive Supranuclear Palsy( PSP)
I have this diagnosis
PSP is an adult-onset, neurological disease that impacts movement, thinking, speech and vision.
> TIP: Cross out the two pages of the other diseases that do not
apply( e. g., if the person has PSP, cross out the CBD and MSA pages)
• PSP is rare. There are about 6 – 10 people per 100,000 living with the disease. However, this is likely an underestimate given that many people with PSP have not yet been diagnosed or are diagnosed with something else.
• It is commonly referred to as an“ atypical parkinsonian condition” because of the overlap of certain symptoms with Parkinson’ s disease. It may be originally misdiagnosed as Parkinson’ s disease because of this.
• Onset of symptoms is typically symmetric.
• The two most common sub-types of PSP are PSP- Richardson’ s syndrome, which involves prominent loss of balance, stiffness and awkwardness with walking, and PSP-parkinsonism, which can involve mild tremor and less vision and swallowing changes. There are other subtypes of PSP, characterized by their dominant symptoms. Ultimately, treatment is tailored to a person’ s individual symptoms and needs, regardless of subtype.
• Pathologically it is classified as a tauopathy( which is abnormal accumulation of the tau protein in the brain, which is also found in frontotemporal dementia).
• PSP is slightly more common in men.
• Average age of onset is 63 years, but can occur as early as age 40.
• Average life expectancy is eight years after symptom onset, with a wide range.
• There is currently no known way to reverse or stop the progression, though many symptoms can be managed with medication, rehabilitation therapies and close monitoring of safety and functioning.
COMMON SIGNS AND SYMPTOMS
> Limited response to levodopa therapy
> Faster progression, especially compared to Parkinson’ s disease
> Slowed or absent balance reactions / postural instability
> Frequent falls( most often backward)
> Clumsy, shuffling or uncoordinated gait
> Parkinsonian symptoms( rigidity, slowness, tremor)
> Loss of eye movements, especially with downward gaze
> Double vision
> Abnormal eyelid control( involuntary closure or difficulty with opening)
> Decreased blinking with“ deer in headlights” look
> Dystonia: involuntary muscle contractions, commonly with neck or hand
> Constipation and changes to urinary function
> Slurred speech or difficulty with control of loudness of voice
> Swallowing difficulty, including drooling and coughing / choking on solids or liquids
> Difficulty expressing thoughts or words( aphasia) or sometimes repeating words or phrases( echoalia)
> Cognitive changes: slowness of thought, difficulty with organization / planning / multitasking, loss of inhibition, poor judgment and impulsivity
>“ Rocket sign”: when patient jumps up quickly from seated position, often falling back in chair
> Depression, anxiety and apathy
Refer to PSP: Some Answers for detailed information on the diagnosis. www. curepsp. org / iwanttolearn
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