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Progressive Supranuclear Palsy

There are a few different conditions that can present like Parkinson's. These are referred to as atypical parkinsonism's or Parkinson's-plus syndromes. Progressive supranuclear palsy (PSP) is considered an atypical parkinsonism. Let's learn more about PSP.




Diagnosis

Just as with Parkinson's Disease, there is a spectrum with PSP, so diagnosis can be difficult. Diagnosis starts with ruling out other conditions like stroke via an MRI, Alzheimer's via a lumbar puncture, and Wilson's disease via blood or urine tests.

However, the most accurate way to confirm is via an autopsy.


There cardinal symptoms of PSP (which only become clear 3-4 years after original symptom onset) that help a diagnosis are

  1. ocular motor dysfunction - decreased ability to look in a particular direction voluntarily and decreased ability to open eyes after voluntarily closing them.

  2. postural instability - this is similar to PD where you can see repeated falls and changes in balance.

  3. akinesia and rigidity - difficulty initiating movement and stiffness are also a sign of PD, which can make diagnosis difficult.

  4. cognitive dysfunction - apathy, slowed thinking, and impulsivity are examples of cognitive dysfunction that can occur.


PSP is thought to be related to changes in the protein tau, while Parkinson's is related to changes in the protein alpha-synuclein. Both have abnormal protein changes, but the type of protein that changes is what contributes to the fast progression, symptoms, and symptom severity in PSP.


People with PSP typically do not respond to levodopa medication



Symptoms

Like PD, PSP is progressive and unique to each person. Symptoms can start subtly and progress overtime to become much more visible. One of the biggest first sign is motor symptoms related to walking. People may experience falls when walking or may feel like you are walking a bit more stiff.

Symptoms of PSP can also include:

  • difficulty controlling eye movements

  • difficulty swallowing

  • depression

  • vision changes

  • hand tremor

  • increased anger

  • emotional outbursts like crying or laughing at unexpected or inappropriate times

  • difficulty controlling eyelids

  • dementia

  • memory changes

  • slurred speed





Stages of PSP


Early stage

This is typically 1 year after the disease onset. Sometimes it can look like normal changes associated with aging. This can be balance changes, vision changes, and voice changes. People may report a difficulty reading and a reduced speaking volume. Other people can show mood and behavior changes like anxiety, apathy, and depression.


Mid stage

This typically occurs during the 2nd and 3rd year after disease onset. Typically people may need to use walking aids and show an increase in vision changes. Swallowing and speech may also see a decline.


Advanced stage

This typically occurs between the 3rd and 6th year after disease onset. People tend to have a really hard time walking and can often require a wheelchair. People also experience significant muscle stiffness and pain. Vision, speech, and swallowing are also significantly impaired. Aspiration (inhaling food or drinks when eating) and pneumonia are a big concern at this state.


End-of-life

Once reaching this stage, people expect to have between 6-8 weeks. Symptoms progress rapidly here and people tend to experience a decline in consciousness and a decline in the ability to eat or drink.




Variants

There are a few different variants of PSP that are important to note


PSP-RS (Richardson syndrome)

A key diagnosis factor of PSP-RS is difficulty or inability to look up or down, but it often only appears three or four years after symptoms begin.


PSP-P (Parkinsonism)

Typically people show a moderate response to levodopa and a slower progression than PSP-RS. Dyskinesia, autonomic symptoms, and visual hallucinations are much less common in PSP-P than in advanced Parkinson's.


PSP-PGF (Progressive gait freezing)

Gait disturbances that develop and progress years before other symptoms is a big sign of PSP-PGF, specifically if they occur without tremor, rigidity, dementia, or eye movement changes.


PSP-F (Frontal presentation)

Behavioral signs including personality changes, socialization changes, behavior changes, and cognition changes are major signs.


PSP-SL (Speech language)

Speech and language changes before motor changes occur with PSP-SL.


PSP with predominant cerebellar ataxia

This is difficult to differentiate from multiple system atrophy but typically people see a lower severity with autonomic symptoms than multiple system atrophy.



Treatment

There is no cure for PSP, but seeing a physical therapist, occupational therapist, and speech therapist can help with function. Botox can be used for rigidity, dystonia, and to help with eyelid control. Talk with your neurologist about different medication options to help manage symptoms.

It is recommended to prioritize palliative care to maximize comfort.



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