Progressive Supranuclear Palsy

Progressive Supranuclear Palsy (PSP) is a progressive, atypical parkinsonian disorder caused by the accumulation of the abnormally folded protein tau in the nervous system. While there are rare cases of genetic mutations causing PSP, it is widely accepted that PSP is not a genetic disease and that the precise cause is unknown. 

There are many faces, or subtypes of PSP, but most patients experience parkinsonism (slowness and stiffness), early troubles with balance leading to uncontrollable falls, changes in speech, and troubles with cognition, namely difficulty in judgement and forming correct language. Other symptoms that can be seen include difficulty swallowing (dysphagia), abnormal posture of the head-neck (aka cervical dystonia), and troubles with eye movements. In advanced disease, a combination of all these symptoms may occur, as can dementia. 

For most, the progression of PSP is relatively slow, over years, but more rapid compared to typical Parkinson Disease and more rapid than all would like. 

PSP Treatment

Although there is no one pill or cure for PSP, we treat the symptoms one faces - whether this be trouble with slowness or stiffness in movement, or other troubles such as falls, cervical dystonia, or even drooling. In most, a trial of carbidopa-levodopa (Sinemet) is warranted to treat symptoms of slowness and stiffness but in contrast to Parkinson Disease, the effect of Sinemet in PSP for these symptoms may be minimal. 

Many patients living with PSP will need frequent evaluations and support services from physical, occupational, and speech-swallow therapists, whether at an outpatient rehab center or in the home. In general, physical therapy is crucial to help with gait, balance, fall prevention, and mobility while occupational therapy helps with activities of daily living like getting dressed, going to the bathroom, and eating. As speech and swallowing are often affected in PSP, speech-swallow therapy is frequently prescribed and can be very helpful to improve speech and prevent aspirating foods-liquids. 

Staying active is critical and any exercise you like to do and tolerate is highly recommended.

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