Progressive supranuclear palsy (PSP) is a very rare neurological disorder, affecting about 20,000 people in the US. This is a disease in which midbrain structures (those in the middle of the brain – neuroanatomy doesn’t have to be hard) deteriorate. The onset is slow and the initial symptoms can be very nonspecific – fatigue, headaches, joint pain, dizziness, depression. In fact, it may be that since this disease typically gets diagnosed when someone is in their 60s (although it can be older or younger) people may be just retroactively tacking the signs and symptoms of aging onto their disease specific symptoms. However the disease does eventually separate from aging – the patient , or more likely the patient’s family, notes memory problems, emotional lability and personality changes, and there begin to be problems with walking, balance, vision and swallowing.
Many symptoms overlap with the much more common Parkinson’s Disease, which can complicate diagnosis. One sign specific to PSP is vertical gaze paralysis, which means the patient cannot voluntarily look down. Due to the location of the lesions above the sections of the brain that control involuntary movements, these can be preserved. Eventually some patients lose the ability to move their eyes voluntarily in any direction.
The clinical course of PSP can vary, but it is progressive, as the name implies, and as time goes by there are more problems with balance, swallowing and cognition. Falls are a major problem, and after a few years most patients lose the ability to walk. Swallowing difficulties increase the risk of aspiration and malnutrition, and many patients will need a feeding tube placed.
PSP is not considered fatal in and of itself, but basically reduces your expected remaining years of life by a half to two-thirds. This is because patients are vulnerable to falls, and especially pulmonary complications such as pneumonia.
The cause of PSP is unknown; undoubtedly there is a reason why some peoples' midbrains deteriorate, but we don’t know what it is. Overall there is no family tendency, although at times there will be a family with more than one member affected, as in our article.
There is no treatment for PSP. Antidepressant medications may help the symptoms a bit for some reason, but drugs for Parkinson symptoms are not effective, possibly because they require more intact midbrain structures to work with. Interventions center on combating symptoms – special prism glasses to look down with, weighted walkers for balance and so on. The balance problems are from the brain, so exercise cannot directly help them, but patients can benefit on a number of levels from physical therapy. Obviously, high quality nursing care can make a big difference as well.
