Progressive Supranuclear Palsy (PSP) is a rare neurodegenerative disease. Only five or 6 people per 100,000 will develop it. The exact cause of PSP is unknown, but the symptoms are caused by misfolding and accumulation of a naturally occurring brain protein called tau. These accumulations become toxic and cause degeneration of brain cells.

Symptoms

PSP is difficult to diagnose because the early stages often look very similar to Parkinson’s disease. One of the main diagnostic features of this disease is visual complaints. Patients with PSP have difficulty looking up or down and often complain of double vision and involuntary closing of the eyelids. Other early complaints are unexplained falls, problems with memory and thinking, and mood problems including impulsiveness, apathy, and depression. Other similar disorders, which may be treatable, should be ruled out when diagnosing a patient with PSP.

Diagnosis

PSP is difficult to diagnose because the early stages often look very similar to Parkinson’s disease. One of the main diagnostic features of this disease is visual complaints. Patients with PSP have difficulty looking up or down and often complain of double vision and involuntary closing of the eyelids. Other early complaints are unexplained falls, problems with memory and thinking, and mood problems including impulsiveness, apathy, and depression. Other similar disorders, which may be treatable, should be ruled out when diagnosing a patient with PSP.

Treatment

There is currently no FDA-approved treatment for PSP. Some patients may respond to anti-Parkinson medications. Levodopa may help with the slowness and stiffness. Still, the effect is generally much less than in Parkinson’s disease, the benefit usually diminishes significantly over time, and patients typically need a much higher dose of medication to receive benefit. Antidepressants may help mood problems in some patients. Some non-drug therapies that are helpful for some individuals include prism glasses for double vision, speech therapy, and walking aids. For patients with marked swallowing problems surgical placement of a feeding tube may help ensure nutrition and reduce the risk of pneumonia caused by food going into the lungs.