Progressive Supranuclear Palsy, sometimes called Steele-Richardson-Olszewski, is a rare degenerative neurological condition often presenting with similar symptoms to Parkinson’s disease. The condition is so named as it begins slowly and continues to get worse (i.e. progressive) and causes weakness (i.e. palsy) by damaging certain parts of the brain above pea-sized structures called nuclei that control eye movement (i.e. supranuclear).
PSP is usually seen in people between the ages of 50-60 or above. The most obvious sign of the disease is an inability to move the eyes properly, which occurs because of lesions in the area of the brain that coordinates eye movements. Such inability could appear as an inability or reduced ability to look up or down, light sensitivity, slow blinking of eyelids and difficulty maintaining eye contact. Some patients may describe this effect as a blurring. Patients also often show alterations of mood and behavior, including depression and apathy. Other key signs of PSP include, unsteady walking and frequent unexplained falls (mainly backward), stiffness of the neck and limbs, slow slurred and/or quiet speech, difficulty swallowing and a general slowness of movements. Some mild dementia is also observed.
The diagnosis of PSP may be difficult. However, the key to establishing the diagnosis of PSP is the identification of early gait instability, with frequent backward falls and difficulty moving the eyes, which are considered the hallmark of the disease.
Causes of PSP
The cause of PSP is not known. Scientists have come up with several theories as to why the brain would degenerate in such a way. PSP could result from exposure to a virus that enters the body and then takes years to produce obvious effects or be the result of complex genetic mutations. None of these theories, however, have been proven.
Treatment for PSP
Although currently there are no specific treatments for PSP, when people with PSP present with Parkinson’s symptoms including tremors, slowness, stiffness, and balance problems they may respond moderately to anti-parkinsonian treatment such as Levodopa. Another group of drugs that has some application in PSP treatment are antidepressant medications.
However, a variety of other forms of therapy can help control the symptoms of stiffness, imbalance, swallowing difficulties etc. A multi disciplinary team including; Physiotherapists, Speech therapists, Occupational therapists, Dieticians etc would benefit the person greatly as is seen with persons with Parkinson’s.
PSP does also predispose patients to serious complications such as pneumonia secondary to difficulty in swallowing (dysphagia), choking, head injury, and fractures caused by falls, thus requiring good attention to the medical and nutritional needs of the individuals to minimize such complications.
The diagnosis of PSP has significant impact not only on the person but also those close to the person with PSP. Progressive disability results in an increasing need for care and support for the person. However such a loss of independence can also be unsettling and challenging for the individual.
Carers and families too may feel stressed, frustrated and chronically tired. Thus making the condition harder to cope.
Support services for both the person with PSP and their caregivers is vital to uphold the quality of care, encourage communication and a positive attitude towards ones treatment and care, and also maintain the pleasurable nature of the relationship.