What is the course?
Depending on the type of supranuclear gaze paresis, a slightly altered course is typical. In the classic progressive supranuclear gaze paresis (Richardson syndrome), gait insecurity occurs first with staggering gait, unstable posture and resulting falls. Vertical eye movements can only be performed at a slower pace and gradually slight cognitive limitations develop, as in early-stage dementia.
Over the course of the next three to six years, the symptoms of speech and swallowing disorders are added, as is complete paralysis of vertical eye movements. The cognitive deficits also progress. In other forms, the movement disorders in particular can occur more severely and at an early stage, while cognitive symptoms appear later in the course of the disease (PSP akinesia with gait blockade). In the same way, cognitive abilities – such as speaking – may be lost at first, while movement disorders occur late in the course of the disease (PSP-PNFA, progressive, non-flooded GSP aphasia).
Does supranuclear gaze paresis limit life expectancy?
Unfortunately, a supranuclear gaze paresis can severely limit life expectancy. As a rule, survival of only six to twelve years after the first appearance of the disease can be assumed. However, since the disease usually does not appear until the sixth or seventh decade of life, many affected persons can still reach a normal age. The cause of death is usually breathing and swallowing difficulties and the resulting infections that occur in the late course of the disease. Before this, falls can put the patient in a wheelchair, which can further promote certain infections.
The inheritance
The exact mechanism of the progressive supranuclear gaze paresis (PSP) is still unclear. As in Parkinson’s disease, the disease leads to the destruction of nerve cells in important areas of the brain (substantia nigra). The substantia nigra is a part of the brain that is located in the so-called brain stem and is a central control center for motor tasks.
It is not yet known why these nerve cells in particular are destroyed. Since these cells are also affected in Parkinson’s disease, it is possible to understand why Parkinson’s disease drugs also work for a limited time in progressive supranuclear palsy (PSP). In progressive supranuclear palsy (PSP) patients a variant of a gene (Tau gene) was found on chromosome 17.
In Germany, a leading research group from Marburg is investigating genetic defects as the background of progressive supranuclear gaze paresis (PSP). Despite the known genetic defect, it does not appear to be inherited. The risk of disease for patients with progressive supranuclear palsy (PSP) is therefore no different from that for the normal population.
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