(Steele-Richardson-Olszewski syndrome)

Progressive supranuclear palsy is a rare degenerative disorder of the central nervous system, which increasingly affects the voluntary eye movements, causing bradykinesia, muscle rigidity and progressive axial dystonia, Pseudobulbärparese and dementia. The diagnosis is made clinically. Treatment depends on the symptoms of symptom relief.

The cause of progressive supranuclear palsy is unknown.

Progressive supranuclear palsy is a rare degenerative disorder of the central nervous system, which increasingly affects the voluntary eye movements, causing bradykinesia, muscle rigidity and progressive axial dystonia, Pseudobulbärparese and dementia. The diagnosis is made clinically. Treatment depends on the symptoms of symptom relief. The cause of progressive supranuclear palsy is unknown. degenerating neurons in the basal ganglia and brain stem; Also, there are neurofibrillary tangles containing an abnormal phosphorylated tau protein. Multiple lacunar infarcts in the basal ganglia and deep white matter may resemble progressive supranuclear palsy, but cerebrovascular disease is degenerative, and it proceeds gradually. Symptoms and signs Symptoms of supranuclear palsy usually begin in late middle age. The first symptom may go up or go down to his difficulties in establishing or downgaze without Reklination neck or problems stairs. Voluntary eye movements, v. a. the vertical, are difficult, the reflective eye movements, however, are not affected. The movements are slow, the muscles become rigid, and it develops an axial dystonia. The patients show a tendency to fall backward. Dysphagia and dysarthria with emotional lability (pseudobulbar) are common. It can develop into a resting tremor. Dementia can happen. Many patients are unable to work within about five years and die within 10 years. Diagnosis Clinical Investigation The diagnosis of progressive supranuclear palsy occurs clinically. Usually an MRI Wirde performed to rule out other diseases. In advanced cases, the MRI shows a characteristic decrease in size of the midbrain, which is best seen on mediosagittalen views and that causes the midbrain takes the form of a hummingbird or emperor penguin. Treatment Supportive treatment Treatment of progressive supranuclear palsy focuses on relieving symptoms, but is not satisfactory. Occasionally levodopa and / or amantadine allow a partial reduction in the rigors. Physiotherapy and occupational therapy can help improve mobility and function and reduce the risk of falls. Since it is a deadly disease, patients should be encouraged to write a living will soon after diagnosis. This ruling should indicate want what kind of medical care to patients at the end of their lives.

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