PSP is a rare neurodegenerative brain disease. It describes a brain dysfunction caused by tau protein clumping. It is estimated that around six to seven out of 100.000 people will develop PSP at some point in their lives. PSP occurs predominantly from the age of 40 onwards.

PSP was discovered in 1963 by Dr. John C Steele, Dr. JC Richardson and Dr. J.Olszewski. PSP is closely related to Parkinson's disease, making it difficult to diagnose. In many cases, the patient has to odyssey through medical practices and clinics until the correct diagnosis is made - often years later.

So far there is no cure for the disease, only a few drugs can alleviate the symptoms.

Research into PSP is currently making great progress. An international working group led by Prof. Höglinger has developed new diagnostic criteria early detection developed. New therapies are being tested. Dr. Gesine Respondek from the Neurocenter of the Technical University of Munich and member of the German Study Group on Atypical Parkinson's Syndromes has identified five different forms (PSP phenotypes).

PSP phenotypes (progressive forms)

The symptoms that patients with PSP suffer from at the onset of the disease can vary greatly. There is a typical progression that was first described in 1963 and is now called the Richardson symptom after the person who first described it. This progressive form of PSP has very typical symptoms. It is not usually confused with other diseases. However, some patients develop the symptoms that are important for the PSP diagnosis only late or not at all. In these progressive forms, the disease is not recognized as PSP until late in many of those affected. The different forms of PSP are also referred to as PSP phenotypes and are described below.

Typical course forms

PSP Richardson Syndrome (PSP-RS)

In the typical form, a characteristic supranuclear gaze palsy develops, which is manifested by limitations in the ability to look up or down at will. Gait is often unsteady and unsteady early in the course of the disease, with a tendency to fall backwards (postural instability). There is increasing stiffening of the muscles (rigor), with the neck and trunk being more affected than the arms and legs. Patients are often only able to sit, stand and walk upright. All movements are slowed down (bradykinesis). Some sufferers can only speak slurred (dysarthria). Difficulty swallowing (dysphagia) is also common. In addition, there may be a reduction in attention, deficits in the planning of actions and changes in personality, such as increased aggression, disinhibited behavior and reduced drive.

Atypical progression forms

There are other forms of PSP progression. They are named after their clinical picture, which is in the foreground at the beginning of the disease. Often the symptoms are similar to those of other diseases, making it difficult for the doctor to make the correct diagnosis. In the further course, however, symptoms can also occur that are typical of Richardson's symptoms.

PSP – Parkinsonism (PSP-P)

In this form, the main signs of Parkinsonism are a slowing of movement (bradykinesia) and a stiffening (rigor). Sometimes tremors can also occur. Patients can initially take one Therapy L-Dopa provides relief from symptoms. This makes it difficult to distinguish the symptoms in the early stages from Parkinson's disease. After more than 2 years, patients can also develop the symptoms of classic PSP. (Richardson syndrome) =: postural instability and supranuclear palsy.

PSP akinesia with gait blockages (PSP-PGF)

This progressive form begins with gait disturbances (slow or unsteady gait) accompanied by "freezing" of the gait and speech. Here, too, the patients only develop the symptoms of classic PSP later. Tremor, rigidity, bradykinesia and memory impairment do not appear in the first 5 years.

PSP – Corticobasal Syndrome (PSP-CBS)

In this form of the disease, the symptoms are accentuated or appear exclusively on one side of the body. Disturbance of the coordination of action sequences (apraxia), involuntary muscle twitching (myoclonus), sensory disturbances of the skin (cortical loss of sensitivity), incorrect posture of the limbs (dystonia) and Parkinson-like symptoms such as rigidity and bradykinesia can occur. At the same time or later, the symptoms of classic PSP develop.

PSP – PSP with speech/speech disorders (PSP-SL)

Speech coordination disorders (speech apraxia), lack of grammatical structures (agrammatism) and distorted speech sounds (sound positions) are the main features of this progression. Bradykinesis, postural instability, and supranuclear palsy develop later.

PSP – the main symptoms

Symptoms can vary greatly from person to person!

However, some abnormalities are observed particularly frequently.

The main symptom is a progressive limitation of voluntary eye movements - this can include:

• Unsteady gait and dizziness
• Falls “out of the blue”, especially backwards
• Difficulty walking, balance problems
• Slowing down of movements, similar to Parkinson's disease
• Vision problems, such as double vision or blurred vision
• speech and swallowing problems
• sleep disorders
• Drive impoverishment, personality change

Symptoms that often lead to the diagnosis of PSP are falling, especially backwards, and an increasing inability to lower your eyes.

dr medical Gesine Respondek and Prof. Dr. medical Günter Höglinger,
Neurology Clinic, Klinikum Rechts der Isar, Technical University of Munich
and German Center for Neurodegenerative Diseases Munich