What is Progressive Supranuclear Palsy (PSP)?
Progressive Supranuclear Palsy (PSP) is also called Steele-Richardson-Olszewski syndrome from the names of the three neurologists who identified and characterized the disease in 1964.
This rare neurodegenerative disorder currently affects 3,000 to 10,000 patients in France and approximately one person in 16,000 in Europe and 1 to 5 persons in 100,000 worldwide. There is about 1.1 new case per 100,000 persons every year.
What are the symptoms of PSP?
The first symptoms occur around age 55 to 70 on average.
There are several forms of PSP and the early signs of the disease can vary significantly. Some of the most common symptoms include:
- Progressive balance disorders and frequent falls
- Slower eye movements
- Sensation of blurred vision
- Dry eyes and increased sensibility to light
- Changes in behavior: apathy, impulsiveness, aggressiveness, restlessness of attention, etc.
- Difficulty of speech
What are the causes of PSP?
PSP is a neurodegenerative disease caused by the degeneration of nerve cells. Similarly to other diseases of the same type (Alzheimer’s, Parkinson’s, etc.), it is accompanied by an abnormal accumulation of Tau proteins in the nerve cells, the causes of which are still unknown.
The disease results in damage in the brain stem that will gradually affect balance, vision, movement, swallowing and speech.
What is the life expectancy?
Following diagnosis, patients have an average life expectancy of 5 to 7 years. There is currently no treatment available to cure or even slow down the progression of the disease.
How is it diagnosed?
PSP is still very little known by the general public, and also by medical professionals. An average of three to four years may pass between the first symptoms and the diagnosis. This difficult diagnosis is primarily based on the age (over 40 years old) and the evolution of symptoms.
Several tests help perform a more accurate diagnosis:
- Neurological exam to assess eye movements, balance, movements, language and intellectual functions.
- Biological samples are useful to detect any disease with similar symptoms that could be treated.
- Brain MRI to assess the possible presence of any tumor, abscess or vascular disease.
- Eye movement exam to record eye movements and confirm the diagnosis.
How is PSP different from Parkinson’s disease?
The symptoms of PSP and Parkinson’s disease are very similar: stiffness, difficult movements, and clumsiness.
However, PSP progresses much more rapidly and speech- and swallowing-related symptoms are more severe. On the other hand, tremors are much less frequent in PSP.
How is PSP managed?
Current treatments help manage the symptoms of the disease but do not cure it:
- Physical therapy for movement disorders
- Speech and occupational therapies for speech and swallowing disorders
- Psychological counseling
How can Alzprotect benefit to the patients?
The Alzprotect team focuses on developing an effective treatment for PSP. Our objective: to relieve the patients and their families who are dealing with this severe condition.
One of the molecules that we are developing, AZP2006, may prove effective for the treatment of this disease. It inhibits the pathway that produces proinflammatory cytokines, prevents the development of neuroinflammation and of Tau protein hyperphosphorylation, and slows down disease progression.
To be made available to the patients, our molecule must undergo a very long process of tests and clinical trials, managed by health authorities such as the ANSM (Agence Nationale de Sécurité du Médicament) in France, the EMA (European Medicine Agency) in Europe or the FDA (Food and Drug Administration) in the United States.
These clinical phases (phase 2 and phase 3) assess the safety and efficacy of a drug candidate. If outcomes are successful, the drug can be marketed.
What is the development stage of your solution?
The ANSM has given its approval for conducting phase 2a clinical trials and the first patients will be recruited during of 2020. Based on records documenting the product development and most importantly the safety and efficacy results, authorities review and approve the documents before authorizing the conduct of clinical trials.
At this time, it is hard to predict how successful a drug will be and when it will be available to all patients.
Alzprotect start phase 2 trials in 2020. Divided into phases 2a et 2b, these trials will run over a period of 3 to 5 years, hoping that the efficacy observed in animals will result in a treatment for humans.
How to participate in a clinical trial?
We receive direct requests from patients and their relatives. It is important to note that by no means can we include a patient or recommend him/her to a clinician.
Only the doctors in charge of those clinical trials can be contacted by patients or their relatives for inclusion in a clinical trial or for access to treatment if available.
We recommend you to contact patients’ associations who can provide information about ongoing clinical trials.
In France
Association PSP France
25 rue Gandon
75013 Paris
Tel.: + 33 142 964 156 (Line open on Saturdays, from 11am to 2pm)
www.pspfrance.org
In Europe
PSP Association
www.pspassociation.org.uk
In the United States
Cure PSP
www.psp.org