Materiały źródłowe

• #1 Progressive supranuclear palsy: diagnosis and management | Practical Neurology

  https://pn.bmj.com/content/21/5/376

  Treating patients with progressive supranuclear palsy (PSP) is both effective and rewarding. This review aims to share our experience in the proactive management of PSP, considering the patient, the family and the medical context in which the illness unfolds. […] Effective management starts with achieving an early, accurate diagnosis. We review new concepts in the diagnosis of PSP that have emerged over the last 10 years. We then consider the differential diagnosis and the role of investigations. We go on to review pharmacological treatment options and non-pharmacological aspects to effective holistic care, support and management. […] PSP cannot yet be cured, but treatment can be helpful. Each patient is different, and so the selection, timing and doses of the following drugs will vary from person to person.

• #1

  https://link.springer.com/article/10.1007/s11940-016-0422-5

  In the absence of an effective drug treatment to target the underlying cause of CBD and PSP, management should focus on optimizing quality of life, relieving symptoms and assisting patients with their activities of daily living (ADL). […] Patients should be managed by a multidisciplinary team consisting of neurologists, physiotherapists (PT), occupational therapists (OT), speech and language therapists (SALT), dieticians, ophthalmologists, psychologists, and palliative care specialists. […] Disease-modifying therapies for PSP and CBD have targeted tau pathology. […] In the absence of approved pharmacological treatments for PSP and CBD, management should be based on relieving symptoms and assisting patients with their activities of daily living. […] Advanced care planning and non-pharmacological supportive therapies remain paramount in the management of PSP and CBD. […] Most patients will be trialed on L-DOPA and amantadine, although there is limited evidence for benefit some patients may experience modest improvement in Parkinsonism. […] Botulinum toxin is helpful in reducing dystonia and in managing sialorrhea and is particularly useful for eyelid dysmotility.

• #1 Progressive supranuclear palsy (PSP): Management and prognosis – UpToDate

  https://www.uptodate.com/contents/progressive-supranuclear-palsy-psp-management-and-prognosis/print

  Progressive supranuclear palsy (PSP) is an atypical parkinsonian disorder characterized by vertical supranuclear gaze palsy and postural instability with unexplained falls. This topic will review the management and prognosis of PSP. […] There are no treatments that alter the natural history of disease in PSP and no drugs that provide significant symptomatic benefits as seen with levodopa in Parkinson disease. However, several nonpharmacologic and pharmacologic supportive measures are available for the treatment of PSP. […] A multidisciplinary approach is essential, involving health care professionals from neurology, physical therapy, occupational therapy, speech pathology, nutrition, neuropsychology, psychiatry, social work, and palliative care. […] Dietitians as well as speech and language therapists can help to manage dysphagia and dysarthria. Specific modalities for dysarthria include facial exercises, Lee Silverman Voice Treatment, written communication, and a talking keyboard. Some experts advise routine swallowing evaluations (eg, every six months) to assess the risk for dysphagia and aspiration, which are major causes of mortality in patients with PSP. Treatments for dysphagia include head posturing, dietary changes, and percutaneous gastrostomy tube placement in more advanced cases. However, a gastrostomy tube does not diminish the risk of aspiration.

• #1 Progressive supranuclear palsy – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/progressive-supranuclear-palsy/diagnosis-treatment/drc-20355664

  Although there is no cure for progressive supranuclear palsy, treatments are available to help ease symptoms of the disorder. The options include: […] Parkinson’s disease medicines, which increase levels of a brain chemical involved in smooth, controlled muscle movements. The effectiveness of these medicines is limited and usually temporary, lasting about 2 to 3 years in most patients. […] OnabotulinumtoxinA (Botox), which may be injected in small doses into the muscles around your eyes. Botox blocks the chemical signals that cause muscles to contract, which can improve eyelid spasms. […] Antidepressants. Some antidepressant medicines may have a modest effect on symptoms such as impulsive behavior. […] Eyeglasses with bifocal or prism lenses, which may help ease problems with looking downward. Prism lenses allow people with progressive supranuclear palsy to see downward without moving their eyes down.

• #1 Progressive Supranuclear Palsy Treatment & Management: Approach Considerations, Pharmacologic Therapy, Consultations

  https://emedicine.medscape.com/article/1151430-treatment

  Treatment of progressive supranuclear palsy (PSP) is challenging at best. Only a few patients respond to dopaminergic or anticholinergic drugs, and responses often are short-lived and incomplete. No medication is effective in halting the progression of the disease; however, several medications, including dopamine agonists, tricyclic antidepressants, and methysergide, may provide modest symptomatic improvement with respect to some of the clinical features. […] Electroconvulsive therapy (ECT) may ameliorate motor symptoms in some patients with PSP. However, long hospitalizations and significant adverse effects (eg, confusion) limit the usefulness of ECT. […] The combination of carbidopa and levodopa generally produces no dramatic symptomatic improvement in patients with PSP, in sharp contrast with its effect in patients with idiopathic Parkinson disease. Accordingly, administration of carbidopa-levodopa may serve as a diagnostic test to help eliminate the possibility of Parkinson disease.

• #1 Progressive supranuclear palsy: diagnosis and management | Practical Neurology

  https://pn.bmj.com/content/21/5/376

  For many cognitive and behavioural symptoms, support, tolerance and environmental measures are more effective and safer than medication. […] We recommend a trial of levodopa in all patients with significant akinetic rigidity, but usually not in people with PSP-F or PSP-SL. […] Amantadine can be helpful, with patients reporting reduced axial rigidity, improved mobility, more energy and less fatigue, or clearer speech. […] PSP-PGF may respond to cholinesterase inhibitors, reflecting the cholinergic nature of pedunculopontine gait-rhythm generators. […] The coordination of non-pharmacological management is as important as drug management. Support for patients and families at different stages of disease will require input from a diverse multi-disciplinary team, from hospital and community services in health and social care. […] The involvement of palliative care teams can be hugely beneficial. Such teams often have a wide range of services, many not available elsewhere, and more readily accessible.

• #1 Progressive Supranuclear Palsy Treatment & Management: Approach Considerations, Pharmacologic Therapy, Consultations

  https://emedicine.medscape.com/article/1151430-treatment

  Some clinicians think that bromocriptine may have somewhat greater effect in individuals with PSP, though the effect is modest and short-lived in most patients. Tricyclic antidepressants have also been used. Other medications that have been tried with limited success include amantadine and trazodone. […] OnabotulinumtoxinA has been found to be useful in the treatment of rigidity (nuchal rigidity in particular) and dystonia (eg, blepharospasm, bruxism, and focal limb dystonia). It may also be useful for sialorrhea. […] A small phase II clinical trial using coenzyme Q10 in patients with PSP showed modest clinical improvement in the short term. […] Patients should follow a well-balanced diet. When feeding becomes impossible because of dysphagia and the high risk of bronchoaspiration, patients should be encouraged to accept gastrostomy as a good option.

• #1

  https://link.springer.com/article/10.1007/s11940-016-0422-5

  There are currently no disease-modifying treatments for progressive supranuclear palsy (PSP) or corticobasal degeneration (CBD), and no approved pharmacological or therapeutic treatments that are effective in controlling their symptoms. […] The use of most pharmacological treatment options are based on experience in other disorders or from non-randomized historical controls, case series, or expert opinion. […] Levodopa may provide some improvement in symptoms of Parkinsonism (specifically bradykinesia and rigidity) in PSP and CBD; however, evidence is conflicting and where present, benefits are often negligible and short lived. […] In fact, poor response to levodopa forms part of the NINDS-SPSP criteria for the diagnosis of PSP and consensus criteria for the diagnosis of CBD. […] There is some evidence that intrasalivery gland botulinum toxin is useful in managing problematic sialorrhea and that intramuscular botulinum toxin and baclofen are helpful in reducing dystonia, including blepharospasm.

• #1 Progressive Supranuclear Palsy | AFTD

  https://www.theaftd.org/what-is-ftd/progressive-supranuclear-palsy/

  There is currently no effective treatment for PSP. In some patients, the slowness, stiffness, and balance problems of PSP may respond to anti-parkinsonian agents such as levodopa, or levodopa combined with anticholinergic agents, but the effect is usually temporary. […] Decreased transmission of GABA in the mid-brain is considered to contribute to the symptoms of PSP and so drugs that work on the GABA neurotransmitter may be helpful. Zolpidem, in limited case studies, has improved speech and facial expressions, as well as akinesia rigidity and dysarthria. However, this drug is short acting and prolonged use is not recommended. In general, speech, vision, and swallowing difficulties usually do not respond to any currently available drug treatment. […] Another group of drugs that has been of some modest success in PSP are antidepressant medications. The most common antidepressants used for PSP have been Prozac, Elavil and Tofranil. The anti-PSP benefit of these drugs seems to be unrelated to their ability to relieve depression.

• #1 Progressive Supranuclear Palsy (PSP) – Brain Foundation

  https://brainfoundation.org.au/disorders/progressive-supranuclear-palsy/

  There is no cure for progressive supranuclear palsy. Instead, treatment is focused on managing symptoms and giving the patient the best possible quality of life. […] Several medications can be helpful, as well as multidisciplinary care such as speech therapy or physiotherapy. As the disease progresses, patients may need a walker or other assistive devices. […] The medications recommended for a person with PSP will depend on their symptoms. PSP can increase your sensitivity to side effects (except for dopaminergic medications), so the general rule is to start low and go slow. […] Medications are used to help a patient manage symptoms that put them at risk or are particularly troublesome. Some medications that are used in PSP treatment include: Levodopa, Amantadine, Zolpidem, SSRIs (antidepressants), Memantine, Botulinum toxin injection, Artificial tears / eye drops, Clonazepam.

• #1

  https://www.nhs.uk/conditions/progressive-supranuclear-palsy-psp/treatment/

  There’s currently no cure for progressive supranuclear palsy (PSP), but there are lots of things that can be done to help manage the symptoms. […] There are currently no medications that treat PSP specifically, but some people in the early stages of the condition may benefit from taking levodopa, amantadine or other medications used to treat Parkinson’s disease. […] Antidepressants can help with the depression that’s often associated with PSP. […] A physiotherapist can give you advice about making the most of your remaining mobility using exercise, while making sure you don’t overexert yourself. […] A speech and language therapist (SLT) can help you improve your speech and swallowing problems (dysphagia). […] As your swallowing problems become more severe, you’ll need additional treatment to compensate for your swallowing difficulties.

• #1 Progressive supranuclear palsy physiotherapy | Active Ability

  https://activeability.com.au/physiotherapy/progressive-supranuclear-palsy/

  Progressive supranuclear palsy is a rare neurological condition that mainly affects people over the age of 60. For people with progressive supranuclear palsy, physiotherapy can help to maintain function and independence for as long as possible. […] Physiotherapy treatment for PSP aims to optimise physical function and independence and relieve any distressing symptoms. […] Physiotherapy treatment for PSP may include therapies designed to improve walking ability and safety, transfers such as getting out of bed and on/off chairs, control of head and body movements, balance and posture, energy levels, muscle strength and flexibility, pain or discomfort, independence, and quality of life. […] The authors concluded these studies provide early evidence supporting the use of physiotherapy rehabilitation programs to improve gait, balance, and gaze control in people with PSP.

• #1 Progressive supranuclear palsy – Wikipedia

  https://en.wikipedia.org/wiki/Progressive_supranuclear_palsy

  Patients with PSP usually seek or are referred to occupational therapy, speech-language pathology for motor speech changes (typically a spastic-ataxic dysarthria), and physical therapy for balance and gait problems with reports of frequent falls. There has been research in the use of robot-assisted gait training. Evidence-based approaches to rehabilitation in PSP are lacking and, currently the majority of research on the subject consists of case reports involving only a small number of patients. […] Case reports of rehabilitation programs for patients with PSP generally include limb-coordination activities, tilt-board balancing, gait training, strength training with progressive resistive exercises, and isokinetic exercises and stretching of the neck muscles. While some case reports suggest that physiotherapy can offer improvements in balance and gait of patients with PSP, the results cannot be generalized across all PSP patients, as each case report followed only one or two patients. The observations made from these case studies can be useful however, in helping to guide future research concerning the effectiveness of balance and gait training programs in the management of PSP.

• #1 Progressive Supranuclear Palsy (PSP): Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/6096-progressive-supranuclear-palsy

  Certain treatments can help various eye issues, including: Botox (botulinum toxin) injections, Eye drops and artificial tears, Special glasses, Wraparound glasses. […] As PSP worsens, you might reach the point where you cant swallow at all. This will prevent you from eating and drinking. Before that point, your healthcare provider may recommend that you get a percutaneous endoscopic gastrostomy (PEG). […] Palliative care is a specialized form of care that provides symptom relief, comfort and support to people living with serious conditions, including PSP.

• #1 Progressive Supranuclear Palsy – EyeWiki

  https://eyewiki.org/Progressive_Supranuclear_Palsy

  We advise against the use of progressive or multifocal lenses in PSP and prefer the use of single vision readers. In addition, eye drops can be used for dry eyes in order to prevent exposure keratitis, and botulinum toxin injections may reduce blepharospasm, dystonia, and retrocollis. […] There are no effective medications in treating PSP directly, but options are available for symptomatic treatment. Dopaminergic replacement therapy is worth trying as it has been shown to mildly improve PSP symptoms. However, these improvements are often short lived. Antidepressants are often prescribed to treat patients with emotional apathy, depression, and pseudobulbar crying. […] Amitriptyline can alleviate neck pain, insomnia, and excessive drooling, and gabapentin is useful in reducing dystonic pain.

• #1 Treatments for Progressive Supranuclear Palsy | Stanford Health Care

  https://stanfordhealthcare.org/medical-conditions/brain-and-nerves/progressive-supranuclear-palsy/treatments.html

  No medication or procedure is available to cure progressive supranuclear palsy (PSP) or completely control its symptoms, but strategies and methods can help manage many of the symptoms. […] To improve balance and improve flexibility of the muscles, medications used to treat Parkinson’s disease may be effective. These include the drug levodopa, which may sometimes be used in conjunction with other drugs. Antidepressants can help the depression or irritability seen commonly in this disease. […] If you have PSP, you may be able to use certain aids to make life easier. Special glasses with prisms may improve your vision. A weighted tool that helps you walk more easily can prevent you from falling backward. Physical therapy and exercise may slightly improve flexibility in some people. […] When symptoms are advanced and swallowing becomes too difficult, you may need a feeding tubea tube that goes from an opening made in the skin of your abdomen into the stomachto provide you with needed nutrition.

• #1 Progressive supranuclear palsy – Wikipedia

  https://en.wikipedia.org/wiki/Progressive_supranuclear_palsy

  Individuals with PSP are often referred to occupational therapists to help manage their condition and to help enhance their independence. This may include being taught to use mobility aids. Due to their tendency to fall backwards, the use of a walker, particularly one that can be weighted in the front, is recommended instead of a cane. The use of an appropriate mobility aid helps to decrease the individual’s risk of falls and makes them safer to ambulate independently in the community. Due to their balance problems and irregular movements, individuals need to spend time learning how to safely transfer in their homes and in the community. This may include rising from and sitting in chairs safely. […] Due to the progressive nature of this disease, all individuals eventually lose their ability to walk and will need to progress to using a wheelchair. Severe dysphagia often follows, and at this point death is often a matter of months.

• #1

  https://www.nhs.uk/conditions/progressive-supranuclear-palsy-psp/treatment/

  Feeding tubes may be recommended for severe swallowing problems, where the risk of malnutrition and dehydration is increased. […] If you’re having problems controlling your eyelids, injections of botulinum toxin (such as Botox) can be used to help relax the muscles of your eyelids. […] Palliative care can be offered at any stage of PSP, alongside other treatments. […] Many people with PSP consider making plans for the future that outline their wishes (both medical and other decisions), and make these known to both their family and the health professionals involved in their care. […] There have been several trials of different medications that aim to help symptoms of PSP or slow its progression.

• #1 Progressive supranuclear palsy – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/progressive-supranuclear-palsy/diagnosis-treatment/drc-20355664

  Speech and swallowing evaluations, to help you learn other ways to communicate and safer swallowing techniques. […] Physical therapy and occupational therapy, to improve balance. Facial exercises, talking keyboards and gait and balance training also can help with many of the symptoms of progressive supranuclear palsy. […] Researchers are working to develop treatments of progressive supranuclear palsy, including therapies that may block the formation of tau or help to destroy tau.

• #1 New diagnostics and therapeutics for progressive supranuclear palsy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5802400/

  Progressive supranuclear palsy (PSP) is now recognized as a spectrum of motor and behavioral syndromes associated with a specific four repeat (4R) tau neuropathology at autopsy. […] There are currently no effective treatments for PSP, but because PSP is strongly linked biochemically and genetically to tau protein abnormalities, there is a growing interest in pursuing clinical trials of new tau-directed therapies for this disorder. […] Such new tau therapies are envisioned to have disease-modifying effects by reducing brain levels of toxic forms of tau or compensating for loss of tau function. […] To be most effective, these treatments will need to be initiated at early stages of disease. […] An important advance has been the development of the new International Parkinsons and Movement Disorder Society (MDS) Criteria for the Diagnosis of PSP that recognize early, suggestive forms and operationalize diagnosis of non-Richardsons PSP phenotypes.

• #1 New diagnostics and therapeutics for progressive supranuclear palsy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5802400/

  Deep brain stimulation of the pedunculopontine nucleus has been attempted in advanced PSP-RS, however there were no clear benefits observed and unacceptable side effects. […] The rapid growth of knowledge about the potential pathogenic mechanisms of PSP has facilitated the implementation of a series of rational clinical studies with hypothesis-driven therapeutic approaches aimed at disease-modification by targeting tau or mitochondrial dysfunction. […] Of these, three clinical trials were adequately powered to demonstrate small to moderate disease-modifying effects: the NNIPPS study of riluzole, a phase 2 trial of tideglusib, and a Phase 2/3 trial of davunetide. […] An important limitation of these studies was the lack of a pharmacodynamic biomarker that could demonstrate that the experimental agent engaged its physiological target and produced its hypothesized biological effect.

• #1 New diagnostics and therapeutics for progressive supranuclear palsy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5802400/

  The rationale for treating PSP with microtubule stabilizers is to compensate for the microtubule dysfunction that might result from a disease-associated loss of tau function. […] A number of microtubule-stabilizing agents have been developed and three have been explored in human neurodegenerative disease clinical trials. […] However, a recent large clinical trial of davunetide for PSP-RS demonstrated a lack of efficacy. […] TPI-287, a new blood brain barrier permeable taxane, has entered phase I clinical trials for AD, PSP and CBD. […] Evidence that tau may propagate between cells in a prion-like fashion raises the possibility that blocking spread of pathogenic tau by specific anti-tau antibodies might be a viable approach to tauopathy treatment. […] Two N-terminal tau directed monoclonal antibodies, BMS-986168 and Abb-8E12, have progressed to Phase 2 clinical trials for PSP.

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  https://link.springer.com/article/10.1007/s40120-024-00614-9

  The monoclonal antibody bepranemab (UCB0107) recently underwent a phase Ib study to evaluate its safety, tolerability, and pharmacokinetics in the treatment of patients with PSP. […] Two N-terminal tau-targeting monoclonal antibodies, gosuranemab and tilavonemab, have entered phase II clinical trials for PSP. […] Active vaccinations against pathological tau epitopes have been investigated, and three potential tau vaccines have begun human clinical trials: AADvac1, ACI-35, and JACl-35. […] Overall, the results showed that tilavonemab lacked efficacy in the treatment of PSP in humans. […] Ultimately, there were no other findings to indicate a response to tilavonemab. […] The current phase III trial of rivastigmine is a randomized, double-blind, placebo-controlled study with 106 participants with probable PSP-RS.

• #1 New diagnostics and therapeutics for progressive supranuclear palsy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5802400/

  Active vaccination against tau epitopes is also under investigation, and two tau vaccines have entered human clinical trials: AADvac1 and ACI-35. […] Other strategies under development for targeting tau pathology include enhanced degradation of misfolded tau or NFTs by various proteolytic systems including autophagy, the unfolded protein response, agents targeting mitochondrial dysfunction, cell replacement and young plasma transfusions designed to reverse brain aging. […] There are no effective therapies for PSP, however, insights into the etiology and ontogeny of PSP over the past decade are bearing fruit in the form of new experimental therapeutic agents that have entered PSP clinical trials. […] Increasing numbers of clinical trials are planned for PSP, offering hope for patients and their families for the possibility of effective PSP therapies.

• #1 New Hope for Progressive Supranuclear Palsy with Innovative Trial | UC San Francisco

  https://www.ucsf.edu/news/2024/09/428366/new-hope-progressive-supranuclear-palsy-innovative-trial

  A clinical trial that will test three drugs concurrently, and could include more, represents new hope for patients with progressive supranuclear palsy (PSP), an incurable neurodegenerative disorder that usually kills within seven years after symptoms start. […] There are currently no drugs to stall the diseases deadly progression. […] The hope is that the study will transform the type of care that patients with PSP receive, said Julio Rojas, MD, PhD, of the UCSF Department of Neurology, Memory and Aging Center and the Weill Institute for Neurosciences, and a principal investigator of the trial. If none of the first three drugs are effective, we will continue trying with other drugs. […] The three drugs will be tested using a platform clinical trial model similar to one used for a study of amyotrophic lateral sclerosis (ALS), a neurodegenerative disorder that is much better known and better funded than PSP, although they both affect similar numbers of people.

• #1 New Hope for Progressive Supranuclear Palsy with Innovative Trial | UC San Francisco

  https://www.ucsf.edu/news/2024/09/428366/new-hope-progressive-supranuclear-palsy-innovative-trial

  Patients on the PSP trial will have a 75% chance of treatment with an active drug and after one year, all participants will have the opportunity to receive a drug. […] Unlike typical clinical trials, platform trials can remain open with multiple new therapies tested in successive cycles if the first ones dont work, said Adam Boxer, MD, PhD, endowed professor in memory and aging at the UCSF Department of Neurology, as well as a principal investigator in the trial. […] The nonprofit organization CurePSP will be collaborating with UCSF and other trial sites to recruit participants. […] Enrollment is expected to start in fall 2025. It will be open to patients with Richardsons syndrome, which affects about 70% of patients with PSP.

• #1 Progressive Supranuclear Palsy: Diagnosis, And Treatment

  https://www.viezec.com/progressive-supranuclear-palsy/

  Surgical options for PSP are limited and primarily experimental. Deep brain stimulation (DBS) has shown promise in some neurodegenerative disorders but has not been widely successful in PSP. Research into experimental procedures, including gene therapy and targeted delivery of therapeutic agents, is ongoing. These approaches aim to modify disease progression and improve symptoms, but they are not yet available for clinical use. Patients considering surgical interventions should do so within the context of clinical trials, where potential benefits and risks are closely monitored. […] Research into stem cell therapy for PSP is in its early stages, with several preclinical studies showing promising results. These studies have demonstrated the potential of stem cells to reduce tau pathology and improve motor function in animal models. Clinical trials in humans are limited but are beginning to explore safety and efficacy. These trials aim to determine the best methods for delivering stem cells to the brain and assess their potential to slow disease progression.

• #1 Progressive Supranuclear Palsy: Diagnosis, And Treatment

  https://www.viezec.com/progressive-supranuclear-palsy/

  The potential benefits of stem cell therapy for PSP include the regeneration of damaged brain tissue, reduction of tau pathology, and improvement in motor and cognitive functions. However, there are significant risks, including the possibility of immune rejection, tumor formation, and unintended differentiation of stem cells. The long-term safety and efficacy of stem cell therapy are not yet established, making it an area of cautious optimism and ongoing research.

• #1

  https://www.psp.org/iwanttolearn/clinical-trials

  Treatment of Disturbed Sleep in PSP: UCSF is recruiting for a study to explore if specific sleep medications can improve your sleep patterns, disease symptoms, or quality of life. This is a 6-week remote clinical sleep trial in PSP, meaning you can fully participate from your own home with frequent phone calls with the study team. […] PROSPER Trial: Ferrer is currently seeking individuals with PSP to participate in the PROSPER study, a clinical study phase II co-designed with patients and care partners to assess how safe and effective an investigational oral medicine called FNP-223 may be for treating PSP patients. After a screening assessment to confirm eligibility, participants will be randomly assigned to receive either the study medicine (FNP-223) or placebo. Participants will have a 50% chance of being randomly assigned to the group being given the study medicine (FNP-223). Both FNP-223 and placebo will be taken orally (as tablets) 3 times a day. Participation in the PROSPER Study includes 52 weeks of treatment period and involves a total of 7 visits in-person and 7 phone calls.

• #1 Progressive Supranuclear Palsy – Parkinson’s Group

  https://parkinsonstreatment.org/progressive-supranuclear-palsy/

  Most recently, the drug Davunetide was tested which reduces tau phosphorylation. But still not clear whether it works for PSP. […] Other areas are also being explored to find a cure for this devastating disease which include stem cells, growth hormones, gene therapies transcranial magnetic stimulation (TMS), and DBS (deep brain stimulation). […] At present DBS is being explored to target a specific area for PSP, DBS in particular is not approved for PSP and largely thought not to be helpful.

• #1

  https://www.nhs.uk/conditions/progressive-supranuclear-palsy-psp/

  There’s currently no cure for PSP, but research is continuing into new treatments that aim to relieve symptoms and slow down the progression of the condition. […] Treatment currently focuses on relieving the symptoms while trying to make sure someone with PSP has the best possible quality of life. […] Treatment will be tailored to meet the needs of each individual and may include: medication to improve balance, stiffness and other symptoms, physiotherapy to help with movement and balance difficulties, speech and language therapy to help with speech or swallowing problems, occupational therapy to help improve the skills needed for daily activities, Botox (botulinum toxin injections) or special glasses to help with eye problems, feeding tubes to help manage dysphagia and avoid malnutrition or dehydration.

• #1

  https://link.springer.com/article/10.1007/s40120-024-00614-9

  No tau-targeting therapies have been effective in modifying the course of PSP. […] Several tau-targeting therapies are in clinical trials. […] The discovery of effective tau-modifying pharmacotherapies could significantly impact the prognosis of patients with PSP and other neurodegenerative disorders such as Alzheimers and Parkinsons diseases and corticobasal degeneration. […] Therefore, the development of PSP therapies is a highly relevant area of research, and this review summarizes the therapies that have been studied and those that are currently undergoing clinical trials for the treatment of PSP. […] Currently, treatment remains mainly symptomatic, and several non-disease-modifying treatments are in clinical trials. […] Nonpharmacological interventions, such as physical therapy, improve symptomatic and functional manifestations of PSP.

• #2 Progressive Supranuclear Palsy (PSP): Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/6096-progressive-supranuclear-palsy

  Progressive supranuclear palsy (PSP) is a rare condition that affects certain areas of your brain. Common symptoms include balance issues with frequent falls, eye movement issues and cognitive changes. There isnt a cure for PSP, but certain treatments can help manage symptoms. […] Theres currently no cure for PSP and no way to slow it down. But several treatments can help manage your symptoms and improve your quality of life. […] Treatments include: Oral medications, Movement therapies, Eye treatments, Percutaneous endoscopic gastrostomy (PEG), Palliative care. […] Antiparkinsonian medications are oral (taken by mouth) medicines typically used to help people with Parkinsons disease. They can sometimes help manage progressive supranuclear palsy symptoms, too. […] The following therapies can help with some symptoms of PSP: Physical therapy, Occupational therapy, Speech therapy.

• #2 Progressive supranuclear palsy (PSP): Management and prognosis – UpToDate

  https://www.uptodate.com/contents/progressive-supranuclear-palsy-psp-management-and-prognosis/print

  Progressive supranuclear palsy (PSP) is an atypical parkinsonian disorder characterized by vertical supranuclear gaze palsy and postural instability with unexplained falls. This topic will review the management and prognosis of PSP. […] There are no treatments that alter the natural history of disease in PSP and no drugs that provide significant symptomatic benefits as seen with levodopa in Parkinson disease. However, several nonpharmacologic and pharmacologic supportive measures are available for the treatment of PSP. […] A multidisciplinary approach is essential, involving health care professionals from neurology, physical therapy, occupational therapy, speech pathology, nutrition, neuropsychology, psychiatry, social work, and palliative care. […] Dietitians as well as speech and language therapists can help to manage dysphagia and dysarthria. Specific modalities for dysarthria include facial exercises, Lee Silverman Voice Treatment, written communication, and a talking keyboard. Some experts advise routine swallowing evaluations (eg, every six months) to assess the risk for dysphagia and aspiration, which are major causes of mortality in patients with PSP. Treatments for dysphagia include head posturing, dietary changes, and percutaneous gastrostomy tube placement in more advanced cases. However, a gastrostomy tube does not diminish the risk of aspiration.

• #2

  https://link.springer.com/article/10.1007/s11940-016-0422-5

  In the absence of an effective drug treatment to target the underlying cause of CBD and PSP, management should focus on optimizing quality of life, relieving symptoms and assisting patients with their activities of daily living (ADL). […] Patients should be managed by a multidisciplinary team consisting of neurologists, physiotherapists (PT), occupational therapists (OT), speech and language therapists (SALT), dieticians, ophthalmologists, psychologists, and palliative care specialists. […] Disease-modifying therapies for PSP and CBD have targeted tau pathology. […] In the absence of approved pharmacological treatments for PSP and CBD, management should be based on relieving symptoms and assisting patients with their activities of daily living. […] Advanced care planning and non-pharmacological supportive therapies remain paramount in the management of PSP and CBD. […] Most patients will be trialed on L-DOPA and amantadine, although there is limited evidence for benefit some patients may experience modest improvement in Parkinsonism. […] Botulinum toxin is helpful in reducing dystonia and in managing sialorrhea and is particularly useful for eyelid dysmotility.

• #2 Progressive Supranuclear Palsy (PSP) | National Institute of Neurological Disorders and Stroke

  https://www.ninds.nih.gov/health-information/disorders/progressive-supranuclear-palsy-psp

  Currently there are no tests or brain imaging techniques to definitively diagnose PSP. A physician will review your medical history and perform a physical and neurological exam. It is important to identify problems with gait, eye movements, speech, and swallowing early. Exams can help rule out other similar disorders. Diagnostic imaging may show shrinkage at the top of the brain stem and help doctors examine brain activity in certain areas. […] There is currently no treatment that effectively stops or slows the progression of PSP, and symptoms usually do not respond well to medications. […] Parkinson’s disease medications rarely help people with PSP. In some individuals, levodopa can treat the slowness, stiffness, and balance problems associated with PSP, but the effect is usually minimal and short-lasting.

• #2

  https://www.nhs.uk/conditions/progressive-supranuclear-palsy-psp/treatment/

  There’s currently no cure for progressive supranuclear palsy (PSP), but there are lots of things that can be done to help manage the symptoms. […] There are currently no medications that treat PSP specifically, but some people in the early stages of the condition may benefit from taking levodopa, amantadine or other medications used to treat Parkinson’s disease. […] Antidepressants can help with the depression that’s often associated with PSP. […] A physiotherapist can give you advice about making the most of your remaining mobility using exercise, while making sure you don’t overexert yourself. […] A speech and language therapist (SLT) can help you improve your speech and swallowing problems (dysphagia). […] As your swallowing problems become more severe, you’ll need additional treatment to compensate for your swallowing difficulties.

• #2 Progressive supranuclear palsy: diagnosis and management | Practical Neurology

  https://pn.bmj.com/content/21/5/376

  For many cognitive and behavioural symptoms, support, tolerance and environmental measures are more effective and safer than medication. […] We recommend a trial of levodopa in all patients with significant akinetic rigidity, but usually not in people with PSP-F or PSP-SL. […] Amantadine can be helpful, with patients reporting reduced axial rigidity, improved mobility, more energy and less fatigue, or clearer speech. […] PSP-PGF may respond to cholinesterase inhibitors, reflecting the cholinergic nature of pedunculopontine gait-rhythm generators. […] The coordination of non-pharmacological management is as important as drug management. Support for patients and families at different stages of disease will require input from a diverse multi-disciplinary team, from hospital and community services in health and social care. […] The involvement of palliative care teams can be hugely beneficial. Such teams often have a wide range of services, many not available elsewhere, and more readily accessible.

• #2 Progressive Supranuclear Palsy Treatment & Management: Approach Considerations, Pharmacologic Therapy, Consultations

  https://emedicine.medscape.com/article/1151430-treatment

  Some clinicians think that bromocriptine may have somewhat greater effect in individuals with PSP, though the effect is modest and short-lived in most patients. Tricyclic antidepressants have also been used. Other medications that have been tried with limited success include amantadine and trazodone. […] OnabotulinumtoxinA has been found to be useful in the treatment of rigidity (nuchal rigidity in particular) and dystonia (eg, blepharospasm, bruxism, and focal limb dystonia). It may also be useful for sialorrhea. […] A small phase II clinical trial using coenzyme Q10 in patients with PSP showed modest clinical improvement in the short term. […] Patients should follow a well-balanced diet. When feeding becomes impossible because of dysphagia and the high risk of bronchoaspiration, patients should be encouraged to accept gastrostomy as a good option.

• #2 Progressive Supranuclear Palsy (PSP) | National Institute of Neurological Disorders and Stroke

  https://www.ninds.nih.gov/health-information/disorders/progressive-supranuclear-palsy-psp

  Injections of botulinum toxin into muscles around the eyes can help with PSP-associated eye closing. […] Some antidepressant drugs may offer some benefits beyond treating depression, such as pain relief and decreasing drooling. […] There are also non-drug treatment options for PSP symptoms. […] Weighted walking aids can reduce the risk of falling backward. […] Bifocals or special glasses called prisms can help reduce difficulty looking down. […] Exercise and physical activity (supervised by a healthcare professional) can keep joints limber. […] People who have significant trouble swallowing and are at risk of serious choking may have a surgical procedure to place a tube into the stomach to allow the person to get nutrients without having to chew and swallow. […] Deep brain stimulation via devices that deliver electrical stimulation to parts of the brain and other surgical procedures commonly used in Parkinson’s have not proven effective in PSP.

• #2 Progressive supranuclear palsy – Wikipedia

  https://en.wikipedia.org/wiki/Progressive_supranuclear_palsy

  Management is only supportive as no cure for PSP is known. PSP cases are often split into two subgroups, PSP-Richardson (the classic type) and PSP-Parkinsonism, where a short-term response to levodopa can be obtained. Dyskinesia is an occasional but rare complication of treatment. Amantadine is also sometimes helpful. After a few years the Parkinsonian variant tends to take on Richardson features. Other variants have been described. Botox can be used to treat neck dystonia and blepharospasm, but this can aggravate dysphagia. […] Two studies have suggested that rivastigmine may help with cognitive aspects, but the authors of both studies have suggested that larger studies are needed. There is some evidence from small-scale studies that the hypnotic zolpidem may improve motor function and eye movements.

• #2 Progressive Supranuclear Palsy

  https://www-staging.uspharmacist.com/article/progressive-supranuclear-palsy

  A randomized, placebo-controlled trial in Germany looked at the short-term effects of coenzyme Q10 in PSP. Results indicated significant changes in the occipital lobe and a consistent trend in the basal ganglia. Coenzyme Q 10 treatment compared to placebo showed clinical improvement according to the PSP rating scale and the Frontal Assessment Battery; although the improvement was slight, it was noted as significant. The researchers concluded that since coenzyme Q 10 appears to improve cerebral energy metabolism in PSP, long-term treatment might have a disease-modifying, neuroprotective effect.

• #2 Progressive supranuclear palsy physiotherapy | Active Ability

  https://activeability.com.au/physiotherapy/progressive-supranuclear-palsy/

  Neurological physiotherapists are experienced at managing disorders like PSP. […] Physiotherapy will often include balance training activities. […] Your physiotherapist will assess your mobility, looking for things like a wide-based or unsteady gait. […] This involves practice of everyday tasks such as rolling over in bed and getting from sitting to standing. […] You might be prescribed exercises to help maintain muscle strength and flexibility. […] Physiotherapists are highly trained in pain. […] Your physio might recommend assistive devices such as a wheelchair, walking frame or other equipment to improve your safety and make life easier at home or work. […] Your physiotherapist might train family members and carers to support your loved one with moving safely around the home and out in the community, completing their exercise program, using assistive devices, and managing pain. […] We focus on strategies to maintain functional ability and safety for as long as possible.

• #2

  https://www.brain.northwestern.edu/dementia/psps/treatment.html

  There are a number of non-pharmacologic ways to support those with Progressive Supranuclear Palsy Syndrome (PSPS). Speech therapy can improve swallowing, language and communication. Occupational therapy can help provide strategies and techniques to compensate for the cognitive and motor changes associated with PSPS. Physical therapy can also improve aspects of motor function. […] Though there is no cure for progressive supranuclear palsy syndrome (PSPS), some medications can be prescribed with the goal of symptom reduction. Levodopa (Sinemet) can be trialed to address the motor symptoms of PSPS. Unfortunately, the response to levodopa is usually very limited. […] Medications can be prescribed to address mood and anxiety symptoms associated with PSPS. These medications can provide relief for affected individuals and their caregivers. Antidepressants can be given to treat depression, anxiety, or irritability. Low doses of antipsychotics may also be considered to help address delusional thinking or aggression.

• #2 Progressive supranuclear palsy – Wikipedia

  https://en.wikipedia.org/wiki/Progressive_supranuclear_palsy

  Individuals with PSP are often referred to occupational therapists to help manage their condition and to help enhance their independence. This may include being taught to use mobility aids. Due to their tendency to fall backwards, the use of a walker, particularly one that can be weighted in the front, is recommended instead of a cane. The use of an appropriate mobility aid helps to decrease the individual’s risk of falls and makes them safer to ambulate independently in the community. Due to their balance problems and irregular movements, individuals need to spend time learning how to safely transfer in their homes and in the community. This may include rising from and sitting in chairs safely. […] Due to the progressive nature of this disease, all individuals eventually lose their ability to walk and will need to progress to using a wheelchair. Severe dysphagia often follows, and at this point death is often a matter of months.

• #2 Progressive supranuclear palsy – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/progressive-supranuclear-palsy/diagnosis-treatment/drc-20355664

  Speech and swallowing evaluations, to help you learn other ways to communicate and safer swallowing techniques. […] Physical therapy and occupational therapy, to improve balance. Facial exercises, talking keyboards and gait and balance training also can help with many of the symptoms of progressive supranuclear palsy. […] Researchers are working to develop treatments of progressive supranuclear palsy, including therapies that may block the formation of tau or help to destroy tau.

• #2 Progressive Supranuclear Palsy | Conditions | UCSF Health

  https://www.ucsfhealth.org/conditions/progressive-supranuclear-palsy

  Bifocals or special glasses called prisms are sometimes prescribed to remedy the difficulty of looking down and certain exercises can help keep the joints limber. […] When a patient experiences severe difficulty swallowing and choking is a risk, a surgical procedure called a gastrostomy may be necessary. This surgery involves placing a tube through the skin of the abdomen into the intestine for feeding purposes.

• #2 Progressive Supranuclear Palsy (PSP): Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/6096-progressive-supranuclear-palsy

  Certain treatments can help various eye issues, including: Botox (botulinum toxin) injections, Eye drops and artificial tears, Special glasses, Wraparound glasses. […] As PSP worsens, you might reach the point where you cant swallow at all. This will prevent you from eating and drinking. Before that point, your healthcare provider may recommend that you get a percutaneous endoscopic gastrostomy (PEG). […] Palliative care is a specialized form of care that provides symptom relief, comfort and support to people living with serious conditions, including PSP.

• #2

  https://111.wales.nhs.uk/encyclopaedia/p/article/progressivesupranuclearpalsy/

  If you’re having problems controlling your eyelids, injections of botulinum toxin (such as Botox) can be used to help relax the muscles of your eyelids. […] Palliative care can be offered at any stage of PSP, alongside other treatments. […] Many people with PSP consider making plans for the future that outline their wishes (both medical and other decisions) and make them known to both their family and the health professionals involved in their care. […] There have been several trials of different medications that aim to help symptoms of PSP or slow its progression.

• #2 Progressive Supranuclear Palsy (PSP) | National Institute of Neurological Disorders and Stroke

  https://www.ninds.nih.gov/health-information/disorders/progressive-supranuclear-palsy-psp

  There have been significant efforts to develop new therapies for PSP to slow down or stop the disease progression. Many of these studies focus on developing anti-tau therapy to stop the accumulation and spread of the tau protein. Other studies have focused on different PSP-related changes in the nervous system, including inflammation and problems with mitochondria (the energy factories in cells). Other studies hope to find ways to improve brain activity using a special type of brain stimulation that does not involve surgery. Some researchers are exploring stem cell-based therapies to help symptoms of PSP.

• #2 New diagnostics and therapeutics for progressive supranuclear palsy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5802400/

  The rationale for treating PSP with microtubule stabilizers is to compensate for the microtubule dysfunction that might result from a disease-associated loss of tau function. […] A number of microtubule-stabilizing agents have been developed and three have been explored in human neurodegenerative disease clinical trials. […] However, a recent large clinical trial of davunetide for PSP-RS demonstrated a lack of efficacy. […] TPI-287, a new blood brain barrier permeable taxane, has entered phase I clinical trials for AD, PSP and CBD. […] Evidence that tau may propagate between cells in a prion-like fashion raises the possibility that blocking spread of pathogenic tau by specific anti-tau antibodies might be a viable approach to tauopathy treatment. […] Two N-terminal tau directed monoclonal antibodies, BMS-986168 and Abb-8E12, have progressed to Phase 2 clinical trials for PSP.

• #2

  https://link.springer.com/article/10.1007/s40120-024-00614-9

  The monoclonal antibody bepranemab (UCB0107) recently underwent a phase Ib study to evaluate its safety, tolerability, and pharmacokinetics in the treatment of patients with PSP. […] Two N-terminal tau-targeting monoclonal antibodies, gosuranemab and tilavonemab, have entered phase II clinical trials for PSP. […] Active vaccinations against pathological tau epitopes have been investigated, and three potential tau vaccines have begun human clinical trials: AADvac1, ACI-35, and JACl-35. […] Overall, the results showed that tilavonemab lacked efficacy in the treatment of PSP in humans. […] Ultimately, there were no other findings to indicate a response to tilavonemab. […] The current phase III trial of rivastigmine is a randomized, double-blind, placebo-controlled study with 106 participants with probable PSP-RS.

• #2 New diagnostics and therapeutics for progressive supranuclear palsy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5802400/

  Deep brain stimulation of the pedunculopontine nucleus has been attempted in advanced PSP-RS, however there were no clear benefits observed and unacceptable side effects. […] The rapid growth of knowledge about the potential pathogenic mechanisms of PSP has facilitated the implementation of a series of rational clinical studies with hypothesis-driven therapeutic approaches aimed at disease-modification by targeting tau or mitochondrial dysfunction. […] Of these, three clinical trials were adequately powered to demonstrate small to moderate disease-modifying effects: the NNIPPS study of riluzole, a phase 2 trial of tideglusib, and a Phase 2/3 trial of davunetide. […] An important limitation of these studies was the lack of a pharmacodynamic biomarker that could demonstrate that the experimental agent engaged its physiological target and produced its hypothesized biological effect.

• #2

  https://link.springer.com/article/10.1007/s40120-024-00614-9

  Studies are currently underway to evaluate whether the management of modifiable risk factors aids in the treatment of patients with PSP. […] Current strategies of PSP treatment include targeting tau protein aggregation, microtubule dysfunction, and tau posttranslational modification by mechanisms that include immunotherapy, gene therapy, and vaccines. […] Several clinical trials are currently underway to identify a disease-modifying treatment for PSP. […] In determining treatment for tauopathies, it is critical to consider the cause of tau-dependent neurodegeneration. […] The first set of tau-based treatments targets tau loss-of-function, which can lead to microtubule destabilization. […] Treatment with microtubule-stabilizing agents may compensate for tau dysfunction in these neurodegenerative diseases.

• #3 Progressive Supranuclear Palsy Treatment & Management: Approach Considerations, Pharmacologic Therapy, Consultations

  https://emedicine.medscape.com/article/1151430-treatment

  Some clinicians think that bromocriptine may have somewhat greater effect in individuals with PSP, though the effect is modest and short-lived in most patients. Tricyclic antidepressants have also been used. Other medications that have been tried with limited success include amantadine and trazodone. […] OnabotulinumtoxinA has been found to be useful in the treatment of rigidity (nuchal rigidity in particular) and dystonia (eg, blepharospasm, bruxism, and focal limb dystonia). It may also be useful for sialorrhea. […] A small phase II clinical trial using coenzyme Q10 in patients with PSP showed modest clinical improvement in the short term. […] Patients should follow a well-balanced diet. When feeding becomes impossible because of dysphagia and the high risk of bronchoaspiration, patients should be encouraged to accept gastrostomy as a good option.

• #3 Progressive Supranuclear Palsy | AFTD

  https://www.theaftd.org/what-is-ftd/progressive-supranuclear-palsy/

  There is currently no effective treatment for PSP. In some patients, the slowness, stiffness, and balance problems of PSP may respond to anti-parkinsonian agents such as levodopa, or levodopa combined with anticholinergic agents, but the effect is usually temporary. […] Decreased transmission of GABA in the mid-brain is considered to contribute to the symptoms of PSP and so drugs that work on the GABA neurotransmitter may be helpful. Zolpidem, in limited case studies, has improved speech and facial expressions, as well as akinesia rigidity and dysarthria. However, this drug is short acting and prolonged use is not recommended. In general, speech, vision, and swallowing difficulties usually do not respond to any currently available drug treatment. […] Another group of drugs that has been of some modest success in PSP are antidepressant medications. The most common antidepressants used for PSP have been Prozac, Elavil and Tofranil. The anti-PSP benefit of these drugs seems to be unrelated to their ability to relieve depression.

• #3

  https://link.springer.com/article/10.1007/s11940-016-0422-5

  Benzodiazepines may also be useful in managing dystonia. […] Myoclonus may be managed using levetiracetam and benzodiazepines. […] Pharmacological agents licensed for Alzheimers disease (such as acetylcholinesterase inhibitors and N-Methyl-D-aspartate receptor antagonists) have been used off-label in PSP, CBD, and other tauopathies with the aim of improving cognition; however, there is limited evidence that they are effective and risk of adverse effects may outweigh benefits. […] The use of atypical antipsychotics for behavioural symptoms is not recommended in the elderly or those with dementia associated conditions and most antipsychotics will worsen Parkinsonism. […] Antidepressants may be useful for behavioral symptoms and depression but are often poorly tolerated due to adverse effects.

• #3 Progressive supranuclear palsy physiotherapy | Active Ability

  https://activeability.com.au/physiotherapy/progressive-supranuclear-palsy/

  Progressive supranuclear palsy is a rare neurological condition that mainly affects people over the age of 60. For people with progressive supranuclear palsy, physiotherapy can help to maintain function and independence for as long as possible. […] Physiotherapy treatment for PSP aims to optimise physical function and independence and relieve any distressing symptoms. […] Physiotherapy treatment for PSP may include therapies designed to improve walking ability and safety, transfers such as getting out of bed and on/off chairs, control of head and body movements, balance and posture, energy levels, muscle strength and flexibility, pain or discomfort, independence, and quality of life. […] The authors concluded these studies provide early evidence supporting the use of physiotherapy rehabilitation programs to improve gait, balance, and gaze control in people with PSP.

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