Progressive Supranuclear Palsy

Progressive supranuclear palsy(PSP) worsens over time and can lead to life-threatening complications, such as pneumonia and swallowing problems. There's no cure for progressive supranuclear palsy, so treatment focuses on managing the signs and symptoms.

PSP is briefly described as an atypical parkinsonian syndrome (Parkinson-plus disorder) typically characterized by progressive.

• Early-onset postural instability,

• Frequent (unexplained) falls,

• Problems with eye movement,

• Axial (involving neck or trunk) rigidity,

• Speech/swallow difficulty, and cognitive decline.

You may have particular difficulty looking downward, or experience blurring and double vision. This difficulty with focusing the eyes can make some people spill food or appear disinterested in conversation because of poor eye contact. This disorder results from deterioration of cells in areas of your brain that control body movement, coordination, thinking and other important functions. Progressive supranuclear palsy is also called Steele-Richardson-Olszewski syndrome

Additional signs and symptoms of progressive supranuclear palsy vary and may mimic those of Parkinson's disease and dementia. Stiffness and awkward movements

• Falling

• Problems with speech and swallowing

• Sensitivity to light

• Sleep disturbances

• Loss of interest in pleasurable activities

• Impulsive behavior, possibly including laughing or crying for no reason

• Difficulties with memory, reasoning, problem-solving and decision-making

• Depression and anxiety

• A surprised or frightened facial expression, resulting from rigid facial muscles

Age is the only proven risk factor for progressive supranuclear palsy. The condition typically affects people around the age of 60 and is virtually not seen in people under the age of 40.

The cause of progressive supranuclear palsy isn't known.

Rarely, progressive supranuclear palsy occurs within a family. But a genetic link has not been established, and most people with progressive supranuclear palsy haven't inherited the disorder.

Unfortunately no pill or cure is established till date. Treatment primarily focus on supportive care.

We still have hope, the research in recent years are established the pathology behind the PSP. Now we know more about proteins involved in the process of disease. However, the cause of this devastating disease remains unclear.

Knowledge of tau protein pathology, in particular, has led to several recent clinical trials aimed at reduction of pathological phosphorylated tau and deposits in brain cells. Drugs that inhibit GSK-3 (glycogen synthesis kinase 3) have received much attention because GSK-3 phosphorylates tau and localizes to tau-positive neurofibrillary tangles (cellular deposits seen in several neurodegenerative disorders including PSP)

After movement disorder specialist diagnoses it as atypical Parkinson’s the approach towards a patients will be initiated by Neuro rehabilitation team and physiotherapist trained in movement disorders. They use a digital gait and balance mat which helps the clinicians follow the disease and researchers learn more about walking and balance issues leading to falls in these disorders.

The patient and family, care takers will have access to all interdisciplinary services of our team at one place:

• Physical therapy

• Occupational therapy

• Speech/swallow evaluations

• Neuropsychology

• Psychiatry

• Social work/case management

• Palliative services

We aim to provide these multiple services (as needed) during your clinic visit, thus providing true comprehensive, interdisciplinary care.

We aim to provide opportunities for patients to participate in clinical research and trials that we hope will lead breakthroughs in diagnostics and treatment for these devastating disorders. Our aim is to offer patients the best possible clinical and research experience.

Most recently, the drug Davunetide was tested which reduces tau phosphorylation. But still not clear whether it works for PSP. With increasing knowledge about the pathologic mechanisms of PSP new targets for therapeutics trials are inevitable.

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. However, studies in Toronto, Canada (Dr. Lozano) are investigating targeting a very small area of the brainstem called the PPN (pedunculopontine nucleus) for gait problems in Parkinson’s disease and potential application to PSP.