lunes, 29 de octubre de 2018

Progressive Supranuclear Palsy (PSP) Management

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By Karolina Brzozowska, M.Sc.Reviewed by Liji Thomas, MD

Progressive supranuclear palsy (PSP) is a rare brain disorder that affects approximately 6 in 100,000 people worldwide. It causes muscle weakness and affects gait, balance and overall movement, as well as the patient’s mood, cognitive skills and behavior. While no treatment is available at this time, various symptoms can be managed with the use of medication or other forms of therapy.

Progressive Supranuclear Palsy (PSP) Management

Managing the various effects that PSP has on the patient’s cognitive, behavioral and motor skills is challenging, especially as the illness progresses over time. The main focus remains on managing the symptoms and ensuring an additional daily support network for the patient.

Ongoing clinical trials remain a hope for those persons affected. The research into PSP today mainly targets tau protein pathology, as it is believed to be an underlying cause of this condition as well as several other neurodegenerative disorders.

Some of the options offered to patients today include:

medication, including that used in the management of Parkinson’s disease,
botulinum toxin type A (botox) injections,
especially prescribed glasses,
physical and occupational therapy,
speech therapy.

Medication in PSP Management

The medication used in aiding patients with Parkinson’s disease stimulates the release of a neurotransmitter called dopamine from the brain. It helps the muscles to relax, ensuring better control of movement and reducing muscle stiffness. However, it is yet to prove effective in the case of PSP. Similarly, botulinum toxin type A (botox) injections are prescribed to aid muscle spasms and stiffness.

This treatment is similar to that advised for patients with muscle spasticity as it induces muscle fiber-type changes. This helps the muscles to relax and reduces the tension and the pain associated with it. Antidepressants may be prescribed to help with the accompanying depression in some cases.

Non-Medication Options in PSP Management

When it comes to the deterioration of vision, the needs of a patient with PSP are very different from just needing prescription reading glasses. Bifocals or a type of special glasses called prisms can prove helpful for patients with PSP to remedy the difficulty of looking down (“downgaze palsy”). The effectiveness may vary from patient to patient, as the root cause remains the damage to the brain which cannot be helped with corrective glasses.

Physical therapy is of increasing interest in the management of PSP, particularly given the evidence of the various benefits it has for patients with Parkinson’s disease. The focus is on enabling the patient to maintain as much fluidity of movement and muscle strength as possible, which in turn enables them to live an independent life for as long as they can. Occupational therapy can also focus on teaching the patient “how to fall”, as this can significantly reduce the risk of further injury.

As the speech and swallowing are both affected, it is sometimes necessary to introduce feeding through a PEG tube. It is a tube that connects the stomach directly with the outside through the abdominal wall. It ensures that appropriate nourishment is administered and eliminates the risk of choking when swallowing.

Lifestyle Changes and Home Remedies

Making additional adjustments to the lifestyle and adopting some useful home remedies can also aid in minimizing the effects of PSP and ensuring the best possible quality of life for the patient. Eliminating clutter and moving objects that may cause the patient to fall, as well as using walking aids, are all helpful in this area.

Good care and additional assistance is essential as the disease progresses.

Sources

"Progressive Supranuclear Palsy Fact Sheet", NINDS, Publication date September 2015. (www.ninds.nih.gov/.../Progressive-Supranuclear-Palsy-Fact-Sheet)
“Progressive Supranuclear Palsy”, Mayo Clinic (www.mayoclinic.org/.../syc-20355659 )
“Progressive supranuclear palsy”, NHS website (https://www.nhs.uk/conditions/progressive-supranuclear-palsy-psp/)
Agarwal S, Gilbert R. Progressive Supranuclear Palsy. [Updated 2018 Sep 20]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2018 Jan. (https://www.ncbi.nlm.nih.gov/books/NBK526098/ )