The legendary singer Linda Ronstadt had a spectacular year in 2019. One of the five honorees of the Kennedy Center Honors was the most popular female singer of the 1970s. A recent CNN Films documentary titled “Linda Ronstadt: The Sound of My Voice” also features Ronstadt. But these honors come with a price. She first experienced vocal difficulties in 2000.
As she revealed to Anderson Cooper of CNN, “The top end of my voice was inaudible to me. I was unable to hear the portion I used to tune myself. At times, I would choke up. It would merely feel like I was cramping.” After receiving a Parkinson’s disease diagnosis initially, she was forced to stop singing in 2009. Her diagnosis was modified to progressive supranuclear palsy, a rare brain condition, in late 2019. (PSP).
Cooper was informed by Ronstadt that her illness has significantly affected her life: “Everything becomes difficult. Showering, tooth brushing—I always come up with inventive new ways to accomplish tasks. I’m like a little kid. It’s difficult to eat; I have to retrain how to do it. If you’re persistent and prepared to do that, you could sculpt a new brain map, but it’s challenging.”
Progressive Supranuclear Palsy: What Is It?
PSP is a rare form of brain disease that impairs thinking, speech, swallowing, vision, mood, behavior, mobility, gait, and balance. Damage to brain nerve cells is the cause of the condition. Certain regions of the brain above nuclei, or clusters of nerve cells, are harmed by the disease (supranuclear). Particularly, these nuclei regulate eye movements. The inability to aim and move the eyes effectively, which people may feel as blurred vision, is one of the disease’s hallmark symptoms.
According to various estimates, PSP affects only three to six persons in every 100,000 people worldwide, or about 20,000 Americans, making it significantly less frequent than Parkinson’s disease (an estimated 50,000 Americans are diagnosed each year). PSP symptoms typically start after the age of 60, though they might start earlier. Men experience it more frequently than women.
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What Signs Are Present?
Although the pattern of symptoms and indicators might vary greatly from person to person, they typically fit into one of four categories. Named after one of the physicians who first recognized PSP as a separate entity in 1963, the Richardson Syndrome. According to the advocacy and support organization NORD, it includes “gait and balance impairment, a wide-eyed staring facial expression, abnormal speech, memory, and cognitive impairment, and a slowing or loss of voluntary eye movement, particularly in the downward direction (supranuclear ophthalmoplegia)”.
Parkinsonism that is atypically present includes slowness, muscle rigidity, and sporadic trembling. They might react to levodopa at first. Patients with corticobasal syndrome (CBS) exhibit bizarre rigidity and dystonia. With symptoms beginning on one side of the body and continuing to be worse on that side, CBS neurodegeneration is clearly asymmetrical. Akinesia and gait freeing: These patients tend to freeze upon turning or passing a threshold and have sluggish gait initiation (such as a doorway).
What Distinguishes PSP from Parkinson’s Disease?
Parkinson’s disease and PSP both induce stiffness, trouble moving, and clumsiness, but PSP advances more quickly than Parkinson’s disease. PSP sufferers frequently stand extremely straight or even occasionally tilt their heads back (and tend to fall backward). “Axial rigidity” is the term for this. Parkinson’s patients typically lean forward. Speech and swallowing issues are far more frequent, and severe, and tend to manifest earlier in the course of PSP than they do in Parkinson’s disease. In Parkinson’s disease, eye movements are almost normal, but they are abnormal in PSP.
Other characteristics that both disorders have in common include late middle age onset, bradykinesia (slow movement), and muscle rigidity. Tremor is uncommon in PSP patients but very common in those with Parkinson’s disease. Levodopa benefits persons with Parkinson’s disease significantly, although it has a small and transient effect on PSP patients. The protein tau accumulates in the brain cells of people with PSP, whereas the protein alpha-synuclein accumulates in the brain cells of people with Parkinson’s disease.
What Brings on PSP?
PSP’s precise origin is unknown. The gradual degeneration of brain cells, particularly in the brain stem, is what leads to PSP symptoms. The substantia nigra, one of these regions, is also damaged in Parkinson’s disease, and damage to this portion of the brain contributes to the motor symptoms shared by PSP and Parkinson’s.
The buildup of aberrant tau protein deposits in brain nerve cells is the disease’s defining feature. Microtubules, which maintain nerve cells’ long processes, or axons, that communicate with other nerve cells, are linked to the protein tau. PSP belongs to the category of diseases known as tauopathies, which also includes other conditions like Alzheimer’s disease, corticobasal degeneration, and other varieties of frontotemporal degeneration, due to the buildup of tau.
PSP is typically sporadic, occurring infrequently and with no known cause; in a very small number of cases, the disease is brought on by mutations in the MAPT gene, which subsequently give the nerve cell incorrect instructions for producing tau. According to a recent study, the condition may be at least partially inherited and may also be influenced by a number of environmental factors.
Exists a Treatment for This?
PSP presently has no effective treatments, although researchers are working to find new ways to control the condition. Medication usually has no effect on PSP symptoms. Ropinirole is one of the Parkinson’s disease medications that is frequently administered. Antiparkinsonian medications like levodopa may partially alleviate the slowness, stiffness, and balance issues caused by PSP in some people, although the benefit is typically mild and transient.
Botulinum injections can be used to treat excessive eye closure. Some antidepressant medications may have advantages besides treating depression, such as reducing drooling and relieving discomfort. Recent PSP therapy efforts have mostly centered on clearing abnormally accumulated tau from the brain.
The safety and tolerability of a drug that reduces tau buildup in preclinical models will be evaluated in a current clinical trial. Improved tau imaging techniques are being investigated in other research in order to monitor the development of the disease and how well it responds to therapy.
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What Is the Outlook?
Within three to five years of the disease’s onset, patients become severely crippled as the condition worsens. Serious problems, such as pneumonia, choking, head injuries, and fractures, are more likely to occur in affected people. Pneumonia is the most common cause of death. PSP patients have the potential to live ten years or longer after the onset of the disease with proper medical and nutritional care.
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