Why Parkinson’s Disease Has Robbed Linda Ronstadt Of Her Singing Voice

Singer Linda Rondstadt has recently been diagnosed with Parkinson's disease.

Singer Linda Rondstadt has recently been diagnosed with Parkinson’s disease.

The news that Linda Ronstadt has lost her powerful voice after a four-decade singing career as a result of Parkinson’s disease shocked not just the singer, but her many fans.

The 67-year-old Grammy-winning singer revealed last week that she struggled with symptoms of the disease for nearly eight years before getting her diagnosis just months ago. The neurological degenerative condition robs sufferers of their speech, mobility and their cognitive abilities.

During my career, I have spent almost 30 years investigating the effects of Parkinson’s disease on the brain and  I have seen great strides in our understanding and treatment of this illness.

What is Parkinson’s disease?

Over 4 million people worldwide suffer from Parkinson’s disease – a so-called hypokinetic disorder (hypo = lack of; kinetic = movement). Parkinson’s disease can be genetic but it can also be triggered by brain injury – as observed in boxers such as Muhammad Ali – and by environment – e.g. prolonged exposure to chemicals such as insecticides, weed killers and some drugs.  However in most cases Parkinson’s disease arises ‘out of the blue’ – i.e. idiopathic Parkinson’s disease. It is a progressive disease the symptoms start out small and get progressively worse but it is rarely fatal.

Three major symptoms

The disease is associated with three major symptoms (i) akinesia – an inability to start a movement – for instance when starting to brush your teeth, (ii) bradykinesia – an excessive braking or slowing of movement – when the brush strokes become shorter and eventually freeze and (iii) tremor – starting out as a trembling finger movements as if rolling a coin or pill – sometimes called ‘pill-rolling’ – which can progress to the whole body. With Parkinson’s disease one minute you are working away in the garden and the next you are literally stuck to the spot – totally unable to move. In these situations daily life can become a challenge that can be difficult to endure.

History

The disease is named after a British surgeon and political activist James Parkinson (1755-1824) who was born in London’s east end. In October 1794 he was questioned under oath before a council of state in connection with a plot to kill the King. He was exonerated but remained a strong advocate for the under-privileged. In 1817 he published An Essay on the Shaking Palsy reporting 6 cases. Although his publication was later considered the seminal work on the disease, it received little attention until a lot more cases came to light.

Where in the brain does Parkinson’s disease occur?

The Nobel Prize was awarded in 2000 to Professor Arvid Carlsson for his research over the past 50 years which confirmed that the ‘core defect’ in Parkinson’s disease is a loss of a chemical called dopamine in two tiny brain regions  – no bigger than a wart – called the substantia nigra. These two regions deep in the back of the brain – one on the left side and one on the right side – contain nerves that make a lot of dopamine and release it at their tips about 4 inches forward in the basal ganglia – located deep in the center of the brain where it helps to convert the intention/motivation needed to perform a movement into actual movement.

The core defect is a loss of dopamine

Dopamine belongs to a family of neurotransmitters called amines that help nerve cells to stay in touch with each other- sometimes called neurotransmitters. Dopamine acts like hydraulic oil which lubricates the basal ganglia nerve network involved in executing a movement. One only needed to go to watch Linda perform on stage at the height of her career to see dopamine in action as her brain effortlessly converted her thoughts and moods into the mesmerizing tonality, rhythm and range that made her so loved by a generation. In Parkinson’s disease the substantia nigra progressively dies, the supply of dopamine to the basal ganglia dries-up and like a car out of oil the engine seizes-up and movement grinds to a halt.

Trapped and disconnected

We need dopamine to survive. Dopamine allows us to talk, sing, socialize, improvise and take risks. When dopamine flows we don’t see things as being limited by circumstance. We have boundless energy and literally anything is possible!  Without dopamine we feel trapped and disconnected. It is not surprising therefore that mood can become low and depression is often associated with this illness.

Medication is expensive

Over the past 40 years neuroscientists been working hard to develop drugs that can safely raise dopamine levels in the Parkinsonian brain – the so-called antiparkinsonian drugs – and better, more effective treatments come on the market every decade.  Drug treatments are effective in managing symptoms but frequent changes are needed as the disease progresses. In the end many combinations of drugs at higher and higher doses may be needed and unpleasant side-effects may become an issue. Medication costs on average €100,000 per year.  Interestingly, the inexpensive anti-inflammatory drug ibuprofen has recently been shown to help stave off Parkinson’s disease possibly by protecting the substantia nigra but ibuprofen can itself produce unwanted side effects, including stomach bleeding.

Alternatives to drug-treatment?

deep  brain stimulation

Neuroscientists believe that the loss of the dopamine-rich nerves in the substantia nigra results is an over-activity of a ‘brake’ mechanism in the basal ganglia which in turn inappropriately shuts down movement.  Deep brain stimulation (DBS) takes advantage of our knowledge of this brain wiring by applying a small electric current into a target area called the subthalamic nucleus located deep within the basal ganglia to switch off the brake and free-up movement. It is an expensive and risky procedure but it can provide a new lease of life particularly to those patients for whom conventional drug treatment no longer seem to work.

The politics of brain research

Probably the most important lesson to be taken from Linda’s illness is that we need to open up a debate on how we as a society fund research into brain illness and highlight the realization that a deeper understanding of the brain IS the difference between a good and a bad quality of life for the sufferers of brain illnesses such as Parkinson’s disease.

I hope that Linda and her loved ones will take hope from the on-going research into Parkinson’s disease by teams of dedicated scientists worldwide.

Click on the link below to hear Professor Billy O’Connor talk about Parkinson’s disease on Mind Matters – a science programme on RTE radio.

http://www.podcastdirectory.com/episodes/mind-matters-on-parkinson-s-disease-3465171.html

Weekly Neuroscience Update

sad music

Sad music might actually evoke positive emotions reveals a new study by Japanese researchers. The findings help to explain why people enjoy listening to sad music, say Ai Kawakami and colleagues from Tokyo University of the Arts and the RIKEN Brain Science Institute, Japan.

Extroverts may be more outgoing and cheerful in part because of their brain chemistry, reports a study by Cornell neuroscientists.

Researchers at Neuroscience Research Australia (NeuRA) have discovered that the region of the brain called the orbitofrontal cortex plays a key role in linking emotion and memories. The finding comes from a new study, which demonstrates that patients with frontotemporal dementia (FTD) lose the “emotional content” of their memories.

Researchers in Oxford have demonstrated a significant improvement in the treatment of advanced Parkinson’s disease with deep brain stimulation.

In a study examining how bilingual children learn the two different sound systems of languages they are acquiring simultaneously, researchers have discovered insights that indicate children can learn two native languages as easily as they can learn one.

Scientists have developed a method with which the chances of success of a surgical procedure for temporal lobe epilepsy can be accurately predicted.

Patient Plays Guitar During Brain Surgery To Treat Parkinson’s

Brad Carter Plays Guitar During Brain Surgery To Treat Parkinson’s

To mark Ronald Reagan UCLA Medical Center’s 500th deep brain stimulation operation last Thursday, surgeons performed the world’s first live-Vined brain surgery.

The operation, which lead neurologist Dr. Nader Pouratian described as “textbook” brain pacemaker implant surgery, was documented with Vine, a six-second video medium, and with Instagram photos on the hospital’s Twitter account in real time on patient Brad Carter, an actor diagnosed with the progressive neurological disorder essential tremor in 2006.

“Not everyone gets to experience a surgery, and more specifically an awake brain surgery,” Pouratian said in a phone interview with The Huffington Post. “I thought it was a great opportunity to share with the world.”

Deep brain stimulation is a therapy for people with Parkinson’s disease, that  involves inserting a pacemaker that emits electronic impulses to affected areas of the brain, lessening tremor symptoms. At UCLA, the patient is conscious during the surgery and is asked to perform tests so that doctors can ensure the pacemaker is properly placed.

Carter’s disease made his hands shake and his eyes twitch, affecting his ability to play the guitar. He had asked that he be allowed to strum his guitar during the operation to see if the pacemaker improved his playing ability.
Vine videos from the surgery show Carter’s guitar skills get stronger as physicians located the best place to leave the pacemaker.

Weekly Neuroscience Update

A run of poor sleep can have a potentially profound effect on the internal workings of the human body, say UK researchers.

As a bird sings, some neurons in its brain prepare to make the next sounds while others are synchronized with the current notes—a coordination of physical actions and brain activity that is needed to produce complex movements, new research at the University of Chicago shows. In an article in the current issue of Nature, neuroscientist Daniel Margoliash and colleagues show, for the first time, how the brain is organized to govern skilled performance—a finding that may lead to new ways of understanding human speech production.

Deep brain stimulation has helped people with severe obsessive-compulsive disorder, and new research begins to explain why. A Dutch study appearing in the Feb. 24 online issue of the journal Nature Neuroscience found the procedure essentially restored normal function in a part of the brain called the nucleus accumbens.

Researchers have identified a possible treatment window of several years for plaques in the brain that are thought to cause memory loss in diseases such as Alzheimer’s. The Mayo Clinic study is published in the Feb. 27 online issue of Neurology.

Though it’s most often associated with disorders like diabetes, Harvard researchers have shown how the signaling pathway of insulin and insulin-like peptides plays another critical role in the body – helping to regulate learning and memory.

Researchers in Scotland and Germany have discovered a molecular mechanism that shows promise for developing a cure for Huntington’s Disease (HD).

Some people do not learn from their mistakes because of the way their brain works, according to research led by an academic at Goldsmiths, University of London. The research examined what it is about the brain that defines someone as a ‘good learner’ from those who do not learn from their mistakes.

Scientists from the University of Oxford say they have discovered how the brain protects itself from damage that occurs in stroke.

A team of French researchers has discovered that the human brain is capable of distinguishing between different types of syllables as early as three months prior to full term birth. 

Weekly Neuroscience News

Scientists identify link between size of brain region and conformity.

Although there are several drugs and experimental conditions that can block cognitive function and impair learning and memory, researchers have recently shown that some drugs can actually improve cognitive function. The new multi-national study, published in the 21 February issue of the open-access journal PLoS Biology, reveals that these findings may implicate scientists’ understanding of cognitive disorders like Alzheimer’s disease.

New connections between brain cells emerge in clusters in the brain according to a study published in Nature on February 19 (advance online publication). Led by researchers at the University of California, Santa Cruz, the study reveals details of how brain circuits are rewired during the formation of new motor memories.

A new study shows that depression is linked to hyperconnectivity of brain regions.

A study in the February issue of Neurosurgery reveals that deep brain stimulation (DBS), commonly used to treat individuals with movement disorders or chronic pain, also affects respiratory function.