How rare sleep disorder led to detection of early sign of Parkinson’s

Donald Dorff could hear the crowd roaring as he grabbed the quarterback’s pass out of the air and ran toward the goal line.

“There was a 280-pound tackle waiting for me, so I decided to give him my shoulder,” the 67-year-old told magazine National Geographic in 1987.

“When I regained consciousness, I was on the floor of my room,” Dorff said. “I had crashed into the dresser and knocked everything off it, broken the mirror and made a huge mess. It was 1:30 in the morning.”

How did a retired grocery manufacturer’s dream from Golden Valley, Minn. become worthy of a profile in a well-known national magazine?

Five years earlier, Dorff had become the first patient diagnosed with an unusual disease called REM sleep behavioral disorder or TCSR .

There is more. Dorff’s case also launched researchers on a journey that uncovered one of the first signs of two devastating diseases: Parkinson’s and a unique form of dementia called “dementia with Lewy bodies.”

Symptoms include screaming, kicking and punching

The discovery of TCSR was made in 1982, when Dorff, after years of “violent moving nightmares,” became a patient of psychiatrist and sleep specialist Carlos Schenck, then working at the Minnesota Regional Sleep Disorders Center in Minneapolis. in the United States.

“During rapid eye movement sleep, or REM sleep, the brain basically paralyzes the body so that we cannot act on our dreams,” said Schenck, now a professor and senior psychiatrist at the Hennepin County Medical Center from the University of Minnesota, to CNN recently.

“But Dorff managed to get up and hurt himself while dreaming, a very strange behavior. We were left scratching our heads about it,” he said. “So we put him in a sleep lab, and lo and behold, all of his physical behaviors emerged during REM sleep, which had never been reported before.”

More patients with the unusual disorder have been identified, many displaying a disturbing set of violent symptoms, Schenck says.

A 70-year-old patient, described by his wife as having a gentle nature during the day, used to punch and kick her up to five times a night, according to Schenck. On one occasion he tried to strangle his wife while dreaming that she was fighting a ferocious bear.

Another patient, also 70 years old, dreamed that he had just knocked down a deer and that his neck was going to break. In reality, it was his wife’s neck, which he discovered when he woke up. (She stopped sleeping in the same bed.)

In 1986, Schenck and his team published their first paper on the newly discovered sleep disorder.

As he followed patients over the years, however, Schenck discovered something more disturbing — the behavior could be a “canary in the mine” for future neurodegeneration.

“Losing REM sleep paralysis, which is the basis of TCSR, is the earliest and strongest predictor of a future diagnosis of Parkinson’s disease and dementia with Lewy bodies,” says Schenck.

TCSR can affect women too

Dementia with Lewy bodies can refer to two different types of dementia: Parkinson’s disease dementia It is dementia with Lewy bodieswhich both affect cognition.

Parkinson’s disease, however, is a slowly progressing disorder that inhibits muscle control, balance and movement — although about 4 in 5 people with Parkinson’s eventually develop dementia, according to Johns Hopkins Medicine.

In both cases of Parkinson’s and both dementias, cells in and around the rod-shaped trunk at the base of the brain begin to die. In addition to controlling breathing, swallowing, heart rate, and eye and facial movements, the brain stem also controls the sleep-wake cycle.

Early brain stem damage caused by Parkinson’s disease can interfere with the body’s innate ability to paralyze muscles while dreaming, thus allowing the body to sit, scream, shake, or otherwise act out during a dream.

Hallucinations, which are common in Parkinson’s disease, may also occur, according to case studies. A 67-year-old man saw faceless hooded or cloaked figures or animals while awake at night. The images disappeared when the man turned on the light or got up to investigate.

These nocturnal behaviors can often be controlled with medication, including high doses of melatonin or the drug clonazepam, which stops seizures and relaxes tense muscles, Schenck says.

“Either one alone or both in combination works 80% to 90% of the time, and there is a long list of alternative treatments,” says the expert, who first published the connection between TCSR and Parkinson’s in February 1996.

“We know the mechanism of TCSR very well and we know how to treat it,” he says. “The next step is to slow or stop the progression to Parkinson’s disease and dementia with Lewy bodies.”

At first, researchers believed that TCSR only affected men, but later realized that women are equally impacted.

“So many women have this problem, but because it is much milder and doesn’t cause injuries as often, they often don’t seek help,” he says. “However, they have lost muscle paralysis in REM sleep and are just as at risk of developing Parkinson’s disease in the future as men.”

A clinical trial is underway at nine medical centers in the United States, Schenck says. Called North American Prodromal Synucleinopathy Consortiumresearch hopes to identify potential treatments for REM sleep behavior disorder and slow its progression to Parkinson’s and dementia.

Seek medical treatment if violent dreams begin

As a child, Donald Dorff shared a room with three brothers and never showed signs of a sleep disorder. On the night of his wedding, however, he frightened his new wife with loud talking, moaning, grinding of teeth and small movements, according to Schenck’s case study.

About 41 years later, he began to have vivid, violent dreams that he acted out. In a dream, he was riding a motorcycle when another motorcyclist tried to collide with him.

“I decided I was going to kick his bike, and at that moment my wife woke me up and said, ‘What in heaven’s name are you doing to me?’ because I was kicking her so hard,” Dorff told Schenck during treatment.

In another dream, Dorff was under attack by a man with a rifle and was preparing to fight back when he suddenly woke up. “I was kneeling next to the bed with my arms outstretched as if I was holding the rifle and ready to shoot.”

One night, Dorff threw himself against a dresser and cut himself, Schenck said. It was then that he found Schenck’s clinic and went there for treatment.

While some people with TCSR develop full-blown Parkinson’s quickly, Dorff was not one of them. He died of prostate cancer, Schenck says, and was able to control his violent nighttime behavior until his death as long as he continued taking his medication.

Dorff’s case helped doctors discover an early key to two devastating disorders, allowing for earlier diagnosis and treatment.

“If you have never been a sleepwalker or sleep talker throughout your life and then suddenly after age 50 you start talking louder and more frequently, you really should be evaluated by your primary care doctor,” Schenck said. .

“Your doctor should consider a neurological evaluation because this may be the first sign of a neurodegenerative disorder.”