This is the first of three blogs about Lewy body dementia.
Some years ago, my friend Molly Grant, was concerned about her husband, Todd, so she asked his sister, Peggy, if she’d noticed any change in him.
“Yes,” Peggy admitted. “He seems to have lost his self-confidence. And his posture has changed; he’s slumped.”
Todd also seemed confused at times. He had what Molly called his cloudy days, but sometimes within hours his mind would clear. In addition, neither of them was sleeping well because he was so restless at night.
When Todd finally agreed it was time to see a doctor, his primary care physician gave him a mini-mental exam—a 10-minute test that screens for cognitive impairment. He scored 25 out of 30.
“That’s just on the edge,” the doctor said. “It could be early Alzheimer’s, but only time will tell. Come back in six months and we’ll check again.”
Once home, Molly wondered how Todd would have scored if he had been tested on one of his cloudy days.
Not long after, Todd tripped and fell. He wasn’t hurt, but when it happened again a week later, Molly was sure it had to do with the way he shuffled when he walked. He was also having trouble with small tasks, like buttoning his shirt, and his falls were making him anxious.
“Something’s wrong with my body,” he told Molly.
A neurologist they consulted was immediately taken by Todd’s shuffling gait and his anxiety. He detected rigidity in his muscles and slowness in his movements.
“Todd, I think you have Parkinson’s Disease,” he announced.
Molly mentioned Todd’s borderline score on the mini mental test, but the doctor said, “Nah, that’s close enough to normal.”
Todd started physical therapy to maintain his range of motion. It was a challenge when his mind was cloudy. Furthermore, a medication the neurologist prescribed for him for his Parkinson’s rigidity made his confusion worse.
His best friend from college was now a neurosurgeon. When he learned of Todd’s symptoms and diagnosis, he wasn’t satisfied. He sent the Grants to Dr. P., a neurologist friend of his.
During the 15 minutes Dr. P. spent with Todd and Molly, he took four phone calls. When it looked as though he was going to dismiss them without a diagnosis, Todd blurted out, “What’s the diagnosis?”
“You have dementia with Lewy bodies.” Pause. “And Parkinson’s.” And he was gone.
It was a shock to Molly and Todd that he had two diseases. Furthermore, they had never heard of dementia with Lewy bodies. A bit shaken, before they left for home, they called his sister Peggy to share the news. By the time they got home, Peggy had googled Lewy bodies and emailed them several links, most importantly that of the Lewy Body Dementia Association.
Lewy body dementia is confusing in several ways. It’s an umbrella term for two closely related diseases: dementia with Lewy bodies (DLB) and Parkinson’s disease dementia (PDD). Both combine the motor symptoms of Parkinson’s disease (PD) and the cognitive symptoms of dementia.
Like Parkinson’s itself, both are caused when a normal protein in the brain—alpha-synuclein–begins to form clumps inside brain cells. Called Lewy bodies, the clumps eventually cause brain cells to die and the brain to shrink.
DLB and PDD cause slightly different symptoms but a big difference in timing.
PDD’s cognitive symptoms show up only after the person has had PD motor symptoms—tremor, shuffling gait, slow movements, rigidity—for at least a year, often not until the person is in late PD. Dementia with Lewy bodies, on the other hand, generally starts with cognitive symptoms, and within a year the motor symptoms of Parkinson’s begin.
Therefore, it’s clear that Todd, whose cognitive problems came first and PD symptoms soon after, has DLB, not PDD, and certainly not both dementia with Lewy bodies and Parkinson’s. For DLB patients, Parkinson’s symptoms are part of the disease.
Todd is not alone in being misdiagnosed at first. An autopsy study showed that health care providers miss DLB in one out of three cases.
Yet after Alzheimer’s disease, DLB is the most common form of neurodegenerative dementia. It’s been called the most common disease no one has heard of.
An estimated 6.7 million Americans have Alzheimer’s disease, and another million have Parkinson’s. Both are well known. Though about 1.4 million people have dementia with Lewy bodies, it’s almost unknown.
Why?
DLB was first documented in autopsy in 1976, but it wasn’t until 1996 that experts reached a consensus on how to diagnose it. Medical schools began to teach about it some years later. That’s why many physicians in practice today don’t know of it.
But even for those who have heard of DLB, it’s challenging to diagnose. In part 2, I’ll describe the core symptoms necessary for a diagnosis, and other supportive symptoms that help lock it in, as well as symptoms that can be misleading.
I’ll also describe how to find a physician who is knowledgeable about dementia with Lewy bodies. It’s important to diagnose DLB early and I’ll explain why.
Maggie Sullivan has come to know Alzheimer’s intimately. She was caregiver and advocate during the eight years her mother lived with the disease. For the past 30 years, she has facilitated caregiver support groups for the Alzheimer’s Association, learning from the experience of more than 300 members of those groups. The opinions she expresses here are her own. Maggie is also a writer whose essays and articles have appeared in the New York Times and elsewhere.
