Many Americans are hearing the term “Lewy Body Dementia” for the first time after the widow of late comedy legend Robin Williams revealed to ABC News that her husband was suffering from the disease at the time of his death. But for the nearly 1.4 million Americans with Lewy body dementia and their loved ones, the devastating toll the illness can take on the mind, body and spirit are all too familiar.
Despite the prevalence of the illness, it remains widely misunderstood, misdiagnosed or unknown by the general public and healthcare professionals. As a national discussion emerges around the disease following news of Williams’s diagnosis, advocates for Lewy body dementia (LBD) sufferers hope more awareness will lead to earlier diagnosis and more effective treatment.
Lewy body dementia is the second most common type of degenerative dementia after Alzheimer’s disease. LBD is a term used to describe any type of dementia where Lewy bodies – or abnormal deposits of a protein known as alpha-synuclein – are present in the brain. It is a progressive dementia for which there is no known cure.
For Ariadne Weaver, the first signs that something was amiss with her husband came years before he was diagnosed with LBD.
“He was always a little spacey, but he started getting even more spacey – he started making decisions that he couldn’t explain to me,” she recalls. During one incident, he was convinced someone had stolen his car and couldn’t manage to find it after over an hour of searching, even though it was parked just a block away.
Nearly ten years later, Weaver says one of the toughest symptoms to deal with has been what she calls the “rollercoaster effect.” At times her husband has been very lucid, at others, practically comatose.
“Through the whole disease process I’ve never known who he’s going to be when he wakes up – its just a hallmark of the disease,” she says.
LBD sufferers experience symptoms that are common both in Alzheimer’s and Parkinson’s disease. Consequently, many patients with LBD are initially misdiagnosed as having Parkinson’s or Alzheimer’s. Symptoms of LBD that are also found in Alzheimer’s sufferers include:
- Progressive memory loss
- Visual hallucinations
- Fluctuations in cognition
- Changes in attention, alertness and mental clarity
- Impaired problem-solving skills
LBD sufferers may experience symptoms that resemble Parkinson’s, such as:
- Slowness of movement
- A shuffling gait
- Stooped posture
- Frequent falls
- An expressionless face
- Autonomic nervous symptoms including blood pressure fluctuations, excessive sweating, urinary incontinence or sexual dysfunction
“A patient with LBD might present with different combinations [of symptoms], so [the disease] doesn’t walk and talk alike,” says Angela Taylor, Director of Programs at the Lewy Body Dementia Association.
There are also a number of early warning signs of LBD that are not common to Alzheimer’s or Parkinson’s. People with the illness often have one or more sleep disorders, according to the LBDA. One of the more common sleep disorders suffered by LBD patients is REM sleep disorder behavior, in which people physically act out their dreams in their sleep. This can cause injuries to the patient and the person they share a bed with, and can occur years or even decades before the onset of dementia, Taylor says.
The LBDA provides a symptoms checklist that caregivers or patients can fill out and bring with them to doctors appointments.
Because LBD often mimics other diseases and since every patient’s mixture of symptoms is different, it can be tough to diagnose. Another factor that can result in misdiagnosis is the general lack of awareness of the disease, Taylor noted.
“There hasn’t been enough awareness raised about this disease, both in the general public and in the health care profession, so that has an impact on diagnosis -- primary care physicians may realize it’s dementia but not realize what type,” she says.
Misdiagnosis can not only delay proper treatment but can also lead to the prescription of medications used to treat Alzheimer’s or Parkinson’s that can prompt a negative reaction in LBD patients. Traditional anti-psychotic drugs are often prescribed to Alzheimer’s patients, for example, to treat hallucinations and delusions. But for unknown reasons, the use of anti-psychotic drugs by LBD sufferers can actually worsen symptoms and in some cases, can even be life-threatening, according to the LBDA.
Taylor notes, however, that the use of newer anti-psychotic drugs to treat LBD is sometimes recommended if a patient’s hallucinations and delusions present a danger to the patient or to others.
Awareness is Crucial
Greater awareness of LBD among the general public and medical professionals is expected to result in more early diagnoses and boost a patient’s chances of getting the proper treatment. In Robin Williams’s case, the late actor began experiencing symptoms in 2013 but was only diagnosed with dementia a few months before he took his own life in Aug. 2014, his widow Susan Williams told ABC News. Williams also suffered from depression and anxiety, both of which are among the psychiatric symptoms commonly experienced by those with LBD, according to the University of California, San Francisco.
Another beloved American actor and “Golden Girl”, Estelle Getty first announced she had Parkinson's and osteoporosis, and was later diagnosed with Alzheimer's. However, doctors later discovered that she, too, had LBD.
Whenever someone presents with what appears to be an abnormal case of dementia or Parkinson’s, they should be referred to a specialist, Taylor says. While there is currently no diagnostic tool that can differentiate between Parkinson’s and LBD, looking at a wider array of both physical and cognitive symptoms can lead to correct diagnosis and the right treatment early on. “Knowledge is power -- the more we understand what is going on around us, the more we can prepare for it, put it in context and cope with it, but when we don’t know what’s going on, it can be devastating,” she says.
Once an accurate diagnosis has been made, getting the best treatment means care from medical professionals with different specialties in order to treat the range of cognitive and physical symptoms associated with LBD. The patient’s doctors can help them and their loved ones determine which course of treatment is best.
In addition to medications used to treat cognitive symptoms, movement symptoms, and hallucinations, other treatments can help LDB sufferers cope with the sleep disorders and depression or anxiety that manifest as a result of the disease.
Support and Resources
The LBDA strongly recommends that families of LBD sufferers get involved in the LBD community in some way and educate themselves about the disease as much as possible.
One way family caregivers of LBD sufferers can do this is by joining a support group where they can exchange information and tips, reduce feelings of isolation and take some much-needed time for themselves.
Robin Riddle, CEO of non-profit Brain Support Network and the leader of an LBD support group in San Mateo, Calif., says joining these types of groups can be critical for LBD caregivers. She says that’s especially the case with LBD because many of the symptoms are embarrassing; from urinary incontinence to REM sleep disorder behavior and paranoid delusions.
“Most of the symptoms [of LBD] are things you don’t want to discuss with adult children, your loved one’s friends or his or her siblings,” Riddle says. “Really the only place you can speak openly is at a caregiver support group meeting for those caring with Lewy Body Dementia specifically.”
Long-term Care Options
As the disease progresses and symptoms worsen, some families opt for home health care or memory care.
“The types of things that increase the likelihood of [LBD patients] being admitted to long-term care are worsening Parkinsonism and also behavioral changes related to delusions or hallucinations,” Taylor says.
Whether or not a family decides to seek long-term care for their loved one suffering from LBD will also depend on the family’s existing care resources and on the patient’s personality, among other factors.
After years of caring for her husband at home and coaxing from her fellow support group members, Ariadne Weaver ultimately moved her husband into a skilled nursing home not far from their Santa Ynez Valley home. He remains there today.
Though she initially agonized over the decision, Weaver says it was the best thing she could do for her husband and for their marriage.
“I was one person working 24 hours a day, and [the nursing home] has nurses, and CNAs, and a dietitian, people cleaning and cooking, people showering him, an activities staff. I was trying to be all those people at once, and I just couldn’t be – now he’s taken care of 30 times better than I could have at home,” she says. “And when I go to see him, it’s like a date.”
Weaver says coping emotionally with her husband’s illness is still difficult and she worries about him all the time, but now she feels secure in the knowledge that he’s getting quality care.
For LBD sufferers who are in the last stages of the illness and their loved ones, hospice care can provide much-needed physical, emotional and spiritual support.
Lewy body dementia has long been misunderstood and remains challenging to diagnose, but experts say increased understanding of the disease can help change this. When more people recognize the signs, symptoms and pitfalls of LBD, doctors are more likely to correctly diagnose sufferers and patients and their families are likelier to get the right treatment and support.