Actor Sir Patrick Stewart, known worldwide for his roles in “Star Trek” and the “X-Men” films, died Wednesday at his home in Los Angeles. He was 86. His family said he had been battling Alzheimer’s disease and Lewy body dementia.
A statement posted Thursday to Stewart’s Instagram account described his final days: “He has been held in the warm embrace of family and friends, surrounded by live music, prayer, tears, laughter, and the kind of profound emotion that only he could inspire after a lifetime of devotion to us all. We will forever cherish the peaceful ease and reflection of his invincible grace.”
Stewart’s death has drawn new attention to Lewy body dementia, a condition that affects roughly 10% to 15% of the more than 7 million Americans living with dementia. Alzheimer’s disease, by contrast, accounts for about 60% to 70% of cases.
What is Lewy body dementia?
Lewy body dementia, or LBD, is a progressive brain disorder caused by abnormal clumps of a protein called alpha-synuclein, known as Lewy bodies, which build up inside neurons. The clumps take their name from Dr. Friedrich Lewy, the German-born physician who first identified them in 1912, according to the National Institute on Aging.
LBD is distinct from Alzheimer’s, which is primarily driven by a buildup of beta-amyloid plaques and tau tangles rather than Lewy bodies.
“LBD has many core clinical features that distinguish this form of dementia from Alzheimer’s disease,” said Dr. Jonathan Fellows of the Michigan Institute for Neurological Disorders. “Patients can experience fluctuations in their cognition, alertness and attention, which can vary from day to day or even from hour to hour.”
Another key difference: Alzheimer’s patients typically struggle to form new memories, while those with LBD can form new memories but have trouble retrieving them.
Symptoms and warning signs
LBD does not progress through the same formally defined stages as Alzheimer’s, but it carries a set of recognizable core symptoms. One is known as the “Lewy lean.”
“The ‘Lewy lean’ is a term used to describe gait and balance problems associated with LBD,” Fellows said. “Patients can have several different variations, including a persistent lean to one side when sitting, standing or walking; difficulty standing upright; difficulty walking straight; and increased falls associated with loss of balance.”
Fellows said patients can also experience detailed visual hallucinations and sleep disturbances that often surface early in the disease.
“A common early feature of LBD includes REM sleep behavior disorder, where patients have vivid dreams and act out their dreams with yelling, kicking or punching during sleep,” he said. “This sleep disorder often precedes the cognitive symptoms by many years.”
Causes and treatment
Researchers have not identified a single cause of LBD, though a combination of genetic, environmental and lifestyle factors is believed to play a role. The disease is not generally considered hereditary, though the American Brain Foundation notes certain genetic variants are linked to higher risk.
The Lewy body deposits interfere with normal brain function and are tied to the loss of neurons that produce acetylcholine, which supports memory and learning, and dopamine, which regulates movement, mood, sleep and motivation.
There is no cure for LBD, so treatment focuses on managing symptoms, often borrowing from therapies used for Alzheimer’s and Parkinson’s disease. Fellows said cholinesterase inhibitors such as rivastigmine or donepezil, though not FDA-approved specifically for LBD, can help with cognition, attention and hallucinations. Levodopa therapy, such as Sinemet, can address Parkinson’s-like movement symptoms. Atypical antipsychotics are sometimes used for hallucinations, delusions and agitation, but Fellows cautioned that traditional antipsychotics should be avoided because they can make LBD symptoms worse. Patients with REM sleep behavior disorder may be treated with melatonin or clonazepam.
Stewart joins a list of public figures who lived with the disease. An autopsy of Robin Williams found he had LBD before his death by suicide in 2014. Radio host Casey Kasem was diagnosed with LBD after initially being misdiagnosed with Parkinson’s disease. “Golden Girls” actress Estelle Getty, country singer Glen Campbell and Hall of Fame pitcher Tom Seaver also lived with the condition.
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