'This disease is a sea monster with 50 tentacles of symptoms that show when they want'. Photo / Getty Images
After one of the most popular actors on the planet took his own life in August last year, millions of shocked fans were left asking questions. Robin Williams, then 63, had been seeking treatment for severe depression. But last week, his widow, Susan Schneider, revealed that her husband was suffering not only from Parkinson's disease that had been diagnosed three months earlier, but also from Lewy body dementia (LBD), a progressively brutal illness that affects memory, movement and motor skills, and causes severe psychiatric symptoms.
Schneider says depression didn't cause Williams's death: "Lewy body dementia killed Robin. That's what took his life."
Strikingly, LBD - sometimes referred to as dementia with Lewy bodies or Parkinson's with Lewy bodies, depending on symptoms - is the second-most common dementia after Alzheimer's and affects more than 127,000 Britons. Yet most of us have never heard of it. James Galvin, a neurology and psychiatry professor at Florida Atlantic University, says: "It's the most common disease you've never heard of."
"This disease is a sea monster with 50 tentacles of symptoms that show when they want," Schneider said. Williams suffered hallucinations, anxiety, depression, loss of motor control and problem-solving skills, sleep, balance and spatial awareness problems, and delusions.
When Paul Moynagh's wife, Imogen, couldn't find her way from a cafe table to the counter on a visit to a National Trust house in Devon in 2006, he thought little of it. Paul, 78, couldn't have predicted the confusing set of symptoms that Imogen, 74, would experience. LBD is often misdiagnosed as Parkinson's or Alzheimer's, and it took doctors almost seven years to confirm her illness.
"Looking back, it began with little signs - loss of spatial awareness is an early symptom... but they were so inconsistent," recalls Paul, a retired surgeon. First, there was a minor trembling in Imogen's hands, then severe sleepiness during the day, along with spasms that made her right foot turn in when she walked. Then she developed depression and suffered panic attacks.
"Imogen has a pragmatic personality," says Paul. "She used to play sports, was a keen gardener, walked everywhere and looked after our two children, Mark and Rachel. Ten years ago, if you had told her she would be afraid of being left alone, she would have laughed."
By 2010, Imogen's reasoning and planning skills were suffering - a key sign of LBD. A keen bridge player, Imogen recalls: "I stopped winning, so I knew something was wrong." (Though her speech is now slow, her sense of humour remains.)
Lewy bodies are protein deposits in the brain, explains Prof David Burn, consultant neurologist and director of the biomedical research unit in LBD at the UK National Institute of Health Research (NIHR). Discovered by Dr Frederic Lewy, a colleague of Dr Alzheimer's, in 1912, the deposits develop inside nerve cells (neurons) in the brain, interrupting messaging and causing neurons to die. A patient's symptoms will depend on which part of the brain is affected.
"When neurons die in the cortex, it causes dementia, but when it occurs in the brainstem, it causes motor symptoms (Parkinsonism)," says Prof Burn. "LBD patients face a rapid deterioration in their cognitive, physical and psychiatric function, and it tends to progress faster than other dementias," he says.
Schneider describes one incident just weeks before Williams's death, when she was in the shower and he was standing by the sink. "Something didn't seem right," she recalls, so Schneider got out of the shower to find her husband's head covered in blood. "He pointed to the door and I said, 'Did you hit your head?' and he nodded." The incident confused her at the time. "But now, finding out all about Lewy body disease, lo and behold, their vision is affected, as is the ability to recognise and identify objects," she says. "Now I get it."
Like Schneider, Paul Moynagh was also baffled by his wife's repeated falls in the years preceding her diagnosis. "She'd had nine different broken bones, breaking her wrist twice, her ankle, and once, when she'd fallen down some stairs, her elbow."
Imogen, like Williams and many LBD sufferers, was initially diagnosed with Parkinson's disease. "She began shuffling when she walked and her voice became weak, both symptoms of Parkinson's," says Paul. Meanwhile, her depression was getting worse, not least because Imogen was so aware of what was happening to her. "It's different from Alzheimer's in that people know exactly what's happening, and one day can be completely lucid and the next be experiencing terrible anxiety and delusions. The more Imogen is aware of her situation, the more she gets depressed."
June Brown, who plays Dot Cotton in EastEnders, lost her husband, actor Robert Arnold, to LBD in 2003. In a moving video made for the charity Lewy Body Society, Brown recalls: "Bob knew what was happening to him and he hated it. He once said: 'I never thought I would go like this.'?" Unlike Alzheimer's sufferers, LBD patients often have lucid memories. "Bob never lost his memory for people's names. It's the most strange disease because he would have moments of confusion and moments of clarity. It's worse than Alzheimer's because of this awareness of what you're going through."
Brown says Robert had paranoia and hallucinations - he was frightened by faces he would see in the windows of a summer house he had built at the bottom of the garden. "One evening we were watching the Baftas on television and the camera panned, settling on various stars, and Bob turned to me and said: 'I think Judi saw us.' He meant Dame Judi Dench. He thought we were there and became very distressed because he wasn't correctly dressed."
When Paul Moynagh's wife began experiencing hallucinations - a tell-tale sign of LBD - he knew that there was more to her illness than Parkinson's. "She would see people in the windows of the conservatory and in our floor - which we made look like natural stone - she saw figures speaking to her.
"In my desperation, I would spend hours Googling Imogen's symptoms until I stumbled on Lewy body dementia," he says. "I'm a doctor and I had never even heard of it, and the neurologist was reluctant to accept it, but Imogen ticked all the boxes." By 2013, a locum psychiatrist finally diagnosed Imogen with LBD. "I went along with my Google list, and she finally made the diagnosis. Two months later, the neurologist finally agreed."
Now, the only way to know that someone had Lewy body dementia is when a post-mortem examination finds Lewy bodies in the brain. According to LBD specialist Ian McKeith, professor of old age psychiatry at the Newcastle University Institute for Ageing, LBD often gets misdiagnosed because doctors don't know which questions to ask. He is in the middle of a study funded by the NIHR to develop a diagnostic toolkit for use in NHS practices.
Although there is no cure for LBD, doctors can treat symptoms using drugs that work on the brain's messaging system, says Prof McKeith. But correct diagnosis is essential. "If antipsychotic or anti-Parkinson's drugs are given to patients with LBD, they can be fatal," he says.
"We were living a nightmare," Susan Schneider said of Robin Williams's final months. Prof McKeith says one study found that when carers looking after someone with LBD were asked to rate their quality of life on a scale of zero to one (where zero was as bad as it could be), one in four rated it as below zero.
Still, Paul Moynagh refuses to refer to life with Imogen as a nightmare. She now needs 24-hour attention and help feeding. They recently celebrated their 50th wedding anniversary. During our interview, she turns to her husband and says slowly, with the difficulty she now has in getting words out: "Without your care, I don't know where I would be."
"Underneath it all, she is still the lovely person that I married," he says. "We still love each other as much as we did before - that hasn't changed. If anything, I love her more."
Where to get help:
• Lifeline: 0800 543 354 (available 24/7)
• Suicide Crisis Helpline: 0508 828 865 (0508 TAUTOKO) (available 24/7)
• Youth services: (06) 3555 906 (Palmerston North and Levin)
• Youthline: 0800 376 633
• Kidsline: 0800 543 754 (available 24/7)
• Whatsup: 0800 942 8787 (1pm to 11pm)
• Depression helpline: 0800 111 757 (available 24/7)
• Samaritans: 0800 726 666 (available 24/7)
If it is an emergency and you feel like you or someone else is at risk, call 111.
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