Robin Williams is my all-time favourite comedian. Williams, who died in August 2014, had the ability to render me breathless with laughter within minutes of appearing on television. A superb mimic, he was also a fine serious actor, as seen in films such as Dead Poet's Society. For me, his virtuoso performance was as Airman First Class Adrian Cronauer in Good Morning Vietnam. Even now, on hearing the film's theme tune I Got You (I feel Good) by James Brown, I am transported back to the scene of him reading his hilarious version of a heavily censored news broadcast to troops serving in Vietnam.
The comedian's wife, Susan Schneider, recently announced that as part of Williams's autopsy the coroner found signs of Lewy body dementia (LBD); she added that doctors who analysed the report told her it was one of the most severe cases of the disease they had seen.
Williams took his own life, but his wife told ABC News: “It’s [LBD] that took his life and that’s what I spent the last year trying to get to the bottom of – what took my husband’s life.”
Difficult diagnosis
Schneider did a series of media interviews this month to raise awareness of the relatively unknown form of dementia. Although the third most common cause of dementia after Alzheimer’s and vascular (multi-infarct) dementia, LBD is a difficult diagnosis.
It’s not unusual for it to be diagnosed initially as Parkinson’s disease (which is primarily a movement disorder) or Alzheimer’s because of how similar the symptoms can be. And it is very much a clinical diagnosis, with absolute proof available only after the person has died and the Lewy bodies are seen at postmortem.
LBD was first discovered in the early 1900s by German neurologist Freiderich H Lewy. He was researching Parkinson’s disease when he came across small deposits of a protein called alpha-synuclein in brain cells of people with the condition.
Later named Lewy bodies, the deposits are found in both LBD and Parkinson’s; they are located throughout the cerebral cortex in patients with LBD, while in those with Parkinson’s disease the protein deposits are primarily found in the substantia nigra region.
Alpha-synuclein accumulates at the end of brain cells where they connect with each other using synapses (the junctions that transmit signals between cells).
How might you suspect someone has LBD? Not only do they experience the memory and judgment problems seen in Alzheimer’s but they are also likely to have difficulties with concentration and visual perception. This manifests itself as difficulty recognising objects and making judgments about where they are in space.
Significantly Robin Williams’s wife said he had started experiencing visual problems, describing one accident when her husband, standing over the sink with a bloody wound on his head, told her he’d “miscalculated” where the door was.
Other symptoms that place LBD in the diagnostic frame are: slowed movement and tremors; recurrent visual hallucinations; sleep disturbance, especially daytime sleepiness; and fainting, unsteadiness and falls. Extreme swings between alertness and drowsiness, with a pattern that changes day to day and even hourly also occur.
There is no single test that can diagnose LBD. It’s a clinical diagnosis based on listening carefully to the patient and a comprehensive physical examination. Any tests that are carried out are for exclusion purposes. MRI and CT scans will help exclude a brain tumour and may show signs of Alzheimer’s, while blood tests looking for a vitamin deficiency which can lead to poor brain function will also be carried out.
With no cure for LBD, treatment centres on managing the variety of symptoms. But even with good clinical care it can be a lonely place. Sharing her understanding of what drove her husband to suicide, Susan Schneider said: “Robin was very aware that he was losing his mind and there was nothing he could do about it.” mhouston@irishtimes.com muirishouston.com