“Terrorist inside my husband’s brain.” That is how Susan Schneider Williams, wife of the late actor Robin Williams, described Lewy Body Dementia, which she says devastated her husband’s life in his final days. Like many people suffering from the disease, Williams’ was wrongly diagnosed. Only the postmortem after his suicide in 2014 revealed diffuse Lewy Body Dementia in almost his entire brain and brain stem.

Lewy Body Dementia is the second most common form of dementia, and accounts for about 20% of dementia cases around the world. In India, an estimated 3.7 million people suffer from dementia, of which approximately 5% or 185,000 people have Lewy Body Dementia, according to a report by the Alzheimer’s and Related Disorders Society of India.

Lewy bodies are deposits of the protein called alpha-synuclein inside brain cells. Alpha-synuclein is normally abundantly present in the brain, especially at the ends of neurons, in a soluble form. When the protein aggregates as insoluble fibrils in brain cells it gives rise to pathological conditions like Lewy Body Dementia and Parkinson’s. Lewy bodies are named after Dr Frierich Lewy, a German scientist, who first identified the protein deposits in the brain in 1912.

Psychiatric presentation

Not unlike Alzheimer’s disease, people with Lewy body disease experience problems with memory and judgement. People with Lewy’s disease could also have symptoms associated with Parkinson’s such as muscle stiffness and problems walking.

But, it is “decades behind Alzheimers or Parkinson’s disease as far as the scientific advances and understanding of the disease,” Angela Taylor told Time Magazine. Taylor is director of programs at the Lewy Body Dementia Association in the United States, a non-profit that works on creating awareness about the disease.

A few months before his death, Williams’ was diagnosed with Parkinson’s disease. Williams too, had a tremor in his left hand.

Like Alzheimer’s disease and Parkinson’s disease, Lewy Body Dementia is a neurodegenerative disorder that results in progressive intellectual and functional deterioration. There is no known cure to the illness, or to stem the progression of the disease.

What differentiates Lewy Body Dementia from the two other forms of dementia are the psychiatric presentations in the patients who experience extreme swings between alertness and confusion or drowsiness, which may happen unexpectedly and change from hour to hour or day to day.

What is more debilitating, are the hallucinations – seeing or hearing things that are not real.

Williams’ had a panic attack, when he was in the middle of filming Night at the Museum 3. He was recommended an antipsychotic medication, which makes symptoms worse for people with Lewy Body Dementia.

Williams’ case, his wife wrote, was extreme. “Not until the coroner’s report, 3 months after his death, would I learn that it was diffuse LBD that took him. All 4 of the doctors I met with afterwards and who had reviewed his records indicated his was one of the worst pathologies they had seen. He had about 40% loss of dopamine neurons and almost no neurons were free of Lewy bodies throughout the entire brain and brainstem.”

Diagnosis tough

If doctors missed Lewy Body Dementia in a highly paid and much loved Hollywood actors, it is an indication of how hard the disease is to diagnose. Like for most other diseases the challenge of diagnosis holds doubly true in India.

“Diagnosing dementia itself is a tough,” said Dr Sirish Hastak, a consultant neurologist from Kokilaben Dhirubai Ambani Hospital in Mumbai. “We see far fewer cases of Lewy Body Disease as compared to Parkinson’s disease.”

Dementia patients are often referred to psychiatrists who are not equipped to handle a pathological problem like Lewy Body Disease, said Dr Hastak. Once diagnosed, the treatments for the disease, which normally consists of medication with chemical inhibitors, are considered more effective as compared to Alzheimers’ and Parkinson’s disease.