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Lewy body dementia: Most misdiagnosed and often misunderstood

lewy body dementia

Lewy body dementia made headlines when actor Robin Williams’ wife revealed that the beloved actor and comedian died from this dreaded disease.

At the time of his demise, his loved ones claimed that it was depression that made him take his own life. But few days later, his wife Susan Williams revealed that he was actually suffering from a rare form of dementia called Lewy Body Dementia (LBD) and had only three years to live.

She claimed that an autopsy revealed clumps of protein in Williams’ brain, which is a hallmark of LBD, also known as dementia with Lewy bodies (DLB). LBD likely contributed to anxiety and depression, which finally pushed him over the edge.

“Lewy body dementia killed Robin, it’s what took his life,” Susan Williams insisted.

Now, the question arises, ‘Why was the condition not spotted earlier?’

Although, LBD is the second most common type of dementia after Alzheimer’s, it is also the most misdiagnosed dementia.

The disease is frequently misdiagnosed as Alzheimer’s and according to the Lew Body Dementia Association (LBDA), it takes 3 doctors and more than a year-and-a-half to accurately diagnose LBD. Since the symptoms are very similar to Alzheimer’s and Parkinson’s disease, most doctors fail to recognize the signs of LBD.

Read more New Protein Target May Reverse Memory Loss In Alzheimer’s

What is LBD?

Named after Frederick H. Lewy, the German-born neurologist who discovered the disease in the early 1900s, Lewy body dementia is a progressive degenerative dementia primarily affecting older adults. It affects around 1.3 million Americans, mostly those aged 65 and older.

Lewy body dementia affects more than 100,000 people in the UK.

Dr. Lewy pinpointed small accumulations of a protein known as alpha-synuclein in brain cells of the patients with LBD. These protein were later renamed Lewy bodies.

These Lewy bodies are also found in people with Parkinson’s disease. For each condition, they are found in different parts of the brain. Lewy bodies for LBD are mainly found throughout the cerebral cortex of the brain, while the Lewy bodies for Parkinson’s are found in the substantia nigra region of the brain.

Scientists do not know exactly how Lewy bodies develop, but build-up of Lewy bodies causes lowered levels of neurotransmitters such as, acetylcholine and dopamine, brain tissue loss and nerve cell damage.

Like other forms of dementia, Lewy body dementia is a neurocognitive disease that progressively impairs memory, language ability, object recognition and motor skills, according to the Centers for Disease Control and Prevention (CDC).

lewy body dementia

Risk factors

Major risk factor for LBD is age. People over the age of 60 are at a greater risk. Other risk factors include being male and having a family member with LBD.

Causes of Lewy body dementia

The major cause of the disease is not yet fully understood, but a genetic link with Park 11 gene has been described. Researchers believe that the disease may be related to Alzheimer’s or Parkinson’s.

  • Lewy bodies are also present in the brains of patients with Parkinson’s, Alzheimer’s and other dementias.
  • Lewy body dementia patients also have the plaques and tangles found in people with Alzheimer’s disease.

Symptoms of LBD

LBD symptoms overlap with those of Parkinson’s disease. Many Parkinson’s disease patients may experience dementia related memory problems and cognitive skills, while many people with LBD will experience motor problems associated with Parkinson’s.

  • LBD often starts with sleep deprivation — vivid dreams that the patient will violently act out while lying in bed.
  • Hallucinations, delusions, recognition will follow, along with dizziness and balance problems. Just taking a few steps inside the room can be challenging.
  • The patient will slowly start to lose his or her mind. With shortened attention span and dissolved problem-solving skills, depression, paranoia and anxiety can attack an already fragile brain.
  • People with LBD may experience memory loss and other forms of cognitive impairment, similar to those seen in people with Alzheimer’s.
  • Patients may experience rigid muscles, tremors, and shuffling movements when walking. These signs get worse with the progression of the disease.

“They present themselves like a pinball machine,” Susan Williams told People magazine. “You don’t know exactly what you’re looking at.” This explains why it is so difficult to diagnose LBD.

Diagnosis of Lewy body dementia

lewy body dementia
Actress Estelle Getty died of Lewy Body Dementia in 2008. She was initially diagnosed as having Parkinson’s disease and Alzheimer’s disease.

There is no single test to determine LBD and diagnosis is limited to a doctor’s educated guess. A doctor may need to perform clinical evaluation to assess physical and neurological symptoms of the patient before making a professional judgment.

Since many doctors are not familiar with LBD, a patient may see several doctors before being positively diagnosed with the disease.

This is best to choose a neurologist for diagnosing LBD. A geriatric psychiatrist, neuropsychologist or a geriatrician may also have experience in this field.

For the diagnosis, a medical professional will typically need to conduct tests and neurological examination such as tests of eye movement, balance, reflex and sense of tough.

However, these tests do not accurately diagnose the disease. At present, a patients’ LBD diagnosis can only be done with certainty after his/her death by performing a brain autopsy.

Treatment

There is no cure for LBD, but treatments can be done for individual symptoms which are very challenging. The key to appropriate LBD management is earlier recognition.

Drugs

  • Cholinesterase inhibitors. These are drugs for treating Alzheimer’s disease. Rivastigmine (Exelon), increases the levels of chemical messengers that scientists believe are crucial for thought, memory, and judgment (neurotransmitters) in the brain.
  • Parkinson’s disease drugs. Drugs such as, carbidopa-levodopa (Sinemet) can aid in lowering Parkinson’s disease symptoms, such as slow movement and rigid muscles. These medications also have side-effects — they may also cause heightened hallucinations, confusion and delusions.
  • Antipsychotic medications. Drugs such as olanzapine (Zyprexa), quetiapine (Seroquel), and others, may improve hallucinations and delusions. However, some LBD patient’s may be dangerously sensitivity to some of these medicines. They may experience irreversible symptoms and confusion related to Parkinson’s.
  • Other medications. A healthcare professional may prescribe medications to treat symptoms related to LBD such as, sleep or motor problems.

Therapies

Some drugs may worsen Lewy body dementia. Therefore, some doctors may suggest non-drug therapies:

  • Environmental modification. Organizing the living area and noise-free environment can help someone with LBD to focus and function well.
  • Caregiver’s response. It is best to avoid correcting or interrogating a person with dementia.
  • Daily routines. Break tasks into easier steps and focus on successes, not failures. Following a routing can reduce confusion in people with dementia.

Home remedies

Each patient with LBD or other form of dementia will experience symptoms and progression differently. Therefore, care giving techniques for each person may vary:

  • Enhanced communication. Always maintain eye contact while talking to a patient with LBD. Speak slowly and softly with simple sentences. Do not hurry for response. Give only one instruction at a time. Use hand gestures such as pointing to objects.
  • Exercise helps everyone. Exercise may improve physical function, more controlled behavior and lessen depression symptoms in a patient with Lewy body dementia.
  • Brain games. Playing games or solving crossword puzzles or other activities that involve thinking skills may help reduce mental decline.
  • Sleep pattern. Behavior issues for LBD patients may worsen at night. Make sure the patient goes to bed peacefully and away from any noise. Leave night lights on, this will help prevent disorientation.
  • Caffeine and daytime naps. Limit caffeine intake during the day and discourage daytime napping. This will help prevent nighttime restlessness.
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Salahuddin Ahmed is a freelance medical writer and blogger, who has been writing about medicine and health for more than a decade. A former New York transplant, he now lives in his native Dhaka. He received a Bachelor's degree from the University of Louisiana and a diploma on eTechnology from NIIT, Dhaka. A voracious eater, Salahuddin only dines at restaurants that offer free refills on rice.

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