Lewy Body Disease

Lewy Body disease is the second most common progressive dementia, after Alzheimer’s disease. Its unique characteristic is that patients with this disease develop movement and memory disorder in the span of two years. The movement disorder may include stiffness, tremor, slowness in gait, small steps, and imbalance. The memory and thinking problems can be similar to those seen in Alzheimer’s, which includes short term memory problems initially, progressing to problem solving, visuospatial problems, as well as language problems and difficulty performing multistep activities.

Cause

Though the underlying cause of this disease is not known, a protein called Alpha Synuclein accumulates in the brain, showing up in the form of Lewy Bodies, which is also seen in Parkinson’s disease. Other abnormal protein referred to as Plaques and Tangles related to Amyloid and tau proteins seen in Alzheimer’s disease. 

Signs and Symptoms

Unique features of Lewy body disease include hallucinations (seeing or hearing things that are not there) which is more common early in this disease process.Other symptoms often seen in Lewy Body disease include:

-Difficulty controlling certain involuntary body functions such as blood pressure, pulse and sweating.

-A unique sleep disorder called Rapid Eye Movement (REM) sleep behavior disorder, which can cause the patient to physically act out the dreams while he/she is asleep.

-Difficulty with attention, which manifest in staring into space for long periods, and excessive drowsiness.

-Depression  is very common in Lewy Body disease. 

Risk Factors

 So far, all we know is that being older than age 60, being male, and having another family member with Lewy Body disease puts one at a higher risk of developing the disease. 

Diagnosis

 The diagnosis is usually made through a complete evaluation, neurologic exam, and neuropsychological exam. Often blood tests such as B12 levels, and thyroid function are done to rule out other possible causes or exacerbating factors, and imaging such as MRI or PET scan can give physicians more information about the disease. Some doctors may order a sleep evaluation test to identify REM sleep behavior disorder. 

Treatment

 There is no cure for Lewy Body disease and we can only treat the symptoms associated with the disease. Usually, the treatment is directed to reduce the symptoms.

The Parkinsonian symptoms of stiffness, difficulty with balance and walking, and tremor are often treated with the usual Parkinson’s disease medications, such as Carbidopa-levodopa (Sinemet). These medications are less effective for these symptoms in Lewy Body disease patients than Parkinson’s patients. Sometimes it can sometimes cause greater confusion and hallucinations.

The symptoms related to cognitive decline and dementia are usually treated with Cholinesterase inhibitors, such as Donepezil (Aricept) or Rivastigmine (Exelon), which increase the amount of an essential neurotransmitter in the brain: Acetylcholine.

The hallucinations and delusions can sometimes be treated by antipsychotic medications such as Quetiapine (Seroquel), Olanzapine (Zyprexa), and others. But great caution should be exercised to avoid significant side effects such as severe and irreversible Parkinsonian symptoms. On rare occasions, these medication have been associated with greater mortality and morbidity in the patients.

Other ways of treating the behavioral issues include redirecting the patient’s behavior, adjusting the environment to be more calming and less anxiety provoking, and occupy the patient with activities that do not cause anxiety. Increasing familial interactions, exercise, and maintaing regular routines help immensely. 

 

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