ALTERNATE NAMES

Autosomal Dominant Diffuse Lewy Body Disease; Cortical Lewy Body Dementia; Dementia with Lewy Bodies; Diffuse Lewy Body Disease; Lewy Body Disease; Lewy Body Variant of Alzheimer Disease; Parkinson Disease with Dementia; Senile Dementia of Lewy Type

DESCRIPTION

Lewy Body Dementia (LBD) is a type of progressive dementia. It is the second leading cause of degenerative dementia in the elderly after Alzheimer disease. LBD causes physiological changes in the brain.

Symptoms include cognitive impairments and abnormal behavioral change. The physiological changes in the brain include a build-up of an abnormal protein in the brain called Lewy bodies. These proteins cause brain cell loss and atrophy, resulting in disruption of perception, thinking, and behavior. The clinical manifestations of LBD are characterized by cognitive impairment and dementia with pronounced fluctuations in alertness and attention; periods spent staring absently; disorganized speech; recurrent visual hallucinations; rigidity and loss of spontaneous movement; and depression.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM CODING

Diagnostic testing: The similarities between LBD, Parkinson disease, and Alzheimer disease can pose a significant challenge when making a definitive diagnosis. People with LBD have the diffuse presence of Lewy bodies in both the sub-cortical and cortical areas of the brain. People with LBD also have more severe dopamine and acetylcholine loss as compared to people with Alzheimer disease.

Diagnostic criteria include:

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  Central features (progressive dementia);

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  Core features (fluctuating cognition, recurrent visual hallucinations, and parkinsonism);

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  Suggestive features (rapid eye movement sleep behavior disorder, increased sensitivity to neuroleptics, and low dopamine transporter uptake in the brain’s basal ganglia); and

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  Supportive features (repeated falls, transient loss of consciousness, hallucinations in other modalities, visuospacial abnormalities, and autonomic dysfunction).

Physical findings: Physical symptoms may include:

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  Muscle rigidity or stiffness;

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  Shuffling walk, slow movement, or frozen stance;

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  Tremor or shaking, most commonly at rest;

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  Balance problems and repeated falls;

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  Stooped posture;

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  Loss of coordination;

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  Smaller handwriting than was usual for the person;

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  Reduced facial expressiveness;

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  Difficulty swallowing; and

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  Weak voice.

ICD-9: 331.82

ICD-10: G31.83

PROGRESSION

Individuals with LBD experience progressive cognitive, intellectual, and physical decline until death. Death usually results from pneumonia, malnutrition, or general body wasting.

TREATMENT

There is currently no cure for LBD. Treatment is symptomatic, and may include:

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  Levodopa-carbidopa combinations to treat movement disorders;

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  Acetycholinesterase inhibitors such as donepezil and rivastigmine to slow progression of cognitive impairments;

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  Clonazepam to manage REM sleep behavior disorder;

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  Physical therapy for cardiovascular, strengthening, and flexibility as well as for gait training;

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  Occupational therapy to maintain skills and promote function and independence;

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  Speech therapy to address low voice volume, poor enunciation, muscular strength and swallowing difficulties.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation:

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  Clinical information from the individual’s medical source(s) documenting a progressive dementia is critical and required for disability evaluation of LBD.

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  Activities of daily living report or a similar report completed by a relative or caregiver is used to document progressive loss of functional ability.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets

11.17

12.02

Equals

11.04

11.06