TN 6 (12-11)
DI 23022.610 Lewy Body Dementia
COMPASSIONATE ALLOWANCE INFORMATION
LEWY BODY DEMENTIA
Lewy Body Disease; Diffuse Lewy Body Disease; Dementia with Lewy Bodies; Autosomal Dominant Diffuse Lewy Body Disease; Cortical Lewy Body Dementia; Lewy Body Variant of Alzheimer Disease; Parkinson Disease with Dementia; Senile Dementia of Lewy Type
Lewy Body Dementia (LBD) is a type of progressive dementia that occurs in people older than 65 years of age. 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 into space; disorganized speech; recurrent visual hallucinations; rigidity and loss of spontaneous movement; and depression.
DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING
The similarity of symptoms among LBD, Parkinson disease, and Alzheimer disease, can often make it difficult for a doctor to make 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.
The diagnostic criteria for this disease is separated into 4 categories:
Central features (progressive dementia);
Core features (fluctuating cognition, recurrent visual hallucinations, and parkinsonism);
Suggestive features (rapid eye movement sleep behavior disorder, increased sensitivity to neuroleptics, and low dopamine transporter uptake in the brain’s basal ganglia); and
Supportive features (repeated falls, transient loss of consciousness, hallucinations in other modalities, visuospacial abnormalities, and autonomic dysfunction).
ONSET AND PROGRESSION
People with LBD experience progressive cognitive, intellectual, and physical decline until death. Death usually results from pneumonia, malnutrition, or general body wasting.
There is currently no cure for LBD. Treatment for the symptoms may include drugs such as levodopa-carbidopa combinations used to treat movement disorders and acetycholinesterase inhibitors, such as donepezil and rivastigmine for improving cognitive impairments. Clonazepam is prescribed to manage REM sleep behavior disorder. Physical therapy may be recommended for cardiovascular, strengthening, and flexibility exercises as well as for gait training. Occupational therapy is used to maintain skills and promote function and independence. Speech therapy is prescribed for low voice volume, poor enunciation, and to improve muscular strength and swallowing difficulties.
SUGGESTED PROGRAMMATIC ASSESSMENT*
Suggested MER for Evaluation:
Clinical information from the treating primary physician, neurologist, or psychiatrist documenting a progressive dementia is critical and required for disability evaluation of LBD.
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:
* Adjudicators may, at their discretion, use the Medical Evidence of Record or Listings suggested to evaluate the claim. However, the decision to allow or deny the claim rests with the adjudicator.