ALTERNATE NAMES

AD; EOAD; FAD; Familial AD; Presenile Dementia; Presenile Alzheimer’s disease; Young-onset Alzheimer’s disease

DESCRIPTION

Early-onset Alzheimer’s Disease (EOAD) is the diagnosis of EOAD for a person younger than age 65, and accounts for approximately 5 to 10 percent of all cases of Alzheimer's Disease (AD). EOAD is a degenerative, irreversible brain disease that usually affects older people and causes a dementia characterized by the gradual loss of cognitive abilities, such as memory, language, judgment, and the ability to function.

Physiological changes in the brain include the rampant growth of two abnormal structures, amyloid plaques and neurofibrillary tangles, which interrupt normal brain activity.

The onset of EOAD is subtle; memory impairment is frequently its earliest manifestation, quickly followed by learning and language impairments. Because people with EOAD are often in the work force, it is not uncommon for the disease to first manifest as a decline or loss in their ability to perform work-related activities. In the earlier stages of EOAD, depression is a common complaint. In later stages, agitation, changes in personality and behavior, restlessness, and withdrawal become evident.

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

Diagnostic testing: There is no specific clinical or laboratory diagnostic test for AD. Diagnosis can only be confirmed by brain biopsy or postmortem examination of the brain. The diagnosis of EOAD is based on the combination of clinical and family history; neurological, cognitive, or neuropsychological examination; and neuroimaging.

Pertinent clinical information includes history of onset and description of cognitive and functional impairments at home and at work. A decline in Mini-Mental Status Examination (MMSE) scores over time is a likely indicator of possible dementia. Neuroimaging, such as computerized tomography (CT) or magnetic resonance imaging (MRI) is useful for observing changes in the brain and excluding other causes of dementia.

ICD-9: 290.0, 290.1, 290.10, 290.11, 290.12, 290.13, 294.1 and 331.0

ICD-10: G30.0, G30.8, G30.9

PROGRESSION

People diagnosed with EOAD experience gradual cognitive decline until death. Death usually results from pneumonia, malnutrition, or general body wasting. The average time of survival after diagnosis of EOAD varies but generally is 8 to 10 years, and many people with EOAD require institutionalization.

TREATMENT

There is no treatment to cure or slow the progression of EOAD. Treatment for the symptoms of EOAD may include drugs such as cholinesterase inhibitors (galantamine, rivastigmine, or donepezil) and an N-methyl D-aspartate (NMDA) antagonist (memantine).

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation:

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  Clinical information documenting a progressive dementia is critical and required for disability evaluation of EOAD. The preferable source of this information is the clinical records from the claimant’s medical source(s).

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  Activities of daily living report or a similar report completed by relative or caregiver.

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  Documentation of dementia by standardized testing such as the Clinical Dementia Rating (CDR) scale with a score of = 1, MMSE with a score of = 24, or equivalent test is helpful but not required.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets

11.17

12.02

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