COMPASSIONATE ALLOWANCE INFORMATION

FRONTOTEMPORAL DEMENTIA (FTD), PICK'S DISEASE -Type A - Adult

DESCRIPTION

Frontotemporal Dementia (FTD) describes a clinical syndrome associated with shrinking of the frontal and temporal anterior lobes of the brain. Originally known as Pick's disease, the name and classification of FTD has been a topic of discussion for over a century. The current designation of the syndrome groups together Pick's disease, primary progressive aphasia, and semantic dementia as FTD. The presence of abnormalities in the nerve cells of the brain, called Pick bodies, distinguishes frontal lobe dementia from other types of dementia. As it is defined today, the symptoms of FTD fall into two clinical patterns that involve either (1) changes in behavior, or (2) problems with language. The first type features behavior that can be either impulsive (disinhibited) or bored and listless (apathetic). It also includes inappropriate social behavior; lack of social tact; lack of empathy; distractibility; loss of insight into the behaviors of oneself and others; an increased interest in sex; changes in food preferences; agitation or, conversely, blunted emotions; neglect of personal hygiene; repetitive or compulsive behavior, and decreased energy and motivation. The second type primarily features symptoms of language disturbance, including difficulty making or understanding speech, often in conjunction with the behavioral type's symptoms. Spatial skills and memory remain intact. There is a strong genetic component to the disease; FTD often runs in families.

ALTERNATE NAMES

Frontotemporal Lobar Degeneration, Dementia with Lobar Atrophy and Neuronal Cytoplasmic Inclusions, Diffuse Degenerative Cerebral Disease, Lobar Atrophy of the brain, Pick Disease of the brain-Type 1, Wilhelmsen-Lynch Disease

DIAGNOSTIC TESTING AND CODING

Diagnostic tests may include:

• physical exam, clinical assessment and blood tests,

• neurological exam that checks awareness and responsiveness, vital signs, reflexes, sensory responses and coordination,

• neuropsychological testing, which assesses memory, ability to reason and judge, problem-solving skills and language skills.
  Brain imagining, such as MRI and CT, may demonstrate shrinkage of the frontal and temporal lobes and also help exclude other causes of dementia such as strokes and brain tumors. PET and SPEC tomography testing may be used to evaluate brain activity.

TREATMENT

No treatment has been shown to slow the progression of FTD. Behavior modification may help control unacceptable or dangerous behaviors. Aggressive, agitated, or dangerous behaviors could require medication. Anti-depressants and tranquilizers have been shown to improve some symptoms.

PROGRESSION

The outcome for individuals with FTD is poor. The disease progresses steadily and often rapidly, ranging from less than 2 years in some individuals to more than 10 years in others. Eventually some individuals with FTD will need 24-hour care and monitoring at home or in an institutionalized care setting.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation: Brain imagining, such as MRI and CT, may demonstrate shrinkage of the frontal and temporal lobes and also help exclude other causes of dementia such as strokes and brain tumors. PET and SPEC tomography testing may be used to evaluate brain activity. Clinical evidence describing general physical and blood tests to rule out thyroid disease, vitamin B12 deficiency and syphilis may be considered. Consideration of family history is appropriate as there is often a strong family predisposition for FTD. Documentation of a clinically appropriate medical history, neurological findings consistent with the diagnosis of FTD, and the results of any electrophysiological and neuroimaging testing may be considered.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets Listing

12.02

11.17

Medical Equals

* 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.