Fall 2009

Improving the Diagnosis of Frontotemporal Dementia

When patients in their 50s or 60s show a progressive decline in behavioral management or language skills without significant memory trouble, it's common for physicians to assume psychiatric problems or Alzheimer's disease and to prescribe antipsychotic medication. Bruce Miller, M.D., clinical director of the Memory and Aging Center at UCSF, believes there needs to be greater awareness that such symptoms may instead indicate frontotemporal dementia (FTD), which implies a different treatment regimen.

"We have data that suggest about 60 percent of cases we see with frontotemporal dementia have been diagnosed as something else and that antipsychotic medications can make patients progressively worse," says Miller.

He notes there are many reasons FTD is often missed in the diagnostic process. "Because they have not been trained to think about it, even the best radiologists can miss the frontally predominant atrophy typical of frontotemporal patients," Miller says. The UCSF Memory and Aging Center can take advantage of state-of-the-art imaging to identify that atrophy early.

In addition, the center, which provides a comprehensive array of services for individuals with cognitive problems and conducts extensive clinical research, offers genetic testing that helps clinicians diagnose FTD and helps families plan for and prevent future problems.

Optimal FTD Management

The center's multidisciplinary assessments — which include physicians, pharmacists, nurses and social workers — enable treatment plans that embrace the full range of factors that can help in managing the condition. Those treatment plans focus on three areas: medication, addressing environmental concerns and, where appropriate, clinical trials that test novel medications.

"With medication, we initially take a very conservative approach and rarely use antipsychotics," says Miller. "We rely more on therapies specific to the neurodegenerative condition, such as serotonin-boosting compounds and NMDA receptor antagonists."

To address environmental triggers, the center helps families identify and address pressing safety concerns, which relieves them of feeling they need to address all symptom-driven changes. Clinicians also help devise therapeutic options like exercise routines and sleep therapies that can help patients maintain their cognitive health.

Finally, the center enrolls patients in promising clinical trials for innovative therapies. The trials range from monoclonal antibodies to vaccines that can stimulate immune system response. "We are beginning to move toward tau-specific therapies in the hope that these therapies can dramatically slow down the degenerative process," says Miller.

When should you refer a patient to the UCSF Memory and Aging Center to be evaluated for frontotemporal dementia?

If a patient shows progressive deterioration of behavior or cognition by observation or history and exhibits at least three of the following symptoms:

  • Early behavioral disinhibition
  • Early apathy or inertia
  • Early loss of sympathy or empathy
  • Early preservative, stereotyped or compulsive, ritualistic behavior
  • Hyperorality and dietary changes
  • Executive or generation deficits with relative sparing of memory and visuospatial functions
Diagnosis is more likely if the patient presents with disproportionate atrophy in frontal or anterior temporal regions (via CT or MRI).

The UCSF Memory and Aging Center's Katya Rascovsky, Ph.D., coordinates the International bvFTD (behavioral variant of frontotemporal dementia) Criteria Consortium — an effort to revise diagnostic criteria for bvFTD. These referral guidelines have emerged from that effort.

For more information, contact Bruce Miller, M.D. at (415) 476–5591.

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