July 11, 2007
Research Update — from ALSA’s National Office
Second International FTD and ALS Conference: Challenges and Progress
Roberta Friedman, Ph.D., Research Department Information Coordinator
The second international conference on frontotemporal dementia (FTD) and ALS convened in London, Ontario, Canada, June 10 through 13. Co-sponsored by The ALS Association in collaboration with the ALS Society of Canada, the meeting allowed clinicians and researchers involved in aspects of these two conditions to learn from each other and from invited outside experts. As ALS patients can develop cognitive and behavioral changes that resemble aspects of FTD, the participants sought to work toward consensus on how best to diagnose and treat the overlapping disorders and how to leverage what is being learned about both into effective therapy.
ALS, a motor neuron disease, is named for the obvious death of the motor neurons that reach from brain to spinal cord and cord to muscle. But areas in the brain, other than those directing movement, can be affected to some degree as well. As investigators learn more about these changes, they will uncover new potential therapies for ALS. These potential therapies were the focus of discussion at the meeting. A working group will now build upon and move recent findings into strategies that will aid patients and their families.
At the conference, ALS clinicians and the neuropsychologists who carry out cognitive and behavioral assessments of patients discussed the tests that are likely to prove most useful for diagnosis and prediction. Pathologists who see the tissue damage in the disease contributed their perspective on the key features and emerging commonalities. Experts in Alzheimer’s disease shared their relevant experiences.
About a third of ALS patients will have mild cognitive impairment. About 20 percent have moderate to severe cognitive impairment with severe meeting the criteria for FTD. No one knows if every patient might become impaired if they live long enough.
Presenters described the range of cognitive and behavioral changes in FTD, which can include obsessive rituals, hoarding, and behavioral rigidity, as well as socially incorrect behavior. Atrophy or shrinking of the frontal and temporal areas of brain can precede or coincide with cognitive change in ALS. Yet frontal atrophy to a certain extent is a normal sign of aging.
Presenters seemed to agree that the hippocampus, a memory center, is a site of early change in ALS with cognitive involvement—and even without it—but that the change is subtle, unlike in Alzheimer’s.
Tiffany Chow, M.D., of the University of Toronto said it is important to have a caregiver available to question as lack of insight is another hallmark of FTD. Cognitive tests must accommodate for the motor and speech impairment of ALS. Morris Freedman, M.D., University of Toronto, developed a test of frontal lobe function based on discerning patterns. People with frontal lobe damage cannot figure out the pattern. Chow suggested other quick tests for FTD changes such as reciprocal tapping as well as hiding a coin and changing the pattern of which hand has it.
Jennifer Murphy, Ph.D., with colleagues at the University of California, San Francisco, has developed a 20 minute test that can predict the outcome of more extensive neuropsychological testing. Murphy pointed out why it matters to determine a patient’s cognitive status: more patients with cognitive impairment refuse choices of breathing and feeding aids and are more likely to have falls and choking episodes. Patients are assumed to be participating in decisions as competent individuals when their judgment may in fact be impaired.
Treatment Options
Richard Caselli, M.D., of Mayo Clinic, Scottsdale, Ariz., emphasized that any treatment of FTD is off label use. There is no clear evidence that the drugs marketed for Alzheimer’s disease can help in FTD. He endorsed simple but critical measures such as knowing when to limit or end driving and the removal of weapons from the household.
Consensus needs to be achieved in which cognitive tests and which pathology hallmarks can best help diagnose the changes in behavior and mental processes that can accompany ALS. Through the fruitful discussions that took place around the formal presentations at this meeting, tools will soon be developed that will aid clinicians and guide caregivers in how to meet the challenges posed by this complex disorder.
More details can be found here. A consensus paper will be published.
