LITTLE ROCK — The announcement by Sen. Pete Domenici, R-N.M., that he has a progressive brain disease and will not seek re-election has left unanswered questions about how the illness is affecting him and whether it could interfere with his plans to remain in the Senate until his term expires in January 2009.
Domenici, who is 75 and in his sixth term, disclosed on Thursday that he had frontotemporal lobar degeneration, a term that describes a family of diseases that damage parts of the brain that control language, behavior, planning, organizing, decision-making and movement.
Patients can have speech or behavioral problems, or both, and the symptoms can vary from subtle to flagrant. There is no treatment or cure. The cause is not known, but in about 40 percent of cases there is a family history of it.
The disorder is also called frontotemporal dementia, and sometimes Pick's disease, for the doctor who first identified a form of it in 1892. Frontotemporal disease causes a different pattern of brain damage than Alzheimer's and does not start with the kind of memory loss that usually signals the onset of Alzheimer's. It is also less common, affecting 100,000 to 200,000 Americans, compared with the 4.5 million afflicted with Alzheimer's.
Domenici said he decided not to run for a seventh term after a medical examination last month found that his disease had progressed.
A spokesman, Courtney Sanders, said: "He's had the condition, been aware of it, for some time. I can't say precisely how long." Sanders declined to explain what led to the diagnosis, what symptoms the senator had or what indicated that the disease had progressed.
"We on staff who work here with him every day haven't found any sort of effect that's interfered with his ability to do his job and do it well," she said.
Sanders said Domenici had regular examinations by a doctor at Johns Hopkins and a checkup in April detected no signs of progression. At that point, he was planning to run for re-election, but he changed his mind after the examination in September.
Dr. Bruce Miller, a neurologist at the University of California, San Francisco, and a leading expert on frontotemporal dementia, called it "a very tough disease" and said it could progress rapidly, twice as fast as Alzheimer's.
"In a study we did, the average person, from the time of diagnosis, 1 lived only about 3/2 years," Miller said. He said many patients died from problems like those in Parkinson's disease that impair swallowing.
Miller is not involved in Domenici's care and said he did not know the details of his case. Butasked if it was reasonable for someone with frontotemporaldementia to continue serving in the Senate until 2009, Miller said, "It would be worrisome, I would say. Not knowing any of the specifics, and given the possibility that this is incrediblymild, it might be OK. I would say that generally, most of our patients don't work."
Miller also said he was surprised by the case, because at 75, Domenici is well beyond the average age at which the disease is usually diagnosed.
Typically, the disease is diagnosed after a patient consults a doctor about speech problems or because relatives have noticed changes in behavior. It is sometimes mistaken, at least initially, for a psychiatric disorder. Mental tests can help identify it, and magnetic resonance imaging scans can detect a characteristic pattern of brain shrinkage. Both tests can monitor the progression of the disease.
Another neurologist who specializes in dementia, Dr. Norman Relkin of New York Presbyterian Hospital/Weill Cornell Medical Center, said, "In some cases I've followed patients for many years and they have been able to maintain a relatively high level of function in positions of responsibility."
But as the disease progresses, it impairs insight and judgment, something patients themselves cannot recognize, Relkin said. "Usually those around them become aware of it," he said.
Patients who continue working may need other people to let them know if they have done something irrational, irresponsible or out of character, Relkin said. And they need regular medical visits to monitor the progression of the disease.
Dr. Jason Karlawish, an associate professor of medicine and medical ethics at the University of Pennsylvania, said: "This a case study for something that will be an issue more and more as people don't necessarily retire at 65. Just because he has a diagnosis doesn't mean he's not capable of doing his job."
Front Section, Pages 5 on 10/07/2007