Research Updates as of August 2005
Depression doesn't dominate in ALS
Only 15 (19 percent) of 80 people with late-stage ALS recently surveyed met standard criteria for a diagnosis of depression, say investigators at the Eleanor and Lou Gehrig MDA/ALS Research Center at Columbia University in New York.
Of those 80, 61 were interviewed at monthly intervals at least twice. In that group, 35 (57 percent) never met depression criteria, while five (8 percent) were consistently depressed. The other 21 people (34 percent) were depressed only at some of the monthly assessments.
The investigators, who published their results in two articles in the July 12 issue of Neurology, concluded that depression is the exception, rather than the rule, in late-stage ALS; and that, when it occurs, it’s often transitory.
They didn’t find that spiritual beliefs, the presence of a spouse as a caregiver, depression in the caregiver, financial status, or participation in a hospice program were an influence on depression status.
The researchers also examined factors that may influence a person with ALS’s decision to hasten death. Of the 80 people surveyed, 53 died during the study period. Of these, 23 (43 percent) had said they thought about ending their lives; 10 (19 percent) had expressed to others a wish to die; and three (6 percent) had actively hastened their deaths.
Participants who expressed a wish to die didn’t differ from others in disease severity, age, gender, race, education, support from Medicaid or Social Security Income (SSI), hospice participation, living at home or in a nursing home, or whether they had a spouse as a caregiver.
The study did confirm greater hopelessness and interest in suicide, as well as a lower importance attached to religion, in those who expressed a wish to die.
The authors say that a desire to hasten dying when one has a terminal disease may not be a feature of depression but may be part of a broader phenomenon they call end-of-life despair.
Neurologists Richard Olney and Catherine Lomen-Hoerth of the University of California at San Francisco, in an accompanying commentary, suggest exploring whether depression treatments have an effect on the wish to die.
They also consider why religion and spirituality, which apparently help cancer patients, didn’t offer protection against a wish to die in the ALS patients studied.
They suggest that a more sensitive measure of “comfort in religion,” as well as more careful probing for subtle cognitive changes, might shed light on this.
They conclude by saying, “Whether an ALS patient’s wish to die is more strongly influenced by depression or end-of-life despair remains to be determined. What is more remarkable is that a majority of ALS patients have a more positive attitude toward life even as the inevitability of death is imminent.”
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Spastin gene implicated in juvenile-onset ALS
A mutation in a chromosome 2 gene for a protein called spastin has been implicated as a potential cause of, or risk factor for, a juvenile-onset (before age 25) form of ALS. The spastin protein is involved in the functioning of nerve cell fibers.
In the July 12 issue of Neurology, Thomas Meyer at Charité University Hospital in Berlin, and colleagues, describe a 73-year-old man with ALS symptoms dating back to age 24 and no family history of ALS.
The mutation they found in one of his spastin genes wasn’t found in any of the 200 people in the comparison (control) group; nor was it found in eight of his relatives whose genes were screened.
Other mutations in the spastin gene can cause the neurological disorder spastic paraplegia.
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Does diaphragm stimulation work in ALS?
Rhythmic stimulation of the diaphragm through electrodes — “pacing” the diaphragm — may improve air exchange in ALS, says Raymond Onders, associate professor of surgery at Case Western Reserve University in Cleveland.
Onders has implanted electrodes in the diaphragms of three people with ALS, all of whom had lung capacities that were 53 percent of normal or less. He says the electrodes send signals through what remains of their phrenic (diaphragmatic) nerve fibers.
Two months after implantation, Onders reports, the rate of decline in respiratory capacity has slowed, all patients are able to vocalize the “ah” sound longer, and all report feeling better. Onders says the system is safe but that long-term gains can’t be assessed until more time has elapsed.
Other experts, however, voice concerns.
“This is not a good idea in my opinion, and the long-term results may show more rapid progression,” says Greg Carter, a physical medicine and rehabilitation specialist who co-directs the MDA/ALS Center at the University of Washington in Seattle.
“Phrenic-nerve pacing requires healthy motor neurons, which is not the case in ALS. It may work for a short time, but the effect will be short-lived. There is a very real danger, in my mind, of accelerating motor neuron death ... due to overtaxing the cells.”
Walter Bradley, a neurologist who directs the Kessenich Family MDA/ALS Center at the University of Miami, says he’s “not as worried about the potential damage in ALS as about the likelihood that it will not work.”
Onders, who hopes to study 10 people with ALS, says he welcomes inquiries from patients and professionals. He can be reached at (216) 844-5797 (Cleveland) or at firstname.lastname@example.org.
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