Massachusetts Man may be First American Victim of `Mad Cow' Disease

      CONCORD, Mass. - Last fall, Don Hodges started losing most of his racquetball games. It had never happened before. On Thanksgiving, he slurred some words. Slowly, he lost the ability to walk, stand or speak.

      Now Hodges, 63, sits behind a tray of hospital food, his face slack. Spelling out words by pointing to letters on a clipboard, he can still list his graduate degrees, explain his conversion to Catholicism, and slam ``limousine liberals'' like Jane Fonda.

      Even that will fade, his doctors say. They believe he has a rare degenerative brain ailment called Creutzfeldt-Jakob disease, which is always fatal.

      Ask Hodges how he thinks this happened to him, and he spells out, ``England, 1995.''

      If he is right - and no one will know until after he dies - he would be the first American known to have contracted a new form of Creutzfeldt-Jakob disease that has frightened Britain since an epidemic of ``mad cow'' disease struck 180,000 cattle there. Since the early 1990s, 79 Britons have been diagnosed with ``new variant'' CJD, which scientists have linked to eating products from infected cattle.

      Although his doctor, whom he trusts, strongly doubts it, Hodges is convinced he got sick from eating beef on a trip to England he won in a seat raffle at a Celtics game in 1995. His family tells friends he has ``mad cow'' disease. His wife has joined a national support group and says she is on ``a personal crusade'' to spread the word about CJD and its links to food - even as doctors say her husband''s symptoms do not match the bovine version and federal health officials insist there has not been a single case in this country.

      She is not alone. At the National Institute of Neurological Disorders and Stroke in Bethesda, Md., Dr. Clarence J. Gibbs has a folder full of e-mailed questions from relatives of people with the classic form of CJD, which develops spontaneously in about one in a million people each year.

      ``They immediately start worrying about beef, beef, beef,'' said Gibbs, chief of central nervous system studies. ``They''re looking for something, and that''s the most convenient to hang onto ... because there is no other answer in their minds.''

      When classic CJD strikes in Massachusetts - about six times a year - questions flow into the Department of Public Health, some from families ``absolutely convinced'' beef is the culprit, said Dr. Alfred DeMaria, director of communicable diseases. The department has investigated several cases and found that none were the beef-related version known as new variant CJD.

      In Acton, Hodges'' friends are asking, '' `How did he get it?'' and `How can I avoid it?'' '' said his wife, Sally Brennan-Hodges.

      Only an autopsy will show for sure whether Hodges, a retired Air Force meteorologist, has the distinctive pattern of brain damage caused by the new variant CJD. But his age and symptoms strongly suggest the classic version, said his neurologist, Dr. Gilmore O''Neill, of Massachusetts General Hospital.

      Classic CJD tends to strike people over 50 and sometimes attacks the motor functions first, often leading to uncontrolled jerking motions and dementia. The new variant tends to strike younger people and often starts with psychiatric symptoms, which have not affected Hodges, O''Neill said.

      He said it is doubtful but ``not impossible'' that Hodges has the new version - although it is hard to generalize about new variant CJD when so few cases have been documented. ``I''d be very foolish to give absolutes,'' he said.

      Originally from Ireland, where mad-cow disease is a preoccupation, O''Neill called U.S. fears of beef-related illness ``understandable,'' especially given the American Red Cross ban on blood donations from anyone who spent six months in England between 1980 and 1996.

      As with many high-profile but rare diseases, the mad-cow connection has physicians and health officials walking a tightrope: They caution against panic, reminding people that the chances of getting CJD are infinitesimal compared with breast cancer or diabetes.

      At the same time, they want to raise awareness of CJD. DeMaria calls for required reporting of all CJD cases; Gibbs wants tighter rules to keep U.S. cattle free of mad-cow disease, including a ban on feeding mammals to other mammals, a practice blamed for the British outbreak.

      Formally called bovine spongiform encephalopathy, the cattle disease was first identified in Britain in 1986 and linked to human CJD in 1998, devastating the British beef industry.

      Scientists believe a similar sheep disease called scrapie somehow passed to cattle that were fed ground-up sheep. The bovine form then spread when rendered cow carcasses were fed to cattle. It exists in nine other European countries. France has identified 73 sick cattle this year, up from 31 last year.

      CJD and related animal diseases kill off neurons and riddle the brain with tiny sponge-like holes. They are caused not by a bacteria or virus, but by prions, proteins normally present in the brain that for some reason change their structure. The malformed prions can then convert healthy ones. They must be boiled for hours in detergent to be destroyed.

      In England, safety measures were introduced in 1989 to keep brain and spinal tissue out of food, but because of the long incubation period, new variant CJD cases in humans are still rising, by an average of 23 percent a year. British scientists have recently toned down predictions of a massive human epidemic, but still say several thousand could die.

      In 1997, the United States banned imports of European cattle, sheep and goat products.

      Most mammal-to-mammal feeding was also banned in the United States, although there are a few loopholes. More than 11,000 U.S. cattle with neurological symptoms have been tested, and none had the disease, said Linda Detwiler, a senior veterinarian at the U.S. Department of Agriculture.

      Sheldon Rampton, co-author of the 1998 book, ``Mad Cow U.S.A.,'' said a bigger danger to Americans is chronic wasting disease, found in deer and elk in Colorado and Maine. But state governments, eager for revenue from hunters, ``don''t want to see a problem,'' he said.

      As for Hodges, he will go no farther than to spell, ``If it is possible to prevent the disease, then the powers that be should do something.''

      The chairman of the Acton Republican Committee and his late first wife had f

c.2000 The Boston Globe