British Study Says Brain Patients Had Psychiatric Symptoms
By LAWRENCE K. ALTMAN ... April 5, 1996
Many of the 10 British patients afflicted with a new variant of a
fatal brain disease possibly linked to mad-cow
disease had behavioral and emotional changes that initially fooled
doctors into thinking they had psychiatric
disorders, according to the first published scientific report about
the cases.
In nine of the patients, early symptoms included personality changes,
depression, difficulty sleeping, withdrawal,
fearfulness, and paranoia, said the report, being published on
Saturday in The Lancet, an international journal
issued in London. Several patients were referred by their doctors to
a psychiatrist.
The doctors were also fooled because brain wave tracings of the
patients did not show the changes that are usually
observed in traditional cases of the human brain disease, known as
Creutzfeldt-Jakob Disease.
The findings were reported to alert doctors to the unusual new face
of a rare disease. Creuzfeldt-Jakob Disease
itself is so rare that physicians find it hard to diagnose.
The report was also intended to alert doctors to the distinctive
pattern of waxy deposits known as amyloid plaques
that were found in the brain along with the usual holes that give the
brain an appearance of Swiss cheese.
Seven of the 10 British cases were not classified as probable
Creutzfeldt-Jakob Disease when first reported to the
special surveillance unit that the British government set up in 1990
to look for any human version of mad-cow
disease. If such a unit had not been in place, it would have taken
much longer for individual doctors to compare
notes to detect the new variant.
In 1989, a British committee concluded that inclusion of animal
protein from sheep afflicted with scrapie in
commercial cattle feed was the source of the mad-cow disease. A ban
on such feed was imposed at that time. If
humans can acquire a variant of Creutzfeldt-Jakob disease from beef
contaminated by mad-cow disease, the
exposure is believed to have been greatest in the 1980s.
In the latest research, several factors led to the recognition that
the patients suffered from a new variant of
Creutzfeldt-Jakob Disease, the researchers, headed by Dr. Rob G. Will
of Western General Hospital, in Edinburgh,
reported.
One was that patients also developed motor abnormalities such as
difficulty maintaining balance. A second was
pain when a patient was touched on the face, arms, and legs. One
patient had pain in the feet that persisted
throughout the illness. Another factor was the rapid course to death,
averaging about one year from onset of
symptoms.
Still another factor was the chemical stains on brain tissue obtained
at autopsies for examination under a
microscope. The stains showed a distinctive pattern, being scattered
throughout the brain but most evident in the
areas known as the basal ganglia, thalamus, and cerebellum.
The plaques resembled those found in scrapie, the sheep disease, and
kuru, a human disease. Kuru is believed to
result from ritualistic cannibalism among the Fore group in the
highlands of Papua-New Guinea. The diseases are
now thought to be due to rogue proteins known as prions.
A striking feature of the six females and four males diagnosed with
the new variant of Creutzfeldt-Jakob Disease
was their young age, which ranged from 16 to 39 years at the time
they fell ill from February 1994 to October 1995.
Health records showed that from 1970 to 1989, only four cases of
Creutzfeldt-Jakob Disease occurred in people
younger than 34 years.
Examination of brain tissue collected at the autopsy of one of the
earlier cases did not show similar amyloid
changes.
The researchers also searched for cases outside Britain and found no
similar pattern in countries that had a similar
surveillance system.
The overall incidence of Creutzfeldt-Jakob Disease rose in Britain in
the 1990s, but most cases were in people older
than 75. The most likely explanation, the researchers said, was that
publicity had led doctors to be more alert to
diagnosing it.
The researchers said they found the cluster of variant cases over the
last 10 months by studying possible cases
reported by physicians and looking at death certificates and
newspaper articles.
The researchers said that all 10 had eaten beef in the last 10 years
but none was reported to have eaten brain. One
had been a strict vegetarian since 1991. One patient worked as a
butcher from 1985 to 1987; another had visited an
abattoir for 2 days in 1987. None had ever worked on farms with
livestock, although one patient had spent a
one-week holiday on a dairy farm where there was no known mad-cow
disease.
In an editorial, Dr. J. Gerald Collee, of the University of
Edinburgh, said that experts suspect that a teaspoonful of
highly infective cattle feed is enough to cause mad-cow disease. But
he said that if the disease can be transmitted
to humans, the amount needed for infection cannot be estimated.
The infectious agent causing Creutzfeldt-Jakob and similar diseases
is believed to be resistant to chemicals in the
digestive track and able to enter the body through lymph tissue in
the tract. Factors such as coexistent infection or
foods and alcohol could facilitate such entry.
Copyright 1996 The New York Times Company
