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26

New Scientist 22 September 1988
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Canada pioneers national screening for Huntington's disease
SCIENTISTS in Canada have begun the
world's f i r s t nationwide screening
programme to identify people at risk o f
developing Huntington's disease. T h e
programme, which began earlier t h i s
month, w a s formally announced i n
Toronto at the 16th International Congress
of Genetics by the project leader, Michael
Hayden o f the University o f British
Columbia.
Huntington's disease is an inherited, ultimately fatal, neurological disorder that
affects approximately one in 10 000 people
in the Western world. Either parent can
pass the disease to a child, who has a 50-50
chance of inheriting the defective gene that

ington's disease or who died from it. A l l
participants must agree to extensive preis responsible for the disease.
and post-screening counselling t o help
Because there is no known cure and them understand their test results and to
because victims of the disease generally do cope emotionally with the findings.
not develop symptoms until middle age,
Blood from participants and their close
predicting the presence of the Huntington's relatives will be collected at 14 genetics
gene before the onset of symptoms is crit- clinics across the country. Analyses will be
ical for many people at risk, says Hayden. conducted in Vancouver using three estabIf parents know that they carry the gene, lished gene markers known as D4S10,
they can choose not to have children or to D4S62 and D4S95.
abort affected fetuses. The new programme,
Depending upon the amount of useful
based on a pilot scheme that began in 1986, genetic information available from family
will be open on a voluntary basis to any members, Hayden predicts the tests could
Canadian who is 18 years of age or older, be "99-per-cent accurate" in establishing an
and who has a parent who either has Hunt- individual's risk of developing the disease.
Theoretically, if Huntington's disease is
caused by a„single genetic mutation, and if
Europe unites its scholars of science
the mutated gene can be isolated and
E U R O P E now has its own Academy of technological options f o r the European cloned, detailed family histories and blood
Sciences. S i r A r n o l d Burgen, t h e Community, lobby national governments samples from relatives will not be required.
master of Darwin College, Cambridge, is to secure more money for basic science, It should be possible to detect the presence
among 55 scholars from 16 European and set standards and recommendations in or absence of the defective gene in samples
nations, who decided this month to elect ethics.
taken solely from the individual at risk.
the academy's first 1000 members. They
According to James Gusella, a neuroBurgen says that the academy will need a
are to meet in June next year.
large endowment to meet annual running geneticist at Harvard who led the team that
Members will include representatives f
costs estimated a t £250 000. Already, described the first gene marker in 1983,
the humanities and the social sciences
Britain's Advisory Board for the Research only a few centres in the US, Britain and
well as the natural sciences. Burgen,
Councils, tie Rothschilds and the Bank of Europe offer screening to the public. None
Sweden
matches the Canadian programme. ❑
founder member of the academy, foresee?e
i terdiscip
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ra
fo
d
linaryb
o
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y-rev have pledged £100 000 in aid. p
senting scientists throughout Europe.
C o m p u t e r liaCkers Claied of HACKERSlawbreakin
Like the U S National Acade o f
Sciences, t h e European academy m a y
in Britain can breathe a sigh r u l i n g on hackers and has appealed instead
publish a journal. It will undertake studies_
of relief. The Law Commission o f f o r comment from the public, before i t
_ _
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decided
f i n a l
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Sylvia Dayton, Toronto

Crigianu a n t i W a l e s n a b

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o

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to makes a

recommendation.

Computer misuse covers a broad spectrum of activities including fraud, and the
laying o f "logic bombs" and computer
"viruses" in computer systems.
• I n July of this year a potential computer
fraud involving the transfer of £32 million
from a Swiss bank was thwarted by police.
Kite-flying on the increase in Wales
Computer "viruses", which are speciallyD E D KITES are on the
written programs designed t o replicate
fteso .1‘. increase again in Britain,
themselves and cause damage to software,
thanks to the country's first
have become more widespread.
The report concludes that most forms of
ever programme to breed rare
computer misuse are embraced by existing
birds of prey artificially. This
law, and so recommends no changes. This
year, 38 red kites were reared
is despite the case of Gold and Schifreen,
in central Wales, the secondtwo men who hacked into British Telebest performance this century.
The programme, under way
com's Prestel computers.
The pair were convicted of forgery, but
for two breeding seasons, is
their convictions were put aside when they
being organised jointly b y
appealed to the House o f Lords, which
scientists from t h e Nature
Conservancy Council (NCC)
decided that hacking could not amount to
and the Royal Society for the
the crime of forgery.
Protection of Birds (RSPB).
Immediately after this decision, in May,
the British Computer Society issued strong
They removed eggs from the
recommendations t h a t hacking should
most vulnerable nests of red
become an offence in Britain.
kites soon after t h e birds
Hacking, the commission says, is the
had laid them, substituting
only category that is not covered by existing
dummies for the stolen eggs.
law, despite a recent conclusion from the
The scientists hatched the
Audit Commission which ranks hacking as
real eggs i n incubators o r
"the single largest computer related crime".
beneath bantam hens. Roger 2'
The Law Commission has pulled together
Lovegrove, the RSPB's officer e
the case for and against its criminalisation.
in Wales, commented: "The f.2
Arguments in favour include the fact that
RSPB and the NCC regard cuunauthorised access to certain information
Bantams have helped kites (above) to recover
this as an important milestone
held on a computer, such as personal data
in the protection of this rare
fibre into nests. The replicas contain covered by the Data Protection Act, could
species."
Time-lapse cameras have been fitted to sensors which relay data about egg be "peculiarly damaging". It also notes that
some nests to monitor the presence of adult temperatures, the movement o f parent many other countries, including the US,
birds and intruders. Also, .the scientists birds, and the frequency with which they Sweden and France have already made
hacking a crime.
❑
❑
have slipped "kite" eggs made from glass turn their eggs during incubation.
concern to Europeans, says Burgen.
Unlike the American academy, its European counterpart will be politically neutral.
Burgen expects its membership to reach
around 5000. The academy could assess

recommend that hacking, breaking into a
computer system without authorisation,
becomes a n offence. I n a report o n
"computer misuse", published yesterday,
the commission has decided not to make a