Læknablaðið : fylgirit - 01.05.2002, Blaðsíða 37

ABSTRACTS / 33RD SNC & 2ND SCNN
64 - Social situation of people who have been diagnosed with
multiple sclerosis
Sigurdardóttir M
Dept. of Neurology, Landspítali University Hospital Grensás, Reykjavík, Iceland
Introduction: The research is a MA study in Social Work at the
Iceland University in Reykjavík.
Research description: To study the impact of Multiple Sclerosis on
social situation by a samle of factors in a representive Icelandic
population by a variety of factors.
Main objectives:
1) To explore in what extent changes in participant s situation are and
the families’ coping style, social support and adjustment to MS.
2) To evaluate if it is possible to improve peoples quality of life.
3) To explore if people have already had sufficient information
about the disease. To look into if people with MS have had
enough support form health professionals.
4) To examine how the individual and the family have adjusted to
the changes the disease can bring to their lives, and which
resources could be helpful.
5) To focus on families situation when one member has MS. What
happens when parents can not take care of their role? Does the
other parent take over the families duties and responsibilties?
6) Are those numerous who have been forced to quit their study or
resign from work because of the disease.
The purpose of this research is to collect information and also to
test hypotheses by correlation of variables. To look into the
influence the disease has on the individual and his/her family
relations. This research can give information about need for service,
support, economic status and participant's support net.
Material and methods: In Iceland are about 380 people (0.136 % of
the population) diagnosed with Multiple Sclerosis. A questionnaire
will be sent to all members of the Multiple Sclerosis Society in
which are about 60% of those diagnosed with the disease in Iceland
are members. Others will receive telephone calls and be invited to
participate. The research is quantitive and the method is a postal
study. Participants will be sent a questionnaires with a letter giving
information about the research. The people who are not able to
answear the questionnaire themselves will be interviewed, if they
agree to attend. Included in the letter will be the permit for this
study given by National Bioethics Committee and Data Protection
Authority.
Results: Results of the study will be presented at the conference.
L65 - Results of 40 years surveillance for Creutzfeldt-Jakob
disease in lceland
Georgsson G
Institute for Experimental Pathology, Keldur. University of Iceland, Reykjavík,
Iceland
Creutzfeldt-Jakob disease (CJD) belongs to a group of diseases of
man and animals, which are either called Prion-diseases, a term
derived from the name, that Stanley B. Prusiner gave the infectious
agent, or Transmissible spongiform encephalopathies (TSE's),
which refers to the characteristic pathological lesions. Initially these
diseases were classified as slow infections, a term introduced by
Björn Sigurdsson, as they fulfil the main criteria for this class of
infections. The sporadic form of Creutzfeldt-Jakob disease (spCJD)
was first described some 80 years ago. Later studies have shown
that CJD does also exist in familial forms due to mutations in the
prion gene and iatrogen forms due to medical treatment including
surgical procedures. Recently, i.e. in 1996, a variant form, vCJD, was
described and there is considerable evidence that it is caused by
consumption of products of cattle with Bovine spongiform ence-
phalopathy (BSE). Cattle did apparently contract BSE through
consumption of meat and bone meal containing carcasses of
scrapie sheep, a natural Prion-disease, which has been known in
sheep in the United Kingdom for approximately 250 years.
Interestingly Kuru, a Prion-disease of people of the Fore-tribe
living in New-Guinea, is also transmitted orally and has been traced
to kannibalistic rituals. The cause of spCJD, however, is still not
kown. The impetus for the initiation of the surveillance for CJD in
Iceland, was a hypothesis published in Science in 1974 dealing with
and trying to explain an unusually high incidence of CJD in Libyan
Jews living in Israel. These Jews had 15-30fold higher incidence of
CJD than Iraqui and other Jews living in Israel. The Libyan Jews
were sheep farmers and the hypothesis was that they might have
contracted CJD through the dietary habit of eating eyeballs of
sheep. In Iceland sheep products have together with fish made up
the bulk of the diet. Singed sheep heads including eyeballs and in
some districts also brain have been considered a delicacy and have
been an integral part of the Icelandic diet. As scrapie of sheep was
apparently introduced to Iceland some 120 years ago and did
spread to most parts of the country some 50 years ago the majority
of the population has certainly been exposed to scrapie of sheep for
several decades. Thus we expected to find either a high incidence
and/or aberrant forms when we started this survey of CJD. The
surveillance consisted of two parts: 1) Retrospective study 1960-
1979; 2) Prospective study 1980-1999. The results were, that two
cases of definite CJD were detected in each period, corresponding
to an annual death rate 0.5 per million inhabitants in the earlier
period and 0.4 in the latter period. The overall figure was 0.44 per
million inhabitants, 2 females and 2 males. The age distribution was
56-73 years and the duration of illness 2-12 months. These
parameters as well as the clinical symptoms and especially the
character and pattern of the pathological lesions were consistent
with the diagnosis spCJD, but not vCJD. This fact and the finding of
a relatively low incidence of CJD in spite of a considerable
exposure to scrapie of sheep allows us to conclude, that we have no
reason to assume that scrapie of sheep can be transmitted to
humans and cause CJD.
L66 - Hereditary Cystatin C Amyloid Angiopathy in lceland.
Blöndal H
Departments of Anatomy and Pathology, University of Iceland, Faculty of
Medicine
Introduction: In his doctoral thesis, Apoplexie und ihre Vererbung,
published in 1935, an Icelandic district physician reported on his
studies of several families in the Breidafjordur area that were
known for sudden deaths of young people from stroke. He con-
cluded that this was an autosomal dominant hereditary disease
which was confirmed by later studies. In 1972 amyloid was identi-
fied in pathological blood vessels in the brain of family members
and the condition then received the name Hereditary cerebral
hœmorrhage with amyloidosis (HCHWA). In 1983 the amyloid
Læknablaðið/Fylgirit 43 2002/88 37