Læknablaðið - 15.08.1989, Page 14

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LÆKNABLAÐIÐ
Þakkarorð. Sigurlaugu Aðalsteinsdóttur,
kennslumeinatækni, og Michael Kissane,
ljósmyndara, eru þökkuð tæknileg aðstoð.
Einnig er þakkaður stuðningur Vísindasjóðs
íslands, Líf- og læknisfræðideild, við þetta
verk.
SUMMARY
Nineteen cases with verified Hereditary Cystatin
C Amyloid Angiopathy are presented. All of the
cases had one or more cerebrovascular insults
starting at the age of 20-41 years and survived
from 10 days to 23 years after the first insult.
Progressive dementia was a prominent clinical
feature in seventeen cases of whom two presented
with dementia. At the last examination the majority
had severe dementia and severely abnormal EEG.
Anti-cystatin C positive amyloid vascular and
perivascular infiltrates were found. The resulting
damage to the microvasculature of the brain and
secondary hemorrhages and infarctions were
considered to be an adequate explanation for the
dementia in these cases.
This hereditary type of amyloidosis has been
regarded as being confined to the CNS. This is no
longer true and it would be more properly named
Hereditary Cystatin C Amyloidosis (HCCA).
HEIMILDIR
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Bjamason Ó: Hereditary cerebral hemorrhage with
Amyloidosis. Brain 1972; 95: 387-404.
3. Guðmundsson G, Jensson Ó: Genetics and
epidemiology of cerebrovascular diseases in Iceland.
Nordic Council Arct Med Res Rep 1980; 26: 22-7.
4. Guðmundsson G. Jensson Ó, and Jóhannesson G:
Epidemiology and clinical aspects of hereditary
cerebral hemorrhage with amyloidosis (HCHWA) in
Icelandic families. Abstract. Acta Neurol Scand 1986;
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5. Blöndal H: Pathological aspects of hereditary central
nervous system amyloid angiopathy. Abstract. Acta
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