Dementia in hereditary cystatin C amyloidosis

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 seve...

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Bibliographic Details
Main Authors: Blöndal, H, Guomundsson, G, Benedikz, Eirikur, Jóhannesson, G
Format: Article in Journal/Newspaper
Language:English
Published: 1989
Subjects:
Adolescent
Adult
Amyloidosis
Antibodies
Brain
Cerebral Arteries
Cystatin C
Cystatins
Dementia
Female
Humans
Iceland
Male
Middle Aged
Retrospective Studies
Online Access:https://portal.findresearcher.sdu.dk/da/publications/6bf80f1f-40ab-4f7d-a7c0-a21d2ea794c4
Description
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. Skin biopsies can now probably be used to demonstrate cystatin C positive amyloid deposits conclusively in the tissues of these patients.

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