Linked Life Data

6496876

Source:http://linkedlifedata.com/resource/pubmed/id/6496876

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
4
pubmed:dateCreated
1984-12-14
pubmed:abstractText
Two Dutch siblings, diagnosed as suffering from Fukuyama type congenital muscular dystrophy (FCMD) on the basis of clinical, computerized tomography (CT), and muscle and brain biopsy findings, are reported. Hypoplasia of the chorioidea was observed for the first time in FCMD. Autopsy of the first case revealed the major pathological changes of FCMD, i.e. micropolygyria, loss of cytoarchitecture, hypoplasia of the pyramidal tract, leptomeningeal thickening. Heterotopias of nervous tissue in the spinal arachnoidal spaces were found. This is the first case in which brain tissue has been investigated on two separate occasions. In the biopsy specimen--at the age of 14 months--myelination was poor and astrogliosis marked. At autopsy--4 years later--myelination proved to be only slightly less than normal. However, white matter hypodensities on the successive CT's did not change. There is no ready explanation for this discrepancy. Typical FCMD is compared to FCMD-like cases from outside Japan. There are arguments in favor of the concept of a continuum of diseases--with the same (unknown) etiology--representing both typical FCMD and other types of congenital muscular dystrophy with CNS lesions.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:status
MEDLINE
pubmed:issn
0387-7604
pubmed:author
pubmed:issnType
Print
pubmed:volume
6
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
406-16
pubmed:dateRevised
2004-11-17
pubmed:meshHeading
pubmed:year
1984
pubmed:articleTitle
Fukuyama type congenital muscular dystrophy--two Dutch siblings.
pubmed:publicationType
Journal Article, Case Reports