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| Predicate | Object |
|---|---|
| rdf:type | |
| lifeskim:mentions | |
| pubmed:issue |
3
|
| pubmed:dateCreated |
1991-3-27
|
| pubmed:abstractText |
The lesions of the cerebral white matter which characterize Pelizaeus-Merzbacher disease are classically observed at pathological examination. An early diagnosis can now be obtained by magnetic resonance imaging (MRI). In an eutrophic first son born at term, stridor, nystagmus-like eye movements and axial hypotonia were noted immediately after birth and pyramidal signs appeared at 2 months, the only extra-clinical finding at that age being prolonged latencies of evoked visual potentials. An MRI exploration, performed at 3 months, showed that compared with the grey matter the white matter emitted a low-intensity signal on the T1-weighted sequence and a high-intensity signal on the T2-weighted sequence (signal inversion), such diffuse and symmetrical MRI abnormalities being typical of dysmyelination. When combined with suggestive clinical signs, these abnormalities confirm the diagnosis of Pelizaeus-Merzbacher disease, even in the absence of a familial history, and make it possible to warn the parents of the poor prognosis and the risk of recurrence among future sons.
|
| pubmed:language |
eng
|
| pubmed:journal | |
| pubmed:citationSubset |
IM
|
| pubmed:status |
MEDLINE
|
| pubmed:issn |
0150-9861
|
| pubmed:author | |
| pubmed:issnType |
Print
|
| pubmed:volume |
17
|
| pubmed:owner |
NLM
|
| pubmed:authorsComplete |
Y
|
| pubmed:pagination |
216-21
|
| pubmed:dateRevised |
2008-2-20
|
| pubmed:meshHeading | |
| pubmed:year |
1990
|
| pubmed:articleTitle |
Pelizaeus-Merzbacher disease. Contribution of magnetic resonance imaging to an early diagnosis.
|
| pubmed:affiliation |
Service de Médecine Néonatale, Maternité Universitaire, Nancy.
|
| pubmed:publicationType |
Journal Article,
Case Reports
|