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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
4
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pubmed:dateCreated |
1990-2-12
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pubmed:abstractText |
A 7-year-old boy suffering from X-linked hypogammaglobulinemia and progressive myoclonic encephalopathy is reported. The onset of neurological disturbances is at four years of age with ataxic gait and myoclonic jerks. The EEG shows a progressive slowing of background activity, bilateral diffuse and repetitive, pseudoperiodic, high amplitude slow waves, myoclonic jerks polygraphically documented. The CT-scan shows generalized cerebral atrophy, white matter hypodensity--principally in the frontal regions -, multiple nodular calcifications, also in the basal ganglia. Two years after the onset of neurological signs, the boy is completely bedridden, spastic, dement and blind; the myoclonic jerks persist. Finally the relationship is discussed with both the previously reported patients with the same affection, and with similar progressive encephalopathy in children suffering from A.I.D.S.
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pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
IM
|
pubmed:status |
MEDLINE
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pubmed:month |
Nov
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pubmed:issn |
0174-304X
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pubmed:author | |
pubmed:issnType |
Print
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pubmed:volume |
20
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
223-9
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pubmed:dateRevised |
2008-1-16
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pubmed:meshHeading |
pubmed-meshheading:2514400-Acquired Immunodeficiency Syndrome,
pubmed-meshheading:2514400-Adolescent,
pubmed-meshheading:2514400-Agammaglobulinemia,
pubmed-meshheading:2514400-Electroencephalography,
pubmed-meshheading:2514400-Epilepsies, Myoclonic,
pubmed-meshheading:2514400-Humans,
pubmed-meshheading:2514400-Male,
pubmed-meshheading:2514400-Sex Chromosome Aberrations,
pubmed-meshheading:2514400-X Chromosome
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pubmed:year |
1989
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pubmed:articleTitle |
Progressive myoclonic encephalopathy in X-linked hypogamma-globulinemia. Case report, review of the literature and its relationship with progressive encephalopathy in children with A.I.D.S.
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pubmed:affiliation |
Clinica Pediatrica dell'Università, Policlinico Borgo Roma, Verona, Italy.
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pubmed:publicationType |
Journal Article,
Review,
Case Reports
|