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| Predicate | Object |
|---|---|
| rdf:type | |
| lifeskim:mentions | |
| pubmed:issue |
1
|
| pubmed:dateCreated |
1991-11-7
|
| pubmed:abstractText |
The syndrome of dopa-responsive dystonia comprises a minority of patients with dystonia, yet it is of considerable diagnostic importance because patients respond dramatically to L-dopa therapy. Benefits from this treatment are lasting, and the problems associated with long-term L-dopa therapy in patients with Parkinson's disease are generally absent. It has been suggested that this condition is due to a defect in the dopamine synthetic pathway, which is bypassed when patients are treated with L-dopa. We have studied [18F]dopa uptake in 6 patients with classic dopa-responsive dystonia (5 familial patients and 1 sporadic patient), aged 18 to 66 years. Data have been analyzed according to a graphic approach, calculating an influx constant for each region studied. We have also studied a seventh, clinically atypical, patient with juvenile dystonia-parkinsonism. Similar data have been calculated for a group of 10 healthy control subjects and 10 patients with Parkinson's disease. The 6 patients with typical dopa-responsive dystonia had a modest but significant reduction in the uptake of tracer into both caudate and putamen, which indicates a defect in the decarboxylation, vesicular uptake, and storage of [18F]dopa. This argues against the proposition that dopa-responsive dystonia is due to an inherited defect of tyrosine hydroxylase alone. In the atypical patient, however, we found a greater reduction of [18F]dopa uptake into both caudate and putamen, comparable with that in patients with Parkinson's disease.
|
| pubmed:language |
eng
|
| pubmed:journal | |
| pubmed:citationSubset |
IM
|
| pubmed:chemical | |
| pubmed:status |
MEDLINE
|
| pubmed:month |
Jul
|
| pubmed:issn |
0364-5134
|
| pubmed:author | |
| pubmed:issnType |
Print
|
| pubmed:volume |
30
|
| pubmed:owner |
NLM
|
| pubmed:authorsComplete |
Y
|
| pubmed:pagination |
24-30
|
| pubmed:dateRevised |
2006-11-15
|
| pubmed:meshHeading |
pubmed-meshheading:1681782-Adolescent,
pubmed-meshheading:1681782-Adult,
pubmed-meshheading:1681782-Aged,
pubmed-meshheading:1681782-Biological Transport,
pubmed-meshheading:1681782-Caudate Nucleus,
pubmed-meshheading:1681782-Decarboxylation,
pubmed-meshheading:1681782-Dihydroxyphenylalanine,
pubmed-meshheading:1681782-Dopamine,
pubmed-meshheading:1681782-Dystonia,
pubmed-meshheading:1681782-Female,
pubmed-meshheading:1681782-Fluorine Radioisotopes,
pubmed-meshheading:1681782-Humans,
pubmed-meshheading:1681782-Male,
pubmed-meshheading:1681782-Middle Aged,
pubmed-meshheading:1681782-Parkinson Disease,
pubmed-meshheading:1681782-Putamen,
pubmed-meshheading:1681782-Tissue Distribution,
pubmed-meshheading:1681782-Tomography, Emission-Computed,
pubmed-meshheading:1681782-Tyrosine 3-Monooxygenase
|
| pubmed:year |
1991
|
| pubmed:articleTitle |
Dopa-responsive dystonia: [18F]dopa positron emission tomography.
|
| pubmed:affiliation |
MRC Cyclotron Unit, Hammersmith Hospital, Villigen, Switzerland.
|
| pubmed:publicationType |
Journal Article,
Comparative Study,
Case Reports,
Research Support, Non-U.S. Gov't
|