Positron emission tomographic studies of dopa‐responsive dystonia and early‐onset idiopathic parkinsonism
Identifieur interne : 004385 ( Main/Exploration ); précédent : 004384; suivant : 004386Positron emission tomographic studies of dopa‐responsive dystonia and early‐onset idiopathic parkinsonism
Auteurs : Fang Snow [Canada] ; Torbjoern G. Nygaard [États-Unis] ; Hirohide Takahashi [Canada] ; Donald B. Calne [Canada]Source :
- Annals of Neurology [ 0364-5134 ] ; 1993-11.
English descriptors
- KwdEn :
- Adolescent, Adult, Corpus Striatum (diagnostic imaging), Diagnosis, Differential, Dihydroxyphenylalanine (analogs & derivatives), Dystonia (diagnostic imaging), Dystonia (drug therapy), Female, Fluorine Radioisotopes, Humans, Levodopa (therapeutic use), Male, Middle Aged, Parkinson Disease (diagnostic imaging), Parkinson Disease (drug therapy), Prognosis, Tomography, Emission-Computed.
- MESH :
- chemical , analogs & derivatives : Dihydroxyphenylalanine.
- diagnostic imaging : Corpus Striatum, Dystonia, Parkinson Disease.
- drug therapy : Dystonia, Parkinson Disease.
- chemical , therapeutic use : Levodopa.
- Adolescent, Adult, Diagnosis, Differential, Female, Fluorine Radioisotopes, Humans, Male, Middle Aged, Prognosis, Tomography, Emission-Computed.
Abstract
There are two major syndromes presenting in the early decades of life with dystonia and parkinsonism: dopa‐responsive dystonia (DRD) and early‐onset idiopathic parkinsonism (EOIP). DRD presents predominantly in childhood with prominent dystonia and lesser degrees of parkinsonism. EOIP presents before age 40 with parkinsonism (often with associated dystonia). Both disorders are exquisitely sensitive to levodopa, although the long‐term prognosis in each appears to be different. Some have suggested, however, that DRD is a form of EOIP. We performed positron emission tomography with 6‐fluorodopa in 10 patients with DRD and 18 patients with EOIP to study the integrity of their nigrostriatal dopaminergic systems. In DRD, we found normal striatal FD uptake. In contrast, patients with EOIP had reduced striatal FD uptake. We conclude that the pathophysiologies of DRD and EOIP are distinct. Although both disorders presumably represent a deficiency of striatal dopamine, the results suggest that in DRD dopa uptake, decarboxylation, and storage mechanisms are intact. This may explain the sustained response of DRD to low doses of levodopa. 6‐Fluorodopa positron emission tomography distinguishes DRD from EOIP.
Url:
DOI: 10.1002/ana.410340518
Affiliations:
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Le document en format XML
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DRD presents predominantly in childhood with prominent dystonia and lesser degrees of parkinsonism. EOIP presents before age 40 with parkinsonism (often with associated dystonia). Both disorders are exquisitely sensitive to levodopa, although the long‐term prognosis in each appears to be different. Some have suggested, however, that DRD is a form of EOIP. We performed positron emission tomography with 6‐fluorodopa in 10 patients with DRD and 18 patients with EOIP to study the integrity of their nigrostriatal dopaminergic systems. In DRD, we found normal striatal FD uptake. In contrast, patients with EOIP had reduced striatal FD uptake. We conclude that the pathophysiologies of DRD and EOIP are distinct. Although both disorders presumably represent a deficiency of striatal dopamine, the results suggest that in DRD dopa uptake, decarboxylation, and storage mechanisms are intact. This may explain the sustained response of DRD to low doses of levodopa. 6‐Fluorodopa positron emission tomography distinguishes DRD from EOIP.</div></front></TEI><affiliations><list><country><li>Canada</li><li>États-Unis</li></country><region><li>État de New York</li></region></list><tree><country name="Canada"><noRegion><name sortKey="Snow, Fang" sort="Snow, Fang" uniqKey="Snow F" first="Fang" last="Snow">Fang Snow</name></noRegion><name sortKey="Calne, Donald B" sort="Calne, Donald B" uniqKey="Calne D" first="Donald B." last="Calne">Donald B. Calne</name><name sortKey="Snow, Fang" sort="Snow, Fang" uniqKey="Snow F" first="Fang" last="Snow">Fang Snow</name><name sortKey="Takahashi, Hirohide" sort="Takahashi, Hirohide" uniqKey="Takahashi H" first="Hirohide" last="Takahashi">Hirohide Takahashi</name></country><country name="États-Unis"><region name="État de New York"><name sortKey="Nygaard, Torbjoern G" sort="Nygaard, Torbjoern G" uniqKey="Nygaard T" first="Torbjoern G." last="Nygaard">Torbjoern G. Nygaard</name></region></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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