Document Detail


Presynaptic and postsynaptic striatal dopaminergic function in neuroacanthocytosis: a positron emission tomographic study.
MedLine Citation:
PMID:  1897909     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Using [18F]dopa, [11C]raclopride, C15O2, and positron emission tomography, we have assessed striatal dopamine storage capacity, dopamine D2-receptor integrity, and regional cerebral blood flow, respectively, of 6 patients with neuroacanthocytosis. The patients with neurocanthocytosis all had chorea and variable combinations of seizures, dementia, axonal neuropathy, and orolingual self-multiation. [18F]dopa positron emmission tomographic findings were compared with 30 normal controls and 16 patients with sporadic, L-dopa-responsive, Parkinson's disease. Caudate and anterior putamen [18F]dopa uptake were normal in patients with neuroacanthocytosis, but mean posterior putamen [18F]dopa uptake was reduced to 42% of normal, similar to that in patients with Parkinson's disease. In patients with neuroacanthocytosis, mean equilibrium caudate: cerebellum and putamen: cerebellum [11C]raclopride uptake ratios were reduced to 54% and 62% of normal, compatible with a 65% and 53% loss of caudate and putamen D2-receptor-binding sites, respectively. Striatal and frontal blood flow was also depressed. The severe loss of D2-receptor-bearing striatal neuron, with concomitant loss of dopaminergic projections from the nigra to the posterior putamen, is consistent with both chorea and extrapyramidal rigidity being features of patients with neuroacanthocytosis.
Authors:
D J Brooks; V Ibanez; E D Playford; G V Sawle; P N Leigh; R S Kocen; A E Harding; C D Marsden
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Annals of neurology     Volume:  30     ISSN:  0364-5134     ISO Abbreviation:  Ann. Neurol.     Publication Date:  1991 Aug 
Date Detail:
Created Date:  1991-10-24     Completed Date:  1991-10-24     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  7707449     Medline TA:  Ann Neurol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  166-71     Citation Subset:  IM    
Affiliation:
MRC Cyclotron Unit, Hammersmith Hospital, London, England.
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MeSH Terms
Descriptor/Qualifier:
Acanthocytes*
Adult
Aged
Brain Diseases / physiopathology,  radionuclide imaging*
Caudate Nucleus / blood supply,  radionuclide imaging
Cerebellum / radionuclide imaging
Cerebrovascular Circulation
Corpus Striatum / physiopathology,  radionuclide imaging*
Female
Humans
Levodopa / pharmacokinetics*
Male
Middle Aged
Putamen / radionuclide imaging
Receptors, Dopamine / analysis
Reference Values
Tomography, Emission-Computed*
Chemical
Reg. No./Substance:
0/Levodopa; 0/Receptors, Dopamine

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