Document Detail

Presynaptic and postsynaptic striatal dopaminergic function in neuroacanthocytosis: a positron emission tomographic study.
MedLine Citation:
PMID:  1897909     Owner:  NLM     Status:  MEDLINE    
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.
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    
MRC Cyclotron Unit, Hammersmith Hospital, London, England.
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MeSH Terms
Brain Diseases / physiopathology,  radionuclide imaging*
Caudate Nucleus / blood supply,  radionuclide imaging
Cerebellum / radionuclide imaging
Cerebrovascular Circulation
Corpus Striatum / physiopathology,  radionuclide imaging*
Levodopa / pharmacokinetics*
Middle Aged
Putamen / radionuclide imaging
Receptors, Dopamine / analysis
Reference Values
Tomography, Emission-Computed*
Reg. No./Substance:
0/Levodopa; 0/Receptors, Dopamine

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