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Presynaptic and postsynaptic striatal dopaminergic function in neuroacanthocytosis: A positron emission tomographic study
Author(s) -
Brooks D. J.,
Ibanez V.,
Playford E. D.,
Sawle G. V.,
Leigh P. N.,
Kocen R. S.,
Harding A. E.,
Marchsden C. D.
Publication year - 1991
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.410300207
Subject(s) - neuroscience , postsynaptic potential , dopaminergic , positron emission tomography , medicine , psychology , dopamine , receptor
Using [ 18 F]dopa, [ 11 C]raclopride, C 15 O 2 , and positron emission tomography, we have assessed striatal dopamine storage capacity, dopamine D 2 ‐receptor integrity, and regional cerebral blood flow, respectively, of 6 patients with neuroacanthocytosis. The patients with neuroacanthocytosis all had chorea and variable combinations of seizures, dementia, axonal neuropathy, and orolingual self‐mutilation. [ 18 F]dopa positron emission tomographic findings were compared with 30 normal controls and 16 patients with sporadic, L ‐dopa‐responsive, Parkinson's disease. Caudate and anterior putamen [ 18 F]dopa uptake were normal in patients with neuroacanthocytosis, but mean posterior putamen [ 18 F]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 [ 11 C]raclopride uptake ratios were reduced to 54% and 62% of normal, compatible with a 65% and 53% loss of caudate and putamen D 2 ‐receptor‐binding sites, respectively. Striatal and frontal blood flow was also depressed. The severe loss of D 2 ‐receptor‐bearing striatal neurons, 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.