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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
4
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pubmed:dateCreated |
1989-2-10
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pubmed:abstractText |
We performed a neurochemical study of the brain of two unrelated patients, living in different continents, with neuroacanthocytosis. The levels of monoamines and their metabolites, gamma-aminobutyric acid and substance P, were measured in several brain areas and the monoamine metabolites in cerebrospinal fluid. The binding of 3H-spiperone to striatal membranes and to lymphocytes was also measured. Both patients had a progressive neurological disorder with onset in the third decade of life and characterized by a complex movement disorder, epilepsy, muscular wasting, and changes in behavior. The movement disorder initially manifested with oromandibular dystonia and limb chorea, but at the time of death was characterized by a severe dystonic syndrome. The chemical changes were similar in the two patients. The most important neurochemical findings were a depletion of dopamine and its metabolites in most brain areas, most notably in the striatum, and elevation of norepinephrine levels in the putamen and globus pallidus. Substance P was markedly reduced in the striatum and substantia nigra. Our findings may provide clues to the neurochemical mechanisms underlying dystonia.
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pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
IM
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pubmed:chemical | |
pubmed:status |
MEDLINE
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pubmed:issn |
0885-3185
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pubmed:author | |
pubmed:issnType |
Print
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pubmed:volume |
3
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pubmed:owner |
NLM
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pubmed:authorsComplete |
N
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pubmed:pagination |
300-12
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pubmed:dateRevised |
2006-11-15
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pubmed:meshHeading |
pubmed-meshheading:2905427-Acanthocytes,
pubmed-meshheading:2905427-Adult,
pubmed-meshheading:2905427-Atrophy,
pubmed-meshheading:2905427-Brain,
pubmed-meshheading:2905427-Caudate Nucleus,
pubmed-meshheading:2905427-Chorea,
pubmed-meshheading:2905427-Diseases in Twins,
pubmed-meshheading:2905427-Dystonia,
pubmed-meshheading:2905427-Erythrocytes, Abnormal,
pubmed-meshheading:2905427-Humans,
pubmed-meshheading:2905427-Male,
pubmed-meshheading:2905427-Neurotransmitter Agents,
pubmed-meshheading:2905427-Receptors, Neurotransmitter
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pubmed:year |
1988
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pubmed:articleTitle |
Neurochemical findings in neuroacanthocytosis.
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pubmed:affiliation |
Departamento de Investigacíon, Centro Ramon y Cajal, Madrid, Spain.
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pubmed:publicationType |
Journal Article,
Case Reports,
Research Support, Non-U.S. Gov't
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