Long-term treatment of juvenile Huntington's chorea with dipropylacetic acid.
Neurology
; 27(2): 193-7, 1977 Feb.
Article
em En
| MEDLINE
| ID: mdl-138101
ABSTRACT
Since the proposed mode of action of dipropylacetic acid, an anticonvulsant, is to increase central nervous system gamma-aminobutyric acid levels, we used this agent to treat identical twins with juvenile Huntington's chorea. Their clinical status did not improve immediately after they received dipropylacetic acid. Furthermore, long-term administration (over a year) of high doses of the agent (up to 2,400 mg per day; 92 mg per kilogram per day) did not seem to alter the slow progression of their disease. Prior to treatment with dipropylacetic acid, the twins had normal cerebrospinal fluid gamma-aminobutyric acid levels. In addition, cerebrospinal fluid 5-hydroxyindoleacetic acid and homovanillic acid were determined before and after 18 hours of high-dose probenecid. The former showed a normal threefold to fourfold increase after probenecid administration, but homovanillic acid had a distinctly subnormal turnover after probenecid, with only a threefold rather than the normal tenfold increase.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Valeratos
/
Ácido Valproico
/
Doença de Huntington
Tipo de estudo:
Diagnostic_studies
Limite:
Child
/
Female
/
Humans
Idioma:
En
Ano de publicação:
1977
Tipo de documento:
Article