Huntington’s disease: Effect of cystearnine, a somatostatin-depleting agent

C. Shults, L. Steardo, P. Barone, E. Mohr, J. Juncos, C. Serrati, P. Fedio, C. A. Tamminga, T. N. Chase

Research output: Contribution to journalArticle

30 Scopus citations

Abstract

Somatostatin levels in the basal ganglia are elevated in Huntington’s disease. A controlled therapeutic trial of the somatostatin-depleting agent, cysteamine, was therefore conducted in five patients, including one with the rigid-akinetic form. Maximum tolerated dosage for 2 weeks produced no consistent change in extrapyramidal or dementia scores. Somatostatin concentrations were not significantly altered in plasma or CSF. Growth hormone levels, on the other hand, more than doubled, suggesting a functionally significant decrease in central somatostatin levels.

Original languageEnglish (US)
Pages (from-to)1099-1102
Number of pages4
JournalNeurology
Volume36
Issue number8
StatePublished - Aug 1986

ASJC Scopus subject areas

  • Clinical Neurology

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    Shults, C., Steardo, L., Barone, P., Mohr, E., Juncos, J., Serrati, C., Fedio, P., Tamminga, C. A., & Chase, T. N. (1986). Huntington’s disease: Effect of cystearnine, a somatostatin-depleting agent. Neurology, 36(8), 1099-1102.