TY - JOUR
T1 - Huntington’s disease
T2 - Effect of cystearnine, a somatostatin-depleting agent
AU - Shults, C.
AU - Steardo, L.
AU - Barone, P.
AU - Mohr, E.
AU - Juncos, J.
AU - Serrati, C.
AU - Fedio, P.
AU - Tamminga, C. A.
AU - Chase, T. N.
PY - 1986/8
Y1 - 1986/8
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=0022536654&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0022536654&partnerID=8YFLogxK
U2 - 10.1212/wnl.36.8.1099
DO - 10.1212/wnl.36.8.1099
M3 - Article
C2 - 2874527
AN - SCOPUS:0022536654
SN - 0028-3878
VL - 36
SP - 1099
EP - 1102
JO - Neurology
JF - Neurology
IS - 8
ER -