Adrenergic dysfunction in hereditary adult-onset leukodystrophy.
We studied the pressor response to norepinephrine infusion in patients with an autosomal dominant adult-onset leukodystrophy. We also examined cardiovascular and catecholamine responses to insulin-induced hypoglycemia. A parallel shift to the left of the norepinephrine dose response curve, in conjunction with low baseline plasma norepinephrine levels, was consistent with denervation supersensitivity, suggesting a distal lesion of sympathetic noradrenergic neurons. Absence of the epinephrine response to insulin-induced hypoglycemia indicated that autonomic neuropathy was attended by severe adrenal medullary dysfunction.
Brown
R T
RT
Polinsky
R J
RJ
Schwankhaus
J
J
Eldridge
R
R
McFarland
H
H
Schlesinger
S
S
Dailey
W A
WA
D000328:Adult; D000368:Aged; D001786:Blood Glucose / Q000378:metabolism; D001794:Blood Pressure / Q000187:drug effects; D001927:Brain Diseases / Q000097:blood / Q000235:genetics* / Q000503:physiopathology; D004837:Epinephrine / Q000097:blood; D006801:Humans; D007328:Insulin / Q000494:pharmacology; D008875:Middle Aged; D009638:Norepinephrine / Q000097:blood / Q000494:pharmacology / Q000502:physiology*
D001786:Blood Glucose; D007328:Insulin; D009638:Norepinephrine; D004837:Epinephrine