Cardiac sympathetic denervation in Ross syndrome demonstrated by MIBG-SPECT

ArticleinJournal of the Autonomic Nervous System 76(2-3):184-7 · June 1999with9 Reads
DOI: 10.1016/S0165-1838(99)00018-1 · Source: PubMed

    Abstract

    We investigated cardiac sympathetic innervation by metaiodobenzylguanidine (MIBG) imaging in a patient with tonic pupils, loss of tendon reflexes, and segmental anhidrosis (Ross syndrome). Despite normal cardiovascular reflex tests, we observed a reduced global myocardial MIBG uptake as well as a regional uptake defect over the posterolateral cardiac territory indicating left ventricular peripheral sympathetic denervation. MIBG imaging seems to be a useful noninvasive diagnostic method for detection of early--possibly subclinical--cardiac autonomic impairment in Ross syndrome and provides further evidence of injury to postganglionic autonomic neurons as the underlying pathological mechanism of the disease.