Article

Cardiac sympathetic denervation in Ross syndrome demonstrated by MIBG-SPECT.

Department of Neurology, University of Erlangen-Nuernberg, Erlangen, Germany.
Journal of the Autonomic Nervous System 06/1999; 76(2-3):184-7. 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.

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