Cardiac sympathetic denervation in Ross syndrome demonstrated by MIBG-SPECT.
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.
Druschky
K
K
Department of Neurology, University of Erlangen-Nuernberg, Erlangen, Germany. achim.druschky@rzmail.uni-erlangen.de
Hilz
M J
MJ
Koelsch
C
C
Platsch
G
G
Neundoerfer
B
B
D019797:3-Iodobenzylguanidine; D006321:Heart / Q000000981:diagnostic imaging* / Q000294:innervation* / Q000503:physiopathology; D006801:Humans; D007007:Hypohidrosis / Q000000981:diagnostic imaging* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D019275:Radiopharmaceuticals; D012021:Reflex, Abnormal / Q000502:physiology*; D013564:Sympathetic Nervous System / Q000000981:diagnostic imaging* / Q000503:physiopathology; D013577:Syndrome; D015899:Tomography, Emission-Computed, Single-Photon; D015845:Tonic Pupil / Q000000981:diagnostic imaging* / Q000503:physiopathology
D019275:Radiopharmaceuticals; D019797:3-Iodobenzylguanidine