Classic Triad of Ross Syndrome with Diffuse Autonomic Dysfunction and Positive Antinuclear Antibody Titre

Abstract

A 23-year-old woman presented with left-sided hemifacial flushing and hyperhidrosis with right-sided anhidrosis. Examination showed a dilated right pupil and a slightly constricted left pupil with bilateral sluggish vermiform movements and light-near dissociation. A neurological examination showed absent deep tendon reflexes. Magnetic resonance imaging of the brain, neck, and spine was normal. These features are consistent with Ross syndrome. Ross syndrome is characterized by Holmes-Adie syndrome (tonic pupil, areflexia of deep tendon reflexes) and segmental anhidrosis and lies within the spectrum of peripheral, partial autonomic dysfunction syndromes together with harlequin and Horner syndromes. In addition to the classic triad of Ross syndrome, the patient had diffuse, systemic autonomic dysfunction with a positive antinuclear antibody test and a positive ribonucleoprotein antibody titre.

KEYWORDS::

• Adie syndrome
• Areflexia
• Autonomic dysfunction
• Hyperhidrosis
• Tonic pupil

Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.