Abstract
This the case of a 6 year old with segmental hypohidrosis in the right side of his body, ipsilateral tonic pupil and hiporreflexia. This triad leads to the clinical diagnosis of the Ross Syndrome. The relevance of this case is the recognition of the hypohidrosis as a sign of an autonomic disorder.
The Ross syndrome is a progressive benign autonomic disorder, the pathophysiology is the denervation of parasympathetic postganglionic cholinergic fibers between the ciliary ganglion and iris (pupil tonic), presynaptic degeneration of dorsal root fibers (hyporeflexia) and degeneration of the postganglionic sweat glands fibers. The cause is unclear.
The course is chronic, with variable extension of the hypohidrosis. Patients may get compromise of thermoregulation and impaired quality of life, that is why the patients and caregivers should be warning and learn the strategies to avoid body overheating.
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