Two Cases of Tonic Pupil: Ross and Ross Syndrome Plus.

Reyaz Ahmad,Kumar Saurabh

doi:10.7759/cureus.22305

Abstract

Ross syndrome is a rare disorder of the peripheral autonomic nervous system characterized by a triad of tonic pupils with light-near dissociation, segmental anhidrosis, and areflexia. Though having a benign course, the disease can cause significant social embarrassment. Both our cases presented with complaints of segmental facial hyperhidrosis. The first case with a one-year history had findings of segmental anhidrosis up to T4 thoracic level, left tonic pupil, and absent right ankle reflex. While the second case with a history of five years had bilateral tonic pupil, absent lower limb reflexes, anhidrosis of left face, neck, and upper trunk up to T4 level, apart from having associated Horner’s syndrome. Minor’s (starch-iodine) test and dilute pilocarpine test were helpful for diagnosis in both cases, indicating areas of anhidrosis and pupillary cholinergic denervation hypersensitivity respectively. Both cases were provided counseling and managed conservatively.

Highlights

Ross syndrome was first described by A.T
It is a disorder of the peripheral nervous system characterized by a triad of the tonic pupil with light-near dissociation, areflexia, and segmental hypohidrosis
Both the patients presented with a triad of the tonic pupil with light-near dissociation, segmental anhidrosis with compensatory hyperhidrosis, and areflexia

Summary

Introduction

Ross syndrome was first described by A.T. Ross in 1958 [1]. The patient gave no history of cough, breathing difficulty, rashes, or joint pain. She had no history of sexually transmitted diseases (STDs). Cranial nerve second examinations showed normal fundus and visual fields in both eyes. Reaction to convergence (near reflex) was present indicating light-near dissociation (Figure 2) Both direct and consensual light reflexes were sluggish in the left eye (indicating third nerve involvement). Starch- iodine test showed absent sweating on the right side of the face, neck, and upper trunk up to T4 level (Figure 4). Cranial nerve second examination revealed fundus, visual fields, and acuity (bilateral 6/6 meters) were within normal limits.

Discussion

Conclusions

Disclosures

Findings

Ross AT

12. Thompson HS