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Is Ross Syndrome an Autoimmune Entity? A Case Series of 11 Patients. | LitMetric

AI Article Synopsis

  • Ross syndrome is characterized by segmental anhidrosis, areflexia, and tonic pupils, with fewer than 60 documented cases, and possible autoimmune links suggested by the presence of antibodies in patients.
  • A case series of 11 patients revealed a near-equal gender distribution, mean symptom onset age of 26, and an average delay of 6 years before reaching a specialist, with most presenting excessive sweating.
  • Despite 89% showing absent sympathetic skin response and only 45% displaying all classic symptoms, the investigation found no autoimmunity markers, indicating that the syndrome's causes may not be purely autoimmune.

Article Abstract

Background: Ross syndrome is diagnosed by the presence of segmental anhidrosis, areflexia, and tonic pupils. Fewer than 60 cases have been described in literature so far. There have been reports of presence of antibodies in such patients, suggesting an autoimmune pathogenesis.

Methods: We describe the clinical profile in this case series of 11 patients with Ross syndrome and discuss the current status of autoimmunity in its pathogenesis and the management.

Results: Of the 11 patients with Ross syndrome there was an almost equal sex distribution (male:female ratio was 1.17:1) and the mean age of onset of symptoms was 26 years. Patients took an average of 6 years to present to a tertiary center. Sixty-three percent of the patients presented with complaints of excessive sweating, whereas only 27% had complaints of decreased sweating over a particular area of the body. Only 45% of the patients had the complete triad of Ross syndrome, which included segmental anhidrosis, tonic pupil, and absent reflexes. Eighty-nine percent of the patients had documented absent sympathetic skin response on electromyography. The various markers of autoimmunity were negative in all patients who were investigated for the same in this series. Ninety percent of the patients were managed conservatively.

Conclusions: These findings suggest that, in Ross syndrome, generalized injury to ganglion cells or their projections are not purely autoimmune-mediated.

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Source
http://dx.doi.org/10.1017/cjn.2016.417DOI Listing

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