17/02/2012
Ross syndrome is an uncommon disorder characterized by the triad of segmental hypohidrosis* (or segmental anhidrosis*), hyporeflexia, and tonic pupils.
Maria Nolano et al. (2006) gave a nice description of Ross Syndrome :
'Ross syndrome is described as a rare clinical disorder of unknown cause, characterized by the triad of tonic pupil, hyporeflexia and segmental anhidrosis. Since Ross's first description, 40 cases have been described in literature. Whether this condition is nosographically distinct from Holmes–Adie syndrome (tonic pupil and hyporeflexia) and from harlequin syndrome (segmental hypohidrosis without pupillar abnormalities) is not clear. Shin et al. (2000) suggested that all these conditions could represent different expressions of the same disorder.'
This last suggestion might be true, because several cases have been described in which the patient developed a Holmes-Adie syndrome first and a Harlequin syndrome later. Perhaps we may say :
Holmes-Adie Syndrome + Harlequin Syndrome = Ross Syndrome ?
In 1958, Alexander T. Ross described for the first time a case of tonic pupil and areflexia of the lower extremities (Holmes-Adie syndrome) and segmental hypohidrosis.
Ross syndrome seems to be very rare, because only a few cases have been described in literature. It's obvious that the separate syndromes of Holmes-Adie and Harlequin appear more often than the combination of these syndromes, the Ross syndrome.
On the other hand there are certainly more cases than already diagnosed :
1.First of all the number of described cases is just a small percentage of the real number of cases : Not every case will be examined for a medical report...
2.Secondly many cases will not be recognised because many GP's and neurologists don't know this very rare syndrome.
3.Moreover many cases of Harlequin syndrome and Holmes-Adie syndrome are only 'investigated' by appearance (without further tests). If these cases would be checked properly (for as well Harlequin syndrome as Holmes-Adie syndrome) certainly more cases of Ross syndrome would be found...
4.If people do not experience problems because of a syndrome, they probably will never visit a GP. In that case they might never know that they have a syndrome.
Maria Nolano et al. (2006) have investigated 12 cases of Ross Syndrome. About the clinical history she wrote :'There were several common features in the clinical history of all our patients: an unpleasant feeling in a warm environment since childhood; excessive sweating in some areas of the body that ‘had been getting better over time’; frequent episodes of fever and sometimes fainting during the summer with an increased heart rate just before the heat became unbearable; dryness of the skin especially of the legs and hands that worsened over time with progressive loss of the ability to leaf through a book; the need to wear sun glasses outside; early fatigue during physical exercise; gastrointestinal disorders such as irritable bowel syndrome or constipation.'
* Anhidrosis is the complete abscence of sweating, as opposed to hypohidrosis which is sweating less than normal.
