Ross syndromeR 61.0

Ross syndrome is a very rare (< than 100 cases have been reported since it was first described in 1958) neurological-dermatological disorder characterized by the simultaneous presence of areflexia and tonic pupil, decreased or absent segmental sweat secretion (hypohidrosis), and other functional disturbances (Nolano M et al 2006). These include: tonic pupillary contraction , attenuated muscle reflexes, orthostatic hypotension, vasovagal syncope, dyspnea, headache, reflux esophagitis, irritable colon and psychiatric changes (Damagatla M et al 2020). The unilateral segmental hypohidrosis is compensated on the opposite side by an increased sweat secretion (hyperhidrosis), which affects the affected patients most subjectively.

Unchanged number and morphology of sweat glands. By means of morphometric analysis, a reduced cholinergic innervation of the sweat glands in the hypohidrotic skin could be proven, findings which indicate a selective degenerative process of the cholinergic sudomotor neurons (Sommer C et al. 2002).

By ophthalmologists and neurologists. Avoid extreme temperatures and exsiccative measures such as long and hot showers, hot baths, etc. Bland care with moisturising external agents such as Ungt. emulsif. aq., if necessary with urea additive.

Hyperhidrotic body areas can often be successfully treated with tap water iontophoresis (direct current, 15 Volt, 25 mA) (for exact procedure see there). The commercially available plastic tubs (with electrode connection) are usually designed for hands and feet and must be handmade to fit the affected body area if necessary.

Alternatively, intradermal botulinum injections can be used, which are distributed fan-shaped under hyperhidrotic areas.

1. Agarwala MK et al (2016) Ross Syndrome: A Case Report and Review of Cases from India. Indian J Dermatol 61:348.
2. Chakravarty A et al (2003) Ross syndrome--a case documentation. Acta Neurol Scand 107: 72-73.
3. Damagatla M et al (2020) Ross syndrome. Neuroophthalmology 44:201-203.
4. Itin P et al (1992) Ross syndrome. Dermatologist 43: 359-360
5. Nolano M et al. (2006) Ross syndrome: a rare or a misunderstood disorder of thermoregulation? A skin innervation study on 12 subjects. Brain 129:2119-2131.
6. Reinauer S et al. (1992) Ross syndrome: redefinition and therapy of associated hyperhidrosis by a modified iontophoresis technique. Z Hautkr 67: 615-621
7. Ross AT (1958) Progressive selective sudomotor denervation. A case with coexisting Adie's syndrome. Neurology 8: 809-817
8. Sommer C et al (2002) Selective loss of cholinergic sudomotor fibers causes anhidrosis in Ross syndrome. Ann Neurol 52:247-250.
9. Weller M, Wilhelm H, Sommer N et al (1992) Tonic pupil, areflexia, and segmental anhidrosis: two additional cases of Ross syndrome and review of the literature. J Neurol 239: 231-234
10. Yasar S et a. (2010) Ross syndrome: unilateral hyperhidrosis, Adie pupils, and diffuse areflexia. JDDG 12: 1004-1006