Ross syndrome
Keywords:
Síndrome de Ross, disautonomía, hiperhidrosis, anhidrosis segmentaria.Abstract
Introduction: Ross syndrome is a rare entity of the nervous system classified within dysautonomic syndromes, with few cases reported in the literature since its description in 1958. It more frequently affects men than women between the ages of 3 and 50 years and, as a characteristic clinical triad, manifests as segmental anhidrosis, Adie’s tonic pupil, and osteotendinous areflexia.
Objective: To present the clinical, pathophysiological, and therapeutic features of a patient with Ross syndrome.
Case report: A 58-year-old male patient is presented with left-sided hypohidrosis and contralateral hyperhidrosis of 20 years’ duration, accompanied by osteotendinous areflexia, chronic diarrhoea, and hypotonic pupils. Due to its rarity and limited awareness, clinical suspicion is warranted, as although this disorder does not cause severe impairments, it affects the quality of life of those affected.
Conclusions: Dysautonomic disorders that produce anhidrosis or hyperhidrosis can accompany multiple diseases in daily clinical practice and, therefore, should be recognized by various medical specialties, such as dermatology, neurology, internal medicine, and ophthalmology, to enable timely diagnosis. Ross syndrome does not appear to be as rare as once thought, with increasing reports emerging in the scientific literature, highlighting the need for further study of disorders of the sympathetic and parasympathetic nervous systems, their underlying pathophysiology, and the therapeutic options available for the timely treatment of affected patients.
Downloads
References
1. Ross AT. Progressive selective sudomotor denervation; a case with coexisting Adie's syndrome. Neurology. 1958;8 (11):809. https//doi.org/10.1212/WNL.8.11.809.
2. Mayer H. Bilateral Tonic Pupils Secondary to Ross Syndrome: A Case Report. Journal of Optometry. 2014; 7(2): 106–107. https://doi.org/10.1016/j.optom.2013.06.005
3. Hamadeh G, Fares J. Concurrencia de hiperhidrosis e hipohidrosis en el síndrome de Ross. Clin Med Res. 2023 Marzo; 21(1):49-52. DOI: 10.3121/cmr.2022.1780. PMID: 37130790; PMCID: PMC10153678.
4. Thaisetthawatkul, P. Pure autonomic failure. Curr. Neurol and Neurosci 2016. 8 (2016): 1-7. https://doi.10.1007/s11910-016-0673-2.
5. Lamotte G, Sandroni P, Cutsforth-Gregory JK, Berini SE, Benarroch EE, Shouman K, Mauermann ML, Anderson J, Low PA, Singer W, Coon EA. Presentación clínica y perfil autonómico en el síndrome de Ross. J Neurol. octubre de 2021; 268(10):3852-3860. DOI: 10.1007/s00415-021-10531-8. Epub 3 de abril de 2021. PMID: 33813643; PMCID: PMC9639164
6. Ababneh O, Khamees A, Qiblawi S. Classic Triad of Ross Syndrome with Diffuse Autonomic Dysfunction and Positive Antinuclear Antibody Titre. Neuro-ophthalmology. 2012;36(4):138–142.
7. Villanueva RJ. Biology of sweat glands and their disorders. I Normal sweat gland function. J Am Acad Dermatol. 1989;20:537-63.
8 Damagatla M, Ganne P, Upparakadiyala R, N P. Síndrome de Ross. Neurooftalmología. 12 de diciembre de 2019; 44(3):201-203. doi: 10.1080/01658107.2019.1669184. PMID: 32395175; PMCID: PMC7204520.
9. Zuhal Filikcia Hans-Henrik Horstenb Mette Lindelofa. Ross Syndrome: A Patient with a 23-Year History Case Rep Neurol 2020;12:132–135. https://doi: 10.1159/000507186.
10. Didem Mullaaziz, Aslı Feride Kaptanoglu, Amber Eker. Hypohidrosis or hyperhidrosis? Ross syndrome. Dermatologica sinica 2016 (34): 141e143. doi.org/10.1016/j.dsi.2015.10.004.
11. Manoj Kumar Agarwala, Leni George, Harshad Parmar, and Vivek Mathew. Ross Syndrome: A Case Report and Review of Cases from India. Indian J Dermatol. 2016 May-Jun; 61(3): 348. doi: 10.4103/0019-5154.182472.
12. Zahid A, Coon EA, Lamotte G. "Ningún hombre es una isla": patrón único de anhidrosis con islas de sudor conservado en el síndrome de Ross. Neurol Sci. 2021 Dic; 42(12):5399-5400. doi: 10.1007/s10072-021-05639-8. Epub 1 de octubre de 2021. PMID: 34595680.
13. Mingming Ma, Jing Yao, Yongkang Chen, Han Liu at all. ¿ Is Ross Síndrome a new type Nucleynopathy? A brief research report. Front. Neurosci. 2020; 08 de julio de 2020. https://doi.org/10.3389/fnins.2020.00635.
14. Hamadeh G, Fares J. Concurrence of Hyperhidrosis and Hypohidrosis in Ross Syndrome. Clin Med Res. 2023 Mar;21(1):49-52. doi: 10.3121/cmr.2022.1780. PMID: 37130790; PMCID: PMC10153678.
15. Ahmad R, Saurabh K. Two Cases of Tonic Pupil: Ross and Ross Syndrome Plus. Cureus. 2022 Feb 16;14(2):e22305. doi: 10.7759/cureus.22305. PMID: 35350498; PMCID: PMC8933650.
16. Lamotte G, Sandroni P, Cutsforth-Gregory JK, Berini SE, Benarroch EE, Shouman K, Mauermann ML, Anderson J, Low PA, Singer W, Coon EA. Clinical presentation and autonomic profile in Ross syndrome. J Neurol. 2021 Oct;268(10):3852-3860. doi: 10.1007/s00415-021-10531-8. Epub 2021 Apr 3. PMID: 33813643; PMCID: PMC9639164.
17. Donadio, V., Incensi, A., Cortelli, P., Giannoccaro, M. P., Jaber, M. A., Baruzzi, A., et al. Skin sympathetic fiber alpha-synuclein deposits: a potential biomarker for pure autonomic failure. Neurology 80, 2013. 725–732. doi: 10.1212/wnl.0b013e3182825127.
18. Kisielnicka A, Szczerkowska-Dobosz A, Purzycka-Bohdan D, Nowicki RJ. Hiperhidrosis: etiología, clasificación y manejo de la enfermedad a la luz de las modalidades de tratamiento modernas. Postepy Dermatol Alergol. Abril de 2022; 39(2):251-257. DOI: 10.5114/ada.2022.115887. Epub 9 de mayo de 2022. PMID: 35645673; PMCID: PMC9131949.
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2026 Revista Cubana de Neurologia y Neurocirugia

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

