Horner syndrome: tribute to Professor Horner on his 190^th birthday

Síndrome de Horner: tributo ao Professor Horner em seu 190^o. aniversário

Abstract

This paper reviews some aspects of the life and work of Professor Johann Friedrich Horner, on the occasion of the 190th anniversary of his birthday and 152 years after the publication of "Über eine Form von Ptosis". It also shows the importance of the historical description of ptosis, myosis and anhidrosis associating those symptoms with sympathetic cervical damage. He pharmacologically confirmed the impairment of sympathetic innervation to the eye and preserved parasympathetic function.

Keywords:
Horner's syndrome; Ptosis; Myosis; Stroke

Resumo

Os autores revisam alguns aspectos da vida e obra do professor Johann Friedrich Horner, pelos 190 anos de seu aniversário e 152 anos após a publicação de "Über eine Form von Ptosis" e mostra a importância da descrição histórica da ptose, miose e anidrose, associando estes sintomas com a lesão do sistema simpático cervical. Ele confirmou farmacologicamente o comprometimento da inervação simpática do olho com função parassimpática preservada.

Palavras-chave:
Síndrome de Horner; Ptose; Miose; Doença cerebrovascular

In 1869 Johann Friedrich Horner (Figure 1), a Swiss ophthalmologist, described the case of a 40-year-old woman with nonspecific headache, ptosis, myosis and right anhidrosis. He also noted a distinct flushing and warmth of the ipsilateral hemiface, observing that the patient was not sweating on that side of her face, which remained cold and pale. He also observed increased skin temperature and dryness of the ipsilateral face. There was no change in facial sensitivity showing integrity of the trigeminal nerve, with right vasomotor paralysis. He pharmacologically confirmed the impairment of sympathetic innervation to the eye after noting poor dilation of the affected pupil following instillation of atropine and preserved pupillary constriction to the parasympathomimetic agent calabar, containing physostigmine. He associated those symptoms with sympathetic cervical damage1.

Figure 1.
Johann Friedrich Horner, from https://www.wikidata.org/wiki/Q123315.

The sympathetic pathway to the eye and its attachments is a three-neuron pathway, which begins in the central nervous system (Figure 2). The first order neuronal fibers originate from the posterolateral hypothalamus. They descend through the brain stem to end at the spinal cord at the ciliospinal center (C8-T2). The second order (preganglionic) neuronal fibers leave the T1 root and travel close to the pulmonary apex through the paravertebral sympathetic chain and the stellate ganglion and end in the upper cervical ganglion. Tumors involving the upper lobe of the lung can interrupt the pathway at this level. Third-order (post-ganglionic) sympathetic fibers leave the ganglion to form a plexus around the internal carotid artery. Dissection of the internal carotid artery is a very important cause that can lead to interruption of the pathway at this level2.

Figure 2.
Schematic diagram of the autonomic innervation of the eye. Adapted from https://en.wikipedia.org/wiki/Internal_carotid_plexus#/media/File:Gray840.png.

The plexus then rises to the cavernous sinus, and follows the ophthalmic division of the trigeminal nerve to the orbit, where it feeds the dilating muscles of the iris and the smooth muscle fibers of the upper and lower eyelids. The vasomotor fibers and the sweat glands to the face take a different course after leaving the ganglion. They follow the external carotid artery to supply half of the face on the same side. Horner syndrome (HS) can result from an interruption, at any level, of the sympathetic fibers supplying the eye and face2.

Diagnosis of HS is based on clinical observation but may be confirmed by pharmacological testing, as the prevailing features are readily apparent. They include miosis, partial ptosis, apparent enophthalmos and anhidrosis (Figure 3). Less constant to rare features include (on the affected side): facial flushing, arteriolar or venular dilatation (best seen in the retina), transient lowering of intraocular pressure, hemi-atrophy of the face and iris heterochromia (in congenital HS). Visual acuity is rarely affected. Other symptoms and signs can be present due to compression of surrounding structures by the offending lesion3. The pupillary abnormality HS is an anisocoria with a smaller pupil on the affected side. This is best identified by comparing the degree of anisocoria in bright light and in darkness with the greatest degree of anisocoria expected 5−7s after the lights are turned off because of a deficiency of sympathetic tone to the dilator muscle in the affected eye.

Figure 3.
Clinical characteristics of HS. Source: Nautiyal A, Singh S, DiSalle M, O'Sullivan J7

Johann Friedrich Horner was the second child of Dr. Solomon Horner (1801-1852) and Magdalena Zellner (1804-1852) born on 27 March 1831. He started as a medical student at the University of Zürich in April 1849. He completed his medical studies in 1854 with a thesis entitled: Über die krümmung der Wirbelsäule im aufrechte stehen (“On the curvature of the vertebral column in the upright position”). His educational tour took him to Vienna, Prague and Berlin, where he met von Gräefe, who taught him the modern, scientific methods of ophthalmology4.

In Paris he visited exponents of the area such as Broca and Desmarres, returning to Zurich in 1856. In 1862 he was appointed extraordinary professor, and in 1873 full professor. In more than 30 years of career he published 38 articles, the best known "Über eine Form von Ptosis" in 1869, like Horner curiosity does not add any reference in this paper1. In 1873, his student William Nicati published the thesis “La paralysie du nerf sympathique cervical”, in which a patient named Anna Brändli, previously described by Horner, returns as patient number 15. In the first chapter there is a historical introduction that clearly shows that the author was familiar with the content of the works of Claude Bernard and François Pourfour du Petit4^,5^,6.

According to Van der Wiel, “an impression about his character can be obtained by reading his autobiography, (...) he was very good with his poor patients, 50% of his private practice, who were treated free of charge. They were even admitted to his clinic, the Hottingerhof, where they were treated free of charge as well. He had an extremely good memory and could remember patients' faces for many years. Horner had a keen judgment of business and people, he did not allow himself to be ruled by emotions and focused on realistic goals”4.

Professor Horner was not only the founder of modern Swiss scientific ophthalmology but also the moving spirit behind improvements in social and medical affairs. Horner died at the age of 55, on December 20, 1886, after a stroke in the left hemisphere4.

Recognizing HS is critical, as those findings point to a lesion in the oculosympathetic pathway. Though often benign or idiopathic, the cause of HS can be very threatening or even lethal, so understanding how to recognize, diagnose, and appropriately evaluate HS is important to all clinicians3. Horner was the first one to give a detailed, scientifically supported account and to accurately interpret the signs of cervical sympathetic nerve damage in a human subject.

Our aim is to draw attention to HS and to paye a modest tribute to the 19th century master of neuro-ophthalmology for the 190th anniversary of his birthday.

References

• 1. Horner JF. Über eine Form von Ptosis. Klinische Monatsblätter für Augenheilkunde. 1869;7:193-8. Deutsch. Available from: https://babel.hathitrust.org/cgi/pt?id=uc1.b4114981;view=1up;seq=213
  » https://babel.hathitrust.org/cgi/pt?id=uc1.b4114981;view=1up;seq=213
• 2. Kong YX, Wright G, Pesudovs K, O’Day J, Wainer Z, Weisinger HS. Horner syndrome. Clin Exp Optom. 2007 Sep;90(5):336-44. https://doi.org/10.1111/j.1444-0938.2007.00177.x
  » https://doi.org/10.1111/j.1444-0938.2007.00177.x
• 3. Martin TJ. Horner syndrome: A clinical review. ACS Chem Neurosci. 2018;9(2):177-86. https://doi.org/10.1021/acschemneuro.7b00405
  » https://doi.org/10.1021/acschemneuro.7b00405
• 4. Van der Wiel HL. Johann Friedrich Horner (1831-1886). J Neurol. 2002 May;249(5):636-7. https://doi.org/10.1007/s004150200079
  » https://doi.org/10.1007/s004150200079
• 5. Proctor C, Chavis PS. Horner syndrome. J Neuroophthalmol. 2013 Mar;33(1):88-9. https://doi.org/10.1097/wno.0b013e318270ab95
  » https://doi.org/10.1097/wno.0b013e318270ab95
• 6. Dafereras M, Sapouridis H, Laios K, Chrysikos D, Mavrommatis E, Troupis T. The pioneer ophthalmologist Johann Friedrich Horner (1831-1886) and the clinical anatomy of the homonymous syndrome. Acta Chir Belg. 2020 Oct;120(5):363-5. https://doi.org/10.1080/00015458.2020.1746528
  » https://doi.org/10.1080/00015458.2020.1746528
• 7. Nautiyal A, Singh S, DiSalle M, O’Sullivan J. Painful Horner syndrome as a harbinger of silent carotid dissection. PLoS Med. 2005 Jan;2(1):e19. https://doi.org/10.1371/journal.pmed.0020019
  » https://doi.org/10.1371/journal.pmed.0020019

Publication Dates

• Publication in this collection
  11 June 2021
• Date of issue
  July 2021

History

• Received
  14 Oct 2020
• Reviewed
  19 Nov 2020
• Accepted
  17 Dec 2020