Horner Syndrome
Horner syndrome — also known as oculosympathetic paresis — is a neurological condition caused by disruption of the sympathetic pathway to the eye and face. It is characterized by the classic triad of miosis, partial ptosis, and anhidrosis +/- enophthalmos
Clinically, it presents with the characteristic triad of ptosis, miosis, and anhidrosis. In some cases, enophthalmos and facial flushing may also be observed. The syndrome reflects impairment along a three-neuron arc extending from the hypothalamus to the eye, and lesions may be classified as central (first-order), pre-ganglionic (second-order), or post-ganglionic (third-order).
Aetiologies range from benign to life-threatening and include trauma, carotid artery dissection, malignancy (e.g., Pancoast tumour), spinal cord lesions, and congenital malformations. In children, birth trauma and neuroblastoma are notable causes. The condition may also occur idiopathically.
Diagnosis is clinical, supported by pharmacologic tests such as apraclonidine, cocaine, or hydroxyamphetamine drops to localise the lesion. Management centres on identifying and treating the underlying pathology.
The syndrome is eponymously attributed to Swiss ophthalmologist Johann Friedrich Horner, who published a comprehensive case in 1869. However, earlier descriptions exist, notably by François Pourfour du Petit, Edward Selleck Hare, and Silas Weir Mitchell, prompting historical debate over attribution. In France, the condition is known as Bernard-Horner syndrome, acknowledging Claude Bernard’s experimental work.
Horner’s syndrome is present in Wallenberg syndrome, Babinski-Nageotte syndrome, Cestan-Chenais syndrome, Klumpke syndrome, Villaret syndrome, and Raeder syndrome.
History of Horner Syndrome
1727 – French anatomist and surgeon François Pourfour du Petit (1664-1741) was the first to describe the ocular effects of cervical sympathetic injury in animals. In his paper Sur ce que le nerf intercostal fournit des Esprits aux yeux, he described ocular effects (miosis and ptosis) following cervical spinal transection in dogs:
Si l’Intercostal étant coupé à un Animal , il s’en ensuit des effets sensibles dans les Yeux, et qui ne puissent être rapportes à aucune autre cause…
1795 – William Cruikshank (1745–1800) Performed cervical sympathetic dissections under the direction of John Hunter, noting ocular changes and structural details of the cervical sympathetic chain, but made no clinical correlations.
1838 – British surgeon Edward Selleck Hare (1812-1838) described the case of a man who had died of a tumour on his neck. On examination he described ptosis and miosis in a patient with a cervical tumour compressing the brachial plexus. The clinical case author Hare, died on September 28, 1838, a day before the communication was published.
…a small tumour in the inferior triangular space on the left side of the neck…the pupil of the left eye became contracted; and the levator palpebrae ceased to perform its office.
1839 – Scottish physiologist John Reid (1809-1849) drew attention to Hare’s case, and asked whether injury to the sympathetic chain in man might not cause constriction of the pupil as he had found it to do in animals.
…compression or section of the trunk of the sympathetic in the neck in dogs and cats is instantly followed by contraction of the pupil, the forcing of the cartilaginous membrane over the inner part of the anterior surface of the eyeball, the retraction of the eyeball deeper into the socket, and a slight approximation of the eyelids.
1852 – French physician and physiologist Claude Bernard (1813-1878) described vasomotor control by the cervical sympathetic nerve in rabbits. Bernard conducted severed the cervical sympathetic nerve and meticulously documented the ensuing physiological changes. He coined the concept of “vasomotor nerves.” and is recognised in France as co-eponym: Bernard-Horner syndrome.
After the section of the cephalic branch of the great sympathetic, it is possible to observe a contraction of the pupil of the corresponding eye, accompanied by a narrowing of the palpebral opening, a retraction of the ocular globe, and an increase of the circulation, as well as of the temperature, in all parts of the corresponding face
1864 – American neurologist Silas Weir Mitchell (1829-1914) detailed the classic clinical triad in a 24 year old soldier with a gunshot wound to the right side of his neck:
The pupil of the right eye is very small, that of the left eye unusually large. There is a slight, but very distinct ptosis of the right eye and its outer angle appears as though it were dropped a little lower than the inner angle…his face became distinctly flushed on the right side only when walking in warm weather…a case of injury of the sympathetic nerve, probably the only one recorded.
1865 – Sir Jonathan Hutchinson (1828-1913) reported further cases with sympathetic denervation but did not claim eponym.
1869 – Swiss ophthalmologist Johann Friedrich Horner (1831-1886) reported the findings of ptosis, miosis, enophthalmos in a 40-year-old peasant woman in Über eine Form von Ptosis. He also observed increased skin temperature and dryness of the ipsilateral face.
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. [1869;7:193-198]
Original
English
Frau Anna Brändli, 40 Jahre alt, eine gesund aussehende Bäuerin mittlerer Grösse… bemerkte ein allmähliges herabsinken des rechten oberen Augenlides, das sehr langsam zunahm… Das obere Lid deckt die rechte Cornea bis an den oberen Pupillarrand… Die Pupille des rechten Auges ist bedutend enger als diejenige des linken… Nun erst erzählte uns die Patientin, dass sie rechterseits nie geschwitzt habe
Die Untersuchungen ergaben also Integrität des sensiblen Trigeminus, transitorische Lähmung des vasomotorischen Fasern im rechtseitigen Trigeminusgebiet;… (es) wundert sich Niemand, wenn ich diese… nie vollständige Ptosis als Lähmung des vom Sympathicus versorgten organischen Musc. palpebral super. ansehe.
Anna Brändli, aged 40, healthy looking peasant woman…noticed a slight drooping of her right upper eyelid, which increased very gradually… The upper lid covers the right cornea to the upper edge of the pupil; the lid is not loose or wrinkled but somewhat sunken into the orbit and is still capable of movement; it is neither injected nor swollen.
The pupil of the right eye is considerably more constricted than that of the left, but reacts to light; the globe has sunk inward very slightly. During the clinical discussion … the right side of her face became red and warm; while the left side remained pale and cool.
The vasomotor disturbance involves not only the trigeminal area, but also the fibres of the cervical sympathetic; this experiment with belladonna and calabar speaks for the dual control of the movements of the iris in man… we are dealing with right dilator paralysis. Ptosis a paralysis of the musculus palpebrae superioris supplied by the sympathetic nerve (H. Muller, Harling), and the appearance of the upper lid as part and parcel of the whole symptom-complex.
1873 – Horner’s pupil William Nicati (1850- 1931) popularised Horner’s work via thesis, acknowledging Pourfour du Petit and Bernard. He published a monograph popularising the eponym “Horner’s syndrome” despite earlier literature largely ignoring the term in English.
Associated Persons
- François Pourfour du Petit (1664–1741) — First to describe the ocular signs experimentally.
- Edward Selleck Hare (1812-1838) — First published human clinical case.
- John Reid (1809–1849) — Experimental confirmation of sympathetic control over ocular function.
- Claude Bernard (1813-1878) — Defined the vasomotor role of the cervical sympathetic.
- John William Ogle (1824-1905) — Reviewed literature and defined the clinical triad in animals
- Silas Weir Mitchell (1829-1914) — Early full human description of the classical triad.
- Sir Jonathan Hutchinson (1828-1913) — Supplemented early human cases.
- Johann Friedrich Horner (1831-1886)— Provided detailed clinical, anatomical, and pharmacological correlation.
Alternative names
- Horner’s syndrome
- syndrome de Horner
- Bernard-Horner syndrome (France)
- Oculosympathetic paresis
- Von Passow syndrome
References
Original articles
- Petit. Sur ce que le nerf intercostal fournit des Esprits aux yeux. Histoire de l’Académie royale des sciences, 1727: 9-13
- Hare ES. Tumor involving certain nerves. London Medical Gazette 1838; 23: 16-18
- Reid J. On the Effects of Lesion of the Trunk of the Ganglionic System of Nerves in the Neck upon the Eyeball and Its Appendages. Edinb Med Surg J. 1839 Jul 1;52(140):36-43.
- Bernard C. Recherches expérimentales sur le grand sympathique et spécialement sur l’influence que le section de ce nerf exerce sur la chaleur animal. 1852
- Ogle JW. On the Influence of the Cervical Portions of the Sympathetic Nerve and Spinal Cord upon the Eye and its Appendages, illustrated by Clinical Cases, with Observations. Med Chir Trans. 1858; 41:3 97-440
- Mitchell SW, Morehouse GR, Keen WW. Chapter IV: Wounds of special nerves; wound of the sympathetic nerve. in: Gunshot Wounds and Other Injuries of Nerves. 1864: 39-44
- Horner JF. Über eine Form von Ptosis. Klinische Monatsblätter für Augenheilkunde 1869;7:193-198
- Nicati W. La paralysie du nerf sympathique cervical. Dissertation inaugurale présentée a la faculté de médecine de Zürich. Lausanne et Paris, 1873.
Eponymous review
- von Passow A. Okulare Paresen im Symptomenbilde des “Status dysraphicus”, zugleich ein Beitrag zur Ätiologie der Sympathikusparese (Horner-Syndrom und Heterochromia iridis). Münchener medizinische Wochenshrift, 1934; 74: 1243-1249.
- Kisch B. Horner’s syndrome, an American discovery. Bull Hist Med. 1951 May-Jun;25(3):284-8.
- Onuigbo WI. John Reid (1809-49) and Horner’s syndrome. Scott Med J. 1958 May;3(5):218-20.
- Durham DG. Congenital hereditary Horner’s syndrome. AMA Arch Ophthalmol. 1958 Nov;60(5):939-40.
- Miller JM. Whose eponym? The case for Edward Selleck Hare. Md Med J. 1994 Jun;43(6):498-9.
FOAMed review and cases
- LITFL Clinical Case – Ophthalmology Befuddler 025
- CCC – Horner syndrome
eponymictionary
the names behind the name
