Horner's syndrome

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Horner's syndrome
Other namesBernard-Horner syndrome (BH), oculosympathetic palsy
Miosis.jpg
Left-sided Horner's syndrome
Specialty Neurology   OOjs UI icon edit-ltr-progressive.svg

Horner's syndrome, also known as oculosympathetic paresis, [1] is a combination of symptoms that arises when a group of nerves known as the sympathetic trunk is damaged. The signs and symptoms occur on the same side (ipsilateral) as it is a lesion of the sympathetic trunk. It is characterized by miosis (a constricted pupil), partial ptosis (a weak, droopy eyelid), apparent anhidrosis (decreased sweating), with apparent enophthalmos (inset eyeball). [2]

Contents

The nerves of the sympathetic trunk arise from the spinal cord in the chest, and from there ascend to the neck and face. The nerves are part of the sympathetic nervous system, a division of the autonomic (or involuntary) nervous system. Once the syndrome has been recognized, medical imaging and response to particular eye drops may be required to identify the location of the problem and the underlying cause. [3]

Signs and symptoms

Signs that are found in people with Horner's syndrome on the affected side of the face include the following:

Interruption of sympathetic pathways leads to several implications. It inactivates the dilator muscle and thereby produces miosis. It inactivates the superior tarsal muscle which produces ptosis. It reduces sweat secretion in the face. Patients may have apparent enophthalmos (affected eye looks to be slightly sunken in) but this is not always the case. The ptosis from inactivation of the superior tarsal muscle causes the eye to appear sunken in, but when actually measured, enophthalmos is not present. The phenomenon of enophthalmos is seen in Horner's syndrome in cats, rats, and dogs. [5]

Sometimes there is flushing on the affected side of the face due to dilation of blood vessels under the skin. The pupil's light reflex is maintained as this is controlled via the parasympathetic nervous system.[ citation needed ]

In children, Horner's syndrome sometimes leads to heterochromia, a difference in eye color between the two eyes. [3] This happens because a lack of sympathetic stimulation in childhood interferes with melanin pigmentation of the melanocytes in the superficial stroma of the iris.[ citation needed ]

In veterinary medicine, signs can include partial closure of the third eyelid, or nictitating membrane.[ citation needed ]

Causes

Scheme showing sympathetic and parasympathetic innervation of the pupil and sites of a lesion in Horner's syndrome. Horner's Syndrome and Autonomic innervation of the eye.svg
Scheme showing sympathetic and parasympathetic innervation of the pupil and sites of a lesion in Horner's syndrome.

Horner's syndrome is usually acquired as a result of disease, but may also be congenital (inborn, associated with heterochromatic iris) or iatrogenic (caused by medical treatment). In rare cases, Horner's syndrome may be the result of repeated, minor head trauma, such as being hit with a soccer ball. Although most causes are relatively benign, Horner's syndrome may reflect serious disease in the neck or chest (such as a Pancoast tumor (tumor in the apex of the lung) or thyrocervical venous dilatation).[ citation needed ]

Causes can be divided according to the presence and location of anhidrosis:[ citation needed ]

Pathophysiology

Horner syndrome is due to a deficiency of sympathetic activity. The site of lesion to the sympathetic outflow is on the ipsilateral side of the symptoms. The following are examples of conditions that cause the clinical appearance of Horner's syndrome: [7]

If patients have impaired sweating above the waist affecting only one side of the body, and they do not have clinically apparent Horner's syndrome, then their lesions are just below the stellate ganglion in the sympathetic chain.[ citation needed ]

Diagnosis

Left-sided Horner's syndrome in a cat as a result of trauma, demonstrating miosis in left pupil. Example of Horner's syndrome in a cat.jpg
Left-sided Horner's syndrome in a cat as a result of trauma, demonstrating miosis in left pupil.

Three tests are useful in confirming the presence and severity of Horner syndrome:

It is important to distinguish the ptosis caused by Horner's syndrome from the ptosis caused by a lesion to the oculomotor nerve. In the former, the ptosis occurs with a constricted pupil (due to a loss of sympathetics to the eye), whereas in the latter, the ptosis occurs with a dilated pupil (due to a loss of innervation to the sphincter pupillae). In a clinical setting, these two ptoses are fairly easy to distinguish. In addition to the blown pupil in a CNIII (oculomotor nerve) lesion, this ptosis is much more severe, occasionally occluding the whole eye. The ptosis of Horner syndrome can be quite mild or barely noticeable (partial ptosis). [7]

When anisocoria occurs and the examiner is unsure whether the abnormal pupil is the constricted or dilated one, if a one-sided ptosis is present then the abnormally sized pupil can be presumed to be on the side of the ptosis.[ citation needed ]

History

The syndrome is named after Johann Friedrich Horner, the Swiss ophthalmologist who first described the syndrome in 1869. [9] [10] Several others had previously described cases, but "Horner's syndrome" is most prevalent. In France and Italy, Claude Bernard is also eponymized with the condition (Claude Bernard–Horner syndrome, abbreviated CBH [11] ). In France, Francois Pourfour du Petit is also credited with describing this syndrome.

Children

The most common causes in young children are birth trauma and a type of cancer called neuroblastoma. [12] The cause of about a third of cases in children is unknown. [12]

See also

Related Research Articles

<span class="mw-page-title-main">Sympathetic nervous system</span> Part of the autonomic nervous system which stimulates fight-or-flight responses

The sympathetic nervous system (SNS) is one of the three divisions of the autonomic nervous system, the others being the parasympathetic nervous system and the enteric nervous system. The enteric nervous system is sometimes considered part of the autonomic nervous system, and sometimes considered an independent system.

<span class="mw-page-title-main">Mydriasis</span> Excessive dilation of the pupil

Mydriasis is the dilation of the pupil, usually having a non-physiological cause, or sometimes a physiological pupillary response. Non-physiological causes of mydriasis include disease, trauma, or the use of certain types of drug. It may also be of unknown cause.

<span class="mw-page-title-main">Pupillary light reflex</span> Eye reflex which alters the pupils size in response to light intensity

The pupillary light reflex (PLR) or photopupillary reflex is a reflex that controls the diameter of the pupil, in response to the intensity (luminance) of light that falls on the retinal ganglion cells of the retina in the back of the eye, thereby assisting in adaptation of vision to various levels of lightness/darkness. A greater intensity of light causes the pupil to constrict, whereas a lower intensity of light causes the pupil to dilate. Thus, the pupillary light reflex regulates the intensity of light entering the eye. Light shone into one eye will cause both pupils to constrict.

<span class="mw-page-title-main">Miosis</span> Excessive constriction of the pupil

Miosis, or myosis, is excessive constriction of the pupil. The opposite condition, mydriasis, is the dilation of the pupil. Anisocoria is the condition of one pupil being more dilated than the other.

<span class="mw-page-title-main">Pancoast tumor</span> Medical condition

A Pancoast tumor is a tumor of the apex of the lung. It is a type of lung cancer defined primarily by its location situated at the top end of either the right or left lung. It typically spreads to nearby tissues such as the ribs and vertebrae. Most Pancoast tumors are non-small-cell lung cancers.

<span class="mw-page-title-main">Levator palpebrae superioris muscle</span> Muscle in orbit that elevates upper eyelid

The levator palpebrae superioris is the muscle in the orbit that elevates the upper eyelid.

Chronic paroxysmal hemicrania (CPH) is a severe debilitating unilateral headache usually affecting the area around the eye. It normally consists of multiple severe, yet short, headache attacks affecting only one side of the cranium. Retrospective surveys indicated that paroxysmal hemicrania was more common in women. However, subsequent prospective research showed an equal prevalence between females and males, with a ratio close to 1:1. Unlike in migraine, it has no neurological symptoms associated with it. CPH headaches are treated through the use of non-steroidal anti-inflammatory drugs, with indomethacin found to be especially effective in eliminating symptoms.

<span class="mw-page-title-main">Harlequin syndrome</span> Medical condition

Harlequin syndrome is a condition characterized by asymmetric sweating and flushing on the upper thoracic region of the chest, neck and face. Harlequin syndrome is considered an injury to the autonomic nervous system (ANS). The ANS controls some of the body's natural processes such as sweating, skin flushing and pupil response to stimuli. Individuals with this syndrome have an absence of sweat skin flushing unilaterally, usually on one side of the face, arms and chest. It is an autonomic disorder that may occur at any age. Harlequin syndrome affects fewer than 1000 people in the United States.

<span class="mw-page-title-main">Ciliary ganglion</span> Bundle of nerves, parasympathetic ganglion

The ciliary ganglion is a parasympathetic ganglion located just behind the eye in the posterior orbit. It is 1–2 mm in diameter and in humans contains approximately 2,500 neurons. The ganglion contains postganglionic parasympathetic neurons. These neurons supply the pupillary sphincter muscle, which constricts the pupil, and the ciliary muscle which contracts to make the lens more convex. Both of these muscles are involuntary since they are controlled by the parasympathetic division of the autonomic nervous system.

<span class="mw-page-title-main">Adie syndrome</span> Neurological disorder

Adie syndrome, also known as Holmes–Adie syndrome, is a neurological disorder characterized by a tonically dilated pupil that reacts slowly to light but shows a more definite response to accommodation. It is frequently seen in females with absent knee or ankle jerks and impaired sweating.

<span class="mw-page-title-main">Superior cervical ganglion</span> Largest of the cervical ganglia

The superior cervical ganglion (SCG) is the upper-most and largest of the cervical sympathetic ganglia of the sympathetic trunk. It probably formed by the union of four sympathetic ganglia of the cervical spinal nerves C1–C4. It is the only ganglion of the sympathetic nervous system that innervates the head and neck. The SCG innervates numerous structures of the head and neck.

The orbitalis muscle is a vestigial or rudimentary nonstriated muscle that crosses from the infraorbital groove and sphenomaxillary fissure and is intimately united with the periosteum of the orbit. It was described by Heinrich Müller and is often called Müller's muscle. It lies at the back of the orbit and spans the infraorbital fissure. It is a thin layer of smooth muscle that bridges the inferior orbital fissure. It is supplied by sympathetic nerves, and its function is unknown.

<span class="mw-page-title-main">Ptosis (eyelid)</span> Drooping of the upper eyelid over the eye

Ptosis, also known as blepharoptosis, is a drooping or falling of the upper eyelid. This condition is sometimes called "lazy eye", but that term normally refers to the condition amblyopia. If severe enough and left untreated, the drooping eyelid can cause other conditions, such as amblyopia or astigmatism, so it is especially important to treat the disorder in children before it can interfere with vision development.

Hypohidrosis is a medical condition in which a person exhibits diminished sweating in response to appropriate stimuli. In contrast with hyperhidrosis, which is a socially troubling yet often benign condition, the consequences of untreated hypohidrosis include hyperthermia, heat stroke and death. An extreme case of hypohidrosis in which there is a complete absence of sweating and the skin is dry is termed anhidrosis. The condition is also known as adiaphoresis, ischidrosis, oligidria, oligohidrosis and sweating deficiency.

<span class="mw-page-title-main">Oculomotor nerve palsy</span> Medical condition

Oculomotor nerve palsy or oculomotor neuropathy is an eye condition resulting from damage to the third cranial nerve or a branch thereof. As the name suggests, the oculomotor nerve supplies the majority of the muscles controlling eye movements. Damage to this nerve will result in an inability to move the eye normally. The nerve also supplies the upper eyelid muscle and is accompanied by parasympathetic fibers innervating the muscles responsible for pupil constriction. The limitations of eye movement resulting from the condition are generally so severe that patients are often unable to maintain normal eye alignment when gazing straight ahead, leading to strabismus and, as a consequence, double vision (diplopia).

<span class="mw-page-title-main">Lumbar ganglia</span>

The lumbar ganglia are paravertebral ganglia located in the inferior portion of the sympathetic trunk. The lumbar portion of the sympathetic trunk typically has 4 lumbar ganglia. The lumbar splanchnic nerves arise from the ganglia here, and contribute sympathetic efferent fibers to the nearby plexuses. The first two lumbar ganglia have both white and gray rami communicates.

<span class="mw-page-title-main">Babinski–Nageotte syndrome</span> Medical condition

Babinski–Nageotte syndrome is an alternating brainstem syndrome. It occurs when there is damage to the dorsolateral or posterior lateral medulla oblongata, likely syphilitic in origin. Hence it is also called the alternating medulla oblongata syndrome.

The hypothalamospinal tract is an unmyelinated non-decussated descending nerve tract that arises in the hypothalamus and projects to the brainstem and spinal cord to synapse with pre-ganglionic autonomic neurons.

Villaret's syndrome combines ipsilateral paralysis of the last four cranial nerves and Horner syndrome. Sometimes cranial nerve VII is also involved. It may also involve the cervical ganglia of the sympathetic trunk. Paralysis is caused by a lesion in the retroparotid space, which is bounded posteriorly by the cervical vertebrae, superiorly by the skull near the jugular foramen, anteriorly by the parotid gland, laterally by the sternocleidomastoid muscle, and medially by the pharynx.

<span class="mw-page-title-main">Roots of the ciliary ganglion</span>

The ciliary ganglion is a parasympathetic ganglion located just behind the eye in the posterior orbit. Three types of axons enter the ciliary ganglion but only the preganglionic parasympathetic axons synapse there. The entering axons are arranged into three roots of the ciliary ganglion, which join enter the posterior surface of the ganglion.

References

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  5. Daroff R (April 2005). "Enophthalmos is not present in Horner syndrome". PLOS Medicine. 2 (4): e120. doi: 10.1371/journal.pmed.0020120 . PMC   1087222 . PMID   15839747.
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  7. 1 2 3 Koka, Kirthi; Patel, Bhupendra C. (2023), "Ptosis Correction", StatPearls, Treasure Island (FL): StatPearls Publishing, PMID   30969650 , retrieved 2024-01-04
  8. 1 2 Lee JH, Lee HK, Lee DH, Choi CG, Kim SJ, Suh DC (January 2007). "Neuroimaging strategies for three types of Horner syndrome with emphasis on anatomic location". AJR. American Journal of Roentgenology. 188 (1): W74-81. doi:10.2214/AJR.05.1588. PMID   17179330.
  9. Horner JF (1869). "Über eine Form von Ptosis". Klin Monatsbl Augenheilk. 7: 193–8.
  10. synd/1056 at Who Named It?
  11. Logan, Carolynn M.; Rice, M. Katherine (1987). Logan's Medical and Scientific Abbreviations . J. B. Lippincott and Company. pp.  58. ISBN   0-397-54589-4.
  12. 1 2 Kennard C, Leigh RJ (2011-06-28). Neuro-ophthalmology: Handbook of Clinical Neurology (Series Editors: Aminoff, Boller and Swaab). Elsevier. p. 452. ISBN   9780702045479.