Horner's syndrome

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Horner's syndrome
Classification and external resources

Left-sided Horner's syndrome

ICD-10

G90.2

ICD-9

337.9

OMIM

143000

DiseasesDB

6014

MedlinePlus

000708

eMedicine

med/1029 oph/336

MeSH

D006732

Horner's syndrome or Horner syndrome is a clinical syndrome caused by damage to thesympathetic

nervous system. It is also known by the names Bernard-Horner syndrome oroculosympathetic palsy.
Contents
[hide]

1 Signs

2 History
3 Causes
4 Pathophysiol
ogy
5 Diagnosis
6 See also
7 References

[edit]Signs
Signs found in all patients on affected side of face include; ptosis (which is drooping of the upper eyelid from
loss of sympathetic innervation to the superior tarsal muscle, also known as Mller'smuscle [1]), upside-down
ptosis (slight elevation of the lower lid), and miosis (constricted pupil), and anhidrosis (decreased sweating on
the affected side of the face), dilation lag (slow response of the pupil to light), Enophthalmos (the impression
that the eye is sunk in) loss of ciliospinal reflex and bloodshot conjunctiva may occur depending on the site of
lesion. Sometimes there is flushing of the face is on the affected side of the face due to dilation of blood
vessels under the skin.
The clinical features of Horner's syndrome can be remembered using the mnemonic, "Horny PAMELa"
for Ptosis, Anhydrosis, Miosis,Enophthalmos and Loss of ciliospinal reflex.[2]
In children Horner syndrome sometimes leads to a difference in eye color between the two eyes
(heterochromia).[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.

[edit]History
It is named after Johann Friedrich Horner, the Swiss ophthalmologist who first described the syndrome in 1869.
[4][5]

Several others had previously described cases, but "Horner's syndrome" is most prevalent.

In France and Italy, Claude Bernard is also eponymised with the condition ("Claude Bernard-Horner
syndrome").

[edit]Causes
Horner syndrome is acquired as a result of pathology but may also be congenital (inborn) or iatrogenic (caused
by medical treatment). Although most causes are relatively benign, Horner syndrome may reflect serious

pathology in the neck or chest (such as a Pancoast tumor(tumor in the apex of the lung) or thyrocervical
venous dilatation).

Due to lesion or compression of one side of the cervical or thoracic sympathetic chain, which
generates symptoms on the ipsilateral(same side as lesion) side of the body.

Lateral medullary syndrome

Cluster headache - combination termed Horton's headache[6]

Trauma - base of neck, usually blunt trauma, sometimes surgery.

Middle ear infection

Tumors - often bronchogenic carcinoma of the superior fissure (Pancoast tumor) on apex of lung

Aortic aneurysm, thoracic

Neurofibromatosis type 1

Goitre

Dissecting aortic aneurysm

Thyroid carcinoma

Multiple sclerosis

Cervical rib traction on stellate ganglion

Carotid artery dissection

Klumpke paralysis

Cavernous sinus thrombosis

Sympathectomy

Syringomyelia

Nerve blocks, such as cervical plexus block, stellate ganglion or interscalene block

As a complication of tube thoracostomy

[edit]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:
First-order neuron disorder: Central lesions that involve the hypothalamospinal pathway (e.g.

transection of the cervical spinal cord).

Second-order neuron disorder: Preganglionic lesions (e.g. compression of the sympathetic chain by a

lung tumor).
Third-order neuron disorder: Postganglionic lesions at the level of the internal carotid artery (e.g. a

tumor in the cavernous sinus).

If someone has impaired sweating above the waist affecting only one side of the body, yet they do not have a
clinically apparent Horner's syndrome, then the lesion is just below the stellate ganglion in the sympathetic
chain.

[edit]Diagnosis
Three tests are useful in confirming the presence and severity of Horner syndrome:
1. Cocaine drop test - Cocaine eyedrops block the reuptake of norepinephrine resulting in the dilation of
a normal pupil. Due to the lack of norepinephrine in the synaptic cleft, the pupil will fail to dilate in
Horner's syndrome. A more recently introduced approach that is more dependable and obviates the
difficulties in obtaining cocaine is to apply the alpha-agonist apraclonidine to both eyes and observe
the reversal of miosis on the affected side of Horner syndrome (the opposite effect to cocaine).
2. Paredrine test:- This test helps to localize the cause of the miosis. If the 3rd order neuron (the last of 3
neurons in the pathway which ultimately discharges norepinephrine into the synaptic cleft) is intact,
then the amphetamine causes neurotransmitter vesicle release, thus releasing norepinephrine into the
synaptic cleft and resulting in robust mydriasis of the affected pupil. If the lesion itself is of the
aforementioned 3rd order neuron, then the amphetamine will have no effect and the pupil remains
constricted. There is no pharmacological test to differentiate between a 1st and 2nd order neuron
lesion.
3. Dilation lag test
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 an actual clinical setting, however, these two different 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.
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 the one on the side
of the ptosis.

Definition of Horner's Syndrome

Horner's syndrome is a eye disorder that consists of enophthalmos (sinking

of the eyeball into its cavity), ptosis (droopy upper eyelid), swelling of the
lower eyelid, miosis (abnormal contraction of the pupil), anhidrosis (absence
of facial sweat) and heterochromia (difference in eye color).
Horner's syndrome is caused by paralysis of the cervical sympathetic nerves.

Description of Horner's Syndrome

To better understand Horner's syndrome, one needs to understand how the

nervous system works.
There are two major divisions within the nervous system. There is the part of the nervous system that
you are aware of and have control over, and there is a part of the nervous system that is under
automatic control, called the autonomic nervous system.
Within the autonomic nervous system there are two divisions, the sympathetic and the
parasympathetic nervous systems.
The sympathetic nervous system controls many of the involuntary activities of the glands, organs and
other body parts. The parasympathetic nervous system also controls the involuntary activities of the
organs, glands as well as blood vessels and other tissues in the body.
The eye has both sympathetic and parasympathetic function (innervation). If something were to block
the sympathetic impulses into the eye, there would be an overbalance of parasympathetic supply to
the eye. The result is Horner's syndrome.
The nervous system in the body does not consist of straight lines to and from the brain. In respect to
the eye, the sympathetic nerves travels from the brain down the spinal column to the chest area and
back up.
The first part of the journey, called the first neuron pathway, starts at the brain and travels down the
spinal cord and into the chest.
The second neuron pathway continues from the chest cavity, over the lungs and up the carotid artery
in the neck.

The third neuron pathway goes from the carotid artery and jugular vein through the middle ear and
then into the eye.
Upon entering the eye the nerve pathways split, one goes to the pupil and one goes to the muscles of
the eyelid. Horner's syndrome may occur as a result of lesions found along the course of the nerve's
route from the brain to the eye.

Causes and Risk Factors of Horner's Syndrome

There are three major types of Horner's syndrome. Each named after its
pathway (first, second, or third) and associated with the parts of the body
within the pathway (central, preganglionic, and postganglionic).
First Neuron Horner's Syndrome (central lesions) can be caused by the occlusion (closure)
of the posteroinferior cerebellar artery at the lower portion of the brain stem (also known as
Wallenberg syndrome), by a transient ischemic attack (brief interruption of the blood supply to
the brain), or by brain tumors.
Second Neuron Horner's Syndrome (preganglionic lesions) may be caused by lung cancer,
thoracic tumors, phrenic nerve syndrome, thyroid enlargement, severe osteoarthritis of the
neck with bone spurs, spinal cord injury or disease, neck trauma caused by injury, surgery, or
severe whiplash.
Third Neuron Horner's Syndrome Group I(postganglionic lesions) may be caused by skull
fracture,cluster headaches, migraines, or middle ear infections.
Third Neuron Horner's Syndrome Group II involves the facial sweating mechanism.
When Horner's syndrome occurs for no apparent reason, it may be been inherited or caused by viral,
immune-mediated or idiopathic (without recognizable cause) neuropathies.

Symptoms of Horner's Syndrome

The symptoms of Horner's syndrome include:

Drooping of the upper eyelid
Swelling of the lower eyelid

Sinking of the eyeball

An absence of sweat on the same side of the face as the affected eye

The pupil becomes smaller (miotic)

Each iris may be a different color

Diagnosis of Horner's Syndrome

For proper diagnosis of Horner's syndrome, the physician will conduct a

thorough medical history (emphasizing past injuries and surgeries) and

examine the patient's neck, thyroid, and lymph nodes for other non-Horner's
conditions.
Additionally a chest x-ray, blood analysis, and eye examination will be done.
The ocular examination may consist of a pupillograph. This test examines the absolute and relative
size and reaction of the pupil to both light/dark and accommodation. When observed in room light, the
anisocoria (inequality of pupil size) in Horner's syndrome may be as little as 1 mm or even less. In
darkness, the anisocoria is increased and the effected pupil dilates more slowly than normal.
Pharmacologic tests involve administering phenylephrine, epinephrine, or hydroxyamphetamine to the
suspected pupil and watching its reaction to these dilation drugs.

Treatment of Horner's Syndrome

Treatment depends on the location and cause of the lesion. In some cases
surgical removal of a tumor is appropriate. If the tumor is malignant,
radiation and chemotherapy may be recommended.
Questions To Ask Your Doctor About Horner's Syndrome

Is Horner's syndrome attributed to another condition?

What are the treatment methods for that particular condition?
What type of treatment do you recommend to alleviate Horner's syndrome?
Is this a lifelong disease?