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Horner's syndrome is a clinical syndrome caused by damage to the sympathetic nervous system. It is also known by the names Bernard-Horner syndrome or oculosympathetic palsy.
Symptoms
It results in ptosis (drooping upper eyelid), miosis (constricted pupil), and occasionally enophthalmos (the impression that the eye is sunk in) and anhidrosis (decreased sweating) on one side of the face,loss of ciliospinal reflex and blood shot conjunctiva.
In children Horner's syndrome sometimes leads to a difference in eye color between the two eyes (heterochromia). This happens because a lack of sympathetic stimulation in childhood interferes with melanin pigmentation of the melanocytes in the superficial stroma of the iris.
History
It is named after Dr Johann Friedrich Horner, the Swiss ophthalmologist who first described the syndrome in 1869. Several others had previously described cases, but "Horner's syndrome" is most prevalent. In France, Claude Bernard is also eponymised with the condition being called "syndrome Bernard-Horner".
Causes
Horner's syndrome is usually acquired but may also be congenital. Although most causes are relatively benign, Horner's syndrome may reflect serious pathology in the neck or chest (such as a Pancoast tumor or thorocervical venous dilatation) and hence requires workup.
- PICA syndrome
- Cluster headache
- Trauma - base of neck, usually blunt trauma.
- Stroke
- Middle ear infection
- Tumors - Example; Pancoast tumor
- Aortic aneurysm, thoracic
- Neurofibromatosis type 1
- Goitre
- Dissecting aortic aneurysm
- Thyroid carcinoma
- Bronchogenic carcinoma
- Multiple sclerosis
- Carotid artery dissection
- Klumpke paralysis
- Cavernous sinus thrombosis
- Sympathectomy
- Syringomyelia
Pathophysiology
Horner's 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).
Diagnosis
Three tests are useful in confirming the presence and severity of Horner's syndrome:
- Cocaine drop test - Cocaine blocks the reuptake of norepinephrine resulting in the dilation of a normal pupil. The pupil will fail to dilate in Horner's syndrome.
- Paredrine test
- Dilation lag test
It is important to distinguish the ptosis caused by Horner's syndrome from the ptosis caused by a lesion to the Occulomotor 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 ennervation to the sphincter pupillae).
See also
References
- Horner JF. Über eine Form von Ptosis. Klin Monatsbl Augenheilk 1869;7:193-8.
External link
- WhoNamedIt entry on Horner's syndrome.
Eponymous medical signs | Neurology | Ophthalmology | Syndromes
"Horner's syndrome"