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Horner's syndrome
- 1. Hornerâs Syndrome - Etiology and Localization Yazeed Alwelaie PGY1
- 2. Introduction
- 4. Hornerâs Syndrome ⢠Also called oculo-sympathetic paresis ⢠Classic triad: â Ptosis â Miosis â Anhidrosis
- 5. Horner's syndrome can be produced by a lesion anywhere along the sympathetic pathway that supplies the head, eye, and neck.
- 6. Neuroanatomy
- 7. Three â neuron pathway Neuro-ophthalmology: the requisites in ophthalmology, Krachmer, JH (Ed), Mosby, St. Louis 2000. Copyright Š2000 Elsevier.
- 8. First â Order Neuron Neuro-ophthalmology: the requisites in ophthalmology, Krachmer, JH (Ed), Mosby, St. Louis 2000. Copyright Š2000 Elsevier.
- 9. Second â Order Neuron Neuro-ophthalmology: the requisites in ophthalmology, Krachmer, JH (Ed), Mosby, St. Louis 2000. Copyright Š2000 Elsevier.
- 10. Third â Order Neuron Neuro-ophthalmology: the requisites in ophthalmology, Krachmer, JH (Ed), Mosby, St. Louis 2000. Copyright Š2000 Elsevier.
- 11. Third â Order Neuron Innervates the iris dilator muscles & MĂźller's muscle Neuro-ophthalmology: the requisites in ophthalmology, Krachmer, JH (Ed), Mosby, St. Louis 2000. Copyright Š2000 Elsevier.
- 16. Ptosis ⢠Ptosis is minor, usually less than 2 mm ⢠Paralysis of the Mßller's muscle, which is innervated by the sympathetic pathway ⢠"upside-down ptosis"
- 17. Anhidrosis ⢠Anhidrosis is present in central or preganglionic (first or second-order) lesions ⢠This sign is frequently not apparent to patients or clinicians
- 18. Neuro-ophthalmology: the requisites in ophthalmology, Krachmer, JH (Ed), Mosby, St. Louis 2000. Copyright Š2000 Elsevier.
- 19. Hornerâs in Children ⢠Impaired facial flushing (Harlequin sign) is often more apparent than anhidrosis ⢠Acute features of sympathetic disruption can also include ipsilateral conjunctival injection, nasal stuffiness, and increased near point of accommodation
- 20. Harlequin sign Source: http://picornot.com/keyword/harlequin+sign
- 23. Localizing/Associated Symptoms ⢠Diplopia, vertigo, ataxia, lateralized weakness suggest a brainstem localization ⢠Bilateral or ipsilateral weakness, long tract signs, sensory level, bowel and bladder impairment suggest involvement of the cervicothoracic cord ⢠Arm pain and/or hand weakness typical of brachial plexus lesions suggest a lesion in the lung apex
- 24. ⢠Ipsilateral extraocular pareses, particularly a sixth nerve palsy, in the absence of other brainstem signs localize the lesion to the cavernous sinus ⢠An isolated Horner's syndrome accompanied by neck or head pain suggests an internal carotid dissection
- 25. Etiology
- 26. First-order syndrome Lesions of the sympathetic tracts in the brainstem or cervicothoracic spinal cord can produce a first-order Horner's syndrome.
- 28. ⢠Strokes, tumors, and demyelinating lesions affecting the sympathetic tracts in the hypothalamus, midbrain, pons, medulla, or cervicothoracic spinal cord are other potential causes of a central Horner's syndrome. ⢠Syringomyelia and cervical cord trauma can also produce a Horner's syndrome.
- 29. Second-order syndrome Second-order or preganglionic Horner's syndromes can occur with trauma or surgery involving the spinal cord, thoracic outlet, or lung apex
- 31. ⢠Lumbar epidural anesthesia can also produce a Horner's syndrome. This is most often described in association with obstetrical procedure.
- 32. Third-order syndrome Third-order Horner's syndromes often indicate lesions of the internal carotid artery such as an arterial dissection, thrombosis, or cavernous sinus aneurysm.
- 33. Carotid Dissection An acute Horner's syndrome with neck or facial pain should be presumed to be caused by carotid dissection until proven otherwise. Between 40 and 60 percent of patients present with an isolated painful third-order Horner's syndrome. Emergent diagnostic tests should be obtained
- 36. Diagnosis
- 37. Pharmacologic Testing ⢠Pharmacologic tests can be useful to confirm the diagnosis and to localize the lesion ⢠Two agents are used: cocaine or apraclonidine drops and hydroxyamphetamine drops
- 38. Confirmation of Hornerâs Syndrome ⢠Pharmacological testing with cocaine or apraclonidine drops can confirm the diagnosis of Horner's syndrome in subtle cases ⢠If the diagnosis of Horner's syndrome is clear clinically, then use of cocaine or apraclonidine can be avoided
- 39. Cocaine ⢠Blocks the reuptake of norepinephrine at the sympathetic nerve synapse ⢠Intact pathway â dilates pupil. No effect on impaired sympathetic pathway
- 40. Apraclonidine ⢠Alternative to cocaine ⢠Direct alpha-adrenergic receptor agonist. Apraclonidine has weak alpha-1 and strong alpha-2 activity ⢠Alpha-1 mediates pupillary dilation, while alpha-2 downregulates norepinephrine release at the neuromuscular junction
- 41. From: Ocular Effects of Apraclonidine in Horner Syndrome Arch Ophthalmol. 2000;118(7):951-954. doi:10-1001/pubs.Ophthalmol.-ISSN-0003-9950-118-7-ecs90240 Date of download: 6/9/2014 Copyright Š 2014 American Medical Association. All rights reserved.
- 42. Localization of the Lesion ⢠First-order neuron (brainstem or cervical cord) ⢠Second-order neuron (chest or neck) ⢠Third-order or postganglionic neuron (above the superior cervical ganglion at the carotid bifurcation).
- 43. Hydroxyamphetamine ⢠It releases stored norepinephrine from the postganglionic adrenergic nerve endings ⢠A normal pupil and a first or second-order Horner's pupil will dilate, whereas a third-order Horner's pupil will not dilate as well as the normal pupil.
- 44. Postganglionic right Horner's syndrome Š2014 UpToDateŽ
- 45. Summary
- 46. ⢠Classic signs of a Horner's syndrome include miosis, ptosis, and anhidrosis. ⢠The miosis is typically mild, associated with a dilation lag and most prominent in dim light. ⢠The ptosis is also mild and also involves the lower lid. ⢠Anhidrosis occurs with first or second-order lesions only
- 48. ⢠The common etiologies of Horner's syndrome are categorized by which of the three neurons is affected. ⢠The differential diagnosis is also distinct in children versus adults26
- 49. ⢠The presence of a Horner's syndrome can be confirmed pharmacologically with either cocaine or apraclonidine eye drops ⢠Hydroxyamphetamine eye drops can help distinguish a third-order (postganglionic) Horner's syndrome from either a first or second-order syndrome.
- 50. ⢠In the absence of a clear history of trauma as the cause of Horner's syndrome, imaging studies will be required.
Hinweis der Redaktion
- Horner's syndrome can result from a lesion anywhere along a three-neuron sympathetic (adrenergic) pathway that originates in the hypothalamus
- The first-order neuron descends caudally from the hypothalamus to the first synapse, which is located in the cervical spinal cord (levels C8-T2, also called ciliospinal center of Budge).
- The second-order neuron travels from the sympathetic trunk, through the brachial plexus, over the lung apex. It then ascends to the superior cervical ganglion, located near the angle of the mandible and the bifurcation of the common carotid artery
- The third-order neuron then ascends within the adventitia of the internal carotid artery, through the cavernous sinus, where it is in close relation to the sixth cranial nerve [1]. The oculosympathetic pathway then joins the ophthalmic (V1) division of the fifth cranial nerve (trigeminal nerve). In the orbit and the eye, the oculosympathetic fibers innervate the iris dilator muscle as well as MĂźller's muscle, a small smooth muscle in the eyelids responsible for a minor portion of the upper lid elevation and lower lid retraction.
- The third-order neuron then ascends within the adventitia of the internal carotid artery, through the cavernous sinus, where it is in close relation to the sixth cranial nerve [1]. The oculosympathetic pathway then joins the ophthalmic (V1) division of the fifth cranial nerve (trigeminal nerve). In the orbit and the eye, the oculosympathetic fibers innervate the iris dilator muscle as well as MĂźller's muscle, a small smooth muscle in the eyelids responsible for a minor portion of the upper lid elevation and lower lid retraction.
- The degree of anisocoria is more marked in the dark than in light. There is associated dilation lag, an asymmetry in pupillary redilation between the two eyes when the light source is moved away from the eye [2]. The Horner's pupil will redilate more slowly (by 15 to 20 seconds) than the normal pupil.
- A) In the light, there is mild anisocoria, with the right pupil being smaller than the left pupil. There is also decreased palpebral fissure on the right. B) In the dark, the right pupil does not dilate well. C) After instillation of 1 percent hydroxyamphetamine in both eyes, only the left pupil dilates, suggesting a lesion involving the postganglionic oculosympathic pathways (third order Horner's syndrome).
- The ptosis is minor (less than 2 mm) and occurs as a result of paralysis of the MĂźller's muscle, which is innervated by the sympathetic pathway. The lower as well as the upper lid is affected, producing the so-called "upside-down ptosis." This further narrows the palpebral fissure. The levator palpebrae superioris is unaffected; weakness of this muscle produces the more profound upper lid ptosis seen in third cranial nerve palsies.
- The sympathetic fibers responsible for facial sweating and vasodilation branch off at the superior cervical ganglion from the remainder of the oculosympathetic pathway; thus, anhidrosis is not a feature of postganglionic or third-order lesions [3]. This sign is frequently not apparent to patients or clinicians.
- The sympathetic fibers responsible for facial sweating and vasodilation branch off at the superior cervical ganglion from the remainder of the oculosympathetic pathway; thus, anhidrosis is not a feature of postganglionic or third-order lesions
- One possible cause of Harlequin syndrome is a lesion to the preganglionic or postganglionic cervical sympathetic fibers and parasympathetic neurons of the ciliary ganglion
- Congenital Horner syndrome. Ptosis, miosis, and heterochromia. Lighter colored iris is on the affected left side A congenital Horner's syndrome should be suspected when anisocoria is associated with heterochromia (unequal iris color, with the affected iris being lighter). This occurs because formation of iris pigment in the first several months of age is under sympathetic control. This may only be apparent if the natural color is relatively dark The most common cause is delivery-related trauma to the neck and shoulder, damaging the sympathetic pathway. Associated injury to the lower brachial plexus can produce weakness in the ipsilateral forearm and hand (Klumpke's paralysis). .
- Congenital ptosis of severe degree, left upper lid.
- The most common cause is a lateral medullary infarction, which produces a Horner's syndrome as part of the Wallenberg syndrome. Typically the patient presents with vertigo and ataxia, which overshadow the Horner's syndrome. Other neurologic symptoms and signs include abnormal eye movements, ipsilateral limb ataxia, and a dissociated sensory loss (loss of pain and temperature sensation on the ipsilateral face and contralateral trunk). Hoarseness and dysphagia are also often present.
- when the intermediolateral columns are affected.
- Pancoast tumor
- due to pharmacologic disruption of the preganglionic neuron as it exits the spinal cord. This is most often described in association with obstetrical procedures; in such cases, a Hornerâs syndrome may indicate high sympathetic blockade.
- Carotid endarterectomy and carotid artery stenting can also produce a Horner's syndrome
- Patients with acute carotid dissection are at a high risk for cerebral infarction, which usually occurs within the first few weeks, often within days, after onset of the Horner's syndrome.
- Other causes of postganglionic Horner's syndrome include neck masses, otitis media, and pathology involving the cavernous sinus. Abnormalities of eye movements, particularly a sixth nerve palsy, commonly occur when the cavernous sinus is involved
- A Horner's syndrome is a common feature of cluster headache, occurring with unilateral eye or temple pain and lacrimation, generally lasting no more than an hour or two
- Whether to take the time to test the pupils pharmacologically or not to confirm a Horner syndrome mostly depends on the duration of the anisocoria and the location of the consultation: âWhen a patient is seen in an emergency department with acute painful anisocoria highly suggestive of Horner syndrome, it is essential to immediately obtain appropriate investigations to look for a cervical artery dissection, or a cavernous sinus lesion. In this setting, pharmacologic testing would only delay appropriate testing and management. âWhen a patient presents to an outpatient clinic with incidentally found isolated anisocoria, confirming the Horner syndrome before obtaining potentially costly and unnecessary tests is helpful. Unlike cocaine drops, apraclonidine drops are readily available, and are a very reliable way to confirm (or to rule-out) a Horner syndrome in adults or even older children. s their administration will interfere with the hydroxyamphetamine test for localization.
- Cocaine blocks the reuptake of norepinephrine at the sympathetic nerve synapse and causes pupillary dilation in eyes with intact sympathetic innervation. Cocaine has no effect in eyes with impaired sympathetic innervation, regardless of the lesion location. One hour after instillation of two drops of cocaine (4 or 10 percent), a normal pupil dilates more than the Horner's pupil, increasing the degree of anisocoria; anisocoria of 1 mm or more after cocaine administration is considered a positive result
- In a Horner's pupil, denervation supersensitivity to the alpha-1 receptor will cause that pupil to dilate (usually by about 2 mm), while alpha-2 stimulation in the normal eye will cause that pupil to constrict slightly (usually by <1 mm). Thus, one to two drops of 0.5 percent apraclonidine instilled in both eyes causes a reversal of anisocoria in patients with Horner's syndrome. Comparison testing in small series of patients suggests that this test compares favorably with cocaine in the diagnosis of Horner's syndrome
- All photographs were taken with room lights on. A, The patient at baseline, showing left ptosis and miosis; note the incidental elevated left upper eyelid fold consistent with levator aponeurosis dehiscence. B, Forty-five minutes after instillation of 10% cocaine to each eye. Failure of the left pupil to dilate indicates Horner syndrome. C, Several weeks later, appearance 1 hour after instillation of 1 drop of 1% apraclonidine to the left eye. Note reversal of baseline anisocoria.
- Â There is no pharmacologic test to distinguish between first and second-order lesions.
- Hydroxyamphetamine eye drops will differentiate between a lesion affecting the first (brainstem or cervical cord) or second-order (chest or neck) neuron and one affecting the third-order or postganglionic neuron (above the superior cervical ganglion at the carotid bifurcation). There is no pharmacologic test to distinguish between first and second-order lesions. Because cocaine may interfere with the uptake and efficacy of hydroxyamphetamine drops, it is recommended that 24 to 72 hours elapse between the two tests. One hour after instillation of 1 percent hydroxyamphetamine,
- In the light, there is mild anisocoria, with the right pupil being smaller than the left pupil. There is also decreased palpebral fissure on the right. B) In the dark, the right pupil does not dilate well. C) After instillation of 1 percent hydroxyamphetamine in both eyes, only the left pupil dilates, suggesting a lesion involving the postganglionic oculosympathic pathways (third order Horner's syndrome). The test is positive for postganglionic Horner's lesions when the anisocoria increases by at least 1 mm. This test has a sensitivity of 93 to 96 percent and a specificity of 84 percent for detecting postganglionic lesions. This test is not reliable in children in whom transynaptic degeneration occurs
- A Horner's syndrome can be caused by a lesion anywhere along the three-neuron sympathetic (adrenergic) pathway that originates in the hypothalamus
- High-yield sites of imaging can be identified based on accompanying signs and symptoms and/or the hydroxyamphetamine test